Scott-Brown's Otorhinolaryngology: Head and Neck Surgery - Vol 2 (3 volume set)

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Scott-Brown 's- Otorh inO,laryngology. Head an.d Neck Surgery

Scott-Brown's Otorhinolaryngology, Head and Neck Surgery 7th edition Lead editor: Michael Gleeson

Volume 1 Part 1 Cell biology, edited by Nicholas S Jones Part 2 Wound healing, edited by Nicholas S Jones Part 3 Immunology, edited by Nicholas S Jones Part 4 Microbiology, edited by Nicholas S Jones Part 5 Haematology, edited by Nicholas S Jones Part 6 Endocrinology, edited by Nicholas S Jones Part 7 Pharmacotherapeutics, edited by Martin J Burton Part 8 Perioperative management, edited by Martin J Burton Part 9 Safe and effective practice, edited by Martin J Burton Part 10 Interpretation and management of data, edited by Martin J Burton Part 11 Recent advances in technology, edited by Martin J Burton Part 12 Paediatric otorhinolaryngology, edited by Ray Clarke

Volume 2 Part 13 The nose and paranasal sinuses, edited by Valerie J Lund Part 14 The neck, edited by John Hibbert Part 15 The upper digestive tract, edited by John Hibbert Part 16 The upper airway, edited by John Hibbert Part 17 Head and neck tumours, edited by John Hibbert Volume 3 Part 18 Plastic surgery of the head and neck, edited by John C Watkinson Part 19 The ear, hearing and balance, edited by George G Browning and Linda M Luxon Part 20 Skull base, edited by Michael Gleeson Index CD-ROM

George G Browning MD FRCS Professor of Otorhinolaryngology, MRC Institute of Hearing Research, Glasgow Royal Infirmary, Glasgow, UK Martin J Burton MA DM FRCS Senior Clinical Lecturer, University of Oxford; and Consultant Otolaryngologist, Oxford Radcliffe NHS Trust Oxford, UK Ray Clarke BSc DCH FRCS FRCS (ORL) Consultant Paediatric Otolaryngologist, Royal Liverpool University Children's Hospital, Alder Hey, Liverpool, UK Michael Gleeson MD FRCS Professor of Otolaryngology and Skull Base Surgery, Institute of Neurology, University College London; and Consultant, Guy's, Kings and St Thomas' and the National Hospital for Neurology and Neurosurgery, London UK; and Honorary Consultant Skull Base Surgeon, Great Ormond Street Hospital for Sick Children, London, UK John Hibbert ChM FRCS Formerly Consultant Otolaryngologist, Department of Otolaryngology,~ Guy's Hospital, London, UK Nicholas S Jones MD FRCS FRCS (ORL) Professor of Otorhinolaryngology, Queen's Medical Centre, University C?f Nottingham, Nottingham UK Valerie J Lund MS FRCS FRCS (Ed) Professor of Rhinology, The Ear Institute, University College London, London, UK Linda M Luxon BSc MBBS FRCP Professor of Audiovestibular Medicine, University of London at University College London, Academic Unit of Audiovestibular Medicine; and Consultant Physician, National Hospital for Neurology and Neurosurgery; and Honorary Consultant Physician, Great Ormond Street Hospital for Children, London, UK John C Watkinson MSc MS FRCS (Ed, Glas, Land) DLO Consultant Head and Neck and Thyroid Surgeon, Department of Otorhinolaryngology/Head and Neck Surgery, Queen Elizabeth Hospital, University of Birmingham NHS Trust, Birmingham, UK

Scott-Brown's Otorhinolaryngology, Head and Neck Surgery 7th edition

Volume 2

Edited by

Michael Gleeson George G Browning. Martin J Burton. Ray Clarke. John Hibbert, Nicholas S Jones. Valerie J Lund. Linda M· Luxon. John C Watkinson

Hodder Arnold www.hoddereducation.com

First published in Great Britain in 1952 by Butterworth 8: Co. Second edition 1965 Third edition 1971 Fourth edition 1979 Fifth edition 1987 Sixth edition 1997 This seventh edition published in Great Britain in 2008 by Hodder Arnold An imprint of Hodder Education, a part of Hachette Livre UK, 338 Euston Road, London NWl 3BH http://www.hoddereducation.com

© 2008 Edward Arnold (Publishers) Ltd All rights reserved. Apart from any use permitted under UK copyright law, this publication may only be reproduced, stored or transmitted, in any form, or by any means with prior permission in writing of the publishers or in the case of reprographic production in accordance with the terms of licences issued by the Copyright Licensing Agency. In the United Kingdom such licences are issued by the Copyright Licensing Agency: Saffron House, 6-10 Kirby Street, London ECl N 8TS Whilst the advice and information in this book are believed to be true and accurate at the date of going to press, neither the author[s] nor the publisher can accept any legal responsibility or liability for any errors or omissions that may be made. In particular (but without limiting the generality of the preceding disclaimer) every effort has been made to check drug dosages; however it is still possible that errors have been missed. Furthermore, dosage schedules are constantly being revised and new side-effects recognized. For these reasons the reader is strongly urged to consult the drug companies' printed instructions before administering any of the drugs recommended in this book. British Library Cataloguing in Publication Data A catalogue record for this book is available from the British Library Library of Congress Cataloging-in-Publication Data A catalog record for this book is available from the Library of Congress ISBN

978 0 340 808 931

1 2 3 4 5 6 7 8 9 10 Commissioning Editor: Joanna Koster Project Editor: Zelah Pengilley Production Controller: Lindsay Smith! Andre Sim Text and Cover Designer: Amina Dudhia Cover photograph © MEHAU KULYK!SCIENCE PHOTO LIBRARY Typeset in 10 pt Minion by Macmillan India Printed and bound in India.

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Contents

How to use this book

PART 13 THE NOSE AND PARANASAL SINUSES - EDITED BY VALERIE J LUND 104

Anatomy of the nose and paranasal sinuses

xi

1313 1315

H Stammberger and Valerie J Lund 105

Nasal endoscopy

1344

Rodney J Schlosser and David W Kennedy 106

Physiology of the nose and paranasal sinuses

1355

Adrian Drake-Lee 107

Measurement of the nasal airway

1372

Ronald Eccles 108

Classification and differential diClgnosis of rhinosinusitis

1380

Ian S Mackay and Valerie J Lund 109

Allergic rhinitis

1386

G/enis Scadding and Stephen Durham 110

Nonallergic perennial rhinitis

1408

Claus Bachert 111

Occupational rhinitis

1415

Hesham Saleh 112

Food allergy and intolerance

1424

Carsten Bindslev-Jensen and Morten Osterballe 113

Rhinosinusitis

1439

Michael S Benninger 114

Fungal rhinosinusitis

1449

Jean Michel Klossek 115

Specific chronic infections

1458

S Bahadur and A Thakar 116

Medical management of chronic rhinosinusitis

1469

Gltinis Scadding 117

Surgical management of rhinosinusitis

1478

Valerie J Lund and Julian Rowe-Jones 118

The frontal sinus

1500

Wolfgang Draf 119

Mucocoeles

1531

Valerie J Lund 120

Complications of rhinosinusitis

1539

Robert Slack and Richard Sim

j

,,' .

-

vi I 121

Contents

Nasal polyposis

1549

Niels Mygind and Valerie J Lund 122

The relationship between the upper and lower respiratory tract

1560

Jean Bousquet and Antonio M Vignolat 123

The septum

1569

Adriaan F van Olphen 124

Nasal septal perforations

1582

Charles East and Santdeep Paun 125

The management of enlarged turbinates

1589

Luisa F Grymer 126

Epistaxis

1596

Gerald W McGarry 127

Nasal fractures

1609

Brent A McMonagle and Michael Gleeson 128

Fractures of the facial skeleton

1618

Simon Holmes and Michael Gleeson 129

Cerebrospinal fluid rhinorrhoea

1636

Andrew H Marshall and Nicholas S Jones 130

Granulomatous conditions of the nose

1645

David J Howard and Valerie J Lund 131

Abnormalities of smell

132

Orbital and optic nerve decompression

1660

Richard L Doty and Steven M Bromley 1677

Valerie J Lund and Geoffrey ERose 133

Dacryocystorh i nostomy

1689

Neil Fergie and Nicholas S Jones 134

Conditions of the external nose

1699

Michael O'Connell 135

The diagnosis and management of facial pain

1718

Tim J Woolford and Nicholas S Jones 136

Medical negligence in rhinology

1730

Maurice Hawthorne

PART 14 THE NECK - EDITED BY JOHN HIBBERT

1737

137

1739

Surgical anatomy of the neck

Chris R Jennings 138

Examination and imaging of the neck

1754

Sheila C Rankin and John Hibbert 139

Neck trauma

1766

Johannes J Fagan and Andrew J Nicol 140

Benign neck disease: infections and swellings

1777

Peter Clarke

PART 15 THE UPPER DIGESTIVE TRACT - EDITED BY JOHN HIBBERT 141

Anatomy of the mouth and dentition

1789 1791

Barry KB Berkovitz 142

Benign oral and dental disease

Crisp ian Scully and Jose-V Sebastian Bagan

1816


60 percent beyond

cells,22

the age of 50 years, therefore suggesting that functional

degranulation,

abnormality in this condition may be associated with the 1 ageing process in the nasal mucosa. 6 [***/**]

which decreases eosinophil activation and viability.23

and

to

induce in

eosinophil

marked

migration

contrast

to

and/or

testosterone,

Compared with pregnancy rhinitis, the evidence linking hypothyroidism with nasal pathology is sparse,

and

the reported increase in nasal secretion associated with

Nonallergic occupational rhinitis

thyroid disease is anecdotal.1 [***/**]

Occupational rhinitis, as the term implies, may be defined as rhinitis caused by exposure to airborne agents present

Drug-induced rhinitis

in the work place. These agents elicit predominantly sneezing, nasal discharge and/or blockage and may act via both immunologic (IgE-mediated) and nonimmunologic mechanisms.

The nonimmunologic triggers are often

irritant or toxic small molecular weight compounds such as aldehydes,

isocyanates,

aircraft fuel and jet stream

exhaust, solvents, etc./' 8,17 or may be physical (long-term exposure to cold air). Whilst the specific mechanisms underlying the effects of these irritants and toxic agents have not been fully elucidated, it is possible that damage and/or stimulation of the epithelial cells and neurons by the irritants may lead to proinflammatory mediators and neuromediators, which may predispose the nasal mucosa to inflammation and infection, subsequently resulting in the symptoms of rhinitis. Indeed, a study investigating the effect of airborne exposure to the nonallergenic microbial agents endotoxin and beta(l,3)-glucan in compost work­ ers

has

reported

that

concentrations

(predominantly neutrophils),

of

total

cells

myeloperoxidase (MPO),

IL-8, nitric oxide (NO) and albumin were significantly higher in compost workers than in controls.18 Similarly, occupational exposure to vanadium pentoxide, a cons­ tituent of fuel oil ash and a known respiratory irritant, has been shown to significantly increase the number of polymorphonuclear

cells

in

nasal

lavage

of

boiler­

makers.19 [****/***]

Hormonal rhinitis Hormonal rhinitis is often associated with pregnancy in particular, although puberty is also known to induce

Several commonly employed medications, such as aspirin, other nonsteroidal antiinflammatory drugs (NSAIDs), beta-blockers, angiotensin-converting enzyme (ACE) in­ hibitors, methyldopa, oral contraceptives, psychotropic agents and nasal topical decongestants (oxymetazoline, naphazoline, xylometazoline) may induce symptoms of rhinitis when they are administered either topically or systemically. The symptoms may be either predictable, as would be the case for known side effects of particular drugs, or unpredictable, based on individual hypersensi­ tivity to certain drugs,

in

particular

aspirin,

which

commonly exacerbates rhinitis and asthma. However, intolerance to aspirin and/or NSAIDs predominantly produces rhinorrhoea, which may be either isolated or part of a complex involving hyperplastic rhinosinusitis, nasal polyps and asthma.1 In contrast, intolerance to ACE inhibitors, methyldopa or oral contraceptives, which is less common than aspirin intolerance, leads predomi­ nantly to nasal blockage.1 Whilst persistent overuse of the topical nasal vasoconstrictors also leads to nasal decon­ gestion by a mechanism involving a rebound effect following withdrawal of these drugs,

excessive use of

these agents may also lead to nasal hyperreactivity and hypertrophy of the nasal mucosa, a condition known as , 'rhinitis medicamentosa .1,8 [****/***/**]

Other forms of rhinitis I\lARES

the symptoms of rhinitis.1,8 A large multicentre study

The term NARES was originally introduced by Mullarkey

has recently indicated that the cumulative incidence of

and colleagu�s, who characterized the condition on the

pregnancy rhinitis was 22 percent, and in women who

basis of a presence of greater than 20 percent eosinophils

Chapter 110 Nonaliergic perennial rhinitis

&

in nasal smears of symptomatic patients with perennial sneezing attacks, a profuse watery rhinorrhoea, nasal pruritis, incomplete nasal obstruction and occasional loss of smell. 1, 7, 8 In addition to these symptoms, a marked feature of the disease is the lack of evidence of allergy, as indicated by negative skin prick tests and/or absence of serum IgE antibodies to specific allergens. The prevalence of NARES has been shown to range between 13 and 33 percent in patients with nonallergic rhinitis?I,24 Although the specific aetiology of NARES is not clear, in view of the features shared by this syndrome and the triad (nasal polyposis, intrinsic asthma and intolerance to aspirin) and because NARES patients frequently develop nasal polyps and asthma later on in life, it has been suggested that NARES may be an early expression of the triad. 24 Indeed, in approximately 50 percent of NARES patients without a history of respiratory symptoms, bronchial responsiveness is associated with an increase in the number of sputum eosinophils, but not with an increase in the number of nasal eosinophils. 25 Some investigators have suggested that NARES is a variant of vasomotor rhinitis, and referred to the condition as 'perennial intrinsic rhinitis'. [***/**/*]

I 1411

EMOTIONALLY INDUCED RHINITIS

Although not studied extensively, emotional factors such as stress and sexual arousal have been documented to affect the nose, likely as a result of autonomic stimulation. 2,27 In patients suffering from post-coital rhinitis, anxiety appears to be a predominant feature, which leads to worsening of their disease. 27 [**/*]

ATROPHIC RHINITIS

Primary atrophic rhinitis is a condition that occurs predominantly in women and is characterized by progressive atrophy of the nasal mucosa and underlying bone of the turbinates. 1,2 This leads to the formation of thick crusts, which leave a constant foul smell (ozaena) in the nose. 1, 2 Furthermore, the nasal cavities are enlarged and there is the sensation of nasal congestion. Although the precise aetiology of this condition is not clear, it has been suggested that this may be a result of infection with Klebsiella ozaenae and other bacteria. However, primary atrophic rhinitis is distinct from secondary atrophic rhinitis, which develops directly as a result of granulomatous nasal infections, chronic rhinosinusitis, excessive nasal surgery, trauma and irradiation.

RHINITIS DUE TO PHYSICAL AND CHEMICAL FACTORS

Nasal symptoms similar to those of rhinitis can be induced by physical and chemical factors in individuals with sensitized nasal mucous membranes. 2 Cold, dry air has been shown to lead to a condition known as skier's nose, in which rhinorrhoea features prominently. Exposure to chemicals, particularly air pollutants derived ." from cigarette smoke and liquid petroleum fuels, have also been shown to directly exacerbate symptoms of rhinitis in nonallergic individuals,2 although their effects have generally been extensively investigated and well documented in the lower airways of allergic individuals. 26 Little information is available on the acute or chronic effects of air pollutants on the nasal mucosa. [***/**/*]

FOOD-INDUCED RHINITIS

Few studies have documented that certain foods and alcoholic beverages can induce nonallergic rhinitis, although the underlying mechanisms are largely unknown. Hot and spicy foods, in particular, which contain capsaicin lead to a watery rhinorrhoea termed 'gustatory rhinitis', probably as a result of the capsaicin stimulating the sensory nerves to release neuropeptides and tachykinins. I,2 In contrast, alcoholic beverages are thought to induce symptoms as a result of vasodilation. Dyes and preservatives, as well as sulphites, appear to playa role in a . very few cases, whereas some foods may contain clinically relevant concentrations of histamine or other biogenic amines. [*** /**1*]

.'/

!

DIFFERENTIAL DIAGNOSIS Allergic rhinitis is the major disease to differentiate from nonallergic rhinitis and may be excluded by appropriate allergy tests. However, the possibility of a local IgE production within the nasal mucosa may account for some cases where skin prick tests or IgE antibodies to common allergens are not positive. Several other conditions, including polyps, sinusitis, congenital and acquired anatomical abnormalities (e.g. nasal septal deviation, adenoid hypertrophy, hypertrophy of nasal turbinates, choanal atresia), b~nign and malignant tumours, granulomas, ciliary defects and cerebrospinal rhinorrhoea, 1,2 are known to mimic symptoms of nonallergic noninfectious rhinitis and therefore have to be excluded by careful examination in order to make the correct diagnosis. Anatomical nasal abnormalities block the flow of nasal secretions and lead to rhinorrhoea, postnasal drip and nasal blockage. In children, congenital choanal atresia can lead to reduced nasal airflow, resulting in nasal blockage. Tumours are not very common in the nasal passages, but when established and growing rapidly they often lead to unilateral nasal obstruction, bleeding and pain. Rhinorrhoea and nasal congestion, in the absence of pruritis, are also characteristic features of nasal mastocytosis, an extremely rare condition, in which eosinophils are absent and tests for IgE-mediated disease are negative.

1412 I

PART 13 THE NOSE AND PARANASAL SINUSES

DIAGNOSIS AS A STEP-WISE APPROACH

toxin

and

particularly

intranasal

CapSaICIn

may

be

beneficial. Studies in patients suffering from NANIPER In daily clinical practice. the diagnosis of nonallergic

have demonstrated that intranasal capsaicin leads to a

rhinitis and its subgroups is mainly based on a thorough

significant and long-term reduction in clinical VAS scores)

case history, followed by

the step-wise exclusion of

If the case history is suggestive of clinically relevant

•

also shown to significantly improve all symptoms and produce a larger vascular response throughout a six­

noninfectious rhinitis: •

compared with placebo.lO In another study) treatment once daily for five weeks with intranasal capsaicin was

possible differential diagnoses) as follows.

check possible stimuli. severity and duration of disease; check drug use (systemic and topic) exposure at work place) hormonal status (pregnancy) hypothyroidism. acromegaly) and involvement of other organs (asthma, hormonal status);

•

exclude other nasal disease (rigid nasal endoscopy);

•

exclude allergy: skin prick test) serum 19E-antibodies to the most frequent inhalant allergens) and ultimately nasal provocation testing in selected cases;

•

exclude chronic rhinosinusitis (computed

•

perform nasal cytology (eosinophilia) and if shown

month follow-up period in patients suffering from severe chronic nonallergic rhinitis with nasal vasoconstrictor abuse.30 In cases where nasal obstruction is resistant to medical treatment and if the inferior turbinate is hyperplastic)

surgical intervention to reduce the size

of the turbinate by various procedures has been shown to be useful. Ethmoidal and vidian neurectomies have also been performed by excision. diathermy and cryotherapy) and have produced results with varying degrees and duration of success.

[****/***J

tomography (CT ) scan); to be positive then perform oral aspirin challenge.

CONCLUSION Despite increasing evidence for the various forms of nonallergic rhinitis) many of these conditions are often

THERAPY FOR NONALLERGIC PERENNIAL RHINITIS

underrated and trivialized) primarily due to the paucity of information on the causative factors) the underlying mechanisms and/or the absence of specific diagnostic

First) an evaluation of the severity of the disease should be

tests.

performed to confirm the need for therapy) in connection

amongst clinicians and researchers regarding classification

Furthermore)

there

are differences in opinion

with counselling on how to avoid nonspecific stimuli.

of disease and in conditions such as occupational rhinitis)

In

in particular) the situation is further compounded as

case

of

drug-induced)

food-induced

or

occupa­

tional rhinitis) specific avoidance measures are employed

a

as first-line therapy.

implications) for both employers and employees alike.

consequence

of

legal

and

financial

compensatory

Several treatments (pharmaceutical, nonconventional

To date) no specific diagnostic test is available) and clinical

and surgery) have been employed for idiopathic non­

diagnosis is based on case history and exclusion of other

allergic rhinitis?B Intranasal anticholinergics (ipratro­

disease. Despite these difficulties) however) recent evi­

pium bromide) may be useful in patients with nasal

dence suggests that relevant diagnostic tools and specific

secretion as the predominant symptom. whereas nasal

information regarding the mechanisms underlying some

decongestants should be avoided or limited to ten days.

of these conditions is more forthcoming and should

Topical steroids and antihistamines are the two main

therefore lead to a better overall understanding and

classes of drugs employed) of which only fluticasone

management of these conditions in the future.

propionate) budesonide) beclomethasone and azelastine) respectively) have been approved by the Food and Drug Administration (FDA). Azelastine nasal spray has been found to be more effective than placebo for control of rhinorrhoea) post-nasal drip) sneezing and nasal conges­ tion in most

patients.

although

its effect

on nasal

•

congestion was rather marginal. 29 T he efficacy of in­ tranasal steroids in patients with vasomotor rhinitis has

•

been inconsistent. Furthermore) whilst the topical nasal steroids are more frequently used for treatment of more

•

severe symptoms. they are mostly useful in patients in whom

an

.

inflammatory pathogenesis is a promi­

nent feature of their

disease)

for

example NARES.

Recent evidence suggests that in patients who do not respond to treatment with nasal steroids, treatment with

Approximat I)' half of rhi Us patient may suffer from nonallergic rhinhis. There are no specific diagno tic test. for nonaU gic rhinitis. A thorough case hi ory is the best dtagno tic tooJ available and lould focus on drug intake, w0rk expo ure nd hormonal tatus to subdivide the onditi()n into ubgn�)Ups. 1\5 the, ymptoms are quite non�pecific, the differCHtial diagnoses have to be 6-xcluded.

•

nonconventional therapies such as silver nitrate) botulin

j

Chapter 110 Nonallergic perennial rhinitis

7.

I 1413

Settipane RA, Lieberman P. Update on nonallergic rhinitis. Annals of Allergy, Asthma and Immunology. 2001; 86:

.I Wherever possible, the treatment should take into account the aforementioned subgroups of nonallergic

*

494-507 . 8.

Rhinitis: the spectrum of the disease. In: Busse WW,

perennial rhinitis.

Holgate ST (eds). Asthma and rhinitis, 2nd edn. Oxford:

.I In idiopathic rhinitis, antihistamines and topical steroids are the two main classes of drugs for

Blackwell Science Ltd, 2000: 6-13. 9.

treatment. Topical steroids are to be preferred if an

human airway neurogenic inflammation. Journal of Allergy

.I Intranasal capsaicin treatment is still considered

.I Surgery is an option in patients with persistent nasal

Sanico A, Atsuta S, Proud D, Togias A. Dose-dependent effects of capsaicin nasal challenge: in vivo evidence of

inflammatory pathogenesis is suggested. experimental.

van Cauwenberge PB, Wang D-Y, Ingels KJAO, Bachert C.

and Clinical Immunology. 1997; 100: 632-41. 10.

Blom HM, van Rijswijk JB, Garrelds 1M, Mulder PGH, Timmermans T, Gerth van Wijk R. Intranasal capsaicin is

obstruction not improved by medication.

efficacious in nonallergic, non-infectious perennial rhinitis. A placebo-controlled study. Clinical and Experimental Allergy. 1997; 27: 796-801. 11.

Gerth van Wijk R, Fokkens WJ. The long-term effects of

Deficiencies in current knowledge and areas for future research

>-

capsaicin aqueous spray on the nasal mucosa. Clinical and Experimental Allergy. 1998; 28: 1351-8. 12.

There is a marked lack of diagnostic tools/procedures

nose is a stimulus for NANIPER but not for controls.

)- There is a marked lack of controlled trials for

>-

Epidemiological studies on prevalence of different

* 13.

Rhinology. 2000; 38: 172-6. Fokkens WJ. Thoughts on the pathophysiology of nonallergic rhinitis. Current Allergy and Asthma Reports.

types of nonallergic rhinitis are required. )- Research on mechanisms underlying these conditions

Braat JPM, Fokkens WJ, Mulder PG, Kianmaneshrad N, Rijntjes E, Gerth Van Wijk R. Forced expiration through the

for determining each type of these conditions. determining diagnosis.

Blom HM, Severijnen LA, van Rijswijk JB, Mulder PGH,

2002; 2: 203-9. 14.

is needed.

Milosevic DN, Janosevic UB, Janosevic SB. Intradermal tests with vasomotor agents in perennial nonallergic rhinitis. Acta Oto-Rhino-Laryngologica Belgica. 2000; 54: 465-71.

15.

Zambetti G, Moresi M, Romeo R, Luce M, Filiaci F. Nonspecific nasal provocation test with histamine. Analysis of

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Bousquet J, van Cauwenberge P, Khaltaev N, the ARIA

Steerenberg P, Doekes G et al. Upper airway inflammation assessed by nasal lavage in compost workers: A relation

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1414 I PART 13 THE NOSE AND PARANASAL SINUSES Hamano N, Terada N, Maesako K, Ikeda T, Fukuda S, Wakita J et al. Expression of histamine receptors in nasal epithelial cells and endothelial cells - the effects of sex hormones. International Archives of Allergy and Immunology. 1998; 115: 220-7. 23. Hamano N, Terada N, Maesako K, Numata T, Konno A. Effect of sex hormones on eosinophilic inflammation in nasal mucosa. Allergy and Asthma Proceedings. 1998; 19: 263-9. 24. Moneret-Vautrin DA, Hsieh H, Wayoff M, Guyot JL, Mouton C, Maria Y. Nonallergic rhinitis with eosinophilia syndrome a precursor of the triad: nasal polyposis, intrinsic asthma, and intolerance to aspirin. Annals of Allergy. 1990; 64: 513-8. 25. Leone C, Teodoro C, Pelucchi A, Mastropasqua B, Cavigioli G, Marazzini L et al. Bronchial responsiveness and airway inflammation in patients with nonallergic rhinitis with 22.

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eosinophilia syndrome. Journal of Allergy and Clinical Immunology. 1997; 100: 775-80. Devalia JL, Rusznak C, Wang J, Khair OA, Abdelaziz M, Calderon MA et al. Air pollutants and respiratory hypersensitivity. Toxicology Letters. 1996; 86: 169-76. Shah A, Sircar M. Postcoital asthma and rhinitis. Chest. 1991; 100: 1039-41. Lieberman P. Treatment update: nonallergic rhinitis. Allergy and Asthma Proceedings. 2001; 22: 199-202. Banov CH, Lieberman P. Efficacy of azelastine nasal spray in the treatment of vasomotor (perennial nonallergic) rhinitis. Annals of Allergy, Asthma and Immunology. 2001 ; 86: 28-35. Lacroix JS, Buvelot JM, Polla BS, Lundberg JM. Improvement of symptoms of nonallergic chronic rhinitis by local treatment with capsaicin. Clinical and Experimental Allergy. 1991; 21: 595-600.

111 Occupational rhinitis HESHAM SALEH

Introduction Risk factors Pathogenesis High risk occupations Effects of air pollution Sick building syndrome Diagnosis

1415 1416 1416 1418 1419 1420

Management Key points Best clinical practice Deficiencies in current knowledge and areas for future research References

1421 1421 1422 1422 1422

1420

The data in this chapter are supported by Medline and Embase searches using the key words rhinitis, occupational rhinitis, rhinitis and occupation, air pollutants and occupational, occupational disease, occupational accident, occupational allergy, occupational exposure, occupational hazard, occupational health, occupational medicine, occupational safety, work environment, industrial medicine, industry, industry and industrial phenomena, work and disability.

INTRODUCTION Occupational rhinitis (OR) has been defined as the episodic work-related symptoms of rhinitis. I Symptoms usually manifest on weekdays and abate during weekends and holidays. When exposure has been prolonged, the condition may become irreversible and symptoms persist. 2 ,3 It frequently coexists with asthma and conjunctivitis, and it has been suggested that occupational rhinitis precedes the development of occupational asthma. I, 3 Although reports of occupational rhinitis turning into asthma are scarce, in one study most of the rhinitis patients were younger than those with asthma, suggesting that rhinitis is the first to develop.4 Occupational rhinitis is commoner than occupational asthma and most reports show that it tends to occur three times more frequently. 3 Occupational asthma rarely occurs as an' isolated disease without occupational rhinitis. lOne report· found that 92 percent of patients with

..

/

occupational asthma have occupational rhinitis and 72 percent had conjunctivitis. 5 [** /*] The prevalence of occupational rhinitis is unknown. Studies have dealt primarily with occupational asthma with less work on rhinitis. It has been estimated that 2-5 percent of all patients with asthma in the USA have workinduced asthma. I It is possible that the incidence of occupational rhinitis is underestimated due to failure to diagnose and because, unlike asthma, it is not a life-threatening condition. Workers may be reluctant to report symptoms for this reason and for fear of job loss, loss of seniority or fear of repercussions from coworkers. 6 Various cross-sectionaf studies of populations from diverse occupations exi~t but, because of the different methodology used, the interpretation of data is difficult. Furthermore, data from epidemiological studies are lacking. Information from the Finnish Institute of Health showed that 20 percent of all cases of rhinitis were occupational in origin. 7 It also showed that the number of

1416 I PART 13 THE NOSE AND PARANASAL SINUSES cases more than doubled between 1987 and 1991. Another study, utilizing the Finnish register, showed that the incidence of occupational rhinitis is higher in men than women (60 and 40 percent, respectively). Also, a gender difference exists for the age at which occupational rhinitis develops. Men have the highest incidence at the age range of 25-29 years, after which the incidence gradually declines. In women, the incidence of occupational rhinitis gradually increases and peaks between 40 and 44 years of age. 4 It has been suggested that this difference is related to the fact that men and women are engaged in different jobs and hence exposed to different kinds of causative agents. 8 [** /*] More than 200 chemicals and organic dusts have been implicated as a cause of occupational rhinitis and asthma. 1 The pathological effects of these are either due to an allergic reaction or irritation of the nasal mucosa. Welldocumented causative agents include laboratory animals, such as rats, mice and guinea pigs, grains (bakers), storage mite (agricultural workers), wood dust, particularly hard wood such as western red cedar (furniture manufacturers), guar gum (workers in the food processing and carpet industry), Bacillus subtilis enzymes (detergent manufacturing workers), latex and chemicals such as acid anhydrides, isocyanates, platinum salts, glues and solv~nts.I, 3, 6 Symptoms may develop after a latency period of two months to 18 years in the case of allergic occupational rhinitis. 1, 6 This observation does not apply to irritant occupational rhinitis which can develop on immediate exposure. [***/**]

Approximately 80 percent of those that have an AED of more than 9 !lm, 50 percent of those with 2-9!lm AED and 40 percent of material with less that 2 !lm stick to the nasal wall. Highly soluble materials, such as ammonia, sulphur dioxide and formaldehyde, also deposit in the upper airway. Smaller particles and less water-soluble gases generally pass to the lower airways. 8 According to the nature of the offending agent, occupational rhinitis may be allergic or irritant. [***]

Allergic occupational rhinitis Allergic occupational particles may trigger an IgEmediated (type 1) immune response. A late-phase response may also develop four to six hours after the initial reaction. 8 The causative agents are classified into high molecular weight compounds (HMWC), such as animal and vegetable proteins, and low molecular weight compounds (LMWC), such as isocyanates and anhydrides. An IgE-dependent mechanism, that can be assessed through skin prick test and radioallergosorbent test (RAST) , has been demonstrated with most HMWCs. 3 , 8 LMWCs can also produce specific IgE antibodies by acting as a hapten to form a hapten-protein conjugate. However, an IgE-mediated mechanism has been elicited only rarely with LMWC. Table 111.1 shows a number of HMWCs and Table 111.2 shows LMWCs implicated in allergic occupational rhinitis with the related occupations. 3 ,6,11 [**/*]

RISK FACTORS Irritant occupational rhinitis Risk factors for developing occupational rhinitis include exposure (intensity and duration), atopy and smoking. 6 Most reports on exposure have focussed on intensity, and associations have been confirmed in laboratory animal workers, wool textile mill workers and bakery workers amongst others. 3 Duration of exposure has seldom been studied. Other studies have shown an association between atopy and specific sensitization to high molecular weight agents in grain elevators, fish and seafood workers, bakers, workers exposed to latex, coffee and others. Although smoking is known to induce the release of inflammatory mediators and to cause mucosal changes, its impact on occupational rhinitis is yet to be shown. The current evidence on this matter is still inconclusive as some studies show higher risk of occupational rhinitis in smokers and some do not. 3 , 6, 9 [**]

PATHOGENESIS The nose is the first portal of entry and is exposed to a constant stream of air. Materials that accompany the air stream tend to impact on the mucus surface as a function of their aerodynamic equivalent diameter (AED) .10

Nonallergic, irritant chemicals may trigger the release of substance P and other inflammatory mediators from sensory C-fibres after binding to chemoreceptors on their surface. I2 This sequence, known as neurogenic inflammation, leads to vasodilatation, oedema and other manifestations of inflammation. The patients complain of burning, stinging or painful sensation in the nasal passages in addition to nasal congestion and rhinorrhoea. I Delayed response does not occur in cases of irritant rhinitis. 8 Although some existing evidence shows the release of substance P in the nose with exposures to nicotine, capsaicin, ether and formaldehyde, no such data are available for other chemicals. Chronic irritant rhinitis requires protracted exposure to one or more irritating chemicals. However, in reactive upper airways dysfunction syndrome (RUDS), rhinitis results from an acute exposure to high concentrations of a respiratory irritant and persists after the exposure. 12 Table 111.3 shows a number of agents implicated in irritant occupational rhinitis with the related occupations. 1, 6,11 [**/*] Corrosive reactions may also occur due to excessive concentrations of irritating and soluble chemical gases. Substances implicated in causing corrosion are ammonia,

Chapter 111 Occupational rhinitis

Animal proteins (rats, mice, guinea pigs, rabbits, hamsters) Other animal derived allergens (pig) Grain dust Flour; alpha amylase

Bacillus subtilis Enzymes (papain, trypsin, pancreatic extracts, lactase) Wool Insects (locust, fruit fly) Arthropods Mites (storage, red spider) Mould spores Latex Guar gum/vegetable gum Green coffee bean Tea Dried fruits Tobacco leaf Cayenne peppers Pollens Sunflower pollen Soy bean dust Saffron flower Cocoa Garlic Milk proteins (alpha lactalbumin, casein) Fish, prawn, crab Bird proteins Raw poultry Cow dander

Table 111.2

Laboratory animal workers, pet shop owners, veterinarians, farmers Swine confinement workers Grain elevators Bakery workers Detergent workers Pharmaceuticals Wool textile workers Laboratory workers Laboratory workers Bakers, farmers, grain elevators, food processors Librarians Health care workers Food processors, carpet workers, printing Coffee production Tea workers Dried fruit workers Tobacco workers Hot pepper workers Gardeners, greenhouse workers Bakers, agricultural workers Farmers, food processors Saffron workers Packers Food workers Chocolate manufacturing, tannery Food processors Poultry breeders, farmers Food processors Dairy farms

Low molecular weight compounds (LMWC) causing allergic occu

Diisocyanates (toluene diisocyanates, diphenyl-methane diisocyanates) Anhydrides (trimellitic anhydride, methyl tetrahydrophtalic anhydride, pyrotomellitic anhydride) Wood dust (western red cedar, several other woods) Colophony Metals (platinum, nickel, chromium) Drugs (psyllium, senna, spiramycin) Chemicals (reactive dyes, carmine, polyamide, polyester, para-amide) Cotton Persulphates Solvents and organic dust Azo dyes Chlorine

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Painters, urethane mould workers Epoxy resin production, plastics, electric condenser workers, chemical workers Furniture making, carpentry Electronics, welders Platinum refinery, metal processing or plating Health care workers, bulk laxatives factory workers, pharmaceuticals Reactive dye products, synthetic fibre Cotton mills Persulphate products, hairdressers Shoe manufacturing Textile dying Pulp and paper production

I 1417

1418 I PART 13

THE NOSE AND PARANASAL SINUSES

Ammonia, nitrogen dioxide, hydrogen sulphide Smoke Cooking vapour Glutaraldehyde, formaldehyde Inorganic acid vapours Sulphur dioxide Solvent vapours Zinc chloride smoke Chlorine, chlorine dioxide, hydrogen sulphide Oxides of nitrogen, ozone, diesel exhaust Asphalt vapours, polycyclic aromatic hydrocarbons Metallic oxide fumes Talc

Agricultural workers Firefighters Food service workers Health care workers Laboratory workers Power plant, oil refinery workers Printers, painters Military personnel Pulp mill workers Traffic wardens, lorry drivers, railroad workers Roofers, pavers Welders Pharmaceuticals workers, cosmetics manufacturers

hypochloric acid, vinyl chloride, organic sulphur containing compounds, acrylamide, cyanide, nitriles and organophosphoside compounds. 1 Disorders of olfaction may occur as a result of rhinitis or as a direct effect of irritants on the olfactory receptors and their central connections. 13 Anosmia and hyposmia have been reported after single high-dose exposure to sulphuric acid, hydrogen selenide, phosphorous oxychloride and a mixture of pepper and cresoL S Other case reports of olfactory disorders present workers chronically exposed to low levels of chemicals such as benzene, benzol, cadmium, carbon disulphide, ethyl acetate, formaldehyde, hydrazine, menthol, solvents and oil of peppermint. s, 14 However, olfactory disorders in occupational rhinitis are otherwise underinvestigated. [**/*] Similar to other patients with allergic and nonallergic rhinitis, patients with occupational rhinitis may manifest nasal hyperreactivity to nonspecific environmental agents such as changes in temperature and humidity, exposure to tobacco smoke and strong odours. 1 The prevalence of this entity is, however, unknown. [*]

The prevalence of occupational rhinitis in farmers is unknown. However, a variety of agricultural exposures have been associated with rhinitis. These include grain farming and handling, livestock breeding, feed manufacture and handling, dairy farming, tea farming, cotton, flax and hemp processing and organophosphorous pesticides. 15 Storage mites, present in storage hay, have been described as a cause of barn allergy. A number of reports on grain elevator workers showed prevalence of sensitization in 9-28 percent? [**]

HIGH RISK OCCUPATIONS

Food-processing workers

Data from the Finnish Register of Occupational Diseases identified furriers, who are exposed to different kinds of animal epithelium, as the highest risk group for developing occupational rhinitis. Livestock breeders and bakers were the occupations following in rank. 4 Other commonly reported occupations include farmers, food-processing workers, wood workers, detergents manufacturers, animal laboratory workers and healthcare workers. [**]

It has been suggested that the food industry accounts for

Bakers A large number of cross-sectional studies show that workrelated symptoms of rhinitis and sensitization are

common among bakery workers. Work-related rhinitis has been shown in 18-29 percent of workers and specific sensitization in 10-38 percent. 3 The offending agents are wheat and cereal flours, cereal amylases, fungal amylases, storage mites contaminating wheat flour and others such as baking additives. [**]

Farmers

the largest number of cases of occupational rhinitis.12 Cases of occupational rhinitis due to guar gum are well documented. It is obtained from the seeds of the tree Cyamopsis tetragonoloba, and used as a food additive as well as a laxative and a colour fixing in agent in the carpet industry. The prevalence of symptoms in workers is, however, less well documented. Symptoms related to other plant-related allergens, such as soybean, coffee bean, garlic, cinnamon and hot peppers, have also been reported. 3 , 12 Occupational rhinitis has also been correlated to fish and seafood proteins. 3 Prevalence of symptoms ill' 5-25 percent of fish factory workers has been reported. 3 [**/*]

Chapter 111 Occupational rhinitis

Wood manufacturers Hard woods, often of tropical ongm, are well-known factors in causing work-related symptoms. Plicatic acid, found in western red cedar, has been suggested as the causative agent of rhinitis in carpenters and furnituremanufacturing workers. 3,16 Symptom prevalence figures are less well documented. Oak, beech and pine have also been implicated. 16 Soft woods, used in joiner shops, have been less reported in relation to rhinitis.16 [**/*]

Detergent manufacturers Biological enzymes are used extensively in the detergent industry as weli as the pharmaceutical and food industry. Bacillus subtilis enzymes and lactase have been commonly implicated in occupational rhinitis. 1, 3, 6 Prevalence rates of occupational rhinitis from 7 to 16 percent have been reported in workers. Specific sensitization ranged from 22 to 52 percent. 3 [**]

Animal laboratory workers Laboratory workers are one of the groups most commonly affected with occupational rhinitis.9 It has been suggested that proteins from virtually any mammalian species may induce an allergic response in human beings. 1 Rats are the most commonly implicated but mice, guinea pigs, rabbits and hamsters are also known to cause occupational rhinitis. 3, 9 Immunological substances implicated include saliva, urine, dander and serum. Reports demonstrate symptom prevalences of 10 to 33 percent and specific IgE antibodies to animal protein in 6-46 percent of workers. 3 [**]

Health care workers

.

Concern about latex allergy in health care workers and patients has risen due to the extensive use of latex gloves. Rubber latex is a naturally occurring cytoplasmic exudate obtained from the tree HeveaBrasiliensis. 1? It is used extensively in the manufacture of surgical gloves and other medical devices (drains, ambu bags, mouth gags and bandages are some) .18 Aerosolized particles of latex proteins are carried in the cornstarch used to powder the gloves and may remain airborne for hours. 18, 19 Significant quantities of airborne latex have been measured in areas using powdered latex gloves. 19 These proteins have been implicated in causing rhinitis, asthma and anaphylaxis. 1?, 18, 19 Exposed personnel may also develop contact dermatitis as a reaction to the chemical accelerators and preservatives that are added to latex to improve its physical property. 18 Anaphylaxis most often affects

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I 1419

patients during open surgery due to mucosal absorption and has been reported in those who have had multiple surgical procedures and in patients with spina bifida. 1? However, anaphylactic reactions were also reported during gloving and inhaling latex in the workplace. I? Health care workers with regular latex exposure have been reported to have symptom prevalence rates of 3-17 percent. 20 Specific skin tests have been shown to be positive in 5-20 percent in contrast to prevalence in the general population of less than 1 percent. I? [**] Other substances that may cause occupational rhinitis in health care workers include formaldehyde and drugs such as psyllium, senna and spiramycin.3 Formaldehyde is also used widely in industry in addition to its use as a sterilizing agent in medicine. It is toxic at high doses and can induce irritant reaction. It has also been suggested that it can act as hapten and cause an IgE mediated reaction. 21 [**/*]

Other occupations Workers reported to have occupational rhinitis in other occupations are detailed in Tables 111.1, 111.2 and 111.3. [**/*]

EFFECTS OF AIR POLLUTION In the first half of the twentieth century, dramatic episodes of severe pollution caused by sulphur dioxide (S02) derived from coal combustion occurred in the western world. Regulations to decrease the use of coal in homes and industry were set and other fuels, such as petrol and gas, substituted coal. The bypro ducts of petrol consumption in transport and industry, however, have replaced the decline of coal-associated pollution. Exhaust fumes consist of oxides of nitrogen, namely nitric oxide (NO) and nitric dioxide (N0 2), ozone (0 3), particulate matter (PM) and volatile organic compounds (VOCS).22 Much of the research carried out on pollution has been on its effects on the lower airway. However, epidemiological studies suggest an association between pollution and rhinitis.23 Laboratory studies have shown that exposures to NO, 0 3 and diesel exhaust particles result in the release of inflammatory mediators in the nasal mucosa. 24 A link between high levels of environmental pollutants and 25 increased prevalence of allergy has also been suggested. Whether this would have an impact on allergic occupational rhinitis remains to be examined. [***/**] Certain occupations, such as tt:affic wardens, railroad workers and lorry drivers, may be exposed to high levels of pollution. Epidemiological studies in this area are almost nonexistent. One study from Thailand showed a high prevalence of symptoms of rhinitis of traffic 26 policemen compared to the general population. Further research is needed in this area. [**]

I

.l1f~)jZkl . i.

1420 I PART 13 THE NOSE AND PARANASAL SINUSES

SICK BUILDING SYNDROME Sick building syndrome (SBS) is described as the high prevalence of eye, nose and throat irritation, fatigue and headache among office workers temporally associated with time at work and is characterized by an absence of abnormal physical findings and laboratory results. 8 It is a diagnosis of exclusion as multiple causative agent have been implicated. More than 300 VOCs were identified as indoor pollutants causing SBS. They originate from building materials, combustion fumes, cleaning compounds, paints and stains, in addition to tobacco smoke. It has been suggested that the type of ventilation is important in the causation of SBS. 27 One study showed that workers in buildings with mechanical ventilation and air conditioning have a higher risk of SBS symptoms than workers in naturally ventilated buildings. 28 [**/*]

DIAGNOSIS Diagnosing occupational rhinitis can be difficult. A combination of history, examination and investigation will aid the diagnosis. Nasal challenge with the suspected agent is particularly central to secure the diagnosis. [** /*]

first -degree relatives. 1, 2 An enquiry about problems of the sense of smell and taste should also be made. [**/*] A detailed occupational history includes the patient's account of a typical working day, the nature of chemicals, duration and intensity of daily exposure and use of protective masks. 8 Substance information sheet or an engineer's report may also be required, particularly in medicolegal cases. Other work factors such as type and efficiency of ventilation, history of accidents and spills, and effects on co-workers should also be considered. 26 A site visit has been recommended if the history of work conditions is unhelpful. 1 [*]

~Examination The physical findings in occupational rhinitis are nonspecific. Nasal examination should include description of mucosal changes, nature of secretions, crusting, epistaxis, ulceration and perforation. These are particularly important in medicolegal cases. 1 Crusting and epistaxis are more common in irritant rhinitis. Eye inspection for conjunctivitis and oral cavity/oropharynx examination for ulceration should also be carried out. Chest examination for signs of asthma is important as well as inspection for dermatitis, which occurs in latex allergy. [*]

History Investigations The patients present with a variable number of symptoms including nasal obstruction, anterior rhinorrhoea, postnasal discharge, crusting, disturbed sense of smell and taste and epistaxis? A detailed history of the patient's complaint in addition to occupational history is essential to aid the diagnosis. Development of symptoms during the working week with improvement at weekends and holidays provide the clue for diagnosis. However, history in itself is not sufficient for diagnosis and could be feigned by workers claiming compensation. The history can be further compounded by the presence of symptoms of rhinitis due to atopy in allergic patients. In patients with protracted exposure, the symptoms may continue during absence from work, further complicating matters. 1 1 The difference in presentation between allergic and irritant occupational rhinitics should also be borne in mind. In allergic patients, rhinitis may take longer to develop and may be manifested by a delayed response a few hours after exposure. On the other hand, patients with irritant rhinitis may develop the symptoms instandy on exposure and do not have a delayed response. 1 A history of eye, throat, skin and chest symptoms should be elicited. It is important to enquire about smoking and drug history including overthe-counter decongestants, substance abuse and other drugs known to cause nasal symptoms such as aspirin, alpha-blockers, beta-blockers, antidepressants and angiotensin-converting enzyme (ACE) inhibitors. Enquiries should be made on history of atopy in the patient and

The skin prick test is useful in allergic occupational rhinitis if an appropriate antigen exists. It is also helpful in diagnosing suspected atopy. Some occupational antigens are available commercially, such as in the case of latex. However, sensitization to an occupational agent is not sufficient to diagnose occupational rhinitis, because it only indicates an immunological response and not necessarily a clinical syndrome. 3 Skin prick tests have been used extensively in cross-sectional studies to assess sensitization to a specific agent. Sensitivity and specificity have been evaluated with differing results. 3 Measuring serum specific IgE RAST has also been used in a number of studies. The sensitivity and specificity is probably less than that of the skin prick test. It has been suggested that a combination of both tests may enhance sensitivity and specificity.6 [***/**] A secure diagnosis cannot be made without an attempt to nasal challenge. 1, 6 This is more conclusive when performed in controlled conditions in the laboratory with known agents. Carefully graded exposure to increasing doses of the suspected agent in specialized ventilated environmental chambers is the best method. Difficulties arise when dealing with unknown or multiple agents?' l3 On these occasions, a work place challenge may be an option. 3 ,15 Unfortunately, no standardized method for nasal challenge is yet recommended and the test is time consuming.-The test must be accompanied by an objective measure of the nasal response, such as acoustic

Chapter 111 Occupational rhinitis

rhinometry. It is suitable for rapid and repeated measurement and its portability makes it ideal for assessment in the work place. 4 Nasal inspiratory flow (NIPF) rate measurement is also useful with nasal challenge. It can also be used by the patient to measure fluctuations of the nasal airway during the working week. 3 Other measures, such as collecting nasal lavage for analysis of cells and inflammatory markers, rhino manometry and brush biopsies, have been used with nasal challenge. They are generally more suitable for research situations and have been used extensively in various trials. 3 [***/**] When an olfactory disorder is suspected and in medicolegal cases, olfaction should be tested using one of the commercially available tests, such as the University of Pennsylvania Smell Identification Test (UPSIT).2 [*]

MANAGEMENT The best management approach for occupational rhinitis is prevention. In established cases avoidance methods should be instituted and medical therapy may be tried. Immunotherapy may have a part to play in treating severe cases of allergic occupational rhinitis. [Grade C]

Prevention It has been suggested that applicants for high risk jobs

should be screened for atopy by history taking and a skin prick test. However, in a study on laboratory animal workers, this method was shown to be inefficient. Rejecting workers who were atopic by history and skin prick test leads to an avoidance of 44 percent of workers who develop rhinitis and asthma and only 23 percent of those who develop rhinitis alone. Eliminating those with a positive skin test only removes 13 percent of those who develop rhinitis.29 Prevention in the work place may be effective and should always be considered. 9 Environmental control measures are undertaken by recognizing potentially sensitizing allergens and controlling the point source of any mucosal irritant. Masks, gloves and protective clothing should be used and ventilation improved. 1, 6, 9 [Grade C] In workers with established diagnosis, certain avoidance measures may be effective. For example, laboratory workers who become sensitive to rats generally react to rat urinary protein rather than epithelium. It has been suggested that it may be possible for them to perform surgery on rats, if the cage containing the rats - which is soaked with urine - is kept out of the laboratory.9 In health care workers sensitive to latex, the use of powderfree gloves by all co-workers has been shown to reduce symptoms and allow allergic patients to continue their work. 17 Low protein latex gloves have also been suggested but studies confirming their effectiveness are needed. I7 Nonlatex gloves are becoming available and may be used as an alternative for sensitive individuals. In some

I 1421

occupations where preventive measures are ineffective, relocation of the worker or even changing jobs may be recommended. [Grade C]

Medical therapy Medical therapy of occupational rhinitis is similar to that of other types of rhinitis. Antihistamines, sodium cromoglycate, topical corticosteroids, anticholinergic spray and saline douches have all been used. 1,6,9 Systemic steroids have also been suggested in severe cases. 6 Treatment may be most effective in allergic occupational rhinitis. 6 However, no studies on the efficacy of these medications in occupational rhinitis are available at present. The choice of agent is often based on trial and error according to efficacy and frequency of side effects. Sedating antihistamines affect work productivity adversely and second-generation antihistamines are preferred. Patients may use them regularly when they have chronic symptoms or periodically before an expected exposure to an allergen. 9 [Grade D]

Immunotherapy Limited information is available on the efficacy of immunotherapy in allergic occupational rhinitis. Fear of severe reactions with continued exposure to the offending antigen at the same time of the administration of immunotherapy have precluded its use. 1 Furthermore, many of the offending antigens are not available for treatment. 9 A recent study showed that latex immunotherapy led to significant improvement in rhinitis symptoms but several adverse effects occurred in the study group.30 However, where alternative jobs are not a consideration, the risks of treatment should be balanced against the benefits.1 [Grade C]

1422 I

PART 13 THE NOSE AND PARANASAL SINUSES

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>

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113 Rh i nasi n usitis

MICHAEL S BENNINGER

1439 144{) 1442 1443 1443

Defi niti0 ns Pathophysiology Diagnosis Natural history Prevalence of rhinos/nus'ltis

The economic and quality of life impact of rhinosinusitis Key points Best clinical practice Deficiencies in knowledge and areas for future research References

1444 1447 1447 1447 1447

The data in this chapter are supported by a MedJine search using the key words sinusitis, rhinosin usitis, bacterial, sinusitis, actute sinusit is, chronic sinusitis, fungal sinusitis an d rh initis.

t emporal differences in the pr esentatio n and, in some on the c li nic al presentation. T here are also

DEFINITIONS

cases, a group of disorders

differences in the histopathology and the bacteriology of

characterized by inflammation of the mucosa of the

acute and chronic rhinosinusitis. Although somewhat

Be cause the inflammatio n

accepted that In the majority of patients, no obvious cause for

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49-58.

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12.

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> Further trials involving randomi zation to further

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Asthma and Immunology. 1997; 78: 270- 6.

13.

Hartwig S, Linden M, Laurent C, Vargo AK, Lindqvist N.

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polypectomy (a double blind clinical trial). Journal of

> The observation that raised levels of NO indicates patent ostiomeatal complexes needs confirmation, as

Laryngology and Otology.

14.

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I ntr od uction

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K ey p oi nt s

1 496

D ia gnosis

1478

Best c l in ica l pra ct ic e

1496

S ur gica l appr oac h es

1 480

D ef iciencies i n current kn owled ge a nd a reas f or f ut ur e

S ur gica l pr oced ur es R hi nosi nusit is i n c hi ld ren

1481 1 495

R ef er enc es

Conc lusi ons

1 496

1 49 6

r esear ch

1496

The data in this chapter are supported by a Medline search as

using the key words rhino sinusitis, sinusitis and surgery, as well data taken from the authors' personal collection of literature.

INTRODUCTION

has been adopted intern ationally in recent yea rs , l, 2 in recognition of the physiology and pathophysiology within the nose and sinuses, where it is impossible to make an absolute distinction between where one starts and the other ends. An increasing recognition of the importance of the lateral wall of the nose and in par ticular the ostiomeatal complex has reinforced the use of this terminology, although clearly one anatomical area may be more obviously affected than another. None­ theless imaging studies on the common cold3 clearly demonstrated that even a viral rhinitis produced inflam­ matory c hanges in over 80 percent of the adjacent sinus. In a preantibiotic era, surgical drainage of the sinuses was a necessity in cases that had failed to resolve spontaneously and were often potentially li fe threatening The indications for surgery have changed somewhat with a host of effective medical therapies now avail­ able, but despite these a cohort of patients remain in whom surgery will be required. Nonetheless, adequa te trial of medical therapy is a prerequisite in most cir­ cumstances, as we recognize that many patients will

continue to need medical therapy even when surgery is undertaken (Figure 117.1).

The term 'rhinosinusitis'

-

DIAGNOSIS While a careful clinical history of

remains the cornerstone diagnosis and all patients will undergo a general

.

Surgery Figure 11 7.1

.Algori thm of ma na gem en t of pat ient s wit h

c hr on ic rhi nosi nusi tis.

Cha pter 11 7 Sur gi cal m anageme nt of rh inosi nu si ti s I

otorhinolaryngological examination, the emphasis has moved towards endoscopy supported by appropriate imaging to confirm the diagnosis, define the extent of pathology and demonstrate relevant anatomy. A

number of international consensus groups and task

1479

However, the diagnostic role of endoscopy cannot be too strongly emphasized and it should be available for routine clinical use both in outpatients and in the operating theatre.

forces on behalf of organizations -with a clinical interest in

this

area

have

considered

the

classification

and

diagnosis of rhinosinusitis. 1, 2, 3, 4, 5 In recent years in the absence of firm pathophysiological criteria, clinical

definitions have relied upon duration and symptom complexes of varying severity ideally corroborated by

endoscopy and imaging.

[**/*]

Radiology The role of plain sinus x-rays has been the subject of considerable discussion as false-positives and false­ negatives

can

occur, particularly in infants and children.12

Neither is it possible adequately to visualize the ostio­ meatal area

(Figure 117.2),

identify anatomical variants

or judge important surgical features, such as prominent or

Endoscopy

dehiscent optic or carotid canals.

Endoscopy was first performed by Hirschmann6 in

1903

using a modified Nitze cystoscope which he used in the nasal cavity and in the maxillary sinus via a tooth socket. This cystoscope had a diameter of

5 mm and an electric bulb deflected 90° by a distal prism with 180° rotation. Reichere in 1902, Sargon8 in 1908 and Imhofer9 in 1910 all used similar instruments and even attempted removal of foreign bodies. In

1922, SpeilberglO was the first to

Zinreich et al.13 popularized a protocol for computer­

assisted tomography (CT) using sections taken in the coronal plane -with - The su.ggestion that environmental fungi may be an initiating factor needs further investigation. >-

,/

is

Chapter 1 2 1 Nasal polyposis

� At present, the immune inflammatory process is

10.

11.

Laryngology and Otology 1984; 98: 783-93.

� It is worth studying whether intranasal corticosteroids 12.

Braun JJ, Haas F, Conraux C. Polyposis of the nasal sinuses. Epidemiology and clinical aspects of 350 cases. Treatment

drops.

and results with a follow-up over 5 years on 93 cases.

� The effect of systemic corticosteroid treatment needs

Annales d'Otolaryngologie et de Chirurgie Cervico Faciale.

to be investigated in placebo-controlled, dose-response studies.

� The possibility that a long-term treatment with a low

Drake-Lee AB, Lowe D, Swanston A, Grace A. Clinical profile and recurrence of nasal polyps. Journal of

antibodies can be a future treatment. can be given in a more efficient way, e.g. as nose

Caplin I, Haynes TJ, Spahn J. Are nasal polyps an allergic phenomenon? Annals of Allergy 1971; 29: 631-4.

undergoing detailed analysis, which is needed in order to study whether, for example, treatment with IL-5

I 1557

1992; 109: 189-99. 13.

Keith PK, Conway M, Evans 5, Wong 5, Jordana G, Pengelly

dose of systemic corticosteroid in selected patients

D et al. Nasal polyps: effects of seasonal allergen

has a beneficial effect which outweighs the risk of

exposure. Journal of Allergy and Clinical Immunology

1994; 93: 567-74.

side effects needs a thorough investigation.

� It is possible that leukotriene antagonists can have a

14.

nasal polyps in adults with cystic fibrosis. Clinical

beneficial effect in subgroups of polyp patient, for

Otolaryngology 2000; 25: 19-22.

example in those who are intolerant to NsAlDs.

� Controlled studies of surgery are required.

Hadfield PJ, Rowe-Jones JM, Mackay IS. The prevalence of

15.

� It is necessary in long-term controlled studies to

Brihaye P, Clement PAR, Dab I, Desprechin B. Pathological changes of the lateral nasal wall in patients with cystic fibrosis. International Journal of Pediatric

analyse what is the optimal combined medical and

Otorhinolaryngology 1994; 28: 141-7.

surgical management. 16.

s�)rensen H, Mygind N, Tygstrup I, Flensborg EW. Histology of nasal polyps of different etiology. Rhinology. 1977; 15: 121-8.

17.

Oppenheimer EA, Rosenstein BJ. Differential pathology of nasal polyps in cystic fibrosis and atopy. Laboratory

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Cody DT, Neel HB, Ferreiro JA, Roberts GT. Allergic fungal

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Cauna N, Hinderer KH, Manzetti GW, Swanson EW. Fine structure of nasal polyps. Annals of Otology, Rhinology

settipane GA. l'Jasal polyps: pathology, immunology and treatment. American Journal of Rhinology 1987; 1:

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Pedersen M, Mygind 1\1. Rhinitis, sinusitis and otitis media in Kartagener's syndrome. Clinical Otolaryngology 1982;

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Rowe-Jones .1M, shembekar M, Trendell-smith N, Mackay

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polyps: Epidemiology, pathogenesis and treatment. The

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Henderson Jr. WR, Chi EY. Degranulation of cystic fibrosis

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Ruhno J, Howie K, Anderson M, Andersson B,

sinusitis: The Mayo Clinic experience. Laryngoscope. 1994;

Vanzieleghem M, Hitch D et al. The increased number of

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epithelial mast cells in nasal polyps and adjacent

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Drake-Lee A, Price J. Mast cell ultrastructure in the inferior turbinate and stroma of nasal polyps. Journal of

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Laryngology and Otology. 1997; 111: 340-5. 30.

Drake-Lee AB, McLauhlan P. Clinical symptoms, free

intranasal budesonide in nasal polyposis. Rhinology. 2000;

histamine and IgE in nasal polyps. International Archives

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Prades .1 M et al. Efficacy and tolerability of budesonide

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Beck L, Stellato C, Beall 0, Schall TJ, Leopold 0, Bickel CA

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adhesion molecules in nasal polyps. Journal of Allergy and Tingsgaard PK, Bock T, Larsen PL, Tos M. Topical

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Otolaryngologica. 1999; 119: 362-8.

Lapp P et al. A double-blind comparison of intranasal budesonide with placebo for nasal polyposis. Journal of

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Allergy and Clinical Immunology. 1990; 86: 946-53. 52.

Denburg J. Nasal polyposis: Cytokines and inflammatory inflammatory disease and its treatment. Copenhagen:

European Journal of Respiratory Disease. 1982; 63: 221-8. 53.

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Deuschl H, Drettner B. Nasal polyps treated with beclomethasone dipropionate aerosol. Rhinology. 1977;

Munksgaard, 1997: 78-87. Lund VJ. Diagnosis and treatment of nasal polyps. British Medical Journal. 1995; 311: 1411-4.

Holopainen E, Grahne B, Malmberg H, Makinen J, Lindqvist N. Budesonide in the treatment of nasal polyposis.

cells. In: Mygind N, Lildholdt T (eds). Nasal polyposis: An

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Rhuno J, Andersson B, Denburg J, Anderson M, Hitch 0,

Bachert C, Gevaert P, Holtappels G, Cuvelier C, van Cauwenberge P. Nasal polyps: From cytokine to growth.

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Drettner B, Ebbesen A, Nilsson M. Prophylactic treatment with flunisolide after polypectomy. Rhinology. 1982; 20:

budesonide treatment reduces endothelial expression of and eosinophil infiltration in nasal polyps. Acta

Weber R, Keerl R, Radziwill R, Schick B, Jaspersen 0, Dshambazov K et al. Vidioendoscopic analysis of nasal

Clinical Immunology. 1996; 98: 766-80.

* 34.

Jankowski R, Schrewelius C, Bonfils P, Saban Y, Gilain L,

Liu C M, Shun CT, Hsu M M. Lymphocyte subsets and

et al. Detection of chemochine RANTES and endothelial

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Filiaci E, Passali 0, Puxeddu R, Schrewelius C. A randomized controlled trial showing efficacy of once daily

15: 17-23. 54.

Lund V, Mackay I. Staging of rhinosinusitis. Rhinology.

Elbr0nd 0, Felding JU, Gustavsen KM. Acoustic rhinometry used as a method to monitor the effect of

1993; 31: 183-4.

intramuscular injection of steroid in the treatment of

38.

Drake-Lee AB. Medical treatment of nasal polyps.

nasal polyps. Journal of Laryngology and Otology. 1991;

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Holmberg K, Karlsson G. Nasal polyps: Surgery or

Rhinology. 1994; 32: 1-4.

105: 178-80. 55.

pharmacological intervention? European Respiratory

nasal polyps with intranasal beclomethasone dipropionate aerosol. Clinical Allergy. 1975; 5: 159-64.

Review. 1994; 4:20: 260-5. 40.

41.

Mygind N, Lund V. Topical corticosteroid therapy of

56.

treatment of nasal polyps with beclomethasone

Lildholdt T, Mygind N. Effect of corticosteroids on nasal

dipropionate aerosol. Acta Otolaryngologica. 1976; 82:

inflammatory disease and its treatment. Copenhagen:

256-9. 57.

Munksgaard, 1997: 160-9.

44.

Otolaryngologica. 1988; 105: 140-3. 58.

nasal spray 0.0250f0 in the prophylactic treatment of nasal

treatment trial of nasal polyps in adults with cystic

polyposis after polypectomy. A randomized double-blind

fibrosis. Rhinology. 2000; 38: 63-5.

parallel, placebo-controlled study. Rhinology. 1985; 23:

Johansen LV, Ilium P, Kristensen S, Winther L, Petersen SV,

49-58. 59.

treatment of small and medium sized nasal polyps. Clinical Lildholdt T, Rundcrantz H, Lindqvist N. Efficacy of topical

Karlsson G, Rundcrantz H. A randomized trial of intranasal beclomethasone dipropionate after polypectomy.

Otolaryngology. 1993; 18: 524-7.

46.

Dings6r G, Kramer J, Olsholt R, S6dersstr6m T. Flunisolide

Hadfield PJ, Rowe-Jones JM, Mackay IS. A prospective

Synnerstad B. The effect of budesonide (Rhinocort) in the

* 45.

Lildholdt T, Fogstrup J, Gammelgaard N, Kortholm B, Uls0e C. Surgical versus medical treatment of nasal polyps. Acta

Badia L, Lund V. Topical corticosteroids in nasal polyposis. Drugs. 2001; 61: 573-8.

43.

Pedersen CB, Mygind N, S0rensen H, Prytz S. Long-term

rhinitis. Clinical Immunotherapy. 1996; 5: 122-36. polyps. In: Mygind N, Lildholdt T (eds). Nasal polyposis: An

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Mygind N, Pedersen CB, Prytz S, S0rensen H. Treatment of

Rhinology. 1982; 20: 144-8.

* 60.

Rowe-Jones J, Medcalf M, Durham S, Richards 0, Mackay

corticosteroid powder for nasal polyps: A double-blind,

I. Functional endoscopic sinus surgery: 5 year follow up

placebo-controlled study of budesonide. Clinical

and results of a prospective, randomised, stratified,

Otolaryngology. 1995; 20: 26-30.

double-blind, placebo controlled study of postoperative

Tos M, Svendstrup F, Arndal H, Orntoft S, Jakobsen J,

fluticpsone propionate aqueous nasal spray. Rhinology.

Borum P et al. Efficacy of an aqueous and a powder

2005; 43: 2-10.

Chapter 121 Nasal polyposis

61.

Lildholdt T, Rundcrantz H, Bende

M,

Larsen K.

medical treatment versus surgical treatment in addition to medical treatment of nasal polyposis. Journal of AI/ergy

Glucocorticoid treatment for nasal polyps. A study of budesonide powder and depot-steroid injection. Archives of Otolaryngology - Head and Neck Surgery. 1997; 123: 62.

I 1559

and Clinical Immunology. 1991; 107: 224-8. 63.

Browne J P, Hopkins C, Slack R, Topham J, Reeves BR, Lund

595-600.

V et al. Health-related quality of life after polypectomy

Blomqvist EH, Lundblad L, Anggchd A, Haraldsson PO,

with and without additional surgery. Laryngoscope. 2006;

Stjarne P. A randomized controlled study evaluating

116: 297-302.

I I

.-..--. the influence of both anatomy and surgery on the aerodynamics of the nose in relation to the interaction between air and mucosa; >- materials for reconstruction of the septum, in the event of autologous materials no longer being available.

REFERENCES Barelli PA, Loch EE, Kern EB, Steiner A (eds). Rhinology: the collected writings of Maurice H. Cottle MD. Warwick, NY; American Rhinologic Society, 1987. 2. VelWoerd CD, Urbanus NA, Mastenbroek GJ. The influence of partial resections of the nasal septal cartilage on the growth of the upper jaw and the nose; an experimental study in rabbits. Clinical Otolaryngology. 1980: 5: 291-302. 3. VelWoerd CD, Urbanus NA, Nijdam DC. The effects of septal surgery on the growth of nose and maxilla . Rhinology. 1979; 17: 53-63. 4. Pirsig W. Surgery of the nose in childhood: growth and late re!'Sults. Laryngologie, Rhinologie, Otologie. 1984; 63: 170-80. 1.

Best clinical practice ,/ After nasal trauma, examination of the internal nose for haematoma is obligatory. A septal haematoma should be treated urgently to avoid a septal abscess. .I In patients with a fever and nasal blockage three to five days after an insignificant nasal injury, a septal abscess should be suspected as a sequela of a missed septal haematoma.

*

Chapter 123 The septum

5.

6.

7.

8.

9.

* 10. 11.

* 12.

Grymer LF, Bosch C. The nasal septum and the development of the midface. A longitudinal study of a pair of monozygotic twins. Rhinology. 1997; 35: 6-10. Grymer LF, Pallisgaard C, Melsen B. The nasal septum in relation to the development of the nasomaxillary complex: a study in identical twins. Laryngoscope. 1991; 101: 863-8. Pirsig W. Growth of the deviated septum and its influence on rn idfacia I development. Facial Plastic Surgery. 1992; 8: 224-32. Huizing EH. Septum surgery in children; indications, surgical technique and long-term results. Rhinology. 1979; 17: 91-100. Maran AGD, LUlTd VJ (eds). Clinical rhinology. Stuttgart: Thieme, 1990. Moore EJ, Kern EB. Atrophic rhinitis: a review of 242 cases. American Journal of Rhinology. 2001; 15: 355-61. Jessen M, Ivarson A, Maim L. Nasal airway resistance and symptoms after functional septoplasty: comparison of findings at 9 months and 9 years. Clinical Otolaryngology. 1989; 14: 231-4. Roblin DG, Eccles R. What, if any, is the value of septal surgery? Clinical.Otolaryngology. 2002; 27: 77-80

13.

* 14. 15.

16. 17.

18.

19.

* 20. 21.

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Bitzer EM, Dorning H, Schwartz FW. Der klinische Erfolg der operativen Korrektur der Nasenscheidewand. Laryngorhinootologie. 1996; 75: 649-59. Dorning H, Bitzer EM, Schwartz FW. Patientenzufriedenheit als Health Care Outcome- das Beispeil der operativen Korrektur der Nasenscheidewand. Gesundheidswesen. 1996; 58: 510-8. Freer OT. The correction of delections of the nasal septum with minimum traumatism. Journal of the American Medical Association. 1902; 38: 636. Killian G. The submucous window resection of the nasal septum. Annals of Otology. 1905; 14: 363-7. Sercer A. Die Dekortikation der Nase. In: Sercer A, Mundlich K (eds). Plastische operation en an der Nase und an der Ohrmuschel. Stuttgart: Thieme, 1962. Padovan IF. External approach in rhinoplasty (decortication). In: Conley J, Dickinson JT (eds). Plastic reconstructive surgery of the face and neck, Vol. 1. Stuttgart: Thieme, 1972: 143-6. Goodman WS. External approach to rhinoplasty. Canadian Journal of Otolaryngology. 1973; 2: 207-10. Huizing E, de Groot JAM (eds). Functional reconstructive nasal surgery. Stuttgart: Thieme Stuttgart, 2003. Gibson T, Davis WB. The distortion of autogenous cartilage grafts: its cause and prevention. British Journal of Plastic Surgery. 1958; 10: 257-64.

124 Nasa I septa I perforations CHARLES EAST AN D SANTDEEP PAU N

Surgical

Definition: Through and through defect or defects of the nasal septum Sym ptomatology Clinical assessment Special investigations Prevalence of septal perforations Management

1582 1582 1583 1584 1584 1585

Surgical repair of septal perforations Key points Best clinical practice

1585 1586 1587 1587

Deficiencies in current knowledge and areas for future research References

1587 1588

The data in this chapter are supported by a Medline search restricted to English papers using the key words nasal, septal, perforation, focussing on diagnosis and surgery. Periodic review publications and textbooks of otolaryngology - head and neck surgery published in English also supported the chapter.

DEFINITION: THROUGH AND THROUGH DEFECT OR DEFECTS OF THE NASAL SEPTUM The exact prevalence of septal perforations is unknown as many perforations are asymptomatic, but in a general otolaryngology practice the clinician will encounter several patients with septal holes each year. Younger and Blokmanis 1 cite an incidence of just over 1 percent, which makes the condition relatively common. It must be remembered that racial traditions and fashion dictate that perforations in various parts of the body are a part of nonnallife and the anterior nasal septum is no exception. The majority of perforations involve the anterior quadrilateral cartilage of the septum. Kuriloff has provided an extensive list of the local and systemic causes (Table 124.1). Currently, the most common cause of perforation in the UK is probably local trauma, although in a' significant percentage, no clear history exists attracting the label of (idiopathic'. The trauma is frequently selfinduced or iatrogenic following surgery to the nasal septum or nasal instrumentation. Injuries to the

cartilagenous skeleton of the nose may result in perforation through a compound fracture of the quadrilateral cartilage in the absence of any external deformity. Recreational drugs, for example crack or cocaine snorted nasally, are becoming increasingly common as a cause of septal necrosis. 3 The prevalence of chronic specific infections producing perforations is smalL but there are a significant number of inflammatory conditions which should be excluded, particularly if the history does not give an easy clue to the aetiology.

SYMPTOMATOLOGY The principle symptoms associated with perforations are crusting, epistaxis and whistling. Other symptoms include a feeling of dryness, emptiness in the nose or a general feeling of discomfort. In a series of 69 septal perforations, Brain 4 showed that 62.4 percent were completely free from any symptoms. Size and position of the hole had a

Chapter 124 Nasal septal perforations I 1583

Table 124.1

Aetiologies of nasal septal perforations. Surface irritants

Infections

Neoplastic

Inflammatory

Nasal surgery Nose picking Septal cauterization (bi latera I) Nasal packing for epistaxis Septal hematoma/abscess

Coca i ne insufflation Cocaine adulterants Heroin inhalation

Syphilis Typhoid Diptheria

Sarcoidosis Crohn's disease Dermatomyositis

Decongestant nasal sprays Lime, cement, glass, salt, dust

Tu bercu losis Rhinoscleroma

Melanoma Adenocarcinoma Squamous cell carcinoma Metastatic carcinoma Lymphoma

Cryosu rgery

Tar and pitch

Lepromatous leprosy

Intubation (nasogastric/ tracheal) Desiccation (ozena, deviated septum)

Fumes (chromic/sulfuric acid)

Leish rna niasis

Arsen ica Is, mercu ria Is

Mucor

Calcium nitrate, cyanide Phosporous, sodium carbonate Copper-smelting fumes

Rhinosporidiosis Alternaria Actinomycosis Asperg i Ilosis Histoplasmosis Cryptocococcosis Coccidioidomycosis Paracoccidioidomycosis Candidiasis

Traumatic causes

Radiation Stab and gunshot wounds Foreign bodies (button batteries)

direct bearing on the presence of symptoms. Anterior perforations and large perforations where the anterior margin is in front of the nasal valve appear to be the most troublesome. This probably relates to the relatively slow mucociliary clearance from the anterior septum, low humidity in the anterior nares compared to controls s and the loss of mucosa, all contributing to dryness and crusting. [***] The accumulation of large crusts around the margin of the perforation produces a sensation of blockage. With large stable perforations, patients may feel the nose is empty or complain of 'blockage', even when nasal airflow is greater than average. These features may be due to turbulence of airflow in the nasal vestibule, although there is little evidence to support this. Huge perforations produce a common nasal cavity resulting in rhinolalia and an inability to clear secretions. 2 [*] Inflammation in the perforation margin leads to recurrent epistaxes, often triggered when the crust separates or is removed. Epistaxis can be quite significant from the sphenopalatine vessels. Large areas of mucosal deficit, particularly where there is cartilage exposure, prevent normal mucus clearance, contributing to localized infection and more inflammation. The local inflammation in the nose is often perceived as a generalized sense of discomfort, felt internally and over the nasal bridge. Over time, necrosis of septal cartilage will lead to enlargement of the hole in 'active perforations', sometimes to such a size that surgical closure is virtually impossible. i

L_-

Rheumatoid arthritis Relapsing polychondritis Wegener's granulomatosis Systemic lupus erythematosus

CLINICAL ASSESSMENT A full history is necessary when investigating septal perforations to cover the wide range of aetiologies. Principally, it is important to identify episodes of nasal trauma, cautery or surgery and, in particular, a history of substance abuse. Though patients may often deny it, aggressive nasal self-toilet with digital trauma or tissues will convert an inflamed mucous membrane to an ischaemic crusty area which ulcerates - the so-called 'pick ulcer'. Continued attempts to remove the crust invariably lead to cartilage exposure, cartilagenous necrosis and subsequent perforation. An overall assessment of the external and internal nasal skeleton should be made together with nasal endoscopy. Endoscopy is the best method to assess the margins and state of the residual septum. Measurement of the dimensions of the hole are performed by introducing a small paper ruler into the contralateral airway and reading the scale through the hole. Saddle deformities, deviations of the nose or septum, columella retraction and intranasal adhesions are commonly seen and may require reconstructive rhinoplasty techniques to correct if surgery is contemplated. Nasal questionnaires using a linear visual analogue scoring system may be used as a measure of symptom severity. Although not essential in routine clinical practice, they do provide subjective assessment for comparing pre- and post-treatment symptoms and are a basic measure of outcome (Figure 124.1).

1584 I

PART 13 THE NOSE AND PARANASAL SII\JUSES

Nasal septal perforation questionnaire

I

NAME:

Please mark you own score by marking a line at the appropriate point.

o = No symptoms, 10 = Worst symptoms. 10 Crusts/scabs 2

Nose bleeds

3

Whistling

4

l\Jasal discomfort

Please mark the appropriate point for your symptoms in Questions 5 and 6

5

My nose feels empty

My nose feels blocked

6

Can clear secretions easily

Can't clear secretions at all

Figure 124.1

Nasal septal perforation questionnaire.

SPECIAL INVESTIGATIONS Because of the wide range of aetiologies for septal perforation, history-taking is crucial. Any signs of inflammation around a perforation, however, should prompt investigation of other anatomical areas in the head and neck, as well as investigations for systemic inflammatory conditions. Nasal perforations may be the first sign of various granulomatous disorders, including Wegener's granulomatosis, and occur with a variety of collagen disorders including relapsing polychondritis, systemic lupus erythematosus (SLE) and dermatomyositis. Of the infective causes, the clinician is guided by the health of the patient, but tuberculosis and syphilis should be excluded. A full blood count, erythrocyte sedimentation rate (ESR), urea and electrolytes, urine analysis, C-ANCA, treponemal investigations, ACE titres and a chest x-ray, together with a nasal swab, provide an adequate baseline. Other symptoms of inflammation in the respiratory tract, for example cough, middle ear effusions, an arthritis or skin rash, should prompt referral to a clinical immunologist. There is little evidence that extensive investigations for patients with healed, stable and asymptomatic perforations contribute to a change in the management, particularly where the aetiology is clear. Routine biopsy of septal perforations to exclude vasculitis has been suggested,6,7 but biopsy rarely reveals anything other than chronic inflammation and is not usually sufficient for a specific diagnosis. Indeed, biopsy may convert an inactive perforation to an 'active state', resulting in significant enlargement of the hole. The role of routine biopsy in idiopathic perforations has recently been questioned. In

two retrospective series, septal biopsy was shown to be nonspecific for Wegener's and was only of value in those perforations with an irregular margin which were clinically malignant. 8 , 9 It is clear that the diagnosis of Wegener's or its reactivation cannot be reliably inferred from biopsy of the nasal septum alone. Practically, therefore, perforations should be biopsied if there is an unexplained aetiology, with persistent inflammation, or if the perforation is irregular. [Grade B] Specimens should be examined for mycobacteria and fungi, and with immunocytochemistry to exclude a neoplasm. 2

PREVALENCE OF SEPTAL PERFORATIONS The prevalence of septal perforations following nasal septal surgery shows wide variation and has been reported as between 1.4 and 25 percent. The prevalence appears higher after submucous resection operations 07-25 percent) than the more conservative septoplasty procedures 0.4-5 percent).l0 [**] The use of nasal splints during septal surgery is known to produce a higher incidence of perforation. l l [***] Splinting is unnecessary in the majority of routine septal surgery unless established adhesions are being treated. Recent retrospective reports 12 have suggested an association between nasal steroid sprays and the development of nasal perforation. It is known that all steroid sprays can cause inflammation over the caudal septum, by direct irritation or their initial vasoconstrictor activity, particularly if there is a septal deviation which takes the brunt of the spray jet. Patients should be directed to use the spray with the opposite hand to the nostril being treated, minimizing the impact

Chapter 124 l'Jasal septal perforations

on the septum. Any ulceration after starting steroid spray use should be allowed to heal by withdrawing the medication.

MANAGEMENT The majority of septal perforations are asymptomatic and require no specific treatment. 4 [Grade C] The more anterior the lesion, the more likely it is to cause symptoms. This probably relates to accessibility by the patient to pick at the nose, but may also be related to fast airflow producing drying in the region of the internal nasal valve. The management can be divided into three groups.

Prevention In a series of 50 perforation repairs at the Royal National Throat, Nose and Ear Hospital, 60 percent had a previous history of septal surgery. 13 Meticulous attention to technique is required in elevating the mucoperichondrial flaps in the correct plane, particularly avoiding overlapping bilateral mucosal tears. Starting the dissection on the easier (usually the concave) side to raise one intact flap first, and using an autograft of cartilage or ethmoid plate to support any tears, is good practice. The mucoperichondrium over large spurs is often very thin, and tears may be inevitable. However, when the spur is removed, there will be a relative excess of mucosa which can be repaired with absorbable sutures. Quilting sutures in the septum after surgery should be tied loosely to allow for post-operative oedema. The authors have seen perforations caused by individual sutures tied tightly in the septum which resulted in ischaemic necrosis. Septal inflammation or ulceration should be treated by withdrawing the source of the irritation and promotion of healing, for example by eradication of pathogenic bacteria, avoidance of aggressive cleaning and use of mucosal protectants (petroleum jelly). Antiseptic silicon barrier creams over six to eight weeks will stabilize the mucosa in the majority of cases. In recalcitrant inflammation, thin reinforced silastic sheeting can be sutured to cover the caudal septum. If there is a large area of cartilage exposure, however, this still may not be sufficient to prevent progression to perforation. When a septal ulcer heals, the scar produced may leave a persistent area of squamous metaplasia which never reverts to a mucosal surface. Periodic and regular use of barrier creams may be necessary.

Nonsurgical =-=-.==

-- -- - - - - - - - - - - - - - - - - - - - - - - - - - - - - -

This, essentially, is aimed at reducing the drying effect in the nasal mucosa to alleviate crusts and epistaxis. Alkaline

.,l,

I 1585

nasal douches, proprietory saline sprays and petroleumbased ointments are commonly used. There are no studies of the effectiveness of this type of regimen, but many patients adopt a twice daily ritual of douching and ointment application. It is usually obvious within six to eight weeks whether this will be effective in maturing the margins of a perforation.

Obturation The principle of obturation is to cover the inflamed mucosal margin. In 1951, Deneke and Meyer14 cited the use of septal obturators in the management of perforations. Inert sheeting (usually silastic) was placed to prevent drying and encourage epithelialization over the cartilage/bony septum to create a mature mucosal edge. Evidence suggests that patients do derive benefit from the use of nasal obturators, but opinion on their usage and effectiveness is divided. Facer and Kern,15 in a retrospective study of 73 patients, reported 72.8 percent of obturators remained in place, but 27.4 percent of buttons were removed or were extruded. [>(-] Brain4 described the long-term outcome of 102 patients with medium or large holes (1 cm perforations or larger). He described a good result in 44.4 and 32.4 percent, respectively. The main benefit from obturation appears to be the control of whistling and epistaxis. Often, crusting and nasal discomfort remain a problem. 6, 16 The feeling of obstruction is improved when crusting is reduced, but the bulk effect of an obturator may increase blockage, particularly in anterior perforations with a narrow nasal valve. Poorly tailored Silastic may irritate the mucosa, particularly with anterior holes close to the membranous septum. Patient interference and movement of the mobile membranous septum against the edge of the obturator can lead to granuloma formation. It is often for these reasons that patients request removal of the obturator.

SURGICAL Enlargement of a nasal septal perforation to prevent whistling was described by Jackson and Coates in 1922,17 but there is little evidence to support its routine use in clinical practice. One retrospective article of widening of the perforation and posterior edge repair in larger holes (IS-50 mm) evaluated the pre- and post-operative symptom scores obtained by a linear visual analogue questionnaire. Benefit was reported for the symptoms of crusting and epistaxis and overall nasal discomfort with a significant reduction in mean scores. This technique appears to offer some value for patients with persistent inflammation who could not retain a nasal obturator and who were deemed unsuitable for surgical repair. [**]

1586 I PART 13 THE NOSE AND PARANASAl SINUSES

SURGICAL REPAIR OF SEPTAL PERFORATIONS The plethora of techniques for septal perforation repair described over the years attest to the fact that there is still a challenging technical problem for the nasal surgeon, although reliable surgical techniques with good outcomes have been published in the last 15 years. The variety of repairs may be classified as: • free grafts: - simple or composite autografts; - allografts; • pedicled flaps: - local nasal mucosal; - buccal mucosal; - composite septal cartilage and mucosa; - composite skin/cartilage; • rotation/advancement of mucoperichondrial or mucoperiosteal flaps. Combinations of techniques have been employed in many small reports. In the larger series of observational outcomes of perforation repairs, two factors emerge that appear to have an important bearing on a successful outcome, to achieve closure in 80-90 percent of cases. The first is that there is sufficient residual mucosa in the nasal fossa which can be mobilized and transposed allowing direct suturing of the mucosal defect (Figure 124.2). The second is the routine use of a connective tissue interposition graft to support the repair. Bilateral mucosal flaps with the main blood supply derived from the sphenopalatine vessels form the basis of most techniques. [*'*] Grafts have been reported using temporalis fascia,18 mastoid periosteum, cranial periosteum,

cartilage (septal, auricular, rib) and bone (either locally or from rib or the iliac crest) .19,20 Small defects can be closed with bipedicled flaps, by making relieving indsions either superiorly or inferiorly. Larger holes up to about 2 cm require larger flaps which are pedicled only posteriorly based on the sphenopalatine vessels, and are effectively transpositionlrotation flaps. 1, 20, 21 Even if complete bilateral mucoperichondrial suturing of the hole is not possible, the use of a connective tissue graft between the leaves allows for epithelialization on the side of incomplete closure, as long as the flaps are proteced from drying out and the reconstruction does not get infected. In the presence of any dehiscence of the sutured mucosal defect, it appears that epithelialization occurs more reliably over fascia than cartilage or bone. Reports using acellular dermal allografts,22,23 instead of fascia appear to give similar success rates. (*] The allografts are expensive, but it must be remembered that donor site morbidity from fascia is not a major issue and the repair is entirely the patients own tissue. The use of other collagen xenografts is still undergoing evaluation. The authors have used at least two layers of either temporalis or temporoparietal fascia, if necessary with a sandwich of cartilage as a composite graft where there is little supporting remaining quadrilateral cartilage (Figure 124.3), for the last six years with a current success rate of 87 percent. Auricular cartilage, which is a useful graft, is often curved and care should be taken anterosuperiorly at the internal valve because any excess thickness at this level risks postoperative nasal block, requiring revision surgery. The nasal fossa is a contaminated area and therefore antibiotic prophylaxis is important. There are no studies as to the ideal length of the course, but the development of sepsis can have a potentially devastating effect on the

I

I

I !

( \ Figure 124.2 Extensive mobilization of the mucosal lining allows a round hole to be closed with sutures in a straight line.

Figure 124.3 Bilateral mucosal flap repair with staggered mucosal rotation/transposition flap closure and connective tissue interposition graft. The fascia should extend to the nasal floor to cover any secondary defect inferiorly.

Chapter 124 Nasal septal perforations I 1587

residual cartilage producing a saddle deformity. Currently, the authors give a ten-day course. 13 There is an anatomical limit to the amount of septal mucosa which can be transposed and this is directly inverse to the size of the septal hole. Large perforations (larger than 2.5 cm), particularly with a large vertical dimension as opposed to horizontal linear holes, still pose a problem. However, by extensive undermining along the nasal floor and inferior meatus inferiorly and under the upper lateral cartilage superiorly, substantial flaps may be created. The careful atraum(ltic elevation of flaps is technically demanding, particularly if the mucoperichondrium is inflamed, atrophic or there is a significant spur of the septum. The surgeon has three options for the approach. 1. Endonasal: 18 This approach is limited principally by nostril size and position of the hole. Design of the endonasal flaps is critical in this approach due to the limited access. Broad-based elevations via hemitransfixion incisions and bipedicled flaps preserving anterior and posterior blood supplies with horizontal relieving incisions together with interposition grafts should give very good results for small holes (up to 0.5 cm). 2. External rhinoplasty approach via a trans columella approach 19 or a columella-philtrum incision: The external approach or its modification by sectioning the columella below the medial crural footplates and connecting to a transfixion and intercartilagenous incisions provides excellent exposure of the septum and lower dorsum, particularly if concomitant rhinoplasty is required and especially when additional grafting is needed to the nasal valve or dorsum. Alar crease incisions only provide limited additional unilateral access and should not be combined with the transcolumella approach because of the risk of ischaemia to the columella flap. It is possible to design larger flaps through this approach and suturing of the mucosal hole is definitely easier than by the endonasal approach. The use of medially based pedicled buccal mucosal flaps have limited use for larger perforations, bu t are useful to repair secondary deficiencies of the membranous septum when the mucosa has been mobilized to close very anterior holes. 3. Midface degloving approach: 24 This is an extensive dissection of the face which has been employed for very large holes (> 2 cm). Used purely as an approach with standard rotation transposition mucosal flaps, it appears to offer little benefit over an external approach for successful closure but with a higher complication rate (vestibular stenosis, 20 percent). Variation of the technique by employing tissue expansion of

the mucosa and skin grafting to the nasal sill appear to improve the success rate and reduce the incidence of nasal vestibular stenosis. Tissue expansion is achieved along the nasal floor using a small (1 x 3 cm) expander over six to eight weeks with a total volume of 4-7 mL. This technique has the advantage of generating more lining for easier mucosal closure, but the extensive relatively traumatic dissection must be weighed against the degree of benefit to be gained by the patient. Reported success in this series was 18 out of 22 patients with holes from 2 to 4 cm diameter. Although there is evidence of good technical success in surgical repair of the perforated septum for small- to medium-sized holes, objective evidence of patient benefit following surgical closure is lim.jted. Lindemann et al. s have shown improvements in nasal physiology particularly in end-inspiratory humidity and a reduction in symptom scores for epistaxis and nasal dryness.

KEY POINTS' More than ,half ofp'ertor"tions" are .. ", asyn\Pto,m~tic PI-/*]

I 1591

In cases of anterior septal deviation and nasal obstruction. the following recommendations may be made: ./ Establish the degree of obstruction and the congestion component in both nasal cavities. ./ Marked septal deviation should be corrected by septoplasty. ./ Minor septal deviations and signs of nasal congestion may have other etiologies. Infection, allergy or hypersensitivity should be kept in mind. If conservative medical treatment is not satisfactory and the objective measurements are indicative of small spatial dimensions anteriorly, surgical turbinate reduction should be considered. [Grade DJ

SNORING AND SLEEP APNOEA Nasal airway patency changes with body position and it is reduced when lying down. There is evidence that compared with normals the postural variation of nasal resistance is more pronounced in patients with allergic rhinitis 20 and in nonapnoeic snorers the recumbent position reduces airway patency,21 whilst awake and asleep. In one study,22 over 90 percent of a group of snorers had abnormally high nasal resistance and acoustic rhinometry curves, suggesting engorgement of the inferior turbinate. Snoring is more frequently present in populations with nasal obstruction and inferior turbinate reduction 18 may reduce the frequency of snoring. Many patients suffering from obstructive sleep apnoea (OSA) treated with continuous positive airway pressure (CPAP) devices develop troublesome nasal obstruction. This is probably an increased vasomotor responsiveness, secondary to the use of CPAP. Local steroids may help,20 but are not always effective in controlling turbinate congestion. There is no evidence that surgical turbinate reduction helps in the long term?3 [**/*J

CHRONIC RHINITIS AND RHINOSINUSITIS

Figure 125.2

Compensatory hypertrophy of the middle and

inferior turbinates on the left, wide side of the nose, opposite to a septal deviation.

Nasal obstruction is often the dominating symptom in chronic inflammation/infection of the nose and -sinuses. Vascular engorgement of the nasal cavity is characteristic, mainly of the inferior tui-binate with its well-developed vascular supply. Allergic rhinitis, pregnancy, antidepressant drugs, rhinitis medicamentosa due to long-term use of nasal vasoconstrictors, are diagnoses that should be kept in mind in patients with chronic nasal obstruction (CNO). The group of CNO includes a variety of pathologic and physiologic conditions and principally CNO is not a

1592 I PART 13 THE NOSE AND PARA NASAL SINUSES

(surgical disease'. There are no definitions of indications for inferior turbinate surgery, except that surgery is performed for the subjective feeling of impaired nasal patency. In an attempt to define CN0 18 with acoustic rhinometry, mucosal congestion was found in 76 percent of subjects with CNO, bilaterally in one-third and unilaterally in two-thirds of them. Whether the turbinates are truly irrevernbly enlarged, temporarily engorged due to disturbance of the nasal mucosa or they are enlarged relative to the skeletal dimension of the anterior part of the nasal cavity are questions that make the precise clinical diagnosis of enlarged turbinates difficult in practice. Nevertheless, different studies show inferior turbinate reduction to be effective in more than 90 percent of cases in relieving the chronic feeling of nasal obstruction refractory to medical treatment. The techniques used are numerous and the long-tenn effect is also variable from weeks to years. One controlled, randomized studyl9 suggests submucous resection of the turbinate (SMOFIT), with outfracture of the inferior turbinate bone is effective in the long term, with few adverse effects on the nasal mucosa [***1**] In infectious chronic rhinosinusitis (CRS), the inferior turbinate may be enlarged secondarily to the infection in the middle meatus. However, the current opinion is that the enlargement recedes after sinus infection has been treated. Stammberger24 states that he hardly ever performs reduction of the inferior turbinate in CRS. Occasionally, persistent engorgement of the posterior end of the turbinate occludes the choana. In this situation, removal with a surgical snare is indicated. The role of the middle turbinate (MT) in the development and persistence of CRS is unknown and the role of middle turbinate surgery for CRS remains controversial. The inferior turbinate is very rarely pneumatized, while aeration of the middle turbinate (or concha bullosa, CB) is a common anatomical variant. On coronal computed tomography (CT) scans25 of patients evaluated for sinus disease, 34 percent had CB on at least one side. There was no difference in the overall incidence of inflammatory disease in the ostiomeatal complex in symptomatic patients between those with and without CB. The role of the concha bullosa is still debatable. However, an abnormally enlarged middle turbinate may obstruct the ostiomeatal complex and create problems with the drainage of the paranasal sinuses. In the era of functional endoscopic sinus surgery, some surgeons consider the MT to play a key role in the outcome of endoscopic surgery for CRS. In a prospective, randomized study26 of 1106 patients with CRS, anterior resection of the middle turbinate had significant beneficial effect minimizing the iatrogenic obstruction due to synechiae. This was found in patients with more severe sinonasal disease, but no difference was found in mild cases of CRS. [**1*]

The following are recommended as reasonable indications for consideration of inferior turbinate surgery in adults with chronic nasal obstruction. Patients should meet all the following requirements: .I nasal endoscopy without signs of infection or neoplasm; ./ failure of conservative medical treatment of adequate duration; ./ The complaint should be disabling. [Grade C]

MEDICAL MANAGEMENT There is evidence that intranasal corticosteroids should be used as the first-choice drug in the treatment of nasal obstruction, provided neoplasms and gross skeletal abnormalities have been excluded. More than 100 double-blind, placebo-controlled studies27 have shown efficacy of intranasal corticosteroids on nasal blockage, sneezing and rhinorrhoea, in allergic and in noninfectious, nonallergic rhinitis. Antihistamines for nasal obstruction are ineffective as compared with topical steroids. Vasoconstrictors may be used for a few weeks and awareness must focus on the risk of developing rhirtitis medicamentosa. Use of topical, isotonic saline spray is a recommendable adjunctive treatment in all cases of chronic nasal diseases. (****]

SURGICAL MANAGEMENT The surgical management of the enlarged inferior turbinates has been debated for more than 100 years. Reviews 28 • 29 in this field show that the focus has been placed more on technological advances than to establish a benefit to the patient. The evidence supporting the efficacy of the different procedures remains debatable. The controversy also surrounds the middle turbinate and this has been addressed under Chronic rhinitis and rhinosinusitis. This section will focus on surgery of the inferior turbinates (IT) for chronic nasal obstruction. The chronology of the appearance of the different surgical methods is shown in Table 125.1. The goal of any surgical treatment of the IT should be to improve nasal obstruction and to avoid complications in the short and long term. The different surgical procedures may be classified into mechanical procedures, destructive procedures and turbinate resection procedures (Table 125.2). The complications are primary or secondary haemorrhage, synechiae and crusting ranging from mild to atrophic rhinitis. The reviews 28,29 assessing the benefits and complications of the different procedures come to widely differing recommendations. One states 'laser turbinate reduction is strongly supported because of its

Chapter 125 The management of enlarged turbinates. 1593

relatively effective results with minimal associated morbidity.,28 By contrast, another review concluded 'that it seems that electrocautery, chemocautery, (subtotal) turbinectomy, cryosurgery and laser surface surgery should not be used) as these techniques are too destructive. Intraturbinal turbinate reduction (intraturbinal turbinoplasty) would seem to be the method of choice.'29 In the time between these reviews the first prospective) randomized study comparing electrocautery) cryotherapy, laser cautery) submucosal resection without and with lateral displacement and turbinectomy was published. The study gives extensive objective results with an observation period of between one and four years. The conclusion is that submucosal resection of the cavernous tissue of the IT with lateral displacement of the turbinate bone achieves a long-lasting improvement of the nasal passage with normalization of the mucociliary transport time and without post-operative bleeding. 19

The surgical procedure is described as follows: through a 3-4-mm incision on the head of the inferior turbinate, the submucosal tissue is dissected from the medial surface and inferior edge of the bone by an elevator. Excess cavernous tissue is removed with Hartmann forceps with resection of the posterior end of the turbinate (Figure 125.3). Merocel packing is used.

Table 125.1 Different surgical methods for treatment of enlarged inferior turbinate. Method

Year(s) introduced

Currently in use

1845-1880

+

1869-1890 1882 1904 1906-1911

+ + + +

1930-1953 1952 1953 1961 1970 1982 1977 1994 1998 2002

+

(a)

Thermal coagulation, electrocautery Chemocoagu lation Turbi nectomy Late ra Iization Su bmucous resection tu rbina te bone Pa rtia I resection Injection of corticosteroids Injection of sclerosing agents Vidian neurectomy Cryosurgery Turbi noplasty Laser su rgery Powered instruments Rad iofrequency Argon plasma surgery

Table 125.2

OF SMR

+ + + + + +

Figure 125.3 (a) Incision on the anterior edge of the inferior turbi nate; (b) subm ucous resection of the cavernous tissue of the inferior turbinate (SMR) and outfracture of the turbinate bone (OF); (c) result.

Surgical procedures for treatment of enlarged inferior turbinate, complications and effect.

Mechanical procedures Lateral ization/ou tfractu re Destructive procedures Electrocautery Cryosu rgery Laser su rgery Resection procedures Subm ucous resection Parti aI resection Total resection

Bleeding

-cr~sti ~g.Jsyn~~h!~~e.·ShQ:r~~~~~~~,~eff\~~t,. : lD-~g:te.r~I- ~~fect +/-

-/+ +

+/+ + ++ +

+ +/+ +/+ +/+

+ + +

+/-

+/+ +/+

+ + ++ ++

++ + +

1594 I PART 13 THE NOSE AND PARANASAL SINUSES ).- Controlled. randomized studies of different surgical treatments. with long-term follow up of groups with well-defined diagnoses should be carried out. ). The perception of nasal obstruction at the brain level needs to be elucidated.

New technological devices, such as powered instruments, radiofrequency and argon plasma, are being applied to turbinate reduction. However, only preliminary results are available. The debate on the surgical treatment of the enlarged inferior turbinate will continue, but the way forward has been shown. 19 [****1***1**]

KEY POINTS • The feeling of nasal obstruction Inay be part of the nOTmal nasal physiology. • Endoscopy of the nose and rhinopharynx is necessary to disclose serious causes of nasal obstruction. • Chronic rhinitis usuai!l.y responds to appropriate medical therapy. • In cases without anatom,ical deformities, if medical therapy is ineffective, appropriate surgical reducti,o n of the turbLnates may be effective in 90 percent of cases.

REFERENCES 1.

2.

3.

*

4.

5.

6. .I A thorough history of symptoms and previous medical therapy is necessary to establish the possible etiology of nasal obstruction. .I Chronic nasal obstruction may be due to neoplasms. sinonasal infections. drug abuse, allergy or nasal hypersensitivity. It is mandatory to examine the nasal cavity and the rhinopharynx before offering a treatment. .I If gross anatomical deformities are found. surgical treatment must be considered. .I In nasal congestion with symptoms of allergic rhinitis or nasal hypersensitivity. treatment with intranasal corticosteroids for between one and two months should be the first choice of treatment. ../ If conservative treatment has been used adequately and the nasal obstruction is disabling, inferior turbi nate red uction shou Id be considered. Prospective. randomized. objectively supported studies will hopefully become available in the future.

7.

8.

9.

10.

11.

12.

13.

Deficiencies in current knowledge and areas for future research )- A normal nasal cavity in terms of congestion needs to be defined. > Reliable measurement of nasal airflow changes after medical and surgical treatment of the turbinates - needs to be established.

* 14.

Haight JS. Cole P. The site and function of the nasal valve. Laryngoscope. 1983; 93: 49-55. Grymer LF. Hilberg O. Pedersen OF. Rasmussen TR. Acoustic rhinometry: Values from adu Its with subjective normal nasal patency. Rhinology. 1991; 29: 35-47. Mlynski G, GrOtzenmacher S, Plontke 5, Mlynski B, Lang C. Correlation of nasal morphology and respiratory function. Rhinology. 2001: 39: 197-201. Eccles R. Nasal airflow in health and disease. Acta Otolaryngologica. 2000: 120: 580-95. A recent review of the different mechanisms influencing airflow and background for nasal obstruction. Stoksted P. The physiologic cycle of the nose under normal and pathologic conditions. Acta otolaryngologica. 1952; 42: 175-9. Eccles R. The central rhythm of the nasal cycle. Acta Otolaryngologica. 1978; 86: 464-8. Hilberg O. Grymer LF. Pedersen OF. Spontaneous variations in congestion of the nasal mucosa. Annals of Allergy, Asthma and Immunology. 1995: 74: 516-21. Flanagan P, Eccles R. Spontaneous changes of unilateral nasal airflow in man. A re-examination of the 'nasal cycle'. Acta Otolaryngologica. 1997; 117: 590-5 . Kennedy OW. Zinreich SJ. Rosenbaum AE. Kumar AJ. Johns ME. Physiologic mucosal changes within the nose and ethmoid sinus: imaging of the nasal cycle by MRI. Laryngoscope. 1988; 98: 928-33. Hilberg O. Grymer LF. Pedersen OF. Nasal histamine challenge in nonallergic and allergic subject evaluated by acoustic rhinometry. Allergy. 1995; 50: 166-73. Guyuron B. Nasal osteotomy and airway changes. Plastic Reconstructive Surgery. 1997; 102: 861-3. Grymer LF. Reduction rhinoplasty and nasal patency: Change in the cross-sectional area of the nose evaluated by acoustic rhinometry. Laryngoscope. 1995; lOS: 429-31. Hilberg O. Grymer LF. Pedersen OF, Elbrond O. Turbinate hypertrophy: evaluation of the nasal cavity by acoustic rhinometry. Archives of Otolaryngology - Head and Neck Surgery. 1990; 116: 283-9. Grymer LF. ilium P, Hilberg O. Septoplasty and compensatory inferior turbinate hypertrophy: A randomized study evaluated by acoustic rhinometry. Journal of Laryngology and Otology. 1993; 107: 413-7. The first attempt to define objectively turbinate mucosal hypertrophy and the first prospective, randomized study on compensatory inferior turbinate hypertrophy.

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Ilium P. Septoplasty and compensatory inferior turbinate

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1997; 254: 89-92. 16. 17.

solutions. Hyperplastic lower turbinate, Chapter 9. Functional endoscopic sinus surgery. New York: Decker BC,

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Holmstrom M, Kumlien J. A clinical follow-up of septal

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rhinomanometric examination in the decision concerning

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Grymer LF, Ilium P, Hilberg O. Bilateral inferior

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endonasal sinus surgery with and without partial middle

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Mygind N, Dahl R, Nielsen LP, Hilberg 0, Bjerke T. Effect of corticosteroids on nasal blockage in rhinitis measured by

* 28.

objective methods. Allergy. 1997; 52: 39-44. Jackson LE, Koch RJ. Controversies in the management

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Hasegawa M, Saito Y. Postural variations in nasal resitance and symptomatology in allergy rhinitis. Acta

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surgical methods applied to inferior turbinate hypertrophy,

Berg S, Cole P, Hoffstein V, Haight JS. Upper airway

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Havas Th E, Lowinger DSG. Comparison of functional

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Zinreich SJ, Mattox DE, Kennedy OW, Chisholm HL, Diffiey

operation. Clinical Otolaryngology. 1988; 13: 115-20.

hypertrophy of the inferior turbinate: Long-term results in

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Stammberger H (ed.). Sinus problems and endoscopic

congestion? Rhinology. 1983; 21: 21-7.

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Graamans K. Does septal surgery influence submucous

surgery with special attention to the value of preoperative

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Hoi MKS, Huizing EH. Treatment of inferior turbinate

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with a different conclusion from the other comprehensive

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126 Epistaxis GERALD W MCGARRY

Background Vascular anatomy Classification of epistaxis Adult primary epistaxis Secondary epistaxis

1596 1596 1599 1600 1605

Key points Best clin iea I practice Deficiencies in current knowledge and areas for future research References

1606 1606 1606 1606

The data in this chapter are supported by searches in the Medline, EmBase and the Cochrane Library databases using the key words epistaxis, aetiology, management, treatment and therapies in main articles, RCTs and systematic reviews. Search results were augmented by a manual search through major references and texts. Cross-checking was carried out with references from review articles.

BACKGROUND Epistaxis is defined as bleeding from the nose. This prosaic definition belies the difficulties associated with one of otolaryngology's most common and most difficult to treat emergencies. The historical literature contains numerous references to epistaxis and its antiquity is reflected in the fact that the simplest treatment for a nose bleed (pinching the ala nasi) is called the Hippocratic technique. While an extensive review of the history of epistaxis is beyond the scope of this book, much of the historical literature remains relevant and students of this subject are advised to review the writings of Morgagni, who even predicted nasal endoscopy in 1761! 1

VASCULAR ANATOMY Arterial supply The internal and external carotid arteries supply the nose via branches which anastomose extensively within the

lateral wall, septum and across the midline. In 1879, James Little identi fied an arterial plexus on the anterior septum as a frequent site of bleeding and the same plexus was described one year later by Kiesselbach? As a result of these descriptions, the area most frequently implicated in epistaxis is known as Little's area or Kiesselbach plexus. In the posterior nasal cavity, the vessels are larger than those in Little's area and can more easily be traced to their external or internal carotid origin. EXTERNAL CAROTID ARTERY

The external carotid artery supplies the nasal cavity via facial and maxillary branches. The facial artery supplies the most anterior part of the septum (nasal septal rami of the superior labial artery), the vestibule (lateral nasal artery) and a small area of the nasal cavity (ascending palatine artery). The maxillary artery supply is via the sphenopalatine and greater palatine branches. The greater palatine arl."ery supplies the anteroinferior part of the nasal floor and septum.

Chapter 126 Epistaxis I 1597

The sphenopalatine artery is the most important supply to the nasal cavity. It enters through the sphenopalatine foramen and immediately divides into posterior septal and posterior lateral rami. 3 The posterior lateral division gives the inferior and middle turbinate arteries. These vessels were studied in detail by Burnham in 1935 and his work remains the definitive reference on this subject. 4 Burnham described how the inferior and middle turbinate arteries run in bony tunnels within the turbinates. Shaheen postulated that these bony conduits could reduce the likelihood of the arteries being involved in epistaxis. 5 The posterior septal branch of the sphenopalatine artery runs mediaUy across the face of the sphenoid to the posterior part of the septum, then takes an undulating course anteroinferiorly in the mucoperichondrium. Its terminal branches anastomose in Little's area.

INTERNAL CAROTID ARTERY

The internal carotid contributes the anterior and posterior ethmoidal branches of the ophthalmic artery. The anterior ethmoidal artery, after arising in the orbit, runs under the superior oblique muscle to the anterior ethmoidal canal in which it traverses the ethmoid and nasal cavities. It terminates in the region of the ethmoid fovea in a meningeal branch and a larger branch to the nasal roof, olfactory cleft and superior turbinate. The posterior ethmoidal artery is smaller than the anterior ethmoidal artery and is present in only 80 percent of individuals. 6 Like the anterior vessel, this artery runs medially, but this time passes above the superior oblique muscle to enter the posterior ethmoidal foramen situated 5 mm anterior to the optic canal and 10/15 nun behind the anterior ethmoidal foramen. Within its canal, the posterior ethmoidal artery is accompanied by the sphenoethmoidal nerve and a branch of the nasociliary nerve. This vessel also divides into a terminal meningeal branch and a branch to the posterosuperior nasal cavity, olfactory sulcus and sphenoethmoidal recess. [**]

circulations is erroneous as the arterioarteriolar anastomotic network allows varying directions of flow to OCcur. Compensatory anastomotic flow via the fadal arteries is thought to explain rebleeding which may Occur following ligation or embolization.7 [**1

Venous drainage The veins follow the arteries within the mucosa. The exception is the periarterial venous cuff surrounding the intraosseous portions of the inferior and middle turbinate arteries. The veins of the lateral wall drain through the sphenopalatine foramen into the pterygoid venous plexus to the internal jugular vein. Anteriorly, drainage is via superior labial and greater palatine veins to the facial vein and ultimately the external jugular system. Of particular note clinically is the retrocolumellar vein running 2 mm behind and parallel to the columeJla. This vein is in a particularly superficial area and is a common cause of venous epistaxis in children.

Key clinical areas WOODRUFF'S PLEXUS

The role of Woodruff's plexus in epistaxis is frequently discussed. B• 9 In 1949, Woodruff1o described a plexus of prominent blood vessels lying just inferior to the posterior end of the inferior turbinate (Figure 126.1). He commented on how this plexus was a frequent site of adult epistaxis and described 14 cases of bleeding from

INTERNAL AND EXTERNAL CAROTID TERRITORIES

The nasal cavity is the location of the principal internal-external carotid artery anastomoses in the head and neck. There has been debate over the relative importance of each supply to the nasal cavity. Shaheen showed that the area supplied by the ethmoidal arteries was much smaller than had previously been thought. His findings were supported by observing the sphenopalatine branches to the superior meatus and superior turbinate, the comparatively narrow calibre of the ethmoidal arteries and the fact that the larger of the two ethmoidal arteries, the anterior, is absent in as many as 14 percent of cadaver dissections. 5 The view that the middle turbinate marks the watershed between internal and external carotid

Figure 126.1

Endoscopic photograph of woodruff's plexus

(right) : IT, inferior turbinate; NP, nasopharynx; WP, Woodruff's plexus.

-1~---'-.-

1598 I PART

13 THE NOSE AND PARANASAL SINUSES

this area. Many authors describe Woodruff's plexus as a frequent source of so-called 'posterior' epistaxis despite the absence of any numerical evidence to support this. In s 1967, Shaheen attempted to study and define Woodruff's plexus but was W1able to identify the plexus in nasal ethmoidal blocks. Recent study with endoscopic photography and anatomical microdissection confirms the existence of the plexus and confirms that it is a venous plexus. I I [**]

LATERAL WALL OR SEPTAL BLEEDING?

There is general acceptance that most epistaxis occurs from Little>s area, but there is debate on the relative importance of various other sites. Some suggest that Woodruff's plexus is important and others nominate the septum or other regions of the lateral wall as prime sites (Figure 126.2). Numerous studies using various methods and examination techniques have produced diverse findings. 9 , 12, 13, 14, 15 For example, Shaheen 1s failed to find any lateral wall bleeding, while Wurman 9 did not report any septal bleeding. Sixty percent of bleeding points identified by EI-Simil/ 6 were septal, whereas 62 percent of those found by Rosnagle et al.17 were on the lateral wall. In a study of 50 patients with adult primary posterior epistaxis, McGarry 18 identified the bleeding point in 94 percent (6 percent not located despite endoscopy), 70 percent bled from the septum and 24 percent from the lateral wall. There was no side predilection (50 percent left nostril and 48 percent right> 2 percent bilateral). These findings support the observation that posterior, like anterior, epistaxis is predominantly septal in origin. [**]

ANTERIOR ETHMOIDAL ARTERY IN ENDOSCOPIC SINUS SURGERY

Detailed knowledge of this vessel is essential for surgeons carrying out endoscopic sinus surgery and for those planning its ligation for epistaxis. The artery is frequently encountered in a mesentery just below the skull base between the ethmoid fovea and the lamina papyracea. Inadvertent damage to the mesentery can lead to troublesome bleeding from the artery. Transection of the vessel during sinus surgery can result in retraction of the bleeding end into the orbit with subsequent pressure haematoma and risk of visual loss. The vessel can be ligated as a treatment for epistaxis via an external (medial canthal) approach or, in the rare occasion where the bony anatomy permits, endoscopically (transethmoidal).19

SURGICAL ANATOMY OF THE SPHENOPALATINE FORAMEN

The sphenopalatine foramen is the portal for the major arterial supply of the nasal cavi.ty. Lateral to the foramen lies the pterygopalatine space. The foramen is formed by a

Figure 126.2 Diagram of sites of bleeding in posterior epistaxis. (a) Lateral nasal wall; (b) septum. Numbers are percentages (6 percent not located).

U-shaped notch in the vertical portion of the palatine bone which is closed posterosuperiorly by the sphenoid bone. The foramen transmits the sphenopalatine artery, vein and the nasal palatine nerve (maxillary division of tq.e trigeminal nerve). Clinically, this foramen is the key to the procedure of endonasalligation of the sphenopalatine artery (ESPAL). SurgicaJ localization of the foramen can be difficult. Bolger et al. 20 studied a small bony projection which lies anterior to the foramen in 96 percent of cases. This landmark is called the 'crista ethmoidalis' and its recognition. during surgery may help in finding the foramen. (**J

--'

.~

Chapter 126 Epistaxis

SURGICAL ANATOIVIY OF THE BLOOD SUPPLY OF THE INFERIOR TURBINATE

Severe secondary epistaxis is a serious complication of inferior turbinectomy. Understanding the vascular supply of the inferior turbinate may help in both the avoidance and management of this complication (Figure 126.3). At its origin, the artery runs anteroinferiorly in the submucosa where it is vulnerable to damage during radical turbinectomy. On reaching the inferior turbinate, it divides into three parallel branches which run in bony tunnels within the substance of the turbinate. These tunnels with their periarterial cuff of fibrous tissue and venous elements may prevent the artery constricting following turbinectomy and may predispose to postoperative haemorrhage. 4 , 14 Attempts to control haemorrhage following turbinectomy should be directed towards the posterosuperior aspect of the inferior turbinate where pressure bipolar to the submucosal segment of the artery should prove effective. [**] [Grade D]

I 1599

variation with age is sufficiently pronounced to classify epistaxis as childhood (less than 16 years) or adult (greater than 16 years).

Primary or secondary Between 70 and 80 percent of all cases of epistaxis are idiopathic, spontaneous bleeds without any proven . . preClpltant or causaI Clactor. 22 This type of bleeding can be classified as primary epistaxis. As our understanding of the aetiology advances, the number of cases of true primary epistaxis will decrease but, at present, this definition encompasses most cases. A small proportion are due to a clear and definite cause such as trauma, surgery or anticoagulant overdose and can be classified as secondary epistaxis. The distinction between primary and secondary epistaxis is more than academic as the management of each type is quite different, for example, techniques used to control primary epistaxis are unlikely to be successful for s.econdaryepistaxis due to coagulopathy.

CLASSIFICATION OF EPISTAXIS Traditionally, epistaxis has been classified on the basis of presumed aetiology and publications include long lists of factors thought to cause the condition. 21 As most cases are idiopathic and since case control evidence does not exist for most of the putative aetiological factors, a causation-based classification is inappropriate. A clinical classification based on the patterns of presentation of epistaxis is more useful (Table 126.1).

Anterior and posterior epistaxis The terms anterior and posterior epistaxis are frequently used, but their definitions are imprecise and inconsistent. Pearson attempted to standardize the term posterior epistaxis as a bleeding point which could not be located Table 126.1

Structured clinical classification.

Classifit{';!: f; ' ~ ~ ~ 1:;-'" -._.-. .,' ".\.-, cases wi'lh a low co~:Plj~{lti0n \h'i,te; :,'H: ,~,;i~ ". ~,;, '., • Unusually severe or ~er.gi-~tent hleeding '-,~:"'~ . should initiate. a scar:1:~{f()r ~e~or~f}~, fa,.ctoi:$;:', -"

~.:

Burnham HH. An anatomical investigation of blood vessels of the lateral nasal wall and their relation to turbinates

KEY POINTS

-

Morgagni JP. The seats and causes of diseases. Vol. 1. Alaba ma; Gryphon Editions Ltd., 1983: 312-54 (first

9.

Wurman LH, Sack JG, Flannery JV, Paulson JO. Selective endoscopic electrocautery for posterior epistaxis.

";' • •

Laryngoscope. 1988; 98: 1348-9. 10.

Woodruff GH. Cardiovascular epistaxis and the nasonasopharyngeal plexus. Laryngoscope. 1949; 15: 1238-47,

Best clinical practice

11.

Chiu TW, Shaw-Dunn J, McGarry GW. Woodruff's nasonasopharyngeal plexus: how important is it in posterior

.I First line: direct therapy (bipolar/cautery, endoscopic if reqUired);

epistaxis? Clinical Otolaryngology. 1998; 23: 272-9. 12.

KR. Jackson RT. Factors associated with active,

refractory epistaxis. Archives of Otolaryngology Head and

./ Second line: ind i rect therapy (anterior packing);

.I Third line: surgical therapy (ESPAL); ./ Fourth line: angiography and embolization.

Jackson

Neck Surgery. 1988; 114: 862-5 . 13.

O'Leary-Stickney K, Makielski K, Weymuller EA. Rigid endoscopy for the control of epistaxis. Archives of

Chapter 126 Epistaxis I 1607

14. 15. 16. 17.

18. 19.

20.

21.

22. 23.

24.

25. 26.

* 27. 28.

29.

30.

31.

32.

Otolaryngology Head and Neck Surgery. 1992; 118: 966-7. Padgham N. Epistaxis: anatomical and clinical correlates. Journal of Laryngology and Otology. 1990; 104: 308-11. Shaheen OH. Arterial epistaxis. Journal of Laryngology and Otology. 1975; 89: 17-34. EI-Silimy O. Endonasal endoscopy and posterior epostaxis. Rhinology. 1993; 31: 119-20. Rosnagle RS, lanagisawa E, Smith HW. Specific vessel ligation for epistaxis and survey of 60 cases. Laryngoscope. 1973; 83: 517-25. McGarry GW. Epistaxis. MD thesis, University of Glasgow, 1996. Woolford TJ, Jones NS. Endoscopic ligation of anterior ethmoidal artery in treatment of epistaxis. Journal of Laryngology and Otology. 2000; 114: 858-60. Bolger WE, Borgie RC, Melder P. The role of the crista ethmoidalis in endoscopic sphenopalatine artery ligation. American Journal of Rhinology. 1999; 13: 81-6. Maran AGD, Lund VJ. (eds). Chapter 28. In: Clinical rhinology, New York: Thieme Medical Publishers, 1990: 101-4. Stell PM. Epistaxis. Clinical Otolaryngology. 1977; 2: 263-73. Weiss NS. Relation of high blood pressure to headache, epistaxis and selected other symptoms. The New England Journal of Medicine. 1972; 287: 631-3. Lepore ML. Epistaxis. In: Baily BJ, Johnson JR, Kohut RI, Pillsbury HC, Tardy ME (eds). Head and Neck Surgery Otolaryngology, Vol. 1. Ch. 34, Philadelphia, J.B: Lippincott Company, 1993: 428-46. Juselius H. Epistaxis: a clinical study of 1,724 patients. Journal of Laryngology and Otology. 1974; 88: 317-27. O'Donnell M, Robertson G, McGarry GW. A new bipolar diathermy probe for the outpatient management of adult acute epistaxis. Clinical Otolaryngology. 1999; 24: 537-41. Kotecha B, Fowler S, Harkness P, Walmsley J, Brown P, Topham J. Management of epistaxis: A national survey. Annals of the Royal College of Surgeons of England. 1996; 78: 444-6. Provides a snapshot of current practice. Nunez DA, McClymont LG, Evans RA. Epistaxis: a study of the relationship with weather. Clinical Otolaryngology. 1990; 15: 49-51. Danielides V, Kontogiannis N, Bartzokas A, Lolis CJ, Skevas A. The influence of meteorological factors on the frequency of epistaxis. Clinical Otolaryngology. 2002; 27: 84-8. Mehanna H, Robinson K, Gatehouse S, McGarry GW. Otorhinolaryngological Research Society (ORS) - A circadian rhythm in adult idiopathic epistaxis? Clinical Otolaryngology. 1998; 23: 280. Manfredini R, Portaluppi F, Salmi R, Martini A, Gallerani M. Circadian variation in onset of epistaxis: analysis of hospital admissions. British Medical Journal. 2000; 321: 1112. Watson MG, Shenoi PM. Drug-induced epistaxis? Journal of the Royal Society of Medicine. 1990; 83: 162-4.

33.

McGarry Gw. Drug-induced epistaxis? Journal of the Royal Society of Medicine. 1990; 83: 812.

Livesey JR, Watson MG, Kelly PJ, Kesteven PJ. Do patients with epistaxis have drug-induced platelet dysfunction? Clinical Otolaryngology. 1995; 20: 407-10. 35. McGarry GW, Gatehouse S, Hinnie J. Relation between alcohol and nose bleeds. British Medical Journal. 1994; 309: 640. 36. McGarry GW, Gatehouse S, Vern ham G. Idiopathic epistaxis, haemostasis and alcohol. Clinical Otolaryngology. 1995; 20: 174-7. 37. Lubianca-Neto JF, Bredemeier M, Carvahal EF, Arruda CA, Estrella E, Pietsch A et al. A study of the association between epistaxis and the severity of hypertension. American Journal of Rhinology. 1998; 12: 269-72. 38. Lubianca-I\jeto JF, Fuchs FD, Facco SR, Gus M, Fasolo L, Mafessoni R et al. Is epistaxis evidence of end-organ damage in patients with hypertension? Laryngoscope. 1999; 109: 1111 -5. 39. Roblin DG, Eccles R. Review: What, if any, is the value of septal surgery? Clinical Otolaryngology. 2002; 27: 77. 40. O'Reilly BJ, Simpson DC, Dharmeratnam R. Recurrent epistaxis and nasal septal deviation in young adults. Clinical Otolaryngology. 1996; 21: 12-4. 41. McGarry GW, Moulton C. The first aid management of epistaxis by accident and emergency department staff. Archives of Emergency Medicine. 1993; 10: 298-300. 42. Smith 1M, Ludlam CA, Murray JAM. Haematological indices in elderly patients with epistaxis. Health Bulletin. 1988; 46/5: 277-81. 43. Carney AS, Weir J, Baldwin DL. Contamination with blood during management of epistaxis. British Medical Journal. 1995; 311: 1064. 44. McGarry Gw. Nasal endoscope in posterior epistaxis: A preliminary evaluation. Journal of Laryngology and Otology. 1991; 105: 428-31. 45. Vandan Abeele D, Clemens A, Tassignon MJ, van der Heyning PH. Blindness due to electrocoagulation following functional endoscopic sinus surgery. Journal of Laryngology and Otology. 1996; 110: 261-4. 46. Tan LKS, Calhoun KH. Epistaxis. Medical Clinics of North America. 1999; 83: 43-56. 47.: Stangerup SE, Dommerby H, Lau T. Hot-water irrigation as a treatment of posterior epistaxis? Rhinology. 1996; 34: 18-20.

34.

48.

49.

50.

51.

McGarry GW, Aitken D. Intranasal balloon catheters: How do they work? Clinical Otolaryngology. 1991; 16: 388-92. Stangerup SE, Dommerby H, Siim C, Kemp L, Stage J. New modification of hot-water irrigation in the treatment of posterior epistaxis. Archives of Otolaryngology-Head and Neck Surgery. 1999; 125: 686-90. Stangerup SE, Thomsen HK. Histological changes in the nasal mucosa after hot-water irrigation. An animal experimental study. Rhinology. 1996; 34: 14-7. Petruson B. A double-blind study to evaluate the effect of epistaxis with oral administration of the antifibrinolytic

1608 I PART

13 THE NOSE A[\ID PARANASAL SINUSES

drug tranexamic acid (cyclokapron). Acta OtoLaryngologica Suppl. 1974; 317: 57-61. 52. Sharp HR, Rowe-Jones JM, Biring GS, MacKay IS. Endoscopic ligation or diathermy of the sphenopalatine artery in persistent epistaxis. Journal of Laryngology and Otology. 1997; 111: 1047-50. 53. Loughran S, Hilmi 0, McGarry GW. Endoscopic sphenopalatine artery ligation-when, why and how to do it. An on-line video tutorial. Clinical Otolaryngology. 2005; 30: 539-43. 54. O'Flynn PE, Shadaba A. Management of posterior epistaxis by endoscopic clipping of the sphenopalatine artery. Clinical Otolaryngology. 2000; 25: 374-7. 55. White PS. Endoscopic ligation of the sphenopalatine artery (ELSA): A preliminary description. Journal of Laryngology and Otology. 1996; 110: 27-30. 56. Strong EB, Bell DA, Johnson LP, Jacobs JM. Intractable epistaxis: Transantral ligation vs. embolization: Efficacy review and cost analysis. Archives Otolaryngology Head Et Neck Surgery. 1995; 113: 674-8. 57. Waldron J, Stafford N. Ligation of the external carotid artery for severe epistaxis. Journal of Otolaryngology. 1992; 21: 249-51. 58. Cumberworth VL, Narula AA, Bradley PJ. Prospective study of two management strategies for epistaxis. Journal of the Royal College of Surgeons of Edinburgh. 1991; 36: 259-60. 59. Scaramuzzi N, Walsh RM, Brennan P, Walsh M. Treatment of intractable epistaxis using arterial embolization. Clinical Otolaryngology. 2001; 26: 307-9.

60.

61. 62.

63.

64.

65.

* 66. 67.

Moreau S, De Rugy MG, Babin E, Courtheoux P, Valdazo A. Supraselective embolization in intractable epistaxis: Review of 45 Cases. Laryngoscope. 1998; 108: 887-8. McGarry G. Nosebleeds in children. Clinical Evidence. 2004; 518-21. Crow WN, Scott BA, Guinto Jr. FC, Chaljub G, Wright G, Rabassa AE et 01. Massive epistaxis due to pseudoaneurysm. Treated with detachable balloons. Archives Otolaryngology Head and Neck Surgery. 1992; 118: 321-4. Garth RJN, Cox HJ, Thomas MR. Haemorrhage as a complication of inferior turbinectomy: A comparison of anterior and radical trimming. Clinical Otolaryngology. 1995; 20: 236-8. Srinivasan V, Patel H, John DG, Worsley A. Warfarin and epistaxis: Should warfarin always be discontinued? Clinical Otolaryngology. 1997; 22: 542-4. Walshe P, Harkin C, Murphy S, Shah C, Curran A, McShane D. Rapid Communication. The use of fibrin glue in refractory coagulopathic epistaxis. Clinical Otolaryngology. 2001; 26: 284-5. Pau H, Carney AS, Murty GE. Review. Hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): Otorhinolaryngological manifestations. Clinical Otolaryngology. 2001; 26: 93-8. Good review of HHT. Lund VJ, Howard DJ. A treatment algorithm for the management of epistaxis in hereditary hemorrhagic telangiectasia. American Journal of Rhinology. 1999; 13: 319-22.

127 Nasa I fractu res

BRENT A McMONAGLE AND MICHAEL GLEESON

Introduction

1609

Com piications

1614

Epidemiology and aetiology

1609

Key points

1615

Classification and pathophysiology

1609

Best clinical practice

1616

Clinical presentation

1611

Deficiencies in current knowledge and areas for future

Investigations

1612

Treatment

1612

1616

research

1616

References

T he data in this chapter are supported by a PubMed search using the key words nasal fracture, focussing on diagnosis and management.

surprising that young men are twice as likely to sustain

INTRODUCTION

a fractured nose as women. Subsequent refracture rates of Treatment of nasal fractures was first recorded 5000 years

5 percent have been reported.4 The peak incidence is in

ago during the early Pharoruc period in Ancient Egypt. The

the IS-3D-year age group when assaults, contact sports

Edwin Smith papyrus describes repositioning of deviated

and adventurous leisure activities are more common.s In

nasal bones with the fingers or elevators, the insertion of

childhood,

splints and the application of external dressings.l Just as

fracture their noses as well and these are often of a

accident-prone

toddlers

not

infrequently

then, nasal fractures are still very conunon.2 Isolated

greenstick nature. Compound and comminuted fractures

fractures of the nasal pyramid account for about 40 percent

are more common in the elderly who are prone to falls.

of all facial fractures. Futhermore, fractures of the nasal bones are often sustained along with other fractures of the facial skeleton.

Delays

in management

can result

in

CLASSIFICATION AND PATHOPHYSIOLOGY

significant cosmetic and functional deformity that is often a cause for subsequent medicolegal action. The manage­

Nasal fractures have been classified in a number of ways,

ment of fractures of the nose is an important part of

for example, by the direction of impact and degree of

everyday ear, nose and throat

force applied, the extent and direction of deformity and

(ENT) practice.

the pattern of the fracture.

Nature of inj ury

EPIDEMIOLOGY AND AETIOLOGY Relatively little force is required to fracture the nasal

Most fractures result from laterally applied forces, over 66

bones,

percent in a series reported by lllum et

as little as

2S-75

Ib/in2•3

It is

perhaps

not

al.6 By contrast,

1610 I

PART 13 THE NOSE AND PARANASAL SINUSES

fractures following frontal injuries accounted for 13 6 percent in this series. Greater force is required to fracture the nose with a blow directed from the front as the nasal cartilages behave like shock absorbers.

Frontal bone

}

ThiCk part -+-- Force

from

this direction

Maxilla

Thin part

Extent of deformity

Line of fracture

A five-point grading system has been developed for the extent of lateral deviation of the nasal pyramid: • •

•

•

•

grade 0: bones perfectly straight; grade 1: bones deviated less than half of the width of the bridge of the nose; grade 2: bones deviated half to one full width of the bridge of the nose; grade 3: bones deviated greater than one full width of the bridge of the nose; grade 4: bones almost touching the cheek.

Pattern of fracture

0es 'Oo

�0-s?J.�

Perpendicular plate of ethmoid Sphenoid sinus

Cartilaginous septum

Vomer

Maxillary crest

Figure 127.1

The distal part of a nasal bone is half the

thickness of the proximal part. When it fractures, the nose is apparently deviated but the impression of deviation is optical. It

Nasal fractures can also be subdivided into three broad categories that characterize the patterns of damage sustained with increasing force. This classification has some practical utility as each category of fracture requires a different method of treatment.

is important to recognize this injury. The edges of the stable segment can often be palpated. Figure courtesy of AGD Maran.

'greenstick' variety and significant nasal deformity may only develop at puberty when nasal growth becomes accentuated.7

CLASS 1 FRACTURES

Class 1 fractures are the result of low-moderate degrees of force and hence the extent of deformity is usually not marked. The simplest form of a class 1 fracture is the depressed nasal bone. The fractured segment usually remains in position due to its inferior attachment to the upper lateral cartilage which provides an element of recoil. The nasal septum is generally not involved. In the more severe variant, both nasal bones and the septum are fractured. The fracture line runs parallel to the nasomaxillary suture ipsilateral to the side of the applied force to a point approximately two-thirds along the length of the nasal bone, where the bone becomes much thicker. The fracture line then connects across to the contralateral side and runs parallel and just below the dorsum. The cartilaginous septum is fractured approxi­ mately 0.5 cm below the dorsum and this aspect of the injury may extend posteriorly into the bony septum, through the perpendicular plate of the ethmoid and skull base. This fracture was first described by Chevallet and bears his name (see Figure 127.1). Class 1 fractures tend not to cause gross lateral displacement of the nasal bones and may not even be perceptible. Deformity generally results from a persis­ tently depressed fragment, which is often due to impaction of the flail segment beneath the residual nasal bone. In children, these fractures may be of the

CLASS 2 FRACTURES

Class 2 fractures are the result of greater force and are often associated with significant cosmetic deformity. In addition to fracturing the nasal bones, the frontal process of the maxilla and septal structures are also involved. The ethmoid labyrinth and adjacent orbital structures remain intact. As a rule of thumb, if the nasal dorsum is deviated laterally greater than half the width of the nose (grade 2 or greater fracture), then a septal fracture must also be present. The pattern of deformity is determined by the direction of the force applied. A frontal impact tends to comminute the nasal bones and cause gross flattening and widening of the dorsum; while a lateral impact produces a high deviation of the nasal skeleton. What may appear to be a simple dislocation of the quadrangular cartilage from the bony septum is in reality a complex 'C-shaped' fracture that extends from the quadrangular cartilage beneath the nasal tip, posteriorly through to the perpendicular plate of the ethmoid, to the anterior border of the vomer and then forward through the lower part of the perpendicular plate of the ethmoid into the inferior part of the quadrilateral cartilage. This pattern of fracture was first described by Jarjavay and bears his name (see Figure 127.2). Septal fracture-dislocations tend to happen at points of weaknes�. They are where the quadrangular cartilage inserts into the cartilaginous dorsum, the bony septum

r

Chapter 127 Nasal fractures I 1611

pneumocranium and cerebral herniation may complicate this type of injury. The management of this type of nasal fracture is covered in Chapter 128, Fractures of the facial skeleton.

Frontal bone

r------

I

Maxilla Line of fracture

History

Nasal bone

�

Perp ndicular plate of ethmoid

Sphenoid sinus

Cartilaginous septum

Maxillary crest Line of Fracture

Figure 127.2

A class 2 nasal fracture involves the thicker

proximal portion of the nasal bone.

CLINICAL PRESENTATION

It

results in true deviation

and also fracture of the perpendicular plate of the ethmoid and quadrilateral cartilage. Figure courtesy of AGD Maran.

and the maxillary crest. Both the nasal bone and septal fractures need to be reduced together in order to achieve a satisfactory cosmetic result.

CLASS 3 FRACTURES

Class 3 fractures are the most severe nasal injuries encountered and usually result from high velocity trauma. They are also termed naso-orbito-ethmoid fractures and often have associated fractures of the maxillae. The external butresses of the nose give way and the ethmoid labyrinth collapses on itself. This causes the perpendicular plate of the ethmoid to rotate and the quadrilateral cartilage to fall backwards. These movements cause a classic, 'pig-like' appearance to the patient, with a foreshortened saddled nose and the nostrils facing more anteriorly, like the snout of a pig. There is also telecanthus, which may be exaggerated further by disruption of the medial canthal ligament from the crest of the lacrimal bone. Two categories of naso-orbito-ethmoid fractures have been recognized by Raveh. 8 In the first type, the anterior skull base, posterior wall of the frontal sinus and optic canal remain intact. In the second type, there is disruption of the posterior frontal sinus wall, multiple fractures of the roof of the ethmoid and orbit that may extend posteriorly to the sphenoid and parasellar regions. Multiple dural tears, cerebrospinal fluid leaks,

It is prudent to establish how and when the injury was sustained, not only for medicolegal purposes but also because there is a limited period of time during which simple reduction is possible. There will almost certainly be some degree of nasal obstruction associated with the injury, but complete obstruction and persisting pain might indicate the presence of a septal haematoma. Enquiry should be made about any obvious change in the shape of the nose and of previous injuries or past nasal surgery. The patient may have a recent photograph that could be helpful to establish their claim. Other injuries may also be present and may have been overlooked. Diplopia, visual disturbance and epiphora suggest orbital trauma. Loose teeth, an altered bite or trismus indicate the need for a dental opinion. The onset of watery rhinorrhoea and loss of the sense of smell, though uncommon, signal possible skull base damage and the need for a more detailed evaluation. Finally, it is worth enquiring about their leisure pursuits and any occupational hazards that might influence any decision to correct the deformity. Key issues to consider when determining history include: • • • • • • • • • • • •

details of how the injury was sustained; nasal obstruction; change in appearance; epistaxis; hyposmia; watery rhinorrhoea; visual disturbance; diplopia; epiphora; altered bite;' loose teeth; trismus.

Examination

The importance of clear and complete records of the clinical examination cannot be overemphasized. What is and what is not a new injury can be difficult to determine at a later date when litigation is contemplated. Inspect the nose for any external deformity. This may be extremely difficult in the acute situation when swelling may hide an underlying abnormality. A second inspection a few days later may be necessary. Gently palpate the nasal bones for

1612 I

PART 13 THE NOSE AND PARANASAL SINUSES

a step deformity and make note of any surgical emphysema that could suggest a more serious injury. Make a detailed record of soft tissue lacerations. Inspect the nasal cavities and check for the presence of a septal haematoma or deviation. Do not fail to make a thorough general ENT /head and neck examination. Key issues to consider when examining a patient include: • • • • • •

deviation, depression, step deformities; mobility, crepitus, specific areas of point tenderness; generalized swelling; skin lacerations; septal fracture/haematoma/abscess/perforation; mucosal lacerations.

INVESTIGATIONS

The need for nasal x-rays is controversial and in many places it is actively discouraged. Unlike other fractures, nasal x-rays are not required in order to make the diagnosis or aid subsequent reduction. In a prospective study undertaken by Logan et aI.,9 it was concluded that x-rays were not cost effective. [**] Their only possible utility is proof of injury in subsequent litigation. If there is clinical evidence of a more serious facial injury a computerized tomography (CT) scan should be acquired. Samples of any watery rhinorrhoea must be collected in those with suspected cerebrospinal fluid (CSF) leak and tested for �2 transferrin (see Chapter 129, Cerebrospinal fluid rhinorrhoea). TREATMENT

A very significant number of patients, 80 percent in the series reported by Karagama et aI.,5 do not require any active treatment. Many do not have a nasal fracture and, in those that do, the fracture may not be displaced. Soft tissue swelling can produce the misleading appearance of a deformity which disappears as the swelling subsides. Reassurance is all that these patients require and some may heed suggestions to avoid further trawna. Topical vasoconstrictor drops are helpful to alleviate congestion and obstructive symptoms. A reexamination about five days later is prudent where there is uncertainty about the need for reduction. A large number of patients will have a preexisting nasal deformity caused by a previous incident. Manipulation of the nose will, at best, only return it to its most recent appearance. Patients that fall into this category are probably better advised to consider a formal rhinoplasty when everything has settled down some months later. Others will be at continued risk of further injury because of an occupational hazard, sport or leisure activity. It is better to advise this group of patients to undergo a definitive septorhinoplasty when the risk of further injury no longer exists.

The indications for surgical intervention in the acute phase are significant cosmetic deformity and nasal obstruction caused by a septal haematoma. Apart from issues mentioned previously, the only other contra­ indications for reduction are medical conditions that would make the patient an unreasonable anaesthetic risk. As a general rule, primary care physicians should refer all patients to ENT departments for evaluation if there is any deformity or significant nasal obstruction. Patients with a suspected septal haematoma should be seen urgently at the first possible opportunity. It is the primary care physicians' responsibility to ensure that their patient is seen quickly, certainly within the first few days and no later than one week. Those patients for whom treatment is considered unnecessary should be given the contact details of the ENT clinic lest deformity become apparent over the ensuing weeks.5 The timing of surgical assessment and subsequent reduction is crucial as there is a narrow window of opportunity to correct the deformity. The optimal time for clinical assessment is around four days, by which time much of the oedema will have subsided and any underlying deformity apparent. As stated previously, failure of referral or timely referral is a common cause of litigation in nasal injuries. Review at four days allows sensible planning for reduction of the fracture on an elective operating list within the next two to three days, if it is to be reduced under a general anaesthetic. By seven days, the bony abnormality will be easily palpable and still movable. Further delay makes effective reduction less likely and sometimes impossible without making osteo­ tomies. In children, healing can take place even more quickly and earlier intervention is indicated. [**/*]

Anaesthesia

Reduction of a fractured nose can be performed under local or general anaesthesia. Local anaesthesia has the advantages of reduced cost and convenience. However, the injections can be painful and the associated distortion of soft tissues can hinder accurate assessment of the reduction. In an awake patient there may be a reluctance to use the force necessary to correct the deformity leading to an inferior outcome. Nevertheless, this method avoids the problem of scheduling patients at short notice on to heavily booked operating lists. Local anaesthetic can be used as a combination of external infiltration with internal application of topical preparations. Lignocaine is injected along the nasomax­ illary groove, infraorbital nerve in its foramen and around the infratrochlear nerve. Jones and Nandapalanlo recently reported the use of topical EMLA (prilocaine and lignocaine) cream or AMETOP (amethocaine) gel to the external nose instead of infiltration. They found less discomfdrt when the manipulation was carried out using the topical, local anaesthetic preparation compared with

Chapter 127 Nasal fractures I 1613

local infiltration. They achieved similar cosmetic and functional

{****]

results.

Within

the

nose,

sprays,

injections, pastes or packs coated with local anaesthetic are

all

acceptable,

using

combinations

of

cocaine,

lignocaine, adrenaline and phenylephrine. The vasocon­ strictive element helps to reduce any bleeding associated with mucosal tears from instrumentation during the reduction, but must be used cautiously in hypertensive patients or those with cardiovascular disease. Local versus general anaesthetic as the preferred means for reduction of nasal fracture is a contentious issue and the literature is divided over the influ ence on outcomes.

Ridder et al.II found that there was no significant difference in overall success rate between nasal fractures reduced with local and general anaesthesia. On the other hand, in a retrospective analysis of 324 patients, Courtney et al.4 found a higher number of patients seeking a septorhinoplasty when the initial reduction was per­ formed

under

anaesthetic (17.2 percent) than (3.2 percent). [**]

local

general anaesthetic

In terms of the overall experience of closed reduction under local anaesthesia, Newton and White12 found that

90.6 percent of patients felt it was less painful than a dental restoration and 96.9 percent would readily under­ go the procedure again if required. In other studies, nearly all patients found local anaesthesia perfectly adequate and 13, 4 1 [****/***/**] Local. anaesthesia can also

acceptable.

be combined with sedation in an outpatient setting. There are even some circumstances where closed reduction can be performed without any anaesthesia, for example, on a

Figure 127.3

Instruments used in nasal fracture manipulation.

(a) Howarth's elevator, (b) Ashe's forceps (septum): (c) Walsham's forceps (nasal bones).

sports field. However, from personal experience, it is painful!

finger

There are easily identifiable groups of patients who are not

suitable

for

reduction

under

local

anaesthesia.

of

the

dominant

hand

is

placed

along

the

instrument in the line of the nose. In this way, the depth that the instrument has to be inserted into the nasal cavity

Children, patients with low pain thresholds or significant

is easily gauged and inadvertent damage to the orbit

anxiety states are better admitted for general anaesthesia.

avoided

Likewise, attempts at reduction of fractures in which there

fractures can be reduced with these techniques.

has been delay in presentation are better performed under

(Figure 127.4). All class

For many class

1 and most class

2

2 fractures, closed reduction alone

rarely achieves a satisfactory result as the final postion of

general anaesthetic.

the nasal dorsum reflects the deformity of the underlying septum.

Methods of reduction

1 15 16, , ,

17 At least 50 percent of these fractures

remain displaced because of overlapping segments of the fractured perpendicular plate of the ethmoid or septal

The general principle of fracture reduction is to mobilize

cartilage,

the fragments first by increasing and then decreasing the

reduction.

degree of deformity. An initial slight increase in deformity away

from the

side

of

the

blow to

disimpact

which

can

only

be

repositioned

by

open

Occasionally, the bones are fixed, especially if the

the

fracture is old, and osteotomies are necessary to release

fragments, followed by steady movement back towards

the fragments before manipulation. These should be

and often slightly beyond the midline is usually required.

performed cautiously to avoid the risk of extension into

In most cases, the nose will be restored to its original

the orbit or, even worse, intracranially.

IS

position. More often than not, this can be achieved by

Splints or packs may be necessary, depending on the

firm digital pressure. Sometimes instruments are neces­

stability of the reduction and the surgeon's preference. A

sary, particularly in those where there has been delay.

splint or plaster applied to the nasal bridge maintains, to

Various

developed

some extent, the position of the nasal bones and prevents

specifically for this purpose, such as Freer, Hills and

accidental displacement. Splints are usually kept in place

Howarth

elevators elevators

and and

forceps Ashe

have and

been

Walsham

forceps

(Figure 127.3). The instrument is held so that the index

for about seven days. It is advisable to refrain from contact sports for at least six weeks.

' __,..

.. _i

:_�___ �__

.

1614

I PART 13 THE NOSE AND PARANASAL SINUSES

some (rhinoplasty' techniques, such as release of upper lateral

cartilages,

hump

removal

and

camouflaging

cartilage grafts?3 Incorporation of these more advanced techniques into the management of the fractured nose requires

expertise,

more

instrumentation

and

more

operating time and this may not always be feasible.

Management of the nasal septum Septal fracture is often missed and is a major reason for poor functional and cosmetic results. Kim et a/?4 found that 46.9 percent of nasal fractures have a concomitant septal fracture. If a septal fracture is present, it is wise to reduce this at the same time as the nasal bones. In a prospective study of 52 nasal fractures, Rhee et a1.25 found that 78.8 percent required septoplasty at the time of reduction

of

nasal

fracture.

In

fact,

a

satisfactory

reduction of nasal bones is often not possible without improving the position of the septum. An often quoted aphorism, 'where goes the septum, so does the nose' is not far from the truth. Septal reduction can sometimes be performed with Ashe's forceps, but often requires a Killian or hemi­ transfixion incision, elevation of mucosal flaps to expose the cartilage and bone fragments, and replacement and/or Figure 127.4

Determining depth of insertion of instrument

into nasal cavity,

removal of cartilaginous and bony fragments, as in a standard septoplasty. Satisfactory repositioning is aided by the removal of thin strips of cartilage in a vertical direction at the bony-cartilaginous junction and along

Closed techniques for nasal reduction have tradition­ ally been used, but open reduction techniques may lead to

the maxillary crest. Quilting sutures prevent a dead space from forming and a haematoma collecting.

improved results, despite the increased time and effort involved. Mayell19 and Eichorn et al.2o found satisfactory results in only a third of cases reduced by closed techniques, whereas Illum21 and Watson et az.22 found 71-90 percent of patients had good results after closed techniques.

[**]

Verwoerd17

identified

the

Poor cosmetic result

following

indications for open reduction:17 •

COMPLICATIONS

Attempts to reduce deformity are not always successful.

bilateral fractures with dislocation of the nasal dorsum and significant (preexistent or recent) septal deformity;

Residual deformity has been recorded in 14-50 percent after closed reduction.I8 Factors which contribute to an unsatisfactory cosmetic result include:

•

infraction of the nasal dorsum;

•

extent of the injury;

•

fractures of the cartilaginous pyramid, with or

•

time delay in surgical reduction;

without dislocation of the upper laterals.

•

poor surgical technique;

•

septal fracture unrecognized and untreated;

For depressed tip or flail lateral fractures that are unstable despite closed reduction techniques, Kirschner

(K) wires

can be used. The wire is inserted under fluoroscopic guidance into the depressed fragment as well as neighbour­

•

preexisting nasal deformity;

•

postoperative trauma (in recovery room or subsequently); scarring and fibrosis.

ing uninvolved bone (maxilla or frontal bone), and the

•

wires are screwed together externally to maintain the

Unfortunately, it is not uncommon for the shape of the

position. The external wire can be covered by dressings or

nose to change over time as a result of subsequent

from injury. The wires are removed after two weeks?

hump formation. Some patients inevitably require a

plaster to protect the wires from disruption and the patient Some authors advocate a more aggressive approach to the treatment of acute nasal fractures which includes

scarring and fibrosis, loss of tip or dorsal support, or septorhinoplasty despite apparent adequate reduction. In these,

conventional

medial

and

lateral

osteotomy

Chapter 127 Nasal fractures I

techniques should not be

used

as

t he bones tend to

1615

haematoma. Gentle pressure on the bulging area will collection is present.

refracture along the old fracture lines. Mackaj6 advocates

ascertai n that it is fluctuant if a

intermediate and lateral osteotomies, effectively shatter­

becomes very unwell with a fluctuati n g fever, severe facial

a triple osteotomy technique, with complete medial,

ing the nasal bones. It is after the allow

wise

to wait at least six months

injury before embarking on septorhinoplasty to

the

fractures

to

heal,

the

oedema

to

settle

completely so the underlying nasal skeleton is evident

and for any fibrosis to develop. The frequency of poor cosmetic res ults can be measured by both

surgeon

and

patient dissatisfaction and subsequent septorhinoplasty

rates. Staffel23 reviewed the literature and found that

when pa t ient satisfaction is reviewed using a question

­

naire they tend to be more satisfied with the result (79 percent) than

their surgeon (37 percent).

Untreated, an abscess may

develop

and the patient

and cranial pain. Rarely, cavernous sinus thrombosis or forms

other

of

intracranial

sepsis

can

ensue.

The

haematoma or abscess must be drained as soon

possi ble.

as

This can be performed under local or general

anaesthetic either by using needle aspira tion or, pre

ferably,

an

­

incis i on. Often the collection will have become

organized and so impossible to aspirate. In this situation,

a small window of mucoperiostium should be excised ensuring

there

is

no

corresponding

defect

on

the

contralateral side which could predispose to a perfora­ tion.

Once drained,

quilting

sutures

from

one side

through cart ilage to the other are inserted to eliminate

the dead space. Packs or splints can be used to provide

gentle pressure on the septum. The patient must be

Nasal obstruction Postoperative nasal obstruction is are many causes that incl ud e:

also common and there

• valve obstruction: collapse of upper lateral cartilages and depressed nasal bones;

reexamined within 48 hours to establish that the

collection has not recurred. The management of a septal

abscess is similar, but with the addition of appropriate an tib i otic therapy. Septal

perforations

may

also

develop

after

nasal

fractures, usually as a result of septal haema tomas and

• septal deviati on; • widened septum (haematoma); • tip ptosis.

their

surgical treatment.

Loss

of cartil aginous septal

support can also lead to a saddle nose deform i t y, as well

These causes need to be considered an d addressed at a

subsequent septorhin oplasty.

as columellar retraction and a broadened septum. This is

why it is imp or tant that all nasal inju ries are assessed within the

fi rst

24-48 ho ur s to exclude these entities and

so that the appropriate referral to an ENT Dep artment can be made.

The correction of a saddle nose deformity generally

Epistaxis Epistaxis at the time of injury may

usually rel at ively

be impres sive but is brief. Occas io nall y , fractures involving

the nasoethmoidal complex can cause laceration to the

requires the incorporation of autologous cartilage from

elsewhere to reconstruct the defect Su itable grafts can be

acquired

from

the

conchaI

bowl

or

costal

region,

although occasionaUy bony septum from the perpend i­

anterior ethmoidal artery. This may result in repeated,

cular plate of ethmoid can be used. Septal perforations

the fracture has been reduced. Sometimes, prolonged

and minor

brisk and significant haemorrhage that only stops once

packing or ligation of the artery is required. Bleeding can

the time of reduction, partic ularly if jnstruments are used . Preparation of the nose with

are often asymptomati c, but can be subject bleeds.

S im ple measures,

irrigations and protective ointments,

crustin g

can give long­

also be troublesome at

standing relief.

vasoconstrictive agents helps to minimize this blood loss.

tion with pedicled mucosal flap s (see Chapter

The p rovision

to

such as sali ne

of a septal button is

favoured by some, while others embark upon reconstruc­

septal perforations) .

124, N as al

Septal complications A submucoperi c h ondrial bleed not infrequently compli­

cates nasal fractures. While most septal haematomas are

relati vely limited, some may str i p the mucoperichon ­

drium over an extended area and dep rive the und erly ing

cartilage of its

source of nutrition. This can lead to u lti mately nasal defo r mity. Septal haematomas present with intense unilateral or bil ate ral nasal obstruction and, on inspection, there is a reddish­ purple, fluctuant swelling of the cau d a l septum. A cartilage necrosis and

deviated

septum

can

be

confused

with

a

septal

" �_

Na��-ir�-9:hJ,es

arc cqmmon an�'-:a*aren,ess of

. :;:�, ; w�E � ��f:;t'�:les

a�.�erit ·.�nsures

�fma 5 cm) usually require a craniotomy with cranialization of .the frontal sinus,~da p~~lcrp.ni,i:U,fla'p. ':: .. • The priinary reaSon for failure.; is .if there .'is, .a high pressure, system and these' more . " . common in thegrotip',With sp(Hl~aneDus_ l.eaks and may requite a' shunt. • It is essential fo "confirm-- the diagnosis -of a CSF leak by -testing fluid using the . immunofixation ofbeta-2' transferrin.

are.

Best cUnical practice .I Endoscopic repair has revolutionized the repair of CSF leaks and minimized the morbidity and mortality associated with surgical repair. .I It is vital to confirm that any clear rhinorrhoea is CSF by testing it, using the immunofixation of beta transferri n. ./ Imaging techniques including high definition CT scans and T2-we[ghted MRI will detect the site of the leak in most patients. Where the site of a leak is uncertain or there is the possibility of more than one defect, then a pre- or peroperative Auorescein lumbar puncture will help in the remaining patients. ./ Rarely, defects of the posterior wall of the frontal sinus are inaccessible using the endoscope and then an extradura I approach is preferable. ./ Large or multiple defects may require a craniotomy when cranialization and a pericranial flap give the best results.

Deficiencies in current knowledge and areas for future research It is often not possible to predict which patients have a high-pressure system that will require a shunt. 61 Whilst the patient has an active CSF leak, the pressure is not raised and measuring it with a lumbar puncture is unhelpful. In the authors' experience, in the first few days after repairing the leak the pressure is often not raised, even in patients who go on to leak again and require a shunt. One study showed a proportion had a raised pressure after the leak was stopped, but interestingly there was no mention of these patients going on to have another leak.62 Whilst approximately 50 percent of repaired spontaneous leaks recur compared with less than 2 percent due to other causes if spontaneous leaks are excluded,61 it is not clear which spontaneous leaks require a shunt when these patients

.J._'_~

....

1642 I PART 13 THE !\JOSE AND PARANASAL SINUSES first present, unless there are signs of hydrocephalus or a raised intracranial pressure. Signs of an empty sella, skull base erosions or benign intracranial hypertension make it more likely that these patients will have a raised pressure system, but they are not specific or sensitive enough for the patient to justify a shunt at the first repair. At present, patients with a spontaneous leak undergo an attempt at repair and if this fails a shunt is inserted at the next attempt. It would be helpful to have a more accurate predictor of which patients would 61 require a shunt.

13.

14.

15.

16.

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Neuroradiology. 1995; 1: 177-81. Stafford Johnson DB, Brennan P, Toland J, O'Dwyer AJ. Magnetic resonance imaging in the evaluation of 837-41. Gupta V, Goyal M, Mishra N, Gaikwad S, Sharma A. MR

closure. Journal of Neurosurgery. 1969; 30: 399-405. Donald PJ. Frontal sinus ablationby cranialisation: a report of 21 cases. Archives of Otolaryngology. 1982; 108: 590-5.

55.

cerebrospinal fluid fistulae. Clinical Radiology. 1996; 51: 38.

Ray BS, Bergland RM. Cerebrospinal fluid fistula: Clinical aspects, techniques of localization, and methods of

Kelley TF, Stankiewicz JA, Chow JM, Origitano TC, Shea J. Endoscopic closure of postsurgical anterior cranial fossa

56.

cerebrospinal fluid leaks. Neurosurgery. 1996; 39: 743-6. Hubbard JL, McDonald TJ, Pearson BW, Laws ER.

evaluation of CSF fistulae. Acta Radiologica. 1997; 38:

Spontaneous cerebrospinal fluid rhinorrhea: Evolving

39.

603-9. Stammberger H. Functional endoscopic sinus surgery. Philadelphia: BC Decker, 1991: 436-8.

concepts in diagnosis and surgical management based on the Mayo Clinic experience from 1970 through 1981.

40.

Hughes RGM, Jones NS, Robertson IJA. The endoscopic

41.

57.

treatment of cerebrospinal fluid rhinorrhoea: The

in repair of cerebrospinal fluid rhinorrhea. A 20-year

Nottingham experience. Journal of Laryngology and

experience. Otolaryngology, Head and Neck Surgery. 1989;

Otology. 1997; 111: 125-8. Bateman N, Mason J, Jones NS. Use of fluorescein for

58.

detecting cerebrospinal fluid rhinorrhoea: A safe technique for intrathecal injection. ORL, Journal for Otorhinolaryngology and its Related Specialties. 1999; 61 :

59.

43.

44.

Laryngology. 1996; 105: 620-3. 60.

Brodie HA. Prophylactic antibiotics for post traumatic cerebrospinal fluid fistulae. Archives of Otolaryngology, Head and Neck Surgery. 1997; 123: 749-52. Moralee SJ. Should prophylactic antibiotics be used in the management of cerebrospinal fluid rhinorrhoea following endoscopic sinus surgery? A review of literature. Clinical Otolaryngology. 1995; 20: 100-2.

Gjuric M, Goede U, Keimer H, Wigand ME. Endonasal endoscopic closure of cerebrospinal fluid fistulas at the anterior cranial base. Annals of Otology, Rhinology, and

Moseley JI, Carton CA, Stern WE. Spectrum of complications in the use of intrathecal fluorescein. Journal of Neurosurgery. 1978; 48: 765-7.

101: 555-8. Burns JA, Dodson EE, Gross CWo Transnasal endoscopic repair of craniofacial fistulae: A refined technique with long-term follow-up. Laryngoscope. 1996; 106: 1080-3.

131-2. 42.

Neurosurgery. 1985; 16: 314-21. Yessenow RS, McCabe BF. The osteo-mucoperiosteal flap

* 61.

Wormald PJ, McDonogh M. 'Bath-plug' technique for the endoscopic management of cerebrospinal fluid leaks.

Journal of Laryngology and Otology. 1997; 111: 1042-6. Mirza S, Thaper A, McClelland L, Jones I'JS. Sinonasal .cerebrospinal fluid leaks: Management of 97 patients over 10 years. Laryngoscope. 2005; 115: 1774-7. This paper gives an account of the problems that relate to spontaneous CSF leaks and raised intracranial pressure.

1644 I 62.

63.

64. 65.

PART 13 THE NOSE AND PARANASAL SINUSES

Schlosser RJ, Wilensky EM, Grady S, Bolger W. Elevated intracranial pressures in spontaneous cerebrospinal fluid leaks. American Journal of Rhinology. 2003; 17: 191-5. Dunn CJ, Alaani A, Johnson AP. Study on spontaneous cerebrospinal fluid rhinorrhoe: Its aetiology and management. Journal of Laryn9ology and Otology. 2002; 119: 12-5. Stankiewicz JA. Cerebrospinal fluid fistula and endoscopic sinus surgery. Laryngoscope. 1991; 101: 250-6. Papay FA, IViaggiano H, Dominquez S, Hassenbusch SJ, Levine HL, Lavertu P. Rigid endoscopic repair of paranasal

66.

67.

68.

sinus cerebrospinal fluid fistulas. Laryngoscope. 1989; 99: 1195-202. Hao SP. Transnasal endoscopic repair of cerebrospinal fluid rhinorrhea: An interposition technique. Laryngoscope. 1996; 106: 501-3. Mattox DE, Kennedy DW. Endoscopic management of cerebrospinal fluid leaks and cephaloceles. Laryngoscope. 1990; 100: 857-62. Simmen D, Jones N. Skull base surgery. In: Simmen D, Jones N (eds). Manual of endoscopic sinus surgery and its extended applications. Chapter 15, Stuttgart: Georg Thieme Verlag, 2005: 240-55.

130 Granulomatous conditions of the nose DAVID J HOWARD AND VALERIE J LUND 1645 1645 1649 1653 1653 1654 1655

Definition Sa rco idosis Wegener's granulomatosis Churg-Strauss syndrome Eosinophilic granuloma Giant cell granuloma Cho lesterol gran uloma

• ,

.

1655 1657 1657

Granulomatous neoplasia Key points Best cl i ni cal practice Deficiencies in current knowledge and areas for future research References

. •

.

.

~

'..

1657 1657

. . . ,- r ••

.r

#.,.

,.

~..

....

I

The data in this chapter are supported by a Medline search using the key words granulomatous disease, Wegener's granulomatosis, sarcoidosis, nose and paranasaJ sinuses. Levels of evidence 1-4 and clinical recommendations grades A-D have been used throughout.

DEFINITION The principal element of a granulomatous condition is self-evidently a granuloma, consisting of macro phages, epithelioid cells and mu1tinucleated giant cells. This histologic configuration is encountered in a number of infective inflammatory and neoplastic conditions of the nose and sinuses (Table 130.1) and also forms part of a spectrum of conditions in which vasculitides plays a role. Inflammation of the blood vessels may be primary or secondary and is further classified according to the size of vessel affected (Table 130.2). Whilst many of these conditions are systemic, they may preferentially target or present in the upper respiratory tract and the ear, nose and throat (ENT) surgeon must maintain a continuous low threshold of suspicion to establish the diagnosis at the earliest opportunity.

frequently involves lymph nodes, skin, lungs, eyes, liver, spleen and the small bones of the hands and feet It is found throughout the world, although there is a higher incidence in the southeast United States and Scandinavia where 64 out of 100,000 people were found to have pulmonary sarcoidosis in Stockholm. The ratio of occurence in black to Caucasian people is 12:1 in the United States. 3 , 4 Cutaneous lesions were first described by Besnier s in 1889 as 'lupus pernio', a term that is still used but the generalized nature of the condition was recognized ten years later by Boeck6 who noted four of his nine patients had nasal disease which preceded the skin lesions. The distribution of clinical manifestations to some extent reflects the bias of the treating physician, but it is generally agreed that nasal manifestations are almost always part of multisystem sarcoid which may have been present for some years 0-20 years, mean 4.4 years).7

SARCOIDOSIS

Age and sex

Introduction

Sarcoidosis is a conclition of young adults between the third and fifth decades with a female preponderance of 2: 1) which is higher in those with upper respiratory tract manifestations.

Sarcoidosis is a systemic condition of unknown aetiology which may affect any part of the body, but most

1646 I

PART 13 THE NOSE AND PARANASAL SINUSES

Table 130.1

Granul omatous co n d itio ns affect i n g the nose a n d s i n u ses. "

Inf�ctivea

",

'

J.'

N�Pla� k • �

•

1

� ',

'

•

Bacteria Tubercu los i s

Myobacterium tuberculosis

Wegener's gra n u lomatosis

T-ce l l lymp homa

Leprosy

Myobacterium leprae

Sarcoidosis

( M i d line let hal

Rh i noscleroma

Klebsiella rhinoscleromatis

Churg-Strauss syndrome

Syp hilis

Treponema pallidum

Cholesterol granu loma

Actinomycosis

Actinomyces israeli

Eos i n ophi l i c g ranuloma

granuloma)

Fungal Asperg i l lus

Asp. fumigatus, flavus, niger

Zygomyc.osis

Conidibolus coronatus Rhizopus oryae

Derrnatacietes

CUlVularia Alternaria Bipolaris

Rh inos porid iosi s

Rhiosporidiosis seeber;

B lastomycosis

Blastomyces dermatitidis

Histop lasmosis

Histoplasma capsulatum

Spo rotrichosis

Sporotrichum schenki;

Cocci d i 0 i d i mycosis

Coccidiodes immitis

Cryptococcus neoformans

Protozoa

Le ish rna niasis

Leishmania spp.

aCovered in Chapter 115, Specific chronic infections and Chapter 114, Fu ngal rhinosinusitis. Modified from Ref. 1, with permission.

Table 130.2

Pri,mary vascul itis according to different vess e l size.

' ;'�4��,�;�e�:i��';�'�;6';'�ro a:l� !p��d.}��f.eJ��

.

Ta kayasu arteritis Giant cell (tem poral) arteritis

'

;

�

M,ed'ium�si'z�d i,I,nd .

'·$lY,la·iL'DI·�.od".y�S�� ,I� Polyarte r itis nod osa C hurg-Strauss

Granulomatous angi itis of the central nervous system

syndrome Wegener's granulomatosis

Small blood '

vessels

i '"

.

. Mi.�c�na·')eo·us.· ·.StcoJl(i�a.'1':v:is cunti5 co n d itio n s . ; . .� .;c .

Bue rge r's

Microscopic

disease

polya n g i it i s

Schonlein-Henoch

Be hr;et's d i sease

synd rome Cutaneous

Kawasaki

leu kocytociastic

disease

an giitis

,"

'•

. " ".

>:.�, .

I nfection-re lated vascu litis Ser um sick ness or d rug hypersensitivity­ related vasculitis Hypocomp leme ntemic u rticarial vasculitis Vasc u l itis associated with r h eumatic connective tissue diseases VaSCU l i t i s associated with other systemic diseases Ma ligna nc.y-rela ted vascul itls Post-t ran s p lant vasculitis Pseud ovasculit ic syndromes ( myxoma, e n d ocard itis, Sneddon synd rome)

Reprinted from Ref. 2, with permission.

Aetiology The aetio lo gy

of sarco ido si s is unknown, but susp1ClOn on various infective agents, chemicals (includ­ ing beryllium and zirconium), pine po ll en and even 8 peanut dust. Whilst the type IV del ayed hypersensitivity reaction is often depressed in sarcoid patients, there is no g e neral de p re ssi o n of celt-mediated i mmunity and type I has fallen

and humeral ant ibo d y responses

are normal. However, protein levels are raised in particular gamma globulin in those of African descent.9 There also a ppe ars to be an increase in c irculat ing i mm u n e complex es in the acute phase with a decrease in circulating lymphocytes, particularly T cells of which 10-20 percent are atypica1.10 One suggegtion for the path og enesis of sarcoid is that it is a process initiated by an ant ig e n presenting ceil, probably total plasma

Chapter 130 Granulomatous conditions of the nose I

an alveolar macrophage. Subsequently, there is an IL-1 and 1L-2 driven T-Iymphocyte proliferation. Monocytes are attracted from the blood by monocyte chemotactic factor (1L-8) and held at the site of reaction by macrophage migration inhibition factor. Monocyte acti­ vation by lPN-gamma occurs and this stimulates calcitriol production, which in turn causes macrophage fusion to epithelioid cells and granuloma formation. The persis­ tence of the granuloma may be ascribed to continued antigenic stimulation, failure of suppressor-regulating mechanisms and/or abnormalities in the regulation of the cytokine network.

In the nose, the mucosa often has a rather character­ istic granular appearance, sometimes referred to as a (strawberry skin' (Figure 130.1). The mucosa refers to the tiny pale granulomas against an otherwise erythematous mucosa. This is generally very friable and leads to complaints of nasal congestion, crusting and mucopuru­ lent discharge which is often blood stained (Table 130.3).

Table 130.4

In 1985, Scadding and Mitchellll recommended the following definition (sarcoidosis is a disease characterized by the formation in all of several affected organs or tissues of epithelioid cell tubercules, without caseation though fibronoid necrosis may be present at the centres of a few, proceeding either to resolution or to conversion into hyaline fibrous tissue: Thus the sarcoid granuloma is characterized by epithelioid cells surrounded by lympho­ cytes and fibroblasts but devoid of caseation. Crystalline or calcified inclusion bodies are sometimes seen, e.g. Schau­ mann bodies. However, the histological picture is not diagnostic and similar granulomas can occur in a number of other conditions including berylliosis and fungal disease.

Frequency of clinical involvement of

extrapulmonary organs in sarcoidosis.

Extrathoracic sites

i I�

Histology

1647

"'''_''''_.' .

Peripheral nodes

73

Skin

32

Liver

21

Eye

21

Spleen

18

Bone

14

Salivary glands

6

Joints

6

Heart

5

Nervous system

5

Kidneys

4

Nose and mouth

3

Lacrimal glands

3

Skeletal muscle Larynx Stomach and intestine Uterus

Clinical features Sarcoidosis is a multisystem disease primarily affecting the lower respiratory tract but which may involve the upper respiratory tract, often more frequently than previously realized.12 The extent to which this occurs varies from report to report (Tables 130.3 and 130.4). Ninety percent of patients with sarcoidosis will have evidence of thoracic involvement either within the lung itself or affecting intrathoracic lymph nodes and classi­ cally the disease has been staged dependent on the extent of this involvement. Table 130.3

Incidence

(0/0)

of symptoms in patients with nasal

sarcoidosis,

Nasal stuffiness and obstruction

88

Crusting

63

Blood-sta'lned discharge

37

Purulent discharge

30

Facial pain

22

Mucoid discharge

15

Anosmia Revised from Ref. 7. with permission.

4

Figure 130. 1

Sarcoidosis. Typical appearances of nasal lupus

pernio,

-

.___.__

.1

____

______ _

1648 I

PART 13 THE NOSE AND PARANASAL SINUSES

The anterior nasal septum may perforate, particularly if traumatized, for example, by surgery which may lead to collapse of the nasal bridge. The nasal bones may be involved by a process similar to the dactylitis seen in the fingers and patients may present with a soft tissue mass or expansion of the nasal bridge associated with thickening and discolouration of t he overlying skin as in classic lupus pernio (Figures 130.1, 130.2 and 130.3). This may respond dramatically to medical treatment. 13 The para­ nasal sinuses may also be affected by the process andlor become secondarily infected. As a consequence, facial pain is experienced by one in five patients with nasal sarcoid and the sense of smell may be affected due to

Figure 130.2

Sarcoidosis. Typical appearances of lupus pernio

on the cervical skin.

mechanical obstruction of the olfactory cleft by crusting or fibrosis or as a result of direct neuropathy. In addition to nasal manifestations, lymphoid hyper­ plasia and adenoidal enlargement can lead to otitis media with effusion and sleep disturbance may result from involvement of the tonsils, soft palate, naso- and oropharynx. The soft tissue of the supraglottic larynx may become thickened and increasingly swollen produ­ cing dyspnoea and change in voice quality.

Diagnosis Unfortunately, no one test is pathopneumonic for sarcoidosis. Diagnosis usually relies on a combination of histology, imaging, haematology and clinical acumen. The most reliable test, the Kveim, has regrettably been withdrawn in the UK over fears of virus and prion transfer though without any substantive evidence for this.14 The angiotensin converting enzyme (ACE) is often elevated sometime during the course of the disease ( rai sed in 83 percent of patients with active sarcoid), IS but it is also elevated in a number of other conditions such as tuberculosis, leprosy and primary biliary cirrhosis. The erythrocyte sedimentation rate, serum globulin and serum and urinary calcium levels are sometimes elevated and a full blood count may show mild anaemia, leucopenia, thrombocytopenia and eosinophilia, but clearly none of these tests are specific. Investigation of the lower respiratory tract including imaging (chest x-ray, computerized tomography (CT)) (Figure 130.4), perfusion studies, bronchoalveolar lavage and gallium-67 scanning, are generally performed com­ bined with biopsy of potentially affected tissue such as the lung, skin and lymph nodes.16 Biopsy of nasal mucosa is only of use if it appears abnormal as random biopsy of macroscopically normal mucosa is usually negative (in 92 percent of cases).7 Plain x-rays of the nasal bones may show rarefraction or punctate osteolysis in up to a quarter of cases17 and this, together with soft tissue changes, may be shown by CT scanning (Figure 130.5). This will also demonstrate secondary involvement of the sinuses though will not distinguish between active disease and secondary infection . Magnetic resonance imaging (MRI) of the brain is increasingly undertaken to establish central nervous system (CNS) involvement.

Treatment

Figure 130.3

Sarcoidosis. Coronal section through nasal septal

cartilage showi ng an erosion by 9 ranu lomatous lesions.

Some patients with limited disease undergo spontaneous remission without specific treatment, though the maj ority are offered some form of systemic therapy. This consists of a combination of oral steroids, methtotrexate and hydroxychloroquine, depending on the severity of the disease. Topical treatment may be offered for nasal

----

I 1649

Chapter 130 Granulomatous conditions of the nose

though endoscopic sinus surgery can be undertaken for secondary bacterial infection with success in selected

cases.18

[**]

WEGENER'S GRANULOMATOSIS

Definition A condition, characterized by granulomatous inflamma­ tion

involving

the

tract

respiratory

and

necrotizing

vasculitis affecting small- to medium-sized vessels (e.g. capillaries, venules, arterioles and arteries) with necrotiz­ ing

glomerulonephritis,

common. 19 Although the 20 after his article on

is

condition bears Wegener's name

rhinogenic granulomatosis in 1939, it should really be attributed to Klinger. 21 The pathological hallmark is the coexistence of vasculitis and granulomas and classically involves

a triad of

airway,

lung

re nal

and

disease.

However, it is i ncreas in gly recognized that limited forms

of the condition can occur.22. 23 Thus,

the true

in stan ce of

the condition is difficult to determine. Whilst up to 1967 only 138 une q uivocal cases had been recorded

in the

literature,24 clearly this was a gross underestimate of the true incidence which is now becoming apparent largely Figure 130.4

Posterior-anterior chest x-ray showi ng typical

pulmonary infiltrates in systemic sarcoidosis.

due to the introduction of the antineutrophil cytoplasmic 25 antibody test (ANCA).

Age and sex The age of presentation in our own cohort of over 90 p atients has ran ged from 15 to 73 years, although it has been reported in children. Halstead's series26 reports significant numbers of patients under 25 years of age and these young patients were usually noted to present with a generalized form.

Aetiology The

aetiology

of

Wegener's

granulomatosis

remains

unknown. Its inflammatory nature and its resemblance to polyarteritis nodosa suggests that it represents some form Figure 130.5

Coronal CT showing sclerosis and osteolysis of

the nasal bones associated with a soft tissue mass in nasal sarcoid.

hypersensitivity

reaction

with

that this

an

immune

been postulated

may be related to inhaled bacteria, which would

explain the frequency with which the respiratory tract is involve d. The deposition of the immune complexes is

symptoms including intranasal steroids, either sprays or drops, glucose and glycerine drops and various forms of nasal douching and irrigation. Nasal symptoms may also be -helped by the systemic medication. Surgery generally is contraindicated for both cosmetic or functional indica­ tions,

of

response to an unknown stimulus. It has

particularly

in the presence of active

disease,

thought to

be resp ons ibl e for vasculitis

in other condi­

tions, but these have only been demonstrated in a

small

proportion of patients with Wegener's granulomatosis. McDonald and De Remee27 studied biopsies subjected to

immunofluorescence microscopy, but rarely found de­ posits

of immunoglublin

or

complement.

aetiological and diagnostic point of view,

From the

an

most

_ __. _ _ 1 ____ ._---=----

1650 I PART

13 THE NOSE AND PARANASAL SINUSES

important finding of recent years was that reported by van der Woude in 198528 who found antibodies reacting with the cytoplasm of ethanol-fixed granulocytes and monocytes in 25 patients with Wegener's granulomatosis. It then became clear that two main forms of ANCA could be found: perinuclear or pANCA and cytoplasmic or cANCA. In general, patients with Wegener's granuloma­ tosis have cANCA, whereas pANCA (and occasionally also cANCA) may be found in some other conditions,

PULMONARY SYMPTOMS The majority of patients with active disease experience pulmonary symptoms at some point, cough, haemoptysis or pleuritic pain. The pulmonary lesions often cavitate and can be seen radiologically in most patients. These may be the presenting symptoms and the diagnosis may be aided by flexible bronchoscopy and lung biopsy. Encapsulated lung abscess formation can occur.

such as microscopic polyartertitis. The tests may be done by indirect immunofluorescence or radioimmunoassay 9 30 and titres correlate well with disease activity.2 ,

RENAL SYMPTOMS Between 30 and 90 percent of patients will develop renal symptoms, although the organs may be spared in more limited forms of the disease. Both casts and red cells appear

Clinical features

in the urine and early treatment is vital since damage is

Although the condition is characterized by necrotizing granulomas with vasculitis

of the

upper and lower

respiratory tracts and in the focal necrotizing glomer­ ulonephritis, any part of the body may be affected and in the active cases this involvement may be widespread at the onset. The frequency with which different sites

are

irreversible. Microscopic evaluation of a midstream speci­ men of urine remains a simple but important test in the diagnosis and management of Wegener's granulomatosis. Renal biopsy is not essential but it must be remembered that Wegener's is one of a number of diseases which produces segmental or diffuse glomerulonephritis.

involved, both at presentation and subsequently, varies with the interest and specialty of the reporting physi31 Clans. As 1ate as the early 1970s, Wegener's granuloma. tosis remained a serious and lethal disease with patients frequently dying within a six- to eight-month period. Rapid diagnosis remains of great importance since a fulminating course with a fatal outcome can occur in as little as 48 hours. Duration of symptoms before a diagnosis is made remains highly variable and can be more than a year in some cases or even longer. The possibility of latent, incomplete and limited forms of the disease, as well as the classic 'full blown' condition is now recognized, though it is unknown whether all limited forms eventually progress and what triggers this trans­ formation. However, it is apparent that most patients

OCULAR SYMPTOMS The most common ocular manifestations of Wegener's granulomatosis are conjunctivitis, dacrocystocystitis, epi­ scleritis and corneal ulceration, but optic neuritis and retinal artery occlusion can also occur. Approximately 20 percent of patients may experience proptosis from a granulomatous mass' within the orbit or extending from the adjacent sinuses

(Figure 130.6). Delayed or inadequate

treatment is responsible for failure to control many orbital systems and loss of vision has been frequently documen­ ted. Blindness, unilateral or bilateral, is an important cause of morbidity in Wegener's patients, inadequate ,and intermittent systemic therapy predispose this problem.

start with relatively minor ENT symptoms, which may be overlooked by both the patient and the doctor. These often

include

a

variable

degree

of

epistaxis,

nasal

obstruction and bloody crusts in the nose. Destruction of the intranasal structures including the septum may follow, leading eventually to nasal collapse. Minor nasal surgery and/or repeated biopsies during this period may add to the problem. In addition, many patients may 3 complain of significant facial pain. 2 Patients frequently complain of progressive malaise, pyrexia,

weight

loss

disproportionate to

and the

notably

clinical

feel

very

findings.

unwell,

This

often

results in a delay in diagnosis. The nose and sinuses are involved in more than 80 percent of patients, though it is important to point out that while there is intranasal destruction of bone and cartilage in some patients with Wegener's leading to septal perforation and a character­ istic nasal collapse, there are none of the gross destructive changes of the midfacial skin seen in T-cell lymphomas and basal cell carcinomas.

ORAL SYMPTOMS A wide variety of oral lesions have been described, the most commonest being a hyperplastic granular lesion of the gingiva beginning in the area of the interdental papillae. If a tooth is lost, the socket may fail to heal. Extensive ulcerative stomatitis has also been reported.

OTOLOGIC SYMPTOMS Approximately one-third of patients may develop acute otitis media or otitis media with effusion. There may be deafness, pain and suppuration. Facial nerve paralysis has also been recorded. Both conductive and a sensorineural hearing loss occur and while otitis media with effusion may be secondary to inflammatory changes within the nasal cavity, Wegener's granulomatosis of the temporal bone

with necrotizing

granulation

tissue

tympanic cavity has been documented.

filling the

Chapter changes

130 G ranulomatous conditions of the nose •

and

are

contraindicated

inflammation and scarring.

leading

to

Localized disease

1651

further with a

positive c-ANCA test has been well documented33 and requires ruling out, particularly in an adult presenting with an apparently idiopathic subglottic stenosis. Ex­ tensive laser resection of subglottic stenosis caused by Wegener's is frequently not only unsuccessful, but aJso causes further stenosis. Gentle dilation, occasional soft internal

stenting and

localized

steroid

injection

are

advised?4

OTHER SYMPTOMS The generaJized vasculitis may produce ulceration of the skin, particularly of the distal arms and legs. Polymyalgia and polyarthritis have been described. Direct nervous

of patients in latter is caused by granulomatous invasion of neuraJ tissues, intracerebral or meningeal granulomas, as well as neuritis vasculitis. Any cranial

system involvement occurs in 10-15 percent some series.

nerve

may

This

be

involved.

The

ability

of

Wegener's

granulomatosis to affect any organ in the body makes it a possible diagnosis in a wide range of obscure and bizarre clinical presentations.

Diagnosis The cANCA test is positive in 95 percent of patients with generalized active disease. This sensitivity falls to 60 percent with localized disease affecting the respiratory tract. Thus, in addition to the cANCA, a full blood count, erythrocyte sedimentation rate (ESR), c-reactive protein

(CRP) level, serum urea and creatinine, as well as any other test relevant to the differential diagnosis, e.g. serum angiotensin converting enzyme (SACE) should be re­ quested. Urine analysis for casts and red blood cells together with renal clearance studies, chest x-ray and other respiratory function tests should be performed. Tissue biopsy may be helpful, bu t in isolation is rarely diagnostic

and

has

been

superseded

by

the

above

investigations. When biopsing the nose, it is important to obtain representative tissue ideally from the septum and turbinates. The main histological features, though not pathopneumonic, are as follows:

Figure 130.6

(a,b) Axial CT scans showing sclerosis and

opacification of the nasal cavity associated with widespread

•

vasculitis is mandatory for the diagnosis and

•

granulomas are of the epithelial cell type being may show fibrinoid necrosis, but can also be non-necrotic; large, irregular and lined with histocytes. They

infiltration of the orbit in Wegener's g ranulomatosis. •

LARYNGEAL AND TRACHEAL SYMPTOMS LaryngeaJ involvement is unusual, but when present the subgl ottis and upper trachea are most commonly affected.

This may present a serious problem with laryngotracheal obstruction. Biopsies frequently show only nonspecific

fibrinoid vascular necrosis is a common finding; the

multinucleated giant cells are often present and eosinophils are numerous.

In those patients with a negative c-ANCA and biopsy, in whom the r e is clinical suspicion of Wegener's, the c­ ANCA should be repeated and patients kept under regular review.35

.f�_,- ,

1652 I PART Table tology

13 THE NOSE AND PARANASAL SINUSES

130.5 shows the American College of

criteria

for

the

class ification

of

Rheuma­

Wegener's

gr anulomatosi s.

with exacerbation of disease in some individuals. None­

theless, regular review and monitoring of haematologlcal,

pulmonary and renal indices is vital [**] Unfortunately, despite many reports of good

initial

control of Wegener's gran ulomatos is using a combination

Imaging

of

prednisolone

azathiprine, the

Computerized tomography a nd MRl demonst rate muco­

and

either

cyclophosphamide

or

majori ty of long-term studies emphasize

the cont inuin g problems faced by these patients. Harri­

(Figure 130.7).37 E ighty- six percent showed

son's experience during 27 years at the Royal Natjonal Throat Nose and Ear Hospital 40 showed the varied course

nonspecific mucosal thickening in the nasal cavity or

which this disease can take. Prednisolone (60-80 mg/day)

destruction and 50 percent new bone for mation in the

rog/day) should produce a dramatic improvement in

the sinus cavities. In addition, the or bit was

acute disease. The ESR may fall slowly and gradual

sal thickening and superadded infection in a series of 28 patients

paranas al sinuses, 75 percent showed evidence of bone

walls of

affected in 30 percent of these pa tients. Whilst

the

with cyclophosphamide (2 mg/kg) or azathioprine (200

reduction of both dru gs has to be based on clinic a l and

d i agnosis of sys temic Wegener's granu lomatosis is made

Labor a tory assessment. It is not possible to generalize, but

cl ini ca lly,

with the exception of

in

a patient without a history of previous

sino nasal surgery the combination of bone destruct ion

the fulminating, progressive cases,

initial control is now the norm . In Harrison and Lund's

and new bone forma tion on CT is virtually diagnostic of Wegener's, especi a lly when accompanied on MRI by a fat signal from the sclerotic sinus wall. These changes may be importan t di a gnostically in localized sinonasal Wegener's, where the clinical diagnosis may be uncertain and the cANCA test can be negative. Imaging of the chest may show progres sive cavitation followed by fibrosis.

Treatment The report by Fahey et

al.38 in

1954, suggesting that steroids

and a variety of cytotoxic drugs could improve short-term prognosis, has subsequent ly resulted in

over 90 percent of

patients with Wegener's granulomatosis obtaining pro­ longed remission. Renal damage prior to commencing treatment is the major prognostic factor and therapy should be begun as soon as the diagnosis is suspected rather than waiting for histologica l verification. The ESR, CRP

all been used to monitor clinical it was hoped that the demonstration of a rising titre of cANCA might be used to predict and pre­ em pt imminent relap se. Unfortunately, this has not proved as useful as hoped. 39 The cANCA frequently becomes negative when the disease is under cont rol, but this is not

Wegener's in the nose with septal destruction hyperostosis and

always the case and conversely may remain negative even

opacification of the sinuses together with orbital infiltration.

a nd c-ANCA test have

progress and

Table

130.5

Figure

130.7

Coronal CT showing typical appearances of

1990 ACR criteria for the classification of Wegener's granulomatosis (number of criteria present rule).a

Cr-iteria·

Definition

'�

"

"

>�

,� ,�'

'��.:.:- " ' : ., ..�: ';'!L';; ;:;� "t��. ,....r

)

" ,

Nasal or oral inflammation

Development of painful or painless oral ulcers or purulent or bloody nasal discharge

Abnormal chest x-ray

Roentgenogram of the chest showing the presence of nodules, fixed infiltrates or cavities

.�:

Urinary sediment

Microhematuria (over five red blood cells/HPF) or red cell casts in the urine sediment

Granulomatous inflammation on

Histologic changes showing granulomatous inflammation within the wall of an artery or in the peri­

biopsy

or extravascular area (artery or arteriole)

aFor classification purposes, a patient shall be said to have Wegener's granulomatosis if he/she. has satisfied any two or more of thes e four criteria. This rule is associ ated with a sensitivity of 88.2 percent and a specifity of 92.0 percent. After Leavi tt et 01.16

Chapter 130 Granulomatous conditions of the nose

I 1653

100

Finally, the nasal symptoms can be managed by topical

percent immediate control in 48 cases and, over a 20-

preparations including an intranasal steroid, glucose and

series,

azathioprine with prednisolone produced

year follow up in some patients, no patients have had

glycerine drops and alkaline/saline douching and irrigation.

significant complications such as alopecia, leucopenia or

It has been recommended that surgeons should not

iatrogenic haemorrhagic cystitis. In contrast, a major

undertake augmentation rhinoplasty and/or closure of the

complication of cyclophosphamide is leucopenia, and

septal perforation unless the disease process has been

alopecia may occur even with low dosage. Approximately

quiescent for some years. A series of 12 out of 13 patients in

40 percent of patients taking this drug may have some

remission from Wegener's ultimately had a successful result

bladder bleeding which is a considerable disadvantage in

from dorsal augmentation without reactivation of disease.44

patient management when the urine is being assessed for the possibility of red cells. In view of the unavoidable side effects of continued steroid and cytotoxic treatment, there is an obvious desire by both the patient and the doctor to reduce or stop treatment as soon as possible. However, there is no doubt that this is a cyclical disease and clinical flare up may result in

considerable tissue reaction,

damage and subsequent fibrosis. These are best avoided. Outstanding examples of this are the fibrosis which in the orbit can result in painful proptosis, visual defects or blindness, and in the lungs with increasing pulmonary fibrosis with consequent severe dyspnoea. Although both ESR and ANCA tests are of value in monitoring the disease, change can occur suddenly and the patient is often aware of variations in their well-being in the absence of altered haematological tests and,

in our

experience, some are particularly sensitive to changes in dose. They should, therefore, be listened to with care and their medications altered appropriately. In Harrison and Lund's series, there are patients who

CHURG-STRAUSS SYNDROME In 1951, Churg and Strauss45 described a syndrome of systemic vasculitis and asthma. Churg-Strauss syndrome is defined as eosinophil-rich and granulomatous inflam­ mation involving the respiratory tract and necrotizing vaculitis affecting small- to medium-sized vessels and associated with asthma and eosinophilia.46 Histologically, the

lesions

interstitial

show a

necrotizing

granulomas

and

giant

cell

eosinophilic

vasculitis, pulmonary

infiltrates. Patients present with bronchial asthma, nasal polyps and eosinophila, combined with systemic vascu­ litis.

There

may

also

be

nasal

crusting

and

septal

perforation, but the condition may be differentiated from Wegener's by

the

other

elements

of

the

syndrome.

Treatment again is primarily with oral steroids and the usual combination of medical and surgical therapy for the nasal polyps as required.

[**]

have, after many years, managed to stop their medica­ tions, but it has been impossible to predict the likelihood of

any

individual

particularly

after

patient

suffering

immunological

late

recurrence,

challenges

such

EOSINOPHILIC GRANULOMA

as

influenza or pregnancy. It requires a delicate balance

Definition

between the intrinisic risks of a hazardous relapse against the

surety

of

drug

toxicity,

and

for

the

moment

permanent follow up remains essential. Whilst cyclophosamide has greater side effects, it is generally used for the more severe disease with renal involvement. It may be used in high-dose intravenous pulsed form in severe disease. Plasma exchange immu­ noglobulin infusion and other drugs, such as methotrex­ ate and cyclosporin, have been or are being used in some centres.

Mycophenolate mofetil is being used as an

alternative to azathioprine by some physicians. Finally, it should be remembered that all patients on long-term

This

is

a

clonal

proliferation

of

Langerhans'

cells

associated with a heterogeneous inflammatory infiltrate of eosinophils, histiocytes, lymphocytes, plasma cells and neutrophils. It is now regarded as a neoplastic condition with a variable clinical course.47,48 It is also considered a manifestation of histiocytosis-X and is sometimes referred to as Langerhans' cell histiocystosis and Langerhans' granulomatosis.

Clinical features

oral steroids should undergo bone densitometry and be offered one of the bisphosphonates, such as alendronate sodium, if there is any evidence of bone 10SS.41 Once remission has commenced, medications may be

Whilst all organs may be affected, eosinophilic granuloma predominantly occurs in bones. The skull is a common site of involvement, in particular the temporal, frontal

reduced but it is important not to do this too precipitately

and parietal bones. A wide age range from infancy to over

or an acute exacerbation may occur. After many years of

80 years has been recorded, but about 85 percent of cases

oral steroids, patients may require a small maintenance

are detected in the first three decades of life and 60

dose of 5-10 mg of predinsolone per day to compensate

percent are young children. Males are affected twice as

for. irreversible adrenal suppression. The possible role of

frequently as females. The usual presentation is a painful

infection in the activation of disease has led to the use of

swelling of the involved bone, often for many months

cotrimoxazole in some milder cases.42, 43

associated with cervical lymphadenopathy. Mandibular

1654 I PART 13

THE NOSE AND PARANASAL SINUSES

lesions produce toothache, gum ulceration and loose

otherwise referred to as 'giant cell reparative granuloma' or

teeth, whereas involvement of the temporal bone may

'giant cell reaction of bone'. It was first described by Jaffe

simulate acute mastoiditis.

in

195351 in the jaws, though other craniofacial sites have

been reported. These lesions commonly occur in children and young adults and are benign despite the presence of

I maging

mitoses which may cause the inexperienced pathologist

Radiological evaluation shows punched out bony lesions and in the jaws radiolucent areas around the teeth. Lesions in the skull often show bevelled margins due to angulated destruction of the cortical bone.

difficulties. The giant cells do not contain multiple nuclei as

in true

giant

cell tumours.

Bilateral symmetrical

involvement of the jaws by giant cell granulomas is seen

in cherubism, a rare inherited childhood condition (Figure 130.8).52.53 True giant cell tumours involving the craniofacial bones are quite rare especially in children

Histology Macroscopically the lesions are soft and yellow or red brown in colour and biopsy material is best obtained by curettage.

Microscopically,

the

Langerhans'

cells

are

mixed with other inflammatory cells including histocytes, eosinophils, plasma cells and neutrophils. The cytoplasm of

the

Langerhans'

Charcot-Leyden

cells

crystals

may

be

may be

eosinophilic

found

in

and

ordinary

histiocytes. There is intense osteoclastic activity at the periphery of the granuloma due to prostanglandin and cytokine production by the Langerhans' cells.49 During the healing phase of the granuloma, the stroma becomes increasingly fibrotic. Prognosis has been associated with increasing numbers of eosinophils.

Treatment Treatment depends on whether or not the eosinophilic granuloma is localized and/or solitary. The ratio of solitary to polyostotic disease is probably > 5: 1 and is sometimes termed 'type II disease'. In addition, some have been noted to spontaneously regress and disappear over time. With unifocal disease the combination of curettage/excision and radiotherapy is usually curative provided that no new lesions develop within approximately one year. However a proportion of the patients will develop a generalized disease with hepatosplengomegaly, lymphadenopathy, skin lesions and further osseous lesions, so called 'type I disease'. The course of this can be rapid with a poor prognosis and additional chemotherapy has been advocated in these circumstances. At present the most effective chemotherapy regime appears to be etoposide and steroids given for periods

of

12 months or more dependent on the

response.50 Recently, alpha interferon and bone marrow transplantation have also been used successfully.

GIANT CELL GRANULOMA Definition Benign granuloma-like aggregates of giant cells in a fibrovascular stroma characterize this benign condition

Figure 13q.8 (a,b) Coronal CT scans of upper and lower jaw showing typical appearances of cherubism.

Cha pter 130 Granulomatous conditions of the nose

and

young

microscopic

adults.

To

appearance

add of

to

giant

the

confusion,

cell

the

granulomas

is

I 1655

duration of symptoms of three years. The lesion may affect the maxilla or frontal sinuses producing expansion

indistinguishable from that of solid variant of aneurysmal

of the bone, cosmetic deformity and displacement of

bone cyst and must also be distinguished from the giant

adjacent structures, such as the globe.

cell lesions associated with hyperparathyroidism.

Imaging

Clinical features Pain and swelling over the affected bone most commonly occurs though diplopia, while frontal headache, hearing loss, vertigo and tinnitus have also been reported. Most patients are under 20 years of age and there is a female to

The lesion produces a cyst-like expansion of the bone andlor sinus which is opaque on CT and does not . enhance with contrast. A very high signal is produced on all MRI sequences.

male preponderance of 2: 1. The maxilla and mandible are most commonly affected followed by the sphenoid and tempora1 bones. 51 ' 5 4

Histology

Imaging

ing

It has a typical appearance of granulation tissue contain­ foreign

body-type giant

cells surrounding

clefts

created by the cholesterol crystals.

The lesions are expansile and lytic with a 'soap bubble' centre and well-demarcated edges.

Treatment Histology

Surgical excision by whatever route facilitates complete

Tissue is unremarkable macroscopically. Microscopically, the granuloma has a cellular fibroblastic stroma contain­

removal of the granulation tissue to prevent 'recur­ rence,.5 5 ,5 6

[**]

ing aggregates of giant cells. These are smaller and have correspondingly fewer nuclei than in a true giant cell tumour.

Biochemical

investigations

(serum

calcium

GRANULOMATOUS NEOPLASIA

phosphate and alkaline phosphase) should be undertaken to

distinguish

it

from

the

brown

tumour

of

hyperparathyroidism.

Introduction Many terms, most notably 'midline destructive granulo­ ma' have been used to describe the condition now shown

Treatment

to

Curettage alone is associated with recurrence in

15

percent of cases and excision should be undertaken where possible. 54

[**]

be a TINK

cell

lymphoma. 5 7, 58

This tumour

is

responsible for the classical destruction of the midface and has caused great controversy and pathological debate. It was first described by McBride59 with a subsequent comprehensive account of the clinical and histological features by Stewart.60 In the past, death resulted from intercurrent infection of disseminated lymphoma due to a failure of diagnosis or insufficiently aggressive oncologic

CHOLESTEROL GRANULOMA

treatment. Recently, similar appearances of aggressive midfacial destruction caused by cocaine abuse have been

Definition

reported in which tests for granulomatous diseases and

Granulomatous reaction to cholesterol crystals preCIpI­ tated

in

the

tissues

are

presumed

to

result

lymphoma have been negative.61

from

haemorrhage andlor trauma.

Age and sex Age and sex

TINK cell lymphoma may occur at almost any age from the first to the ninth decade, though the median is fifth or

In 12 personal cases, the age ranged from 26 to 56 years (mean 38 years) with a male preponderance and a mean

sixth decades. A male preponderance has been reported in some series though not others. 40

1656 I

PART 13 THE NOSE AND PARANASAL SINUSES

Clinical features

antigen should be applied as approximately 80 percent

Whilst patients usually present

phenotypes. Histologically, the infiltrates are polymorphic

of the peripheral T-cell lymphomas will show aberrant

with aggressive destruc­

tion of the middle of the face, classically the disease has been divided into three stages. 1. Prodromal. This may last for many years with the

patient complaining of persistent nasal obstruction and rhinorrhoea for which

the patient

may be offered surgery.

2. A period of activity when areas of necrosis develop on and around the nasal cavity associated with purulent discharge, crusting and tissue loss. Progressive destruction of the nasal framework, palate, upper lip extending into the pharynx and

and atypical cells tend to be arranged in a necrotizing angioinfiltrative growth pattern.

Infiltrates consist of

neoplastic atypical T-lymphocytes mixed with plasma celis, small lymphocytes, histiocytes, eosinophils and also some immunoblasts.62 Granulomas and

giant cells are not

present in T-cell lymphomas; thrombosis and necrosis are, however, common findings.

An association with

Epstein-Barr virus has been reported and may be of help in the early differential diagnosis of the condition.63,64

The exact classification of these peripheral T-cell lym­ phomas remains under evaluation.

orbit and skull base occur often associated with pyrexia secondary to infection

(Figures 130.9 and

130.10). 3. The terminal stage. Haemorrhage associated with the gross mutilation of the face and exhaustion lead eventually to death. The process may take a number of years and be associated with systemic

Imaging Dramatic and progressive destruction of the midline soft tissue and bone without a gross tumour mass are typical, though similar to that seen in some cases of Wegener's.

metastases.

T re atment Diagnosis These lesions, previously described as midline granulo­ The nasal T-cell lymphoma still presents a diagnostic

mas, were initially treated with low-dose radiotherapy.65

problem because the atypical cell infiltrates are often

This occasionally produced a dramatic response, but no

dispersed in necrotic areas. Good quality representative

long-term cures.

biopsy material from tissue beneath the slough and crust

Nowadays,

we recommend that all

patients must be treated with radical full course radio­

is essentiaL

Immunohistochemistry using a panel of

therapy of 55 Gy or more with wide

monoclonal

antibodies

including the nose, sinuses and palate. Recurrence or the

Figure 130.9

lINK cell lymphoma affecting the midface with

ea rly ulceration.

against

T-cell

differentiation

Figure 130.J 0

field coverage

lINK cell lymphoma of the midface with

significant central destruction.

.

,

..

�

Ch a pter 130 Gra n u lomato u s co n d i ti o ns of the nose I

development of disseminated

lymphoma worsens the

1657

REFE R E N CES

prognosis and chemotherapy may be advocated in those lesions classified as high grade.66 There are few large clinical series with five - year survival rates ranging from 46

*

1.

Lund VJ . G ra n u l o m a tous d ise a se a nd tu mo u rs o f th e nose and para nasal s i n uses. I n : Ken nedy DW, Bolger WE,

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KEY PO I NTS •

".-t r t

Many pat ients wi t h syste m i c gra n u lo m atous

' .'l= , "

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•

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•

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Jaffe ES, Chan .l K, Su I J . R e port of t h e works h o p on nasa l a n d rel ated extra nod a l a n g i ocentric T/natu ra l ki l l e r ce l l

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lym p h o m as. Defi n itions, d iffe re n ti a l d i a g n osis a n d e p id e m i o l ogy. American Journal of Surgical Pathology. 1 99 6 ; 2 0 : 1 03 - 1 1 . 59.

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o f t h e maxi l l a ry a n d fronta l s i n u ses. Oto-Rhino­ 56.

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I 1659

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of Pathol ogy, 2000, 220.

131 Abnorma Iities of smell RICHARD L DOlY AND STEVEN M BROMLEY

Introduction Anatomy and physiology Olfactory disorder classification Clinical evaluation of smell function Causes of smell disturbance Treatment of smell disorders Key points

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1660 1661 1664 1664 1666 1670 1672

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Best clinical practice Deficiencies in current knowledge and areas for future research Appendix Acknowledgements References

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Many of the papers referred to in this chapter come from the author's own laboratory. Most other references were derived from multiple searches on PubMed and Psychlnfo.

INTRODUCTION The ability to detect environmental chemicals is a primary function of the nose. A proper functioning sense of smell allows a person to discriminate between thousands of largely organic, low molecular mass, volatile compounds and provides information regarding: (1) the safety of a substance or environment (for example, spoiled food, leaking natural gas); (2) the aesthetic properties of everyday objects (for example, rose, dirty laundry); and (3) elements of basic communication (for example, mother/infant interactions). When combined with gustatory and somatosensory stimuli, the sense of smell determines the flavours of foods and beverages, and aids the process of digestion by triggering normal gastrointestinal secretions. Moreover, the olfactory system contributes significantly to a person's quality of life. Among 750 patients presenting to our centre with largely olfactory problems, more than 68 percent experienced altered quality of life, 46 percent described changes in appetite or body weight,

and 56 percent reported influences in daily living or psychological well-being. l Loss of smell can result in significant psychological disruption and even generate feelings of physical and social vulnerability and victimization. 2 The importance of smell is also emphasized by the consequences of its loss in those who depend on it for their livelihood (for example, cooks, homemakers, firefighters, plumbers, wine merchants, chemical plant workers, etc.). Loss of smell can also adversely affect nutrition, especially in the elderly.3 Although otorhinolaryngologists can be the first physicians who patients with smell complaints visit, some patients find themselves making repeated appointments to multiple physicians until their olfactory problem is adequately addressed. Unfortunately, olfactory health is generally disregarded by physicians. This is beside the fact the abnormalities of smell are ubiquitous. Recent estimates suggest that there are at least 2.7 million (1.4 percent) adults in the United States with olfactory dysfunction; presumably similar numbers exist worldwide. 4 Also, most patients who complain of decreased

Chapter 131 Abnormalities of smell.

'taste' function actually have an unrecognized impairment of smell. l Diminished 'taste' is typically due to loss of flavour sensations derived from retronasal stimulation of the olfactory receptors, rather than impairment of tastebud-mediated sensations, per se. 3 Importantly, a patient's lack of olfactory function can be an early sign of a number of serious disease states, including nasopharyngeal carcinoma, Alzheimer's disease, Parkinson's disease, frontal meningiomas, multiple sclerosis (MS) and sinus infections. 5,6 While some patients initially present to their physicians with a frank complaint of smell impairment, others can be completely unaware of their dysfunction, making routine clinical assessments of olfaction imperative. The necessity of the physician to properly evaluate for abnormalities of smell function is also supported in the medicolegal arena, with claims of accidental and iatrogenic smell disturbance often resulting in substantial financial awards. Routine clinical quantitative measurement of smell function can now be easily performed in the office setting, allowing a physician to: (l) validate and characterize a patient's olfactory complaint; (2) identify patients who might be malingering; (3) quantify and document known presurgical smell impairment; and (4) longitudinally follow the course of smell function in the midst of a therapeutic intervention or during recovery from previous loss. This chapter summarizes important aspects of olfactory anatomy and physiology, describes the common olfactory disorders encountered in clinical practice, and provides current practical techniques for the evaluation and management of smell disturbance.

ANATOMY AND PHYSIOLOGY The nose: Structure in relation to smell The human nose contains two complex, independent nasal passages which are dynamic conduits that sub serve both respiration and olfaction. The nasal cavities not only warm and humidify inspired air, bu t they help to eliminate most airborne pathogens and environmental pollutants - a number of which can be toxic to the olfactory system? The olfactory neuroepithelium exists within a small region of nasal mucosa (said to be approximately 2 cm 2) in the upper recesses of the nasal chambers lining the cribriform plate and sectors of the superior turbinate, middle turbinate and septum (Figure 131.1).8 This specialized epithelium is situated behind the approximately I-mm wide olfactory cleft, making it difficult to observe even with modern endoscopic devices. 9 While most of the airstream coming into the nose is shunted through the passages around the inferior and medial turbinates and along the septal wall, only 10-15 percent of the air reaches the olfactory

Figure 131.1

1661

CT scan of paranasal sinuses and associated

nasal structures. The asterisk is within the right maxillary sinus, below the right eye. The inferior portion of the middle turbinate is indicated by the white arrowhead and the inferior turbinate by the circle. Note the attachment of the middle turbinate to the cribriform above. A short white arrow is in the left anterior ethmoid sinus and points to the anterior ethmoidal neurovascular bundle as it emerges from the left orbit and courses along the roof of the ethmoid. The central small open arrow is located in the anterior cranial fossa directly above the bony crista gaili. The long thin arrow situated with the olfactory cleft points to the cribriform plate. The five-pointed star in the right olfactory fossa is adjacent to the vertical lamella of the cribriform plate. Repri nted from Ref. 8, Copyrig ht

©

1995 by

Marcel Dekker. Inc.

neuroepithelium. lO Thus, minor changes in nasal architecture and airflow can result in substantial airflow blockage to olfactory regions without much impairment in nasal respiratory ability.7,11 For example, relatively small polyps located in the superior meatus can deflect incoming air currents away from the olfactory cleft, resulting in smell impairment.l1 On the other hand, too patent of an airway, as seen with excessive turbinate removal, can improperly shunt air away from the olfactory cleft, resulting in a decrement in olfactory acuity.12 It is important to remember that while chronic rhinosinusitis can lead to swelling of the mucosa and a mechanical conductive block, there is evidence for local inflammatory toxicity to the olfactory neuroepithelium that may not be alleviated with surgical intervention. 13 A significant amount of retronasal airflow from the nasopharynx occurs during swallowing and deglutition. This retronasal route is vital to the production of flavour from swallowed foods - adding smell to both taste and touch. The human nasal passage is intermittently affected, at least in some individuals, by the 'nasal cycle'. This cycle, an autonomic ultradian rhythm of periodic

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1662 I PART 13 THE NOSE AND PARANASAL SINUSES alternating side-to-side nasal turbinate engorgement and disengorgement, is said to occur in 80 percent of the normal population with a cycle frequency ranging from 40 minutes to four hours. 7 The nasal cycle itself, which is less frequent in the very young and the very old, does not appear to affect overall olfactory sensitivity in the normal nose; however, intermittent engorgement in the context of a pre-existing structural distortion can result in a noticeable, albeit transient, blockage of a nasal passage (for example, when turbinate adhesions are present).

Four neural systems within the nose Most land mammals have four specialized neural systems within the left and right sides of the nose: (1) the main olfactory system (cranial nerve I or eN 1); (2) the accessory olfactory system (i.e. the vomeronasal system); (3) the trigeminal somatosensory system (eN V); and (4) the nervus terminalis or terminal nerve (eN 0).14, IS While eN I mediates common odour sensations (for example, vanilla, rose and chocolate), eN V mediates both chemical and nonchemical stimuli in the form of somatosensory sensations (for example, irritation, burning, cooling, tickling, touch). eN V is also responsible for inducing reflexive responses, such as secretions of mucus and halting of inhalation, that help to prevent or minimize chemically or thermally induced damage to the linings of the nose and lungs. The vomeronasal system is nonfunctional in humans, while a rudimentary vomeronasal tube is present on each side of the septum with an opening into the human nose, it has no centrally projecting nerve and humans do not possess an accessory olfactory bulb, the target of such a nerve. eN 0 was discovered after the other cranial nerves had been named and consists of a loose plexus of ganglionated nerves that, in most mammals, is in close proximity to the vomeronasal organ and nerve. It has been suggested by some that eN 0 may be a vestige of an ancient nerve whose function was lost or superseded by other parts of the nervous system, although this argument is weak, given that it is so well conserved among a wide range of vertebrates, including humans.

Olfactory neuroepithelium and neural transduction Before neural transduction can begin, odourants must: 1. enter the nose during either active (sniffing) or

passive (diffusion) processes; 2. pass through the olfactory cleft; 3. move from the air phase into the largely aqueous phase of the olfactory mucus.

Mucus is important in that it ensures a moist and protective environment for the olfactory neuroepithelium, and aids in dispersing odourants to the olfactory receptors. From the mucus, odourous chemicals either diffuse or are transported by specialized proteins (termed 'odourant-binding proteins') to the receptors. The olfactory neuroepithelium has an ultrastructure of variable uniformity and, contrary to many textbooks, cannot be discerned reliably from the surrounding respiratory epithelium by the naked eye. It is a pseudo stratified columnar epithelium, supported by a highly vascularized lamina propria. Throughout life, islands of respiratory-like epithelial metaplasia appear within the epithelium, presumably as a result of cumulative viral, bacterial and other insults. 14 In the adult, at least six distinct classes of cells - defined morphologically, biochemically and functionally - can be identified within the neuroepithelium (Figure 131.2): (1) the bipolar sensory receptor neuron is derived embryologically from the olfactory placode, is of central nervous system (eNS) origin and extends odourant receptor-containing cilia into the mucus; (2) the supporting or sustentacular cell insulates the bipolar receptor cells from one another, regulates some elements of mucus production and may aid in the degradation of odourants; (3) the duct cell of Bowman's glands secretes most of the mucus within the olfactory receptor region; (4) the poorly understood microvillar cell, located at the surface of the epithelium, sends tufts of microvilli into the nasal mucus; (5) the horizontal (dark) basal cells, one of two main classes of stem cells within the basement membrane of the epithelium, and (6) the globose (light) basal cells, a multipotent basal cell that can give rise to neurons and nonneuronal cells, including the horizontal basal cells. 14 The number of olfactory receptor cells exceeds that of any other sensory system except vision; collectively, the surface area of the cilia is quite large - exceeding 20 cm 2 in the human. IS Seven domain transmembrane olfactory receptors, which reflect the expression of the largest known vertebrate gene family (on the order of 1000 genes or pseudogenes, accounting for approximately 1 percent of all expressed genes), are found on the 10-30 cilia that project into the nasal mucus from each of these cells. 16 Approximately 6 million receptor cell axons ultimately coalesce into 30-50 fascicles, termed the olfactory fila, which traverse the cribriform plate and pia matter to synapse with second-order neurons within the glomeruli of the olfactory bulb. The olfactory receptor neurons primarily use the neurotransmitter glutamate to excite olfactory bulb (OB) neurons, while dopamine appears to be a necessary modulator of olfactory nerve input. 17 The process of actually transforming the chemical energy of receptor binding into a neural signal- signal transduction - requires a. complex cascade of events, some of which involves activation of G proteins (for example, that of an

Chapter 131 Abnormalities of smell

I 1663

The olfactory bulb and central projections

Low-power electron micrograph (x 670) of a longitudinal section through a biopsy specimen of human olfactory mucosa taken from the nasal septum. Four cell types are indicated: ciliated olfactory receptors (e), microvillar cells (m), supporting cells (s) and basal cells [b). The arrows point to ciliated olfactory knobs of the bipolar receptor cells. d, degenerating cells; bs, base of the supporting cells; Ip, lamina propria; n, nerve bundle; bg, Bowman's gland. Photo courtesy of David T Moran.

Figure 131.2

olfactory-specific subtype, Golf) and various secondmessenger enzymes. lS , 19 It is important to recognize that the olfactory nerve cells, as well as the proximal extraneural spaces, can serve as conduits for the movement of viruses and exogenous agents from the nasal cavity into the brain. This was reCOgnized many years ago as a major route of polioviruses into the brain, leading to programmes to cauterize the olfactory epithelium of school children with zinc sulphate in Toronto and other major cities to avert contracting polio during epidemics. This 'olfactory vector' route has been proposed as a potential explanation for both the olfactory loss and the aetiology of some forms of common neurodegenerative diseases, such as A1zheimer's disease and idiopathic Parkinson's disease,2o although evidence for this hypothesis in these cases remains largely circumstantial.

The olfactory bulbs are complex structures located on the ventral surface of the frontal lobes directly over the cribriform plate. The first synapse of the incoming bipolar olfactory receptor cell neurons occurs within spherical structures making up a distinct layer of the bulb - the glomeruli. A given receptor cell projects to only one glomerulus and any given glomerulus appears to receive most of its input from a restricted region of the epithelium. The main afferent second-order neurons are termed 'mitral' and 'tufted' celis. A considerable amount of convergence of information occurs at the level of the glomeruli. The mitral and tufted celis, in turn, send collaterals that synapse within the periglomerular and external plexiform layers) resulting in 'reverberating' circuits in which negative and positive feedback occur. The apical dendrites of the mitral and tufted cells are influenced by interneurons and centrifugal fibres, most of which are GABAergic or dopaminergic?l It is generally believed that the olfactory system is unique among sensory systems in that information from the sensory receptors is sent directly, and primarily ipsilaterally, into cortical regions without synapsing in the thalamus. However, some cortical projections from primary to secondary (i.e. orbitofrontal) cortex do ultimately relay through the thalamus) and there are some contralateral projections via the anterior commissure. The latter arise largely from pyramidal cells of the anterior olfactory nucleus (AON), a structure whose cells are primarily in the rostral olfactory peduncle. Higherorder brain regions in addition to the AON that are targetted by the mitral and tufted cells of the bulb include (from rostral to caudal): (1) the piriform cortex; (2) the olfactory tubercle; (3) the entorhinal area; (4) the amygdaloid cortex (a region contiguous with the underlying amygdala); and (5) the corticomedial nuclear group of the amygdala. There is a rich supply of centrifugal fibre projections from sectors of the olfactory cortex and other central structures to the olfactory bulb which modify and control olfactory input. 21,22 Structures involved in centrifugal activity include the AON, piriform cortex, lateral entorhinal cortex, regions of the amygdala, raphe nuclei, locus ceruleus and regions of the hypothalamus?2 Third-order projections occur, in a reciprocal fashion, to numerous regions, including the mediodorsal nucleus of the thalamus, the posterior and medial hypothalamus, the hippocampus, and the orbitofrontal cortex, the latter of which also receives second-order projections. Areas of the cortex that result in smell perception when stimulated include the prepiriform and intermediate piriform cortices. Lesions of the olfactory system anterior to the olfactory trigone (including the neuroepithelium, fila) bulb and tract) can result in total lack of smell on the affected side. However, lesions within olfactory structures more posterior to the olfactory trigone do not typically cause comp 1ete 1oss. 22' 23

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1664 I PART 13 THE NOSE AND PARANASAL SINUSES

OLFACTORY DISORDER CLASSI FICATION Olfactory disorders are classified according to standard schemata. It is important to differentiate between a patient's chemosensory complaint and the findings of objective testing, which are not always in congruence. Anosmia refers to an inability to detect qualitative olfactory sensations (i.e. absence of smell function). Partial anosmia defines an ability to perceive some, but not all, odours; hyposmia or microsmia refers to decreased sensitivity to odours. Hyperosmia reflects increased sensitivity to common odours. Dysosmia (sometimes termed cacosmia or parosmia) is distorted or perverted smell perception to odour stimulation. Phantosmia is a dysosmic sensation perceived in the absence of an odour stimulus (also known as olfactory hallucination) and olfactory agnosia refers to an inability to recognize an odour sensation, even though olfactory processing, language and general intellectual functions are essentially intact, as in some stroke patients. Other less commonly used terms include heterosmia - a condition where all odours smell the same; presbyosmia - a decline in smell sense with age and osmophobia - a dislike or fear of certain smells. Presbyosmia is less specific than the other terms noted above (for example, it does not distinguish between anosmia and hyposmia) and is laden, by definition, with the notion that it is age per se that is causing the age-related deficit. Olfactory dysfunction can be either bilateral or unilateral (sometimes termed binasal or uninasal). Bilateral testing usually reflects the better functioning of the two sides of the nose.

CLINICAL EVALUATION OF SMELL FUNCTION History Proper assessment of a patient's smell function requires (1) a detailed clinical history, (2) quantitative olfactory testing and (3) a thorough physical examination emphasizing the head and neck with appropriate brain and rhinosinus imaging. It is important to recognize that patients frequently confuse 'taste problems' with true smell loss, and that many deny any olfactory disturbance until formal testing proves otherwise. Several focussed questions can help establish the nature of the olfactory disturbance. Was olfactory functioning previously normal? Does the patient have a problem with smell, taste or both? What is the timing of onset, duration of impairment and pattern of occurrence? Sudden olfactory loss can be consistent with possible head trauma, ischaemia, infection or a psychiatric condition. Gradual loss may indicate a progressive and obstructive lesion in or around the nasosin us region, particularly if the loss is unilateral. Intermittent loss may suggest an inflammatory process in association with ?-asal and sinus disease. Is the problem

seasonal, suggesting an allergic seasonal rhinitis? Is there a history of precipitating antecedent events, such as head trauma, viral upper respiratory infections, chemical or toxin exposures and nasosinus surgeries? Does the patient have any nasal discharge that is mucous-appearing (for example, allergy), purulent (for example, infection), or clear (cerebrospinal fluid (CSF) rhinorrhoea after traurna)? Does the patient use drugs of abuse, such as intranasal cocaine, ethanol or tobacco? Each of these substances has been associated with some form of olfactory impairment; for example, chronic alcoholism can result in significant impairment in smell discrimination 24 and cigarette smoking results in a loss of olfactory ability that is proportional to the cumulative smoking dose. 25 Cessation of smoking can result in improvement in olfactory function over time. It is essential to determine all medications used by the patient since many reportedly cause smell and taste disturbance. Indeed, smell or taste loss or disturbances are reported in the Physicians' Desk Reference (PDR) as a potential side effect of hundreds of medications. 26 Does the patient have comorbidities that can contribute to smell impairment, such as renal failure, liver disease, hypothyroidism, diabetes or dementia? Delayed puberty in association with anosmia (with or without midline craniofacial abnormalities, deafness and renal anomalies) suggests the possibility of Kallmann's syndrome or some variant thereof. Is there a recent history of epistaxis, discharge (clear, purulent or bloody), facial numbness or weakness, nasal obstruction, allergies, headache or irritation (i.e. signs that may have localizing value)? A positive family history suggests a genetic aetiology. A history of headache suggests sinusitis, migraine or intracranial tumour. Are smells present without an obvious stimulus? A simple partial seizure or aura may not be obvious and other signs of ictal activity should be explored (for example, limb shaking, difficulties with speech, unresponsiveness, automatisms, loss of consciousness and deja vu). Given the strong relationship of Alzheimer's disease and idiopathic Parkinson's disease to smell impairment, one should also look for memory- and parkinsonismrelated complaints in older patients. A physician should also be aware of pending litigation and the possibility of malingering since olfactory loss is a compensable injury.

Physical examination and evaluation Patients complaining of smell disturbance typically require a general assessment of the head and neck and more detailed otolaryngological and neurological examinations. Are there any signs of trauma such as healing wounds, scarring or distorted nasal or skull architecture? Inspection of the nasal passages can begin with a simple nasal forceps examination to view the peripheral nasal cavity for signs of polyps, congestion, deviation of septum or inflammation. However, this method is limited, and

Cha pter 131 Abnormalities of smell •

often nasal endoscopy, employing both flexible and rigid scopes, is needed to ensure thorough assessment of the olfactory meatal area. A deviated septum, per se, does not imply a smell disturbance since the olfactory cleft can remain patent; however, the additional presence of polyps, masses and adhesions of the turbinates to the septum may adequately obstruct airflow. Rarely, foreign bodies can be present, particularly in the context of a psychiatric disturbance. Nasal mucous membranes must be examined for colour, surface texture, swelling, inflammation, exudate, erosion, ulceration, epithelial metaplasia and atrophy. The presence of mucopus above the Eustachian tube orifice suggests posterior ethmoid and/or sphenoid sinus disease. However, mucopus below the Eustachian tube implies involvement of the ostiomeatal complex. A pale mucous membrane suggests allergy, usually as a result of oedema within the lamina propria. Atrophy of the lamina propria is suggested by unusual spaciousness, dryness and crusting, as is seen in atrophic rhinitis. Exposures to environmental or industrial pollutants can result in metaplasia within the epithelium, in addition to swelling, inflammation, exudates, erosion and ulceration. The neurological evaluation should focus on cranial nerve function, with particular attention to the optic nerve (CN II), trigeminal nerve (CN V) and the facial nerve (CN VII) (see next paragraph for CN I). Visual acuity, visual field and optic disc examinations aid in the detection of possible intracranial mass lesions resulting in increased intracranial pressure (papilloedema) and optic atrophy, especially when considering Foster Kennedy syndrome (described below). General sensation over the face should be assessed (for example, cotton, pinprick, temperature). The nasal tickle, performed by using cotton to tickle the inside of one nostril, is also useful since an asymmetric withdrawal response suggests impairment of trigeminal fibres within the nose. The face should be symmetrical and full strength, with intact taste on the

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anterior two-thirds of the tongue bilaterally. Signs of frontal lobe injury and memory impairment may also aid in the diagnosis. Biopsy of the olfactory epithelium is occasionally helpful. However, the interpretation of such biopsies is complicated by sampling issues and the fact that metaplasia of respiratory-like epithelium occurs throughout the olfactory epithelia in persons with no olfactory problems. Some laboratory tests, such as blood serum tests, may also be helpful in evaluating for underlying medical conditions suggested by history and physical examinations, such as infection, nutritional deficiencies (for example vitamin B6, B12), allergy, diabetes mellitus and thyroid, liver and kidney disease. 3 Contrary to many textbooks, there is no proven efficacy of zinc or vitamin A treatments, except in cases where frank deficiencies are lacking.

Quantitative olfactory testing Several standardized and practical psychophysical tests have been developed over the last several years, including a number of brief self-administered tests ranging from the three-item Pocket Smell Test™ to the 40-item University of Pennsylvania Smell Identification Test (UPSIT)?7 The UPSIT is commercially known as the Smell Identification Test™ and is the most widely used olfactory test, having been administered to an estimated 400,000 patients since its development (Figure 131.3).28 The UPSIT can be selfadministered in 10-15 minutes by most patients in the waiting room, and scored in less than a minute by nonmedical personnel. Available in American, British, Chinese, French, Italian, German, Spanish and Japanese versions, this test consists of four booklets containing ten microencapsulated (scratch and sniff') odourants apiece. Test results are in terms of a percentile score of a patient's performance relative to age- and sex-matched controls,

Fig u re 131.3

The four booklets of the

40-odourant University of Pennsylvania Smell Identification Test (UPSIT; commercially known as the Smell Identification Test

rM ).

Each page

contains a microencapsulated odourant that is released by means of a pencil tip. This test. which has been administered to approximately 400,000 patients since its development, is the most widely used olfactory test in the world, with English, french, German, Japanese and Spanish versions available. The UPSIT is considered to be the 'eye chart for the nose'. Photo courtesy of Sensonics. Inc., Haddon Hts., NJ 08035 USA. Copyright

©

2000 by Sensonics, Inc.

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1666 I PART 13 THE NOSE AND PARAI\lASAL SINUSES and olfactory function can be classified on an absolute basis into one of six categories: normosmia, mild microsmia, moderate microsmia, severe microsmia, anosmia and probable malingering. Since chance performance is 10 out of 40, very low UPSIT scores reflect avoidance, and hence recognition, of the correct answer, allowing for determination of malingering. The reliability of this test is very high (test-retest, r=0.94). Although most olfactory problems are bilateral, and bilater81 testing reflects the better functioning side of the nose, in some instances unilateral testing is warranted. 23 To accurately assess olfaction unilaterally, the naris contralateral to the tested side should be occluded without distorting the patent nasal valve region. Such occlusion not only prevents air from entering the olfactory region from the naris (orthonasal stimulation), but prevents active movement of odour-laden air into the occluded side from the rear of the nasopharynx (retronasal stimulation). An easy way of doing this is to seal the contralateral naris using a piece of Microfoam™ tape (3M Corporation, Minneapolis, MN, USA) cut to fit the naris borders. The patient is instructed to sniff the stimulus normally and to exhale through the mouth. While, in most cases, olfactory dysfunction can be adequately characterized by the UPSIT, some physicians employ, alone or in combination with the UPSIT, a smell threshold test. A commonly employed test uses phenyl ethyl alcohol as the odourant and establishes the threshold employing a staircase procedure. 29 Thresholds determined using this procedure are similar in principle to audiometric thresholds using the von Bekesy procedure. Like the UPSIT, threshold testing can be done either bilaterally or unilaterally. The recording of olfactory event-related potentials (OREP) is available in some specialized medical centres as an additional means of assessing the integrity of the olfactory system. Using brain electroencephalography (EEG), the test consists of discerning synchronized brain activity recorded from overall EEG activity following brief presentations of odourants. Unfortunately, OREP testing requires complex, specialized and expensive equipment capable of delivering a well-delineated 'square wave' odourant pulse into the nose within the background of continuously flowing warmed and humidified air. Although OERPs can be useful in some cases in detecting malingering and are generally sensitive to alterations in olfactory function, unlike their visual and auditory counterparts they are unable to discern where in the pathway the anoII).aly exists that is causing the problem.

Imaging studies There are multiple ways of medically imaging patients with smell disturbance. Plain radiographs are rarely useful due to an inability to capture details of the osteomeatal

complex. Computed tomography (CT), on the other hand, has proven to be the most useful and cost-effective technique to assess sinonasal tract inflammatory disorders, and is superior to magnetic resonance imaging (MRI) in the evaluation of the bony structures (for example, ethmoid, cribriform plate, olfactory cleft). In particular, coronal CT scans are useful in evaluating paranasal anatomy. MRI is better to evaluate soft tissue and is the technique of choice to image the olfactory bulbs, tracts and cortical parenchyma. Positron emission tomography (PET), single-proton emission computed tomography (SPECT) and functional MRI (fMRI) presently have limited usefulness outside research institutions.

CAUSES OF SMELL DISTURBANCE There are a number of known aetiologies, many nonmutually exclusive, for olfactory disturbance. These are listed in the appendix to this chapter. The majority of cases of presumably permanent chronic anosmia or hyposmia are due to prior upper respiratory infections, head trauma and nasal and paranasal sinus disease - most causes reflecting permanent damage to the olfactory neuroepithelium. l [****] Despite extensive evaluations, a substantial proportion remains idiopathic. In general, loss of olfactory function can be subdivided into two classes: (1) conductive or transport impairments from obstruction of the nasal passages (for example, chronic nasal inflammation, polyposis, etc.); and (2) sensorineural impairment from damage to the olfactory neuroepithelium, central tracts and connections (for example, viruses, airborne toxins, tumours, seizures, etc.). In some circumstances, it is difficult to classify an olfactory disorder into one of these classes, because of both blockage of airflow to the receptors and damage to the receptors or other elements of the olfactory neuroepithelium, can be present. Chronic rhinosinusitis, for example, can produce damage to the olfactory membrane in addition to blocking airflow, and altered membrane function can, over time, lead to degeneration within the olfactory bulb, a central structure. Although we are currently able to treat many causes of olfactory disturbance due to conductive factors or inflammation of the olfactory epithelium, most olfactory disorders due to sensorineural factors remain untreatable. In most cases, hyperosmia reflects a patient's heightened response to an odour, rather than an increased ability to smell, per se. This problem has been reported in some conditions associated with a change in hormone balance, such as in pregnancy and Addison's disease (adrenal-cortical insufficiency), as well as migraine, drug withdrawal, epilepsy (intericatal period), multiple chemical sensitivity and psychosis. While hyperosmia is relatively rare, dysosmia is more common. Usually

Chapter 131 Abnormalities of smell

dysosmia reflects dynamic alterations of degeneration or regeneration within the olfactory neuroepithelium over time and it is not uncommon for patients who eventually develop anosmia to report having experienced weeks of dysosmia preceding the experience of anosmia. Dysosmia implies an olfactory system that is intact at least to some degree, as total smell loss does not typically accompany most cases of dysosmia. l [***] Severely debilitating, long-lasting and intractable chronic dysosmias have been treated by surgical ablation of regions of the olfactory neuroepithelium, or by surgical removal of a diseased olfactory bulb or bulbs. 30 Olfactory hallucinations or phantosmias can occur from a problem anywhere along the olfactory neural pathways, from the nose to the cortex. Sometimes they are associated with ictal epileptiform activity (for example, simple partial seizures), nasal sinus disease (for example, infection) and head trauma. If someone believes a smell is present (hallucination) and persistently gives this smell personal reference to outside events, despite contradicting evidence, this patient may suffer from olfactory reference syndrome - a depression-related disorder. In some cases, there may be a pathological correlate in the form of right hemispheric lesions. 3l Olfactory agnosia is an extremely rare phenomenon, although is appears to be less commonly investigated than visual and auditory agnosia. It is associated with lesions of the right inferior temporal lobe and is often linked to prosopagnosia (agnosia for familiar faces). The more common disorders or entities associated with olfactory impairment are discussed in detail below, beginning with the more frequent ones.

Upper respiratory infection Upper respiratory viruses, such as those associated with the common cold and influenza, are considered the most common aetiology of permanent olfactory loss in man. Other infectious causes that have been reported include hepatitis, herpes simplex encephalitis, pneumonia and variant Creutzfeldt-Jacob disease. It is not clear what predisposes someone to virus- and bacteria-induced smell dysfunction or the precise mechanisms behind it. Often the smell-affecting respiratory illness is described as being more severe than usual. In a study of olfactory biopsies of four patients with anosmia and 11 patients with hyposmia due to a viral illness, Jafek and colleges 32 noted that patients with anosmia had markedly reduced numbers of receptors and those receptors were abnormal compared with those patients with hyposmia. Given the ability of the olfactory receptor neurons to regenerate, spontaneous recovery of some smell function is theoretically possible over a prolonged period. However, complete recovery is less likely the longer the patient has the loss and is inversely related to the degree of dysfunction. In general, it is necessary to exclude other

I 1667

aetiologies prior to making a diagnosis of post-viral anosmia. Human immunodeficiency virus (HIV)-infected patients are said to show an early impairment in odour thresholds and a later decline in odour identification and discrimination function which appears to parallel a general reduction in cognitive abilities. 33

Head trauma Head trauma often results in smell loss, particularly where rapid acceleration/deceleration of the brain occurs (i.e. coup/contrecoup injury) (Figure 131.4). Blunt trauma to the occiput has been found to produce greater olfactory loss than trauma to the front of the head. 3s Following head trauma, the loss of smell is usually, but not always, immediate. However, it may take a while for the patient to recognize the presence of the dysfunction. Common mechanisms include disruption from shearing forces of the olfactory fila through the sinonasal tract, and direct contusion and ischaemia to the olfactory bulb and frontal and temporal poles. Fracturing of the cribriform plate is not a prerequisite for smell loss. The prevalence of olfactory loss following head trauma is around 15 percent and is proportional to the severity of the injury.3s, 36 [***] As would be expected, such prevalence is much higher in patients referred to specialized smell and taste centres for evaluation and treatment. For example, among 268 patients evaluated at our centre who had experienced head trauma, 66.8 percent had anosmia, 20.5 percent hyposmia and 13 percent had normal smell function. MRI images may reveal damage to the olfactory bulbs, tracts and areas of the temporal and frontal 10bes. 36 Animal research shows that intracranial haemorrhage and ischaemia can lead to degeneration of the olfactory epithelium without transection of the olfactory nerves. 37 [****] Iatrogenic trauma, such as surgery, can cause smell impairment and has been seen with such procedures as . ' 38 SlllUS surgery and cramotomy.

Nasal and sinus disease While the olfactory impairment that follows a viral syndrome or head trauma can be classified as sensorineural, the dysfunction that results from nasal and sinus disease is usually considered conductive - in other words, there is impaired airflow to the olfactory receptors. Theoretically, any inflammatory or obstructive process in the nose can result in a disturbance of smell function, with common examples being allergic rhinitis, rhinosinusitis, nasal polyposis, intranasal tumours and previous nasal surgery. In the evaluation of 240 patients with allergic rhinitis, Rydzewski and colleagues 39 found that 21.4 percent of patients had smell impairment and there was a significant correlation between olfactory thresholds and levels of eosinophils in the blood and nasal discharge.

1668 I

PART 13 THE NOSE AND PARANASAl SINUSES

(bl

Mechanisms of post-traumatic olfactory dysfunction. (a) Injury to the sinonasal tract; (b) tearing of the olfactory fila; (c) cortical contusions and brain hemorrhage. Redrawn from Ref. 34.

Figure 131.4

Treatments in the form of surgery (for example, excision of polyps) or medication (for example, administration of topical or systemic steroids) may improve olfactory function in some cases; however, complete return is not typical. Thus, while chronic rhinosinusitis can result in nasal airflow blockage, there is also a component of direct toxicity to olfactory neurons and impaired ciliary motility resulting in abnormal clearance of mucus. These combined factors make anyone treatment modality limited. In one study, it was found that systemic steroids can temporarily reverse conductive olfactory impairment in 83 percent of patients, while topical steroids helped in only 25 percent of patients - making systemic steroids a useful diagnostic too1. 40 The severity of histopathological changes within the olfactory mucosa of patients with chronic rhinosinusitis is positively related to the magnitude of olfactory loss, as measured by the UPSIT. 4l Biopsies from the neuroepithelial region of patients 'with nasal disease are less likely to yield olfactory-related tissue than biopsies from controls. 42 Anosmic rhinosinusitis

patients generally exhibit a more pathological epithelium (for example, disordered arrangement of cells and increased islands of respiratory epithelium) when compared to nonanosmic patients with rhinosinusitis. 43 Excessive dryness of the nasal mucosa - as seen in atrophic rhinitis, Sjogren's syndrome and repeated nasal surgery - can cause olfactory dysfunction, since a moist receptor environment aids chemoreception and transd uction.

Tumours and mass lesions A number of tumours in and around the olfactory bulbs or tracts can cause olfactory disturbance. Examples include olfactory groove meningiomas, frontal lobe gliomas and suprasellar ridge meningiomas arising from the dura of the cribriform plate. Due to the olfactory nerve's close location to the roof and medial wall of the orbit, as well as the optic nerves and tracts, structural

Chapter 131 Abnormalities of smell

lesions affecting smell may also affect vision. Also, olfactory tumours may extend into the frontal lobes resulting in symptoms of dementia and possibly the release of primitive reflexes (for example, grasping, snout and glabellar). Mass lesions need not be in the olfactory tracts to cause smell impairment. Ishimaru and colleagues 44 identified two patients with hyposmia from tumours to the right frontal lobe; the hyposmia reversed with craniotomy and tumour resection. Mass lesions around the olfactory region can result in a FosterKennedy syndrome, which consists of: (1) ipsilateral anosmia; (2) ipsilateral optic atrophy and (3) contralateral papilloedema secondary to raised intracranial pressure. 45 Pseudo-Foster-Kennedy syndrome has been reported in patients with increased intracranial pressure who had previous unilateral optic atrophy.46 Similarly, tumours in central regions of olfactory processing (for example, mesial temporal lobe) can also potentially affect smell. Headache often accompanies a complaint of smell loss when the aetiology is a mass lesion. Lymphoma has been known to infiltrate into olfactory areas and cause dysfunction. Similarly, granulomatous diseases - such as syphilis, sarcoidosis, systemic lupus erythematosus (SLE) and Wegener's granulomatosis - often result in anosmia. In patients suspected of having a neoplasm, neuroimaging is essential.

Neurodegenerative diseases Olfactory deficits have been described in a number of neurological disorders (see Appendix). Importantly, olfactory dysfunction may be the first clinical sign of Alzheimer's disease (AD) or idiopathic Parkinson's disease (PD). Smell testing is useful in identifying persons who have PD, as well as those who either have, or are at risk for having, AD. In PD, bilateral olfactory deficits occur before the onset of most of the classical neurological signs and symptoms and are unrelated to disease stage, use of anti-parkinson medications, duration of the illness and severity of the motor symptoms, such as tremor, rigidity, bradykinesia or gait disturbance. 5, 6 [***] Measurement of smell function can be useful to distinguish PD among the other disorders that share many nonolfactory symptoms and signs, such as progressive supranuclear palsy, multiple system atrophy, parkinsonism induced by the proneurotoxin 1-methyl4-phenyl-1,2,3,6-tetrahydropyridine (MPTP), and essential tremor. 6,20 [***] Given a male patient suspected of PD based on history and physical examination who is less than 60 years of age, an UPSIT score of 31 carries a 91 percent sensitivity and 88 percent specificity of correctly diagnosing a patient suspected of having PD. 6 In a woman suspected of PD who is less than 60 years old, an UPSIT score of 33 carries a 79 percent sensitivity and 85 percent specificity.6 [***]

I 1669

With regard to AD, Graves and colleagues 47 found that olfactory dysfunction in the presence of one or more APOE-e4 alleles was associated with a very high risk of subsequent cognitive decline, and smell testing identified people who came to exhibit later cognitive decline better than did a global cognitive test. [***] Devanand and collegues48 found that patients with mild cognitive impairment scored lower on the UPSIT than did the controls, and that patients with low UPSIT scores ( < 34) were more likely to develop AD than the other patients. When low UPSIT scores were accompanied by a lack of awareness of olfactory deficits on the part of the patients, they found that this predicted the time to development of AD. UPSIT scores from 30 to 35 showed moderate to strong sensitivity and specificity for diagnosis of AD at follow up. [***] In later life, Down syndrome patients show similar clinical and pathological changes to AD patients and have lower performance on a modified UPSIT compared to mentally-retarded matched controls. 49 [****] The olfactory loss associated with multiple sclerosis is directly proportional to the number of MS-related demyelinating lesions in central brain regions associated with olfactory processing (for example, inferior middle temporal lobe and periorbital frontal cortex).50 [****] Olfactory function actually increases and decreases as the plaque numbers increase and decrease. 51 Therefore, knowledge of a patient's UPSIT score can be predictive of the plaque load in the olfaction-related regions. [****] Schizophrenia is associated with significant deficits in odour identification and threshold sensitivity. 52 [****] The fact that there is an inverse correlation with UPSIT scores and disease progression suggests that there may be progressive neurodegenerative changes within olfactoryrelated pathways and smell testing can potentially be used as a marker of disease progression. 52

Epilepsy and migraine Olfactory auras, also described as hallucinations, are rare. When they are present, they are often associated with seizures and headaches. Olfactory auras consist of sudden unexplained sensations of smell that are usually, but not always, unpleasant and are rarely isolated events. 53 In epilepsy, mesial temporal lobe structures involved in the usual processing of odour information such as the amygdala and hippocampus - have been implicated as the generators of ictal olfactory sensations (simple or complex partial seizures) that often evolve into secondarily generalized seizures. 54 Common aetiologies include mesial temporal sclerosis and tumours. 54 Some cases of epilepsy have been associated with hyperosmia during the interictal period, although most patients with long-standing epilepsy and intractable

1670 I PART 13 THE NOSE AND PARANASAL SINUSES elderly patients, including ones who are healthy and taking no medications (Figure 131.5).57 Under the age of 65 years, approximately 1 percent of the population has major difficulty in smelling. Between 65 and 80 years, this increases remarkably, with about half of the population experiencing a demonstrable decrement in the ability to smelL Over the age of 80, this figure rises to nearly 75 percent. 54 [***] Despite the association with age, the complaint of smell loss should never be attributed simply to age, as often an accumulation of damage over the years is the culprit and a single event, such as a bad cold, can be the precipitating factor. In general, the age-related changes in smell function are reflected not only in damage to the olfactory receptors but related decreases in number of glomeruli within the olfactory bulb. 58 Interestingly, age-related occlusion of the foramina of the cribiform plate has been observed, pinching off the axons of the olfactory receptor cells as they enter the brain cavity. Whether age-related factors increase the susceptibility of the epithelium to damage from exogenous agents is not clear, but likely. What is clear, however, is that smell loss in older people adversely affects the quality of life. Chemosensory loss has been implicated as a cause - particularly in the elderly - of malnutrition, weight loss, impaired immunity and worsening of a medical illness. 3

seizure activity, such as candidates for temporal lobe resection, are hyposmic. 53 The effect of migraine on smell function is less well understood. The concept of certain smells provoking or exacerbating a migranous headache - osmophobia - may exist similar to that of photophobia or phonophobia.

Other causes and considerations Fewer than 5 percent of patients who present to a specialized centre for smell and taste disorders have a chemosensory disturbance that can be directly attributed to an exposure of a toxic compound. l There are numerous chemical agents that have been reported to affect smell function, including acrylates, cadmium, benzene, formaldehyde, solvents and nickel dust, among others (see Appendix).55 Tobacco smoking results in diminished olfactory ability as a function of cumulative smoking dose and, importantly, smoking cessation can result in improvement in olfactory function over time. 25 [***] Medications are also a common cause of smell disturbance and should be considered early, especially in the context of a new drug therapy (see Appendix).56 Several endocrine disorders exhibit disorders of smelL For example, Kallmann's syndrome, or hypogonadotropic hypogonadism with anosmia, is an X-linked or autosomal recessive neuronal migrational disorder associated with aplasia of the olfactory bulb and hypogonadism (pituitary involvement). [****] Similarly, patients with septo-optic dysplasia (de Morsier's syndrome) can have hyposmia, visual symptoms and precocious puberty. Age-related deficits in smell function are well documented and decreased smell function is present in most

TREATMENT OF SMELL DISORDERS The most effective treatments available are those for conductive anosmia, where there is an obstruction of airflow through the nose to the olfactory neuroepithelium. As noted above, the typical causes of mechanical obstruction include nasal sinus disease,

40 ill

OJ

c

~

35

..ill

t

Similar amounts of bone may be removed via external frontoethmoidectomy approaches, such as that of Lynch­ Howarth. Where more extensive bone removal is required, a craniofacial approach allows decompression of the optic 41 42 '

nerve up to the optIC ' c h'Iasm on one or both SI'des.

.

.

opacified on imaging and, while the condition has been described

as

happening

spontaneously,

it

may

be

associated with obstruction of the ethmoid infundibulum after orbital decompression.2o Goldberg

et

[** /* ] al,36 suggested that retention of an

inferomedial strut of bone between the maxillary and ethmoid during orbital decompression provides the globe

Results

with support and prevents hypo globus. It would, how­

Relatively few large series of this procedure have been published and these have largely related to post-traumatic decompression.43, 44. 45, 46

In

a

personal

series

of

14

patients undergoing endoscopic decompression, the pro­

cedure was most successful for those with thyroid eye

disease and sphenoid meningioma in which long-term 4 improvement in vision was attained. 7 Primary or secondary optic nerve decompression was undertaken in seven patients undergoing craniofacial resection for sino nasal neoplasia, two of whom had already lost one eye. Although in four cases disease ultimately rendered the

patients

blind,

the

decompression

preserved

or

improved failing vision in two individuals until close

to their deaths and offered useful palliation.48

ever, appear that maintenance of maxillary aeration rather than direct support of orbital tissues - is more significant in avoiding this problem as evidenced by the deve1opment 0 f 1'd'lOpat h'IC cases.

50 51 .

In both idiopathic and secondary imploding antrum syndrome there is a paucity of sinus symptoms. The idiopathic condition is associated with a high incidence of abnormal nasal anatomy, the majority of patients having a nasal septal deviation to the affected side and many having an abnormal lateralized middle turbinate

0 (Figure 132.8).5 After bone-removing orbital decom­

pression, globe

has

an

initially

occasionally

symmetrical been

recession

followed

by

of

the

increasing

unilateral enophthalmos, usually on the left side, Where

CT scanning confirms occlusion of the maxillary sinus

drainage, an endoscopic middle meatal antrostomy may be

Complications These include acute postoperative visual loss, diplopia and, theoretically, a cerebospinal fluid leak (although, in practice, rarely encountered),

performed to drain the mucus and stabilize the situation.

Restoration of the globe position may be difficult after three-wall decompression, as there is less neighbouring bone to facilitate reconstruction; mobilization of orbital contents and antral placement of a Whitehead's varnish pack for three weeks (via an anterior antrostomy) has, in

J____

•__.>__, _ _

1686

I PART 13 THE NOSE AND PARANASAL SINUSES

Deficiencies in current knowledge and areas of future research >- The exact cause of changes in the fat and muscles in thyroid eye disease remains unknown and requires further elucidation. ). In view of the probable benefits of high-dose steroids after major injury, a placebo-controlled randomized trial comparing medical and surgical therapies for optic canal decompression is hard to justify.

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Figure 132.8

with lateralized middle turbinate and lowered orbital floor in an

tmao many icasntaeisn) prthoeveimd preffoevectidveposiprtioonduciof nthgesuglffiobecie?nOt fibrosis vibePatsiinioegnnttsfeomusml oporwitnagrywarrebutposneidoctioofcniasaniogpotnalofelyntthiraeelqglinuiocrberiena)gstehciionsrusdoublecutaliolnye with prisms or further surgery.

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wt'-r:�:;tog�'e'r;,'WithiconsideJaQle , , .vaofsq:lliti¢:·�ond-ition�' nt . e : ; :i!'�i;!i��f'1�i:�;;;;r;; , adacchiryevocedy,-swtoitlrhinlenosdohastomyaJ.and- results the be ' fl o or b l o wo ut ' . KE¥ POfNTS: '.

•

:

.....

"""!�;. 0,/ - .'

: . � .�.

-.

...'-·.r ·:

.. ,

' ;. �

-

� I"

" ?" '" �

.

..

"

_':'�

••

�l.

-

-

10.

.-

' ' An>-�N'1{�utg�� and",ciphth,�lIDQlo�srmay',c

'

'- "

81: 1658-64.

'

:; -' .: '

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•

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"

cO,tnp�ta.ti9ns mucocoele$,

'ba-¢terlaL-rhinosiUP-_sltisi,: �"

�nd

de'¢Qmpre�si.on;':'although tair

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*

32.

33.

34.

35.

36.

37.

38.

39.

40. *

41 .

42.

43.

44.

45.

46.

Orbital and optic nerve deco m p ression I 1687

ophthal mopathy. American Journal of Ophthalmology 1 993 ; 1 1 6 : 533-47. Lund VJ , Larkin G, Fel l s P, Adams G. Endoscopic orbital deco m p ression. Journal o f Laryngology and Otology 1 997 ; 1 1 1 : 1 051 -5. Stark B, Ol ivari N. Treatment of exophthal mos by orbital fat removal . Clinical Plastic Surgery. 1 993 ; 20: 285-9. Troke l S, Kazi m 1Vl , Moore S. Orbital fat removal : deco m p ression for G raves' orbitopathy. Ophthalmology. 1 993 ; 1 00 : 674-82. I\l u ne ry WR, Nunery CW, Martin RT, Truong TV, Osborn DR. The risk of d i p lopia fol l owing orbital fl oor and med ial wal l decompression in su btypes of ophthal m i c G raves' disease. Ophthalmic Plastic Reconstructive Surgery. 1 99 7 ; 1 3 : 1 53-60. Goldberg RA, Shorr N , Cohen NS. The med ial orb ital strut in the prevention of post decom pression dystopia in dysthyroid op hthal mopathy. Ophthalmic Plastic Reconstructive Surgery. 1 99 2 ; 8 : 32-4. Kraus OJ, B u l lock JD. Treatment of thyroid ocu lar myopathy with adj ustab le and nonadj ustab le sutu re strabismus s u rg e ry. Transactions of the American Ophthalmological Society 1 993 ; 9 1 : 67-84. Bracken M B, She pard MJ, Holford TR, Leo-Su m m ers L, A l d rich EF, Fazl M et al. Ad m inistration of methyl prednisolone for 24 or 48 h o u rs or ti ri l azad mesylate for 48 h o u rs in the treatment of acute sp inal cord inju ry. Journal of the A merican Medical Association. 1 99 7 ; 2 7 7 : 1 597-604. Levin LA, Beck RW, Jose ph M P, Seiff S, Kraker R. The treatment of trau matic optic ne u ropathy: the International Optic Nerve Trau ma Study. Ophthalmology 1 999 ; 1 06 : 1 268-77. Lang J. Clinical anatomy of th e nose, nasal cavity and paranasal sin uses. Stuttg art : Th ieme, 1 98 9 : 1 28. Lund VJ , Howard OJ , Wei WI, Cheesman AD. Craniofacial resection for tu mors of the nasal cavity and paranasal sinuses - a seventeen year expe rience. Head and Neck Surgery. 1 99 8 ; 20: 97-1 05. Li KK, Teknos TN , Lai A, Lau retano A, Terre l l J , Joseph M P. Extracranial optic nerve decom pression: a 1 O-year review of 92 patients. Journal of Cran iofacial Surgery. 1 999; 1 0 : 454-9. Tandon DA, Thakar A, M ahapatra AK, G hosh P. Trans­ eth m oidal optic nerve deco m p ression. Clinical Otolaryngology. 1 994; 1 9 : 98-1 04. Luxenberger W, Stam m berger H, Jebeles JA, Walch C. Endoscop ic optic nerve deco m p ression: the G raz experience. Laryngoscope. 1 998; 1 08 : 873-82. M au rer J, Hinni M , M ann W, Pfeiffer N. Optic nerve decompression in trau ma and tumor patients. European Arch ives of Oto-Rhino-Laryngology 1 999; 2 5 6 : 341 -5. Kountakis SE, lVlai l i ard AA, EI-Harazi SM, Long h ini L, U rso RG. Endoscopic optic nerve decompression for trau matic b l indness. Otolaryngology and Head and Neck Surgery. 2000 ; 1 23 : 34-7.

1688 I

PART 1 3

THE NOSE AND PARANASAL SINUSES

47.

Rose GE, Lund VJ. Endoscopic transnasal orbital decom pression for visual fai l u re due to sphenoid w ing mening iom a. Eye. 2006; 20 : 1 2 1 3- 1 9. Lund VJ, Howard DJ, Chees m an AD. Proceedings 4th 48. International Conference on Head Et Neck Cancer. Head Et Neck Cancer Proceedings. The orbit. Eval u ation and intraoperative m anagement. 1 996; 4: 1 020-7. 49. Soparkar CNS, Patrinely JR, Cuaycong MJ, Dai ley RA, Kersten RC, Ru b in PA et 01. The silent sinus synd rome. A

50.

51.

cause of spontaneous enophthal mos. Ophthalmology. 1 994; 1 0 1 : 772-8. Rose GE, Sandy CJ , Hal berg L, Moseley I. Cl inical and rad iolog ical ch aracte ristics of the i m ploding antru m , or 'si l ent sinus', synd rome. Ophthalmology. 2003 ; 1 1 0 : 8 1 1 -8. Davidson J K, Soparkar CN, Wi l l i ams J B , Patrinely JR. Neg ative sinus pressure and normal predisease i m ag ing in silent sinus synd rome. Arch ives o f Ophthalmology. 1 999; 1 1 7 : 1 653-4.

133 Dacryocystorhinostomy

NEIL FERGIE AND NICHOLAS S JONES

Introduction

1689

Dacryocystorhinostomy in children

1696

History

1689

Common canalicular obstruction

1696

Surgical anatomy

1690

Key points

1696

Indications

1690

Best clinical practice

1696

Preoperative assessment

1691

Deficiencies in current knowledge and areas for future 1697

Operative techniques

1691

Variations in surgical technique

1694

References

1697

Complications common to all approaches

1695

Further reading

1698

The role of antimitotic agents

1695

The data

research

in this chapter are supported by a Medline search using the MeSH headings combinations of DCR, canaliculodacryocystorhinostomy, Lester J ones, epiphora, n asolacrimal 5 tlu o roura cil

dacryocystorhinostomy,

-

,

-

,

mitomycin C and antimetabolites.

INTRODUCTION

bone removal following the creation of a middle meatal

Epiphora (excessive tearing) is a common complaint. For

matous conditions, such as Wegener's granulomatosis and

some this is a minor inconvenience but for others it can

sarcoidosis.

be

extremely

antrostomy, mid face fractures, malignancy and granu lo

troublesome,

and

a

source

of

social

embarrassment as it can alter refraction and mean that

the patient has to wipe their eye(s) perp e tu ally Epiphora .

­

The incidence of nasolacrimal obstruction is estimated to involve approximately

10 percent at to 35-40 percent at 90 years of age l

40 years increasing

.

can also be secondary to the excessive production of tears or arise from proximal obstruction in the drainage system at the punctum or common cannaliculus. While obstruction of the nasolacrimal system may present

with epiphora,

it may

also

present

with

a

mucocoele (Figure 133.1a), pyocoele or recurrent acute dacryocystitis (Figure 133.1b).

In the majority of cases the cause of the obstruction is unknown. Such idiopathic obstruction becomes more conunon

with

preponderance.

increasing Other

less

age

and

common

shows

a

causes

female include

surgical trauma, especially following excessive anterior

HISTORY The first report of dacryocystorhinostomy (DCR) was by Caldwell

in 1893?

Caldwell

created

a

An

ENT surgeon

rhinostomy

using

by an

profession

.

intranasal

approach by removing a por tion of the inferior turbinate and following the nasolacrimal duct to the lacrimal sac.

As can be imagined this would have involved considerable skiU given the equipment available at the time and it did not gain popularity.

Toti,3 in

1904, is credited with the

__ 0______

O _00 io ___

1690

I PART 13 THE NOSE AND PARANASAL SINUSES

They continue for 10 mm before uniting to form the common canaliculus, which has a short course of 3-5 mm angled forwards before entering

the posterior-lateral

aspect of the lacrimal sac. Occasionally there is no common

canaliculus with the

superior

and inferior

canaliculi entering the lacrimal sac independently. The nasolacrimal duct exits from the inferior point of the lacrimal sac and continues in an inferior direction with a slight lateral or medial angulation for 12 mm before entering the nose 10-15 mm behind the anterior end of the inferior turbinate and 16 mm above the floor of the nasal cavity high in the inferior meatus. It is guarded at this point by Hasner's valve. The lacrimal bone that articulates

inferiorly

with

the

base

of

the

inferior

turbinate forms the posterior wall of the bony canal while the frontal process of the maxilla forms its anterior boundary. The lacrimal sac itself lies protected in the concavity of the lacrimal fossa in the medial orbital wall. The lacrimal fossa is formed by the lacrimal bone and by the frontal process of the maxillary bone. The latter forms the anterior lacrimal crest and the remainder of the anterior part of the lacrimal fossa and comprises very dense bone. Rarely, the maxillary bone can form the whole of the lacrimal fossa. The posterior aspect of the inferior portion of the lacrimal sac lies on the very thin lacrimal Figure 133.1

(a) Nasolacrimal mucocoele; (b) dacryocystitis.

bone

and

this

area,

which

is

immediately

anterior to the attachment of the uncinate process, is easily entered using an endonasal approach. The lacrimal

first description of an external approach more than a decade after Caldwell had described the operation via the endonasal approach. The external approach was subse­ quently modified by many surgeons including Dupuy­ Dutemps and Bourget,4 who emphasized the importance of sutured mucosal flaps. With the development of modern nasal endoscopes almost 100 years later a resurgence of interest in the endonasal approach has taken place. The early results by this approach included those described by Steadmans and McDonagh and Meiring6 although the instruments in the 1980s were not ideal and these have since been refined with improved results. Massaro et

az.7 produced the first report using a laser

technique to aid endonasal DCR using an argon blue­ green laser, and Gonnering et

fossa is 15 mm in length, 4-8 mm in breadth and 2 mm

in depth with the sac having two-thirds of its height above the insertion of the middle turbinate and one­ third below. The sac is encapsulated in a dense layer of fascia with contributions

from

the

medial

canthal

tendon,

the

orbicularis oculi muscle and the periosteum of the medial orbital wall. This has the effect that infection within the sac tends to remain confined to the sac causing increasing pressure and pain but without spread into the orbit or face. In

approximately 8 percent of cases, an anterior

ethmoidal air cell, the lacrimal cell, lies medial to the lacrimal bone and has to be opened in order to gain access to the lacrimal fossa.

al.s subsequently reported

using both the CO2 and KTP laser.

INDICATIONS In general, a DCR is indicated where there is symptomatic

SURGICAL ANATOMY

distal obstruction of the nasolacrimal duct that is not relieved by simple probing and syringing.

It is not

The lacrimal drainage system consists of the superior and

indicated

inferior canaliculi , common canaliculus, lacrimal sac and

obstruction lies elsewhere in the canaliculi or puneti as

nasolacrimal duct. Commencing with a punctum in each

the operation will not bypass these areas.

eyelid (approximately 6 mm from the medial canthus) the

as

the

sole

procedure

where

the

site

of

In many patients there is some proximal obstruction

canaliculi initially run at 90° to the lid margin for 1-2 mm

associated. with distal blockage. In such cases, gentle

before turning to run parallel with the lid margin.

probing and dilatation in conj unction with a DCR and

Chapter 133 Dacryocystorhinostomy I

insertion of stents can be performed although the results

lower success rates as thick bone will make an endonasal

of such an approach are not as favourable in cases of pure

approach more difficult.

distal blockage. Where functional obstruction exists as

approach should be considered.

evidenced by free flow on syringing along with failure of the pump system on scintigraphy, a

1691

In these cases an

external

A dacrocystogram can demonstrate diverticula, steno­

DCR may be

sis, strictures, dacryoliths or a tumour but it will rarely

performed but the results of such an approach tend to

give any useful information prior to surgery unless there

be variable, indicating that relying on gravity to drain the

is a mass within the sac or an atypical feature such as a

tears is often insufficient although one recent report

bloody discharge. Dacryoscintigraphy using the radionucleotide 99MTc

provides excellent results.9

90 percent of tears drain through the inferior

may be a helpful adjunct in assessing whether there is a

canaliculus, obstruction of this or the common canalicu­

functional problem. This is the case when there is free

As

lus can be bypassed but requires a more extensive

flow on syringing along with abnormal fluoroscein dye

procedure (see under Common canalicular obstruction).

disappearance tests. If there is a bloody discharge from the punctum malignancy must be excluded especially in the presence of an irreducible swelling at the medial canthus. Computed

PREOPERATIVE ASSESSMENT

tomography (CT) scanning is not part of the routine

Syringing and probing together are the main methods of identifying

the

site

nasolacrimal system.

of

any

obstruction

within

flushing saline through the

inferior canaliculus.

inability

the

to

flush

the

Syringing is achieved by gently through

inferior

An

canaliculus

indicates obstruction at the site of the punctum or inferior canaliculus while reflux of saline through the other canaliculus indicates the obstruction is more distaL Gentle skilled probing with a

'0' Bowman's probe will (Figure

assist in confirming the level of the obstruction

133.2). It is crucial that the mucosa is not damaged and that a false passage is not created. Massaging of the sac may produce a discharge from the

work-up for DCR but is appropriate if there are specific concerns with regard to the possibility of malignancy or if there has been a facial fracture. Fine rigid dacryocystocopes of

0.7 mm diameter have

allowed examination of the proximal lacrimal drainage system and direct visualization of obstruction at this level although such techniques are still evolving. A number of other methods of evaluating the lacrimal system have been described including ultrasound, mag­ netic resonance imaging, magnetic resonance dacryocys­ tography and CT dacryocystography. These techniques do not have any value in the routine preoperative investiga­ tion of patients.

[**/*]

puneti consistent with chronic dacrocystitis. A mucocoele or pyocoele produces a swelling inferolateral to the medial canthus

(Figure 133.1a).

Nasal endoscopic examination is mainly aimed at excluding anatomical abnormalities that may interfere with successful endonasal surgery but will also detect any rare pathology that may affect success rates. A history of trauma involving the lateral nasal wall is likely to result in

OPERATIVE TECHNIQUES The techniques available are: • • •

external D CR; endonasal DCR with conventional instruments; endonasal laser-assisted DCR.

These are compared in

Table 133.1 and explained in more

detail below.

External DCR An external DCR can be performed making a low Howarth's incision. The lacrimal sac with its attached periosteum is dissected free from the lacrimal fossa and is retracted laterally. A rhinostomy of 1.5 cm is created taking care not to damage the nasal mucosa. A vertical slit is made in the exposed nasal mucosa and, similarly, a corresponding vertical slit is made in the lacrimal sac and the flaps created are sutured together to create an epithelially lined rhinostomy. Visibility and access of the intranasal anatomy is restricted and a lacrimal air cell can Figure 133.2

Probing of the inferior canaliculus.

be overlooked.

i ' __ • •• ____.1. _______

1692

I PART 13 TH E NOSE AN D PARANASAL SI N USES

Table 133.1

Comparison of techniques. 1

.' Endonasai 6'CR

External DCR

'1"

;J

... 1

Local

Anaesthetic

General

General or local with sedation

Expense

Low

low

Initial high capital outlay

External scar

",

X

X

Operating time

l'

�

10, 11

��

14,15,16

17, 59-100%

Success rate

>

H aemorrh age

l'

-+

��

Day case

No

Potential

Yes

Disruption of lacrimal pump function

yt'

X

X

1',

82_95CVo'2,13,

90%

lB, 19,20.21

increased;�, decreased.

Figure 133.3

I ncising the mucosa over the left anterior

Figure 133.4

Iau!mal crest (cadaver).

Endonasal OCR with conventional instruments An incision is made in the mucosa overlying the anterior

lacrimal crest

(Figure 133.3) and a posteriorly based

mucoperichondrial flap is raised. The anterior lacrimal crest can be identified as a white vertical ridge of bone immediately

anterior

Removing the bone of the left anterior lacrimal

crest with a punch (cadaver).

to

the

middle

turbinate.

anterior lacrimal crest is removed using a punch

posterior flaps. These flaps of sac mucosa are then placed in contin ui ty with the mucosa of the nasal wall

133.6),

(Figure

A stent may then be inserted. With this approach

the lacrimal sac is widely exposed and the common canaliculus can often be seen.

The

(Figure

133.4) and more superiorly a coarse diamond drill helps

Endonasal laser-assisted OCR

as the bone is thicker there. Posterior to the anterior lacrimal crest is the uncinate process, and just lateral to

The

canaliculus

is

dilated

to

allow

passage

of

a

the uncinate process is the thin lacrimal bone that forms

vitreoretinal light probe. The light probe should idea lly

the remainder of the medial aspect of the lacrimal fossa.

be passed

This bone is paper thin and is easily resected. To create a

inferiorly to avoid trauma to the inferior canaliculus and

large rhinos t omy also requires excision of the upper part

to encourage placement of the ostia in a dependent

of the ant eri or lacrimal crest using a diamond burr

position avoiding the potential of a 'sump'

al though in children the bone can be removed with a

This is then advanced into the lacrimal sac and

th rough the superior canaliculus and angled

within the sac. the point

Kerrison punch. The sac is exposed and has a dark red

of light can be seen endoscopically act ing as a guide for

(Figure 133.5). The sac is divided vertically with either a sickl e knife or a

the area to fashion the rhinostomy

colour and is firmer than nasal

m ucosa

(Figure 133.7). In 8

percent, a lacrimal or anterior agger nasi cell lies between

45° beaver scalpel. A probe placed within the sac, tenting

the sac and the lateral nasal wall and will result in a

it medially, facilitates incision. Microscissors are then

diffusion of this li gh t

used both inferiorly and superiorly to create anterior and

be opened prior to gaining entry to the lacrimal fossa .

(Figure 133.8). This cell will need to

Chapter 133 Dacryocystorhinostomy I

Figure 133.5

1693

Incising the left lacrimal sac (cadaver),

Figure 133.7

Transmitted light from light probe within the

left lacrimal sac viewed endoscopically.

Figure 133.6

Flaps fashioned and retracted to widely expose

the left lacrimal sac (cadaver).

Confusion

can

arise

in

mistakenly

following

the

reflection of the aiming beam of the laser in the belief that it is the transmitted light from the vitreoretinal light probe in the sac. Reducing the illumination from the

Figure 133.8

Diffusion of light through a large right lacrimal!

agger nasi air cell.

endoscope will help visualize the light probe if it is dim because of a lacrimal cell or a thick-walled mucocoele. A variety of different lasers have been described for this

roughen the surface and cause trauma to the common

surgery. At optimum power the laser ablates tissues, but at

canaliculus when it is removed. It is replaced with a

suboptimum levels, which can happen if the beam is at an

lacrimal probe that can be used to tent the sac medially

angle, not focused or at suboptimum power settings,

and help localize the area of mucosa that is ablated as well

burnt tissue can accumulate. The laser cannot ablate

as making further widening of the ostium easier.

charcoal and so with continued use heat is dissipated

The rhinostomy is enlarged to 5-8 mm diameter. A

through the charred tissue with the risk of thermal injury.

silicone stent may then be passed through both superior

Gentle curettage of the charcoal can be performed and the

and inferior canaliculi to create a loop at the medial

laser can then be used again. Suboptimal ablation can be

canthus and be retrieved from the nose with fine nasal

used to advantage

forceps and secured

if the surgeon wants to

achieve

haemostasis. Suction is required to remove the laser plume.

Once the sac is exposed, the light probe is

withdrawn to avoid damage to its metal sleeve, which can

; I

(Figure 133.9). The loop should not

be excessively tight as it can cause granulations at the rhinostomy canaliculi.

site

and

can

'cheese-wire'

through

the

1694

I PART 13 THE NOSE AND PARANASAL SINUSES

that avoids these problems and provides stereoscopic vision but is more cumbersome to manipulate. Some workers do not insert a stent while others leave a stent in for several months. There is no clear evidence to recommend either approach over the other.22 The size of the ostium has been reported by some authors to affect success rates but again there is no clear information to support this. 22 Transcanalicular DCR involves passing a laser fibre through the canaliculus in a similar manner to the passage of the light probe in endonasal laser-assisted DCR. A 600

micron optical fib re is ideal for this purpose.

The position of the laser is confirmed endonasally. The laser is then used to create a rhinostomy towards the nasal cavity and enlarged. This technique was initially described 23 24 , and while attractive, there remain a number

in 1992,

of concerns regarding this technique. Damage to the canaliculus can occur if there is a leak of laser energy from the laser fibre and there is a tendency for the fibre to be directed posteriorly in the direction of the orbit, which can result in ablation of the posteromedial wall of the lacrimal sac or entering the orbit if not performed with Figure 133.9

A silicone stent in place following a left

endonasal visualization to control its direction.

endonasal laser OCR.

The

stoma created tends to be small and posterior. There does not, therefore, appear to be any clear advantage to this method, with the increased possibility of inadvertent damage resulting from it.

CHOICE OF LASER FOR ENDONASAL LASER-ASSISTED

Balloon dacryocystoplasty involves the passage of an

DACRYOCYSTORH INOSTO MY

The laser should have the ability to vaporize soft tissue and bone, should have good haemostatic properties, be deliverable

through

a

flexible

laser

fibre

and

be

inexpensive. The CO laser has poor haemostatic properties, is poor 2 at bone ablation and cannot be delivered through an optical fibre, making it unsuitable for this purpose. The argon laser produces ablation by contact with tissue and has poor bone ablation. The Nd:YAG laser has good tissue ablation but poor haemostatic properties. The Ho:YAG, the KTP/532 and the diode lasers are all suitable for this procedure. The Ho:YAG laser effectively vaporizes bone and has good haemostatic properties but has the disadvantage of a tendency to spatter requiring repeated cleansing of the endoscope lens. The KTP/532 star pulse laser has similar advantages but avoids this problem. The diode laser has a single-use fibre increasing the expense per procedure of this laser. The erbium:YAG laser is likely to be ideally suited to this surgery but as yet no suitable delivery system exists for its use. [**/*]

angioplasty balloon catheter over a protected guidewire. Inflation

of

the

balloon

dilates

the

stenosis.

This

procedure can be performed under local anaesthetic as a day case. Results of this technique have shown mixed initial success rates with a predisposition to restenosis. Reported success rates range from 23 to 70 percent, but with variable lengths of follow-up and inclusion criteria. Of the larger studies Lee et

al.25 reported a

23 percent

success rate in 81 eyes at two years, Janssen et

al.26 a 70

percent in 100 eyes over a 5-48 month follow-up, Ilgit et

al.27 a 66 percent success in 80 eyes at 6-18 months and al.28 a 44 percent success at 12 months in 52

Fenton et

eyes. The largest study involving 430 eyes in 350 patients had a one-year patency of 48 percent and a five-year patency of 37 percent.29 While a simple and safe procedure, the disappointing results, in particular the high recurrence rates, limit the usefulness of dacryocystoplasty at present. The external approach usually

requires a general

anaesthetic with an overnight stay. The facial scar usually heals well unless the line of incision is not broken in the shape of a silhouetted seagull and placed relatively high

VARIATIONS IN SURGICAL TECHNIQUE An endoscope is most commonly used as it is manoeuvr­

able, but the lens requires repeated cleaning as it becomes obscured by the laser plume, blood and debris. An operating microscope with a 300-mm lens may be used

on the side of the nose. If the incision is not broken and is nearer the medial canthus a web can occur. Extensive dissection around the medial canthus is required with the potential for injury to normal lacrimal pump function. In some cases there is haemorrhage, resulting in bruising, swelling ano-, occasionally, nasal packing is necessary. Revision surgery by the same approach can be difficult as

Chapter 133 Dacryocystorhinostomy

opposed to endonasal techniques where it is straightfor­ ward. The main advantage is that consistently high success rates have been achieved by this approach but whether this is reflected in normal practice is debatable as the results from an audit of a nonselected group suggest.20

I 1695

•

migration of stents and cheese-wiring if it is too

•

sump syndrome when the rhinostomy is high and

•

tight;

mucus collects within the sac; haemorrhage.

While routine biopsy is not required, excellent views of the lacrimal sac are obtained allowing biopsy of the lacrimal sac if it looks abnormal. The advantages of endonasal laser dacryocystorhinost­

THE ROLE OF ANTIMITOTIC AGENTS

omy (ELDCR) are that there is no external scar, it can be

The two most common causes of failure in DCR surgery

carried out as an outpatient and in those who are not fit

are closure of the surgically created osteotomy with soft

for general anaesthetic, e.g. with severe cardiovascular

tissue

disease or on warfarin. It produces minimal bleeding and

Antiproliferative agents applied at the osteotomy site

and

obstruction

at

the

common

canaliculus.

low primary and secondary haemorrhage rates, a short

may reduce the fibrosis and hence reduce the failure rate.

operating time and less disruption of medial canthal

This has been demonstrated in other areas of ophthal­

anatomy and lacrimal pump function. The disadvantages

mology where the application of antimitotic agents in

of this approach are that laser precautions are required,

trabeculectomy improved success rates in

the expense of the laser and, although there is a wide

Mitomycin C (MMC) and 5-fluorouracil (5-FU) have

glaucoma.

variation in success rates, there appears to be, overall, a

been equally successful in this regard.34 Both agents have

higher failure rate due to stenosis and scarring at the 1 1 1 rhinostomy site. 8, 9,20,2 ,30

been

Endonasal DCR has many of the advantages of ELDCR but avoids the disadvantages of the external approach. It does have a longer operating time and increased risk of

shown

to

have useful

activity

in

vitro.

Seven

randomized controlled trials comparing the effect of antimitotic agents against controls have been identified. The most impressive results for supporting their use in

external surgery were those obtained by Liao et aI.35 in a

haemorrhage when compared with ELDCR but appears

prospective randomized controlled study which showed a

to have success rates rivalling those of external DCR with

significant

most series reporting success rates of 80-90 percent. It is a 1 1 1 1 1 1 good technique! 2, 3, 4, 5, 6,3

application of MMC of 0.2 mg/mL for 30 minutes. In

improvement

in

success

rates

with

the

the group treated with MMC 95.5 percent were symptom­

In the literature, comparison of the results of different

free compared with 70.5 percent in the conventional

approaches available is far from straightforward with a

untreated group. The results obtained statistical signifi­

dearth of prospective studies comparing the techniques.

cance, in part, due to the low success rate in the

In addition, reported results of individual techniques

conventional group. [***] A further four studies have

use different selection criteria, different reporting of

been reported using antimetabolites in external DCR

successful outcomes and different times as the end

surgery. Of these, all suggested a small benefit in the

point. That being said, the only prospective randomized

MMC group but none were significant. Different doses

trials conducted to compare external and endoscopic

and times of application ranging from 0.2 to 1 mglmL

DCR

were

undertaken

by

Hartikainen

et

aI.32,33

These trials reported success rates for external DCR and ELDCR of 91 and 63 percent, respectively (p=0.016).32 In

and

from

two

to

30

studies.36, 37,38,39 [***]

minutes

were

used

in

these

Two further randomized controlled trials were identi­

with

fied that used these agents in endonasal approaches. The

those having endonasal DCR the success rates were 91

first, a randomized double-blind placebo-controlled trial,

versus 75 percent (p=0.18),33 although numbers were

looked at laser procedures with follow-up at 12 months40

comparing

patients

undergoing

external

DCR

Uncontrolled

with 5-FU being applied at a dose of 0.5 mg/mL for five

observational series show widely varying results for any

minutes. Overall success rates of 76 percent in the treated

small

with

32

cases

in

each

group.

given technique with the general impression supporting

group versus 63 percent in the placebo group were

the conclusions of the above citation, however, clear

obtained. While showing benefit in the treated group this

evidence is lacking. [**/*]

failed to reach statistical significance although a type B error

cannot

be

excluded.

In

another

study

using

conventional instruments and an endonasal approach,

COMPLICATIONS COMMON TO ALL APPROACHES Complications include:

0.5 mg/mL of 5-FU was used for two and a half minutes. No difference was found after a minimum follow-up of 1

nine months for either primary or revision surgery.4 [***] Without

further

information

it is difficult to be

•

failure - this may be due to granuloma formation or

•

scarring at the site of the rhinostomy;

good theoretical evidence to support their use and in vitro

adhesions between traumatized nasal surfaces;

experiments but no clear benefit has been demonstrated.

categorical about any benefit of these agents. There is

1696

I PART 13 THE NOSE AND PARANASAL SINUSES

Their use has not yet been adopted

in DCR surgery

routinely.

recently,

tube

insertion

has

been

reported

via

an

endonasal approach47 but this method awaits medium­ and long-term evaluation.

DACRYOCYSTORHINOSTOMY IN CHILDREN Epiphora is common in newborns with an incidence as

high

as

20

percent

being

reported.

Congenital

nasolacrimal duct obstruction usually happens at the distal end of the nasolacrimal duct and is membranous. Fortunately, more than 95 percent neous resolution in the first

will undergo sponta­

12 months, and in the

remaining cases a further 60 percent in the succeeding 12 months.42

[**]

No treatment should be considered until at least 12 months of age in view of the high resolution rates and

the observation that no detrimental effect has been found in those who did not resolve when this policy has been

Ca naliculodacryocystorh i nosto my This involves resection of the stenosed region of the common canaliculus with primary anastomosis over a stent in conjunction with

a DCR.48 This has proved useful

for distal canalicular obstruction with greater than 8 mm of patent proximal canaliculus10 and the more medial the obstruction, the greater the success rate. Trials attempting to laser common canalicular pathol­ ogy using the O.7-mm rigid fibreoptic endoscope with a delivery channel are underway, but this technique is yet to be of proven benefit.

[*J

adopted. Thereafter) the standard treatment is syringing and probing, usually under general anaesthetic in this age group. It may be beneficial for this to be undertaken as a collaborative procedure between the ophthalmologist and

KEY POINTS

ENT surgeon42 so that probing through Hasner's valve

•

can be visualized and the inferior turbinate outfractured under direct vision. Such an approach has the advantages that the correct passage of the probe can be confirmed

•

Therapeutic procedures, such as an infracture of the inferior turbinate, syringing and stenting, can be under­ taken at the same time. If stenosis is apparent then, in

•

[*]

COMMON CANALICULAR OBSTRUCTION •

Medial mucosal common canalicular obstruction is the

•

most common cause of proximal obstruction and is due

•

a

rates.

long-term is idea] in those with coagulopathies and those who are unfit for general anaesthetic. DCR is primarily carried out for distat nasolacrimal duct obstruction and therefore' an accurate preoperative diagnosis of the site' of obstruction is essential. Endonasal techniques are the least invasive:' >- ": The role of antimitotics,_ is uncertain; , An expectant policy sh6u1d --be! adopted'cin''.,: children, at least untjl-12· niohlhs�O{ age.-' .'� ' The best approach fOf.:'COITl.O}oh , e�n �icillaf" , obstruction ,is uncertain, and reIies on the IJ��.:, �"',,:,,;: -:,\ '1 result of further�sf!I4l,���(�'? .;", ",·! ": : , . , . �yphHis />a(ely. ��volvesc,the, ext,e-rnal nose. '

�

._' I

'• • ,

Terti

_

__

.

�Hr� apd �ongenital syp hilitic lesions may

. resul( i.ri. �xiernal' nasal

,'Qestr-ucti�n:�:":.;c_ '.:

�

','

deformit,y,. or. tissue .

' y

•

,

Best clinical practice

Figure 134.3

.I Chronic infective processes involving the nose are

Keratoacanthoma. Globular lesion with central

keratin-fi lied pi ug.

ra re but req ui re consi d eration in im munocomprom ised patients.

.I Antituberculosis therapy is indi cated in the treatment of lupus vulgaris.

associated with increased incidence. A possible role for a viral agent has been suggested, although this has not been

satisfactorily proven. Histopathological

ana1ysis reveals a tumour denoted by

p roliferating keratinizing cells centred on hair follicles.

Lesions are confined to the dermis) and neoplasia is

NEOPLASMS OF THE NOSE

signified by the presence of occasional mitotic figures and

some cellular atypia. Histological findings are similar to

Keratoacanthoma

those of SCC, lending support to the belief that the two

pathologies have a similar origin;

however,

this still

Keratoacanthoma) or molluscum sebaceum, is a benign

remains a hypothesis.

that follows a specific growth pattern terminating in

coloured or red lesion, which grows rapidly for up to six

cutaneous tumour, probably arising from hair follicles, complete resolution

(Figure 134.3). It is one-third as

Keratoacanthoma presents as a firm, round, flesh­

weeks. The epidermis is glistening and telangectasia are

(SCC).5 Such

evident beneath the surface. T he lesion becomes globular

mostly in white races) with men affected two to three

however, surrounding tissue remains normal. Resolution

common as squamous cell carcinoma

lesions happen from middle age onwards and are seen times more commonly than women.

[**]

Lesions predominantly target sun-exposed areas with

and develops a central hom-filled or keratotic plug,

takes place, on average, three months later, although

complete healing can take up to a year. Shedding of the

75 percent being found on the nose, lip, cheek and eyelids.

keratotic plug signifies recovery and characteristically a

of

particular1y

Ultraviolet radiation is therefore implicated in the genesis keratoacanthoma,

although

industrial

occupations

involving contact with tar and mineral oils are also

scar is left. Rarer variants include recurrent forms, seen

in occupationally

related

lesions

or

in

Muir-Torre syndrome. T he latter condition describes an

Chapter 134 Conditions of the external

nose. 1705

assoCIatIOn between sebaceous adenomas and internal

malignancy. Occasionally, tumours may reach a size of 50 mm or more. The

differential diagnosis is

with squamous skin

carcinoma, however, the rapid growth phase and resolu­

tion characteristics of keratoacanthoma indicate a benign course. Treatment is by simple curettage but this may compromise

samples

for

histopathological

analysis.

Radiotherapy and 5-fluorouracil have been employed

with

success,

particularly

for

persistent or recurrent

forms. Excision biopsy should be used if doubt as to

the diagnosis persists and this also provides an opportu­ nity to leave a more controlled scar. Figure 134.4

Actin i c (so l a r) keratosis Solar damage to the skin, particularly in fair-skinned individuals, can induce dysplastic epidermal cell change, and 1

percent of cases proceed to

frank squamous

malignancy.6 Initial injury is designated by

an

aggregation

of small telangiectatic vessels that later develop into a brown or yellow macule with a tough adherent scale. Lesions extend to several centimetres in size and rarely happen in isolation. Often, patients possess a weather­ beaten look and exhibit skin yellowing due to solar elastosis. The scale thickens and may be sufficiently hypertrophic to form a cutaneous horn. Control of actinic

keratosis

with

liquid nitrogen,

curettage,

or

topical 5-fluorouracil for large areas, is effective, but in all cases prevention should be encouraged as keratosis may rescind with appropriate sunscreens and reduced sun exposure.

Ulcerated SCc. Surro unding tiss ue is markedly

indurated.

[**]

apparent. Differentiation from keratoacanthoma is often difficult but sec follows a slower clinical course. Small growths can be removed by curettage although this reduces specimen quality for histological investiga­ tion. Surgical excision should include a 3-5-nun cuff of normal tissue and deep margins must be considered. On the nose primary closure is often not feasible, but good restoration of aesthetic integrity is possible with employ­ ment of local or regional tissue. Optimal results require precise replacement of excised tissue structures

with

equivalent material. Radiotherapy and cryotherapy are satisfactory treatment alternatives. T he presence of well-defined, flat erythematous lesions in older patients with a history of sun exposure and

histological evidence

of intraepidennal

carcinoma

is

termed Bowen's disease. The initial macule enlarges in an irregular manner and is associated with a whitish scale. Approximately 5 percent of cases will develop invasive Sec.IO Treatment by rapid freezing with liquid nitrogen,

Squamous cel l carcino m a

cauterization, topical 5-fluorouracil or surgical excision

Squamous carcinoma results from malignant change in the epidermal keratinocyte and has an incidence of 100,000 population in the

are

rapidly

increasing,

UK/

in

36 per

provides reasonable control although recurrences are common.

[""*]

although case numbers

particular,

in

Australia.8

Epidemiological data and aetiological factors are similar

Basa l ce l l carcinoma

to Bee but exposure to hydrocarbons, particularly tar radiation are

T he commonest skin cancer in white races is BCe and

additionally implicated in see pathology. Proliferation

and mineral

oils,

thermal

factors

and

recent figures indicate maximal incidence in Australia

of neoplastic cells generally arises in damaged skin

with 726 cases per 100,000 population.ll It is a neoplastic

including scar tissue, areas of chronic ulceration and

proliferation of cells that resemble basal cells of the

benign dermatoses

epidermis, although the precise origin is still unclear.

(Figure

134.4).

Furthermore, zones of

active chronic keratosis and Bowen's disease are recog­

While locally invasive, metastases happen in only 0.1

nized sites for potential development of sec and, overall,

percent of cases. 12 Basal cell carcinoma is commoner with

metastases are found in 10 percent of patients.9 Initial

presentation,

as

an

erythematous

[***1**]

plaque,

verrucous growth or domed nodule, is often innocuous but concern arises with subsequent surface crusting, ulceration and bleeding. The lesion is characteristically firm and surrounding tissue is unexpectedly indurated, while in later stages fixation to underlying structures is

advancing

age

and affects

men

more

than

women,

however, current data reveal changing trends with an increasing incidence in younger age groups, women13 and

in Australia, Europe and the USA.14

[**)

The single most important aetiological factor for developing Bee is solar radiation. It is suggested that intermittent excessive sun in childhood is more important

_I __ -_O ___

1706

I PART 13 THE NOSE AND PARA NASAL SINUSES

than cumulative exposure. IS Predisposing phenotypic influences include light skin colour) poor tanning ability) blonde or red hair, freckling in childhood and Celtic ancestry. As would be presumed, southern Mediterranean and black races are relatively untroubled by BCc.16

Acquired immunodeficiency syndrome, immunosuppres­

sive therapy and arsenic exposure are related to BCC formation, particularly on sun-protected sites of the body. Tumours also display a bias for areas of skin damage including vaccination sites, chronic ulcers or burn scars. Studies of inherited conditions that predispose to BCC formation)

i.e.

albinism)

naevoid BCC syndrome or

Gorlin's syndrome and xeroderma pigmentosa, indicate that inactivation of a tumour suppressor gene on the long arm of chromosome 9 may be a significant feature in the genesis of BCc.17

[***]

Thirty-two percent of BCC presents on the nose,

Figure 134.5

Typical nodular BCe.

Figure 134.6

Morpheaform BCe. These are difficult to id enti fy

adjacent cheek and orbit.ls A variety of lesions may be seen and occasionally these are unevenly pigmented.

The

nodular variety is typically a slow-growing dome-shaped papule with a telangiectatic surface and pearly border. The lesion initially appears innocent but fails to heal, then crusts and eventually ulcerates (Figure 134.5). Other nodular forms pursue a relatively innocuous path for much of their clinical course,

presenting as a small

erythematous papule or plaque, a keratotic patch or as a pedunculated lesion resembling a pyogenic granuloma. The superficial type of BCC manifests itself as a scaly plaque with raised edges. In this form, margins tend to be indistinct and several neoplastic foci may reside within the lesion

making

excision

difficult.

Morpheaform

BCC

appears as a white or yellow plaque which develops telangiectasia, sometimes ulcerates and forms a scar-like lesion that is difficult to define and diagnose (Figure

134.6). This group appears exclusively on the face, presents

late and extends deeply. Less commonly, histopathology

and

define.

identifies a basosquamous BCC containing elements of basal and squamous tumour cells, which pursues a more

reconstruction. In general, basosquamous tumours and

aggressive clinical course than BCC alone.

morpheaform BCCs are more aggressive tumours and

[**]

Numerous treatment regimes are employed to establish

require wider excision. [Grade

D]

histological diagnosis and provide complete resolution of the tumour. Intralesional injection of interferon and photodynamic therapy provide good control of BCC, but

Melanocytic lesions

such measures are still not as successful as traditional techniques. Shave biopsy or curettage and cautery can

These may involve the nose and cheek and arise from

adequately remove small lesions but for larger BCC,

alteration in growth patterns of the melanocyte) which

surgery provides the best aesthetic results and radio­

produces a range of benign, premalignant and overtly

therapy may be employed in older patients. A 4-mm

malignant lesions. Recognition and distinction is im­

margin of clear tissue is recommended and excision

portant) as malignant melanoma is eminently treatable

should

when identified early in its natural history.

involve

the

dermal

layer.19

In

aesthetically

important sites) such as the nasal tip, primary closure may not be possible and local flap repair) free grafts or temporal flaps may be required. Moh's surgical technique

of sequential tumour excision under frozen section control is appropriate for tumours in difficult areas such as the medial canthus. This allows precise excision of the tumour mass and conservation of normal tissue for

Melanocytic naevi are a heterogeneous group that include congenital and acquired lesions. Congenital naevi appear at birth but also include early-onset lesions that are histologically similar and appear in the first five years of life. Initially pale, they enlarge) darken and develop terminal hairs. T he giant form is usually present from birth and may reach a size of 20 cm. It is particularly

Chapter 134 Conditions of the external nose I

1707

disfiguring and malignant transformation happen in 4-6 percent of cases. 20 Smaller forms are prevalent on the face and differ in that malignant change is less common and tends to arise later

during adolescence. [**]

Acquired naev i present an

mos tly during childhood and

adult has an average of 20-30 naevi.

2L

Both this

number and the lesions remain stable through life and rarely become

malignant . Naevi result from proliferation Cells

of melanocytes at the dermo-epidermal junction.

remainin g at this location , junctional naevi, appear as dar k

brown

macules

with

spots

of

black

pigment.

solely in the dermis, form

Intradermal n aev i, situated

macules, papu les or wart-like growths that may or may not be pigmented. Compou nd naevi are composed of junctional and dermal elements and result in raised

brown lesions. In childhood, a rapidly growing red papule or nodule on the face is a spindle or Spitz naevus. This is entirely benign but histologically tends to be con fused with early

malignant

melanoma. [**]

Atypical naevi are a group of lesions that are unevenly pigmented, larger and more irregular than the usual acquired naevi. They are associated with a degree of inflammation

and

analysis

reveals

features

that

are

uncharacteristic of benign growths. In a familial setting these lesions are linked to a high risk for

m al i gnan t

melanoma, but in sporadic cases there is a lesser, although st ill significant, chance of sinister t ransforma tion.

Figure 134.7

Superficial malignant melanoma with varying

colour and irregular outline.

horiz on tal growth phase and close inspection reveals a variety of hues w ithin the lesion, i.e. black, blue, brown and white. Nodular types are rare on the face and appear as a red nodule with a marked vertical growth pattern. These are deep tumours that grow rapidly and have a poor prognosis. Lentigo melanoma is found in older patient s, often on the face, as a flat brown-black patch which extends horizontally for months to years. Nodule formation

heralds

deep

invasion

conve r sion. Histological

diagnosis

is

requir ed

malignant

and and

SUSpICIOUS

lesions 1-2 em in diame t er should be excised with a clear circumferential

MALIGNANT MELANOMA

Malignant melanoma is the leading fatal illness arising in skin and its incidence is rising faster than any other neoplasm. Mean age at presentatio n is in the sixth dec a de although an increasing number of cases are presenting in younger age groups. There is a female prepo n derance and the most important aetiological factor is intense inter­ mittent sun exposure

in

fair-skinned individuals. A

mal ignant

treatment.

ma rgin of 1-2 mm The depth of the .

melanoma is essential for planning further

Confirmed lesions less than I-mm deep can be

removed with a l-cm margin of normal tissue. For lesions greater

than l-mm deep) opinion is divided and trials are Patients

currently comparing excision margins of 1-3 cm.

must be fully examined for related disease which will involve other treatment mo dalities.

positive family h i story is identified in 2-5 pe r ce nt of individuals. Pathologically,

malignant melanoma is charac terized by

malignant melanocytes invading the dennis, and the depth 22 of the lesion is the main prognost ic indicator. Lesions

Kaposi's sarcoma Kaposi's sarcoma (KS) is a rare tumour, possibly arising

less than 0.76-mm th ickn es s have a 100 percent five-year

from lymphatic endothelial cells) and consis ts of several

survival which decreases to 80 percent for melanomas loS-mm deep.23 Growt h is horizontal

epidemic spread of this tum our among young male

and vertical, however, increase in lesion height indicates

homosexuals was recognized as part of a new disease - the

between 0.76 and

enhanced invasive potential and decreased import ant

survival.

[**]

subtypes each runni ng a distinct clinical course. The

acquired immunodeficiency syndrome (AIDS). Evidence

malignant

suggests that a sexually communicated infectious agent

melanoma from other lesions are asymmetry, irregular

cotran sm itted with the HIV causes this form of KS?4

The

features

dist inguishing

outline, varying colour and a diameter more than 1 cm. Tn addition, pruritus, bleeding and inflammation should not

be ignored in suspicious cases. Malignant melanoma may not present as a pig mented lesion and less comm only can involve mucosal surfaces such as the nose. S everal forms exi st and the superficial spreading type is

Recently, human herpesvirus-8/KS-associated herpes virus has been postulated for this infective role.25 Human

immunodeficiency

[***1*]

virus-related

KS

com ­

monly presents on the feet and occasionally involves the head and neck region, p art icularly the ears and nose. Early lesions appear as small pink or red macules, or

main variant to involve the face (Figure 134.7).

brown papules. These progyes s to multiple violaceous

Commencing as a brow n macular lesion it exh ibits a long

nodular lesions, which subsequently coalesce and are

the

___

--

_.1 __ �.-

1708 I

PART 13 TH E NOSE A N D PARANASAL S I NUS ES

aesthetically unacceptable. Diagnosis is usually obvious but biopsy may be required. Ionizing radiation, poly­ chemotherapy and antiretroviral medication are em­ ployed and produce variable long-term benefits. Vascu l a r l es i o n s

This is a heterogeneous group that includes neoplastic lesions and abnormalities of vascular structure. Capillary haemangiomas are hamartomas and most commonly arise on the head and neck, affecting 2.6 percent of all live births. They are noted soon after birth as pink to red macular lesions that rapidly increase in size. The lesion becomes raised, papular or polypoid and takes on a darker hue appearing dark red or purple. Tumours then enter a quiescent phase and subsequently regress with 70 percent disappearing by the age of seven. Treatment is rarely required unless important structures are compromised. In such cases, or when tumours persist, surgery is the most effective therapy. Cavernous haeman­ giomas are less common than their capillary counterpart and consist of large dilated venous channels or sinusoidal blood spaces. Lesions lie deeper within the dermis and appear during the first months of life as an ill-defined, smooth bluish-red growth. Rapid expansion ensues but involution is unfortunately much less common. In addition, cavernous haemangiomas may be linked to certain congenital conditions, i.e. Klippel-Trenaunay­ Weber or Maffuci's syndromes. Treatment, whether it be surgery, radiotherapy or cryotherapy is difficult and the result often disappointing. Pyogenic granuloma can involve the nose, particularly in children, young adults and pregnant women, present­ ing as a dark red polypoid and lobulated mass of newly formed capillary vessels that may reach several centi­ metres in size. Its precise aetiology is unclear although trauma and infection have been suggested to play a role. The granuloma grows rapidly, ulcerates and eventually crusts, suggesting a malignant process, however the histology is entirely benign. Although involution happens, particularly post-partum, surgical excision or curettage and cautery are usually required. Naevus flammeus is a vascular abnormality causing dilation and swelling of areas of skin and presents in two forms. The 'stork bite' or 'salmon patch' is a vascular coloured area sometimes located on the glabella but more usually on the nape of the neck. It is extremely common and those involving the nose mostly resolve in the first year. The more unusual and unsightly 'port-wine stain' encompasses the nose and adjacent cheek in a distribution that follows the trigeminal nerve. Commencing as pink patches the lesion persists, darkens to a deep violaceous colour and later becomes nodular, adding further to the aesthetic disfigurement. In addition, some cases are associated with cerebral vascular abnormalities as in the Sturge-Weber syndrome. Treatment is difficult and the

usual modalities are employed with limited success. Surface tattooing may help and while the introduction of the argon laser has improved on previous results it is still not a complete answer. Hereditary haemorrhagic telangiectasia, or Rendu­ Osler-Weber syndrome, is an autosomal dominant condition characterized by abnormal small vessels located in the skin, mucous membranes and viscera. The vessel abnormality occurs in different forms which generally present at about adolescence with spontaneous haemorrhage. Regarding the nose, the most usual problem is epistaxis but inspection of adjacent skin may reveal the characteristic petechiae. Pulmonary arteriove­ nous fistulas are common and gastrointestinal involve­ ment is to be expected. Treatment is aimed at managing bleeding episodes or anaemia, and electrocauterization or laser therapy of individual lesions provides cosmetic benefit.

Fibroa d n exa l tu m o u rs

These tumours arise from supporting subcutaneous connective tissue structures. In themselves they are rarely important but may mimic neoplastic pathology or present as unsightly blemishes. Few are exclusive to the nose and they are generally classified after diagnostic eXCISIOn. Dermatofibromas are partially pigmented, keratotic lesions that reach sizes of up to 1 cm and generally persist through life. Histology reveals spindle cells and such growths belong to the benign fibrous histiocytoma group. A common lesion, usually on the side of the nose in elderly people, is the fibrous papule. They appear in groups as small flesh-coloured 5-mm growths. Once growth is completed, the lesions remain stable but tend to persist. Trichoepitheliomas are a rare entity which present as small, rounded pinkish nodules on the cheeks, eyelids and nose. They are entirely benign but tend to increase in size and number and sometimes form abortive hairs. Differentiation, clinically and histologically from BCC can be difficult although the presence of hair-like structures is reassuring. Angiofibromas are small flesh-coloured or reddish­ brown dome-shaped papules 2-3 mm in size. These diagnostic lesions are located on the cheek and side of the nose, particularly in the nasolabial crease. They form part of an autosomal dominant condition known as tuberose sclerosis that may exist with seizures, mental retardation, cardiac and renal abnormalities. Family members require examination and genetic counselling. Cysts

Cutaneous' cysts are located either intradermally or within the subcutaneous tissues and present in two main forms.

1 709

Chapter 1 34 Cond i tions of the external nose I

Epidermoid

are

cysts

by

lined

stratified

squamous

KEY

ep ithelium and contain keratin debris. Men are mostly affected and such cysts generally a ppear a fter puberty. In

some

with

prepuber tal

i n testina l

maJ ignant

cases

there

is

th a t

h ave

the

polyps

cha nge,

a

an

potential

known

condition

•

association

as

for

[n

Dermoid cysts are rare and often p rese n t from birth

•

in size

and p ossibly originate fro m incl usion of epidermal tissues embryonic

development.

•

A

presumed dermoid cyst over the nasal roo t sh ould be treated cautiously as th is may represent a nasal glioma or

•

meningoencephalocoele with potential co m m unica tion to the cranial cavity. Imaging and needle asp i rat io n o f the

of the les ion

mass are necessary to confi rm the sou rce before proceeding to excisi on. Milia are small versions

1 -2-mm cys ts th at are mlma ture

o f epidermoi d

cysts and

are

reco gn i zed

trauma,

cond i tio ns

_: ,­

pa t ients.

•

Bee is

•

Bee is

•

Nose

the commonest skin cancer, locally i nvasive but rarely

a nd midface a r e common sites [o r Bee. Di fferent clinka J form of Bee -p r,est.'flo t and diagnosis can be di fficult

•

dermabrasion,

burns or bullous disease. Treatment involves shelling o u t

... �!

the cyst with the tip o f a hypodermic needle. A s they present in considerable numbers, this is a t ime-consu m ­

Melanocytic lesions

ing process.

•

Mel a nocyt ic lesions present i n nu merous

face and trunk. Multiple tumo urs o f the face with a

•

similar histology are found in dark-skinned races but are

-.

Ve rtical groWth is

Sebo rrhoeic keratoses

•

These are benign tumours which p redo minately a ffect

•

elderly people and arise from an increase o f normal keratinocytes in the epidermis. Males a nd fe males a re equally affected and lesions are commoner in wh ite races.

•

They present in any location but are most frequent on the

in younger

a ge

asymp tomatic, Typically, the

groups. however,

These

keratoses

itching

ca n

be

are

m os tly

a

feature.

/.

-.

-. . � :� . -

fo rm s . Recogni t i o n is i mport ant t o identi fy potcnt i ally neoplast ic lesions. Congenital naevi have the po t en t.i al fo r ma l ignan t change. Acquired naevi develop mostly d u r i n g c h i l d h ood and rem ai n s t a b l e d u ri ng ad ult life. Malig.nant cha nge is r -a re. MaJ i gn an t melanoma is t he leading fatal skin dis�ase. Neo p �as ti,c growth is horizontal and vertic�,

known as dermatosis papula n igra, and generally appear

/ ,

metasta� izes.

as

smooth white globules around the fa ce. They appear spontaneo usly but can follow

these

Lesions.

lesion

appearing on the nose as a midline mass anywhe re fro m

during

t he i n c i d e n ce of

'

va riety of

Keratoacanthoma is a ra p idly growi ng benign ' that s ubsequentl.y resolves. It may r m i m i c Sec. Lo ng-term s u n exposure can produce spec.ific ' 'ac t i n ic' cutaneous change and I percen t of , caS(1S proceed toO frank squ a m o us maligna ncy'- " Squamous carcinoma usually a 6ses in sun­ d a m a ge d skin and metas t as i zes i n 1 0 pe rce n t of cases. I n t raepidermal sec, Bowen's disease, becomes frankly invasive i n 10 perce n t of

•

prevent recurrence.

sites

general,

a

a l ign a n t

is increasing.

syndrome. Su rgical excision of the ep idermoid cyst is

aberrant

m

ben i g n , premal i gna n t and

often required and the entire cyst wall is rem oved to

in

with

Solar exposu lie is associated

Ga rdner's

the glabella to columella. They rarely exceed 4 cm

..' ..

POI NTS

" progn�:)sis� ' -

�

associa,��d Wi�h poorer .

.

.

:,

}

_.

"

The n a t u re of the lesion d ictates the extent of

>- F o r m a l i g n a n t co nd itions va rious treatment m o d a l ities

Cotra n RS. M etastasisi ng basa l ce l l carc i n o m a s. Cancer. 1 9 6 1 ; 1 4 : 103 6-40.

1 3.

a n d preservi ng a esthetic i n teg rity. resection a n d the m a rg i n of hea lthy tissue that m ust

M a rks R, Sta p l es M, G i les CG. Tre nds in n o n - m e l a nocytic skin ca n ce r t reated in Austra l ia : the second n a t i o n a l

the ski n . British Journal of Cancer. 1 9 9 6 ; 73 : 1447-54. 1 6.

In fa cia l p l astic s u rg e ry, surg i c a l exce l l e n ce req u i res the knowledge a n d expe ri e n ce that e n compasses a ra n g e of spec i a l ities i n c l u d i n g oto rh i n o l a ry n g o l ogy,

H o g a n DJ , To T, G ra n L, Wong D, La ne PR. R i sk factors for basa l ce l l ca rci n o m a . International Journal of Dermatology. 1989 ; 2 8 : 591 -4.

17.

dermatol ogy, oncology a n d p l astic s u rg e ry.

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R oe n i g k R K, Ratz J L, B a i l i n PL, Whee l a n d RG. Trends i n the p resentation a n d treatment of basa l ce l l carc i n o m a s.

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Wa l ley J, Porter J. Chemoprophyla xis in tu bercu l osis a n d

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d iseases and vascu l itis in the nasa l m u cosa. I n : Rya n TJ

n aevi in a hea lthy B ritish population. British Journal of

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Roo k A, Champion RH. Keratoacanthoma. M o n og ra p h 1 0. M a rks R , Fo ley P , G ood m a n G , H a g e B H , Sel wood TS.

m e l a n o m a . Annals of Surgery. 1970 ; 1 72 : 902-8. 23.

of the extrem ities: a c l i n i copath o l o g i c study usi n g levels

co nse rvative m a n a g e m e nt. British Journal of Dermatology.

of i nvasion ( m i c rostage). Cancer. 1 97 5 ; 3 5 : 666-76. 24.

Roberts D L. I n cidence of non-melanoma s k i n ca ncer i n Dermatology. 1990; 1 2 2 : 3 9 9 -403.

t ra nsm itted i nfecti o n . Lancet. 1 9 9 0 ; 3 3 5 : 1 2 3 -8. 25.

Moore PS, Cha ng Y. Detection of herpes vi rus- l i ke

M a rks R, Sta ples M, G i les GG. Trends in n o n - m e l a n ocytic

seq u e n ces in Ka posi's sarcoma in patie nts w i t h a n d

skin ca n ce r t reated in Austra l i a : The seco nd nation a l

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su rvey. International Journal of Cancer. 1993 ; 53 : 585-90. 9.

B e ra l V, Pete rm a n TA, Berke l m a n R L, Jaffe H W. Ka pos i 's sa rco ma a m o n g persons with AI DS - a sexua l ly

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W a n e bo HJ, Wood ruff J , Fort n e r J G . M a l ig n a nt m e l a n o m a

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B reslow A. Thickness, cross-sectio n a l a reas and depth of i nva sion i n the prog n osis of cuta neous m a l i g n a nt

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M a cKie R M , E n g l ish J, Aitch ison TC, Fitzs i m o n s CP, Wi lson

Ka n a n M W, Rya n TJ. The l oca l isation of g ra n u l o m atous (ed.). Microvascular injury. Lon don : WB Sa u n d e rs, 1 97 6 :

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Wa rd G E , H e n d rick JW. Res u l ts of treatment of ca rci n o m a of the l i p. Surgery. 1 9 50; 27 : 3 2 1 -42.

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S� rtw e l l PE. Raci a l d iffe re n ces in sa rcoidosis. Annals of the Ne w York Academy of Sciences. 1 97 6 ; 27 8 : 368-70.

Chapter 134 Con d itions of the exte rn a l n ose

* 27.

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Kerd e l FA, M osch el la S L. Sa rco id osis ; a n u pdated review.

1717

S nyder 3 rd G G , M cCa rthy R E , Too m ey J M , Rothfi e l d N F.

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l'Jasa I se pta I perforation i n system ic I u pus eryt h e m atosus.

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B u rg e SM, Frith PA, M i l l a rd PR, Woj n a rowska F. M u cosa l

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Log a n RA, G riffiths WAD. C l i m atic factors a n d rosa cea . I n :

i nvolve m e nt in syste m ic a n d c h ro n ic cuta neous l u p us

Ma rks R , P l e w i g G (eds). Acne and related disorders.

eryt h ematosus. British Journal o f Dermatology. 1 9 89 ; 1 2 1 :

Lo n d o n : D u n itz, 1 9 89 : 3 1 1 .

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3 5.

I

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Ja nsen T, P l ewig G. Rosa cea : c l a ssification a n d treat m ent.

De B e rker D, B u rg e S , D issa n eyeka M . Th e seq u e l a e of

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ch ro n i c cuta neous l u p us erythematosus. Lupus. 1 99 2 ; 1 :

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M i l l a rd LG, Rowe l l I'J R . Abnormal l a boratory test res u l ts a n d t h e i r re lationsh i p to pro g n osis i n d i scoid l u pus eryt h e m atosus. Archives o f Dermatology. 1 9 7 9 ; 1 1 5 :

FU RTH ER READ I N G

1 055-8. 31.

Rowe l l N R . La boratory a b n o rm a l ities i n the d i a g n osis a n d m a na g e m e n t o f l u p u s erythematosus. British Journal o f Dermatology. 1 9 71 ; 8 4 : 2 1 0- 1 6.

* 32. 33. 3 4.

* B u rget GC, M e n i ck FJ. Aesthetic reconstruction of the nose. St. Lou i s : Mosby-Yea r Book, 1 994.

* C h a m p i o n R H , B u rton J L, B u rns DA, B reath nach S M (eds). Rook/

Wa l la ce DJ , H a h n BH (eds). Dubois' lupus eryth ematosus.

Wilkinson/Ebling textbook of dermatology. Oxfo rd : B l ackwe l l ,

P h i l a d e l p h i a : Lee a n d Feb i g e r, 1 99 3 .

1 988.

Love LA. l'J ew e nvi ro n m enta l a g e n ts associated w i t h

* Freed berg 1M, Eisen Al, Wolff K, Fra n k Austen K, G o l dsm ith LA,

l u pus- l i ke d isorders. Lupus. 1 994; 3 : 467-7 1 .

Katz S I et al. (eds). Fitzpa trick's Dermatology in general

Tuffa n e l l i D L, D u bois EL. Cuta neous m a n ifestations of

medicine. l'J ew Yo rk; Lo n d o n : McGraw- H i l i , 1 999.

syste m ic l u pus erythematosus. Archives of Dermatology. 1 9 64 ; 9 0 : 3 7 7-86.

1 35 The diag nosis and managem en t of fac ial pa i n

TI M J WOO LFO R D AN D N I C H O LAS S J O N ES

I n trod u cti o n

1 71 8

Te nsi o n -type he adache

1 724

Backgro u n d

1 71 8

M i dfa ci a l se g m e n t p a i n

1 72 5

H istory ta king i n faci a l p a i n

1 71 9

Atyp i c a l faci a l p a i n

1 72 7

R h i n olog ical pa i n

1 720

Key points

1 727

De n t a l pa i n

1 7 21

Best c l i nical pract ice

1 7 27

Vascu l a r p a i n

1 722

Defi ciencies i n c u rrent know ledge a n d a reas fo r fu t u r e

N e u ra l gias

1 7 24

Pa i n caused by t u m ou rs

1 724

1 7 28

resea rc h

1 728

References

This chapter is based u p o n a Medline search using the key words facial pain, sinusitis, paranasal sinuses, migraine, cl uster headache, neu ralgia a n d focu sin g on diagnosis, sin us s urgery and management.

I NTRO D U CTI O N

facial p ain and headaches are usually caused by sinusitis. The fact that there is an ENT sign in outpatients does not

Patients

with

facial

pain

are

frequently

refer red

to

limit the p resenting conditions to this sp ecialty. If all cases

otorhinolaryngologists. Most patients arrive in the clinic

of facial pain are assumed to be sinogenic, those with

with an i nitial diagnosis of si nusitis, although in reality

other conditions

few of these patients have si nogenic pain. This chap ter

ment and many patients

aims to assist the reader in the accu rate diagnosis of facial

inappropriate surgery.

p ain and the management of this co ndit ion.

will not receive correct medical treat­ will continue to be subjected to

The majority of p ainful stimuli from the face are transmitted to the spinal tract of the brain stem via afferents in the trigeminal nerve. Pain afferents from the

BACKG ROU N D

VIIIth, IXth and Xth cranial nerve also relay in the spinal

Reaching the co rrect diagnosis i n patients with facial pain

poorly localized ache because the afferen t nerves inner­

is challenging because many patients come with fixed

vating them are unmyelinated. Facial skin is supplied by

tract. Facial pain fro m deep structures tends to be a dull ,

A

ideas about the cause of their pain. The majo rity are

myelinated

convinced they have sinusitis, a not unreasonable view

neurophysiological m odel has been proposed by Olesen I

because this diagnosis has o ften been co nfirmed by their

to explain the interaction of the various nociceptive

general practitioner o r other specialists. A proportion of

n erves

that

p roduce

a

sharper

inputs causing tension headaches and migraine

pain .

and this

patients have already undergone unsuccessful nasal or

can use full¥ be extended to help in the understanding and

sinus surgery and this reflects the widely held view that

diagnosis of fa cial pain. This theory describes central

Chapter 135 The diagnosis and management of facial pain I 1719

sensitization of the trigeminal nucleus by myofascial, vascular or supraspinal input. It is postulated that an imbalance between these various inputs causes pain. Psychological influences that reduce supraspinal inhibition of the trigeminal caudal nucleus may either increase pain from established disease or provoke pain in the absence of disease. [H/*] To add to this, surgery, trauma and inflammation may promote nociceptive stimulus of the trigeminal caudal nucleus and initiate pain. To manage patients with facial pain effectively, the psychological aspects of this condition must be considered, and in some patients these may be an important factor. A good example is a patient with facial pain who has previously sustained a nasal injury during an assault, without resolution of the psychological insult. The pain is more likely to persist if there are ongoing compensation or legal issues that are outstanding. Similar problems can arise after rhinoplastic or other sinonasal surgery, particularly where the patient is dissatisfied with the outcome. For some patients facial pain is an expression of their emotional distress or anxiety, and in some patients their continuing pain may enable them to obtain attention or secondary gain. The pain is very real to the patient and an empathetic approach is essential. [H/*]

•

•

•

HISTORY TAKING IN FACIAL PAIN In common with many areas of medicine, the key to a correct diagnosis in patients with facial pain is taking an accurate history. It may not be possible to reach a definitive diagnosis at the first visit, and retaking the history at a subsequent consultation with the patient's diary of their symptoms will often clarify the situation. In making a diagnosis it is helpful to classify facial pain into broad categories, namely rhinological pain, dental pain, vascular pain, neuralgias, pain caused by tumours, midfacial segment pain and atypical facial pain. Although these groups mix both anatomical and pathological criteria, a structured approach to history taking remains a key ingredient to making a diagnosis. A practical approach will be described, stressing the need to think beyond the sinuses. It is useful to have a mental algorithm to plan questions and to use these to work through a diagnostic checklist of possible conditions. 2 Below is a guide to general history taking, with more detail given in the sections relating to each of the specific conditions. In reality, many patients do not fit neatly into one category and judgement without the benefit of a firm base of evidence is required as to which treatment offers the best chance of success. • Where is the pain and does it radiate anywhere? Asking the patient to point to where the pain is most severe is usefuL Often this will differ from

•

•

information contained in the referral letter. So-called 'sinus' pain may be headache or pain from the cervical spine or temporomandibular joint (TMJ). Bilateral facial pain is commonly midfacial segment pain. Sinogenic pain may be unilateral or bilateral, whereas migrainous pain and TMJ dysfunction tend to be unilateral. The manner in which a patient outlines their pain, and the gestures used, can inform the examiner about the emotional significance of the symptom. Is the pain continuous or intermittent? Pain described as continuous or present on a daily basis is unlikely to be sino genic or migrainous in origin, and more likely to represent midfacial segment pain or atypical facial pain. Migraine often happens with increased frequency premenstrually, and cluster headaches do indeed cluster, with patients often having weeks or months of remission. Pain that is constant, predominantly unilateral, and particularly if it is progressive, can be due to a tumour and this possibility must be considered. Character of the pain? Vascular pain tends to be throbbing in nature, with cluster headaches being particularly severe. Midfacial segment pain is often described as pressure or a band -like pain. Trigeminal neuralgia causes a stabbing pain that is initiated by a trigger point. What precipitates or is associated with the pain? Sino genic pain is associated with the rhinological symptoms of nasal obstruction and infected catarrh, and tends to be worse with upper respiratory tract infections. Vascular pain may have a preceding aura and is often associated with nausea. Trigger factors, such as certain foods, withdrawal of stress and sleep disruption, are well recognized. Cluster headaches are frequently triggered by alcohol and wake the patient. The pain of TMJ dysfunction is exacerbated by chewing and trigeminal neuralgia provoked by trigger points. What relieves the pain? Patients with facial pain due to sinusitis generally respond to medical treatment such as antibiotics. Although midfacial segment pain initially responds to simple analgesics, patients with this condition often report that these drugs are unhelpful by the time they are referred for a specialist opinion. Patients with migraine will retreat to a darkened room and lie down to help cope with t~eir symptoms. What effect does the pain have on daily life? Patients with atypical facial pain often describe their pain in dramatic detail as severe and unrelenting despite sleeping well and living a relatively normal life. In contrast, some patients with this condition are unable to work and blame the pain for a breakdown in a close relationship. Severe crippling pain that wakes the patient, often a man, is typical of cluster headache. [H/*]

1720 I

PART 13 THE NOSE AND PARANASAL SINUSES

RHINOLOGICAL PAIN Sinusitis The majority of patients who present to an otorhinolaryngology clinic with facial pain and headaches believe they have 'sinus trouble'. Chronic sinusitis can cause facial pain, particularly during an acute exacerbation, although this is overdiagnosed. The symptoms of acute sinusitis are well recognized. Typically, there is nasal obstruction, infected catarrh and facial pain of variable intensity in association with an upper respiratory tract infection. Chronic sinusitis is however often painless, with pain only present during acute exacerbations. This pain is often a unilateral dull ache around the medial canthus of the eye, although there may be more severe facial pain with maxillary toothache. An increase in severity of the pain on bending forward is traditionally thought to be diagnostic of sinusitis but this is nonspecific as many types of facial pain and headache are made worse by this action. The key points in the history of sino genic pain are an exacerbation of pain during an upper respiratory tract infection, an association with rhinological symptoms and a response to medical treatment. Examination of the face is often normal in patients with chronic sinusitis. Facial swelling is usually due to another pathology such as dental sepsis or, more rarely, malignancy. Nasendoscopy is mandatory in the diagnosis of sinusitis. A normal nasal cavity, showing no evidence of middle meatal mucopus or inflammatory changes makes a diagnosis of sino genic pain most unlikely, particularly if the patient is currently in pain or had pain within the past few days. Even the presence of inflammatory changes or infection does not indicate with any certainty that the pain is sinogenic, and all findings must be considered in conjunction with the history. On occasion it is useful to review the patient and repeat the nasendoscopy when they have pain to clarify the diagnosis. Figure 135.1 shows normal middle meatus viewed endoscopically. It is extremely unlikely that this patient's facial pain would be due to paranasal sinus disease. Figure 135.2 shows middle meatal sepsis viewed endoscopically. The issue of imaging continues to cause controversy. Plain sinus x-rays are used to confirm a diagnosis of acute bacterial sinusitis; however, the poor sensitivity and specificity of sinus x-rays make them redundant in the diagnosis of chronic sinusitis. 3 ,4 Interpretation of the appearance of the sinuses on computed tomography (CT) scans must also be treated with caution. Approximately 30 percent of asymptomatic patients will demonstrate mucosal thickening in one or more sinuses on CT scanning. The presence of this finding is certainly not an indication that pain is sinogenic in origin or that surgery is indicated. 5,6 Several other clinical observations suggest that a diagnosis of sinusitis causing chronic facial pain should

Figure 135.1

Normal middle meatus viewed endoscopically.

Middle meatal sepsis viewed endoscopically. This patient could well have facial pain. Figure 135.2

only be made after careful consideration. Chronic rhinosinusitis is common in young children and invariably resolves as the immune system matures. Children very rarely complain of facial pain, even in the presence of florid rhinosinusitis. One must also remember that rhinological symptoms are common, affecting perhaps one-fifth of the adult population. These patients suffer from rhinosinusitis due to various conditions including allergic rhinitis and conditions associated with nasal polyps. It is again notable that the vast majority do not complain ~f significant pain? Headaches are also common in the general population and linking these with unrelated

Chapter 135 The diagnosis and management of facial pain

nasal symptoms can lead to an incorrect diagnosis of sinusitis. Vascular pain is frequently associated with autonomic rhinological symptoms, such as congestion and rhinorrhoea, leading to diagnostic confusion. 8 The management of rhinosinusitis is considered in Chapter 117, Surgical management of rhinosinusitis however, in the current context it is important to stress that surgery should only be considered in the minority of patients where there is good evidence they have sino genic pain and when medical treatment has failed. It is interesting to note that many patients who undergo inappropriate surgery for nonsinogenic pain experience temporary relief from their symptoms, although the pain normally returns. In some patients, surgery does not significantly affect the pain and in a few the pain is made far worse. 8 , 9 Patients whose pain is increased by surgery are subsequently particularly difficult to manage with medical treatment. [***1**1*]

I 1721

produce a sharp, well-localized pain often caused by a lost or cracked filling. Once the periodontium is involved the pain becomes localized to the area of the affected tooth, which throbs and is tender to percussion. [**]

Phantom tooth pain This pain follows a dental extraction although on enquiry there is often a history of some pain that preceded the extraction. Although necrotic bone and neural elements have been found in some patients with this syndrome, the majority of cases represent a form of atypical facial pain. A dental extraction has often been performed following pressure from the patient who is convinced they have diseased teeth. Such patients may eventually find their way to otorhinolaryngology clinics for the management of 'sinus pain', and unfortunately, many subsequently undergo equally inappropriate sinus surgery. [*]

Pain after trauma or surgery Temporomandibular joint dysfunction A small minority of patient's who have sustained nasal trauma or have undergone surgery continue to experience pain long after the direct effect of the injury or surgery have settled. 8,9 There is usually little abnormality on examination and different theories exist about whether the alteration in the neurological pathway is central or peripheraL These patients often suffer from psychological distress following injury or are dissatisfied with the result of their surgery. This problem seems to be particularly common following cases of assault, and ongoing litigation may have a bearing on how symptoms are reported. Caution is required in the management of such patients, especially if further surgery is being considered. Correction of cosmetic abnormalities may not alleviate the pain and the psychological status of such patients must be assessed carefully. These patients often fall into the category of atypical facial pain (see later under Atypical facial pain) and should be managed accordingly. [**1*]

DENTAL PAIN

The pain of TMJ dysfunction may be localized to the joint or extend over the periauricular area extending to the temporoparietal and cervical regions. Temporomandibular joint dysfunction may also present with deep otalgia. 10 In the majority of cases the pain is unilateral and while chewing often exacerbates the symptoms, clicking is a nonspecific sign. Poor dental occlusion with a crossbite or poorly fitting dentures may be implicated in TMJ dysfunction. Examination reveals a tender TMJ on direct palpation or lateral jaw movement. The muscles surrounding the joint also may be tender, and pain caused by palpation of the lateral pterygoid muscle insertion at the posterior end of the upper buccal sulcus is said to be a specific sign of TMJ dysfunction. In practice, the majority of patients with TMJ dysfunction diagnosed in an otorhinolaryngology clinic are best managed by oral and maxillofacial surgeons. Treatment involves correction of aggravating factors and resting the joint by avoiding yawning and prolonged chewing. Occlusal devices such as bite-raising appliances often help. [**]

Painful teeth While many patients attending oral and maxillofacial surgery clinics have facial pain originating from the teeth, patients with pain attributable to this cause are rare in otorhinolaryngology. Usually the diagnosis is clear as the offending tooth is painful to percussion. However, pain originating from the dental pulp may produce poorly localized pain causing misdiagnosis. This pain rarely crosses the midline but can radiate to surrounding structures and, in particular, the opposite jaw (e.g. maxilla to mandible). In contrast, dentino-enamel defects

Myofascial pain This condition is five times more common in postmenopausal women and has a strong association with stress. It has many features in common with TMJ dysfunction. A widespread, poorly defined aching in the neck, jaw and ear with tender points in the sternomastoid and trapezoid muscles may be initiated by malocclusion or poor deltopectoral posture. Treatment is along similar lines to TMJ dysfunction. [**]

1722 I

PART 13 THE NOSE AND PARANASAL SINUSES

VASCULAR PAIN Migraine Migraine is a common condition affecting approximately 6 percent of men and 18 percent of women. 11 The classical variety accounts for 25 percent of migraine cases and there is often a family history of the condition. In classical migraine an aura precedes the onset of headache and this may include visual disturbances, such as fortification, scotomata (blind spots within the field of vision) or visual field defects. The aura may also include unusual tastes and aromas. The headache is usually a throbbing, unilateral pain although rarely it may be bilateral and constant. Even if the pain is not typically migrainous, patients will normally have nausea, photophobia or phonophobia (sensitivity to sound).12 Figure 135.3 shows the features of migraine. As the name suggests, common migraine happens more frequently than classical migraine, accounting for 75 percent of cases. Patients with common migraine have the same throbbing headache and nausea but do not experience the visual aura or other neurological symptoms. Migraine can last up to 72 hours.13 Migraine attacks can be induced by a number of trigger factors including various foods, sleep disturbance and withdrawal of stress (,Saturday morning headaches') and premenstruation. Not infrequently there is an association of migrainous headaches with a woman's menstrual cycle and this finding may point to the aetiology of the pain. Management of migraine involves avoidance of trigger factors and medical treatment aimed either at the acute

episode or prophylactic treatment. Simple treatment in the acute phase includes aspirin and antiemetics. Serotonin agonists, such as sumatriptan and rizatriptan, are frequently effective in treating acute attacks. Betablockers or pizotifen are usually the first-line treatment for prophylaxis provided there are no contraindications to their use. Prophylactic treatment is considered if symptoms happen more than three times a month with a duration of more than 48 hours. 13, 14 Referral to a neurologist is recommended for refractory cases. [****/***] [Grade B]

Cluster headache Cluster headache typically affects men between 30 and 50 years old. The term cluster headache is something of a misnomer as the pain is commonly frontal, temp9ral, extends over the cheek or even into the teeth. Frequently, there is lacrimation, rhinorrhoea and nasal obstruction, symptoms that have led to misdiagnosis of sinusitis. A temporary Horner's syndrome has been described on the side of the pain. IS, 16 Patients describe a distressing, throbbing, unilateral pain that wakes them. In extreme cases patients feel like 'banging their head against a wall'. The pain may last for 15 minutes up to two hours. Clusters of attacks often continue for several weeks followed by months or years of remission. Alcohol should be avoided during a cluster period and treatment is along the same lines as migraine with triptans for acute attacks and pizotifen to prevent recurrent clusters. Figure 135.4 shows the features of cluster headache. [****/***] [Grade B]

Piercing

Supraorbital Facial Frontal Parietal

Photophobia Pallor or flushing

Occipital Temporal

Figure 135.3

The features of migraine.

Chapter 135 The diagnosis and management of facial pain

I 1723

diagnostic biopsy if there is a strong clinical suspicion that the condition is present. [***] [Grade C] Burning

o

Piercing

Red eye Watery eye Dry/runny nose

Ptosis

Unilateral Figure 135.4

The features of cluster headache.

Paroxysmal hemicrania This uncommon condition happens almost exclusively in women and causes multiple attacks of excruciating pain in the frontal, temporal or ocular regions. The unilateral pain has several similarities to cluster headaches, lasting 15-30 minutes and happening several times a day. The autonomic symptoms of lacrimation, nasal congestion and rhinorrhoea may cause diagnostic confusion. 16, 17 The first-line treatment is with indomethacin which is usually effective; indeed, chronic paroxysmal hemicrania was originally classified on the basis of a response to this drug. More recently, the overlap between cluster headaches and chronic paroxysmal hemicrania has been recognized with reports of chronic paroxysmal hemicrania responding to sumatriptan. 18 ,19 [***] [Grade B]

Temporal arteritis Although patients with this condition rarely present to an otorhinolaryngology clinic, rapid diagnosis is essential to avoid progression of the disease with involvement of the ophthalmic artery and visual loss. The majority of patients with temporal arteritis are women aged over 50 years with fever, malaise and severe temporal pain. Examination shows the temporal artery to be thickened and exquisitely tender. Investigation reveals an elevated erthrocyte sedimentation rate. The diagnosis is confirmed on histological examination of an arterial biopsy that reveals intimal hyperplasia and fragmentation of the internal elastic lamina. Steroids in high doses (60 mg prednisolone daily) should be commenced prior to a

Siuder's neuralgia and 'contact point pain' In 1908, Sluder20 first described a collective group of neuralgic, motor, sensory and gustatory symptoms and signs including facial pain. His hypothesis was that these clinical findings were caused by inflammation of the sphenopalatine ganglion. This condition is now considered to be of vascular origin, hence inclusion in this section. Sluder named this clinical presentation 'sphenopalatine ganglion neuralgia', although, interestingly, he did not record a case with a combination of all the features he described. In the years that have followed many have accepted 'Sluder's neuralgia' as a discrete clinical entity. Some have also extended his theory of sphenopalatine inflammation to include mucosal contact with the lateral nasal wall as a cause for facial pain, despite the fact that Sluder did not describe the presence of contact points. Sluder recommended that pain should be treated with topical treatment of the sphenopalatine ganglion using cocaine or formaldehyde, followed by an injection of phenol if this failed. He described a variable response to treatment, and blamed the recurrence of pain on disease causing further inflammation of the sphenopalatine ganglion.21 In recent years attempts have been made to manage Sluder's type syndrome with topical, surgical or stereotactic radiotherapy treatment aimed at specific ablation of the sphenopalatine ganglion. 22 , 23, 24 The success of these various treatments has been mixed with many patients only gaining limited or short-term relief. [**/*] A review of the literature also reveals many publications claiming successful treatment of facial pain by surgical correction of contact points pressing on the lateral wall of the nasal cavity. Many of these publications have questionable methodology, with small numbers of patients and various clinical presentations being included. Most report short follow-up periods and no control group. The routine use of the nasendoscope, not available to Sluder and the previous generation of rhinologists, frequently reveals nasal cavity mucosal contact points in the pain-free patient. There is also now research evidence that questions the whole concept of mucosal contact points causing pain. 25 ,26 The finding that mucosa-tomucosa contact within the nasal cavity does not cause pain is not unduly surprising as there is nowhere else in the body where mucosal contact has this effect. [***/**] Septal surgery has also been reported to help headaches in the absence of contact points,27 but few rhinologists would attribute pain to this cause. No pathological theory can be offered to explain this outcome although the placebo effect of surgery is well recognized, as is cognitive dissonance,z8 One possible

1724 I PART 13 THE NOSE AND PARANASAL SINUSES explanation for this is that surgery transiently alters neurological input into the trigeminal caudal nucleus, thereby temporarily relieving pain. This neuroplasticity is further modified by the emotional and psychological stimuli that inevitably surround surgery, particularly where relief of pain is the main objective. [*] The clinical syndrome described by Sluder has several autonomic features in common with vascular pain described previously in this section (see above under Vascular pain), particularly cluster headache. Sluder's neuralgia is now classified as cluster headache. 29 [*]

NEURALGIAS Trigeminal neuralgia The characteristic presentation of trigeminal neuralgia with paroxysms of severe lancinating pain induced by a specific trigger point is well recognized. In more than one-third of sufferers, the pain arises in both the maxillary and mandibular divisions, while in one-fifth it is confined to the maxillary division. In a small number of patients (3 percent) only the ophthalmic division is affected. Typical trigger points are the lips and nasolabial folds, but pain may also be triggered by touching the gingivae. A flush may be seen over the face, but there are no sensory disturbances in primary trigeminal neuralgia. Remissions are common but the condition can also increase in severity. Patients with trigeminal neuralgia, and particularly younger patients, should undergo magnetic resonance imaging to exclude any other pathology such as disseminating sclerosis. The association of disseminating sclerosis with trigeminal neuralgia is well established. Disseminating sclerosis is identified in 2-4 percent of patients with trigeminal neuralgia and in a small proportion of these patients trigeminal neuralgia is the first manifestation of the disease. Patients with multiple sclerosis are younger than the general trigeminal neuralgia population and the pain is more frequently bilateraL 30 Tumours such as posterior fossa meningiomas or neuromas are found in 2 percent of patients presenting with typical trigeminal neuralgia, reinforcing the need for imaging to exclude such pathology.31 Carbamazepine remains the first-line medical treatment, with gabapentin now being employed more frequently. In cases refractory to medical treatment, referral to specialist centres for consideration of other treatment modalities such as microvascular decompression or stereotactic radiotherapy may be appropriate. 32 [****/***]

Glossopharyngeal neuralgia This very rare neuralgia is characterized by a severe lancinating pain in the tongue base, tonsillar fossa and

posterior pharynx. It may be precipitated by swallowing or yawning and the bouts may last for weeks with a tendency to recur. Treatment is along similar lines to trigeminal neuralgia with imaging to exclude any other pathology, including multiple sclerosis. Drug treatment is used initially, with surgery reserved for refractory cases. 33 [***]

Postherpetic neuralgia This is pain following a herpes zoster infection, and is defined as pain recurring or continuing at the site of shingles after the onset of the rash. Up to 50 percent of elderly patients who have had shingles may develop postherpetic neuralgia, although fortunately, most recover during the first year. Antiviral agents greatly relieve the pain of acute shingles, and there is evidence they reduce the risk of subsequent postherpetic neuralgia. 34 Various medical treatments may be helpful, in particular, tricyclic antidepressants, carbamazepine or gabapentin. Referral to a pain clinic is often appropriate for what can be a particularly distressing condition. [**** /***]

PAIN CAUSED BY TUMOURS Although tumours rarely present with facial pain, constant, progressive pain, particularly if associated with other suspicious symptoms or neurological signs should alert the clinician. A past history of malignancy may raise the possibility of metastases. A thorough examination and appropriate imaging is mandatory to exclude the possibility of a tumour. Some lesions, such as a neuroma, can have a long natural history and pain may have been present for several years. Patients with carcinoma of the paranasal sinuses often present with advanced disease. Unilateral blood-stained mucus is a common early presentation. Proptosis, epiphora, facial paraesthesia or swelling and a loose tooth or ill-fitting denture may represent more advanced disease. Pain is usually a late feature.

TENSION-TYPE HEADACHE This is typically described as a feeling of tightness, pressure or constriction, which varies in intensity, frequency and duration, and may be at the vertex or forehead, eyes or temple, and there is often an occipital component. It often lasts many hours and analgesics are of little or no benefit although many patients continue to take them, often in large quantities. It is usually present on waking. Figure 135.5 shows the characteristics of tension-type headache. The subgroup that is referred to an otorhinolaryngologist often have chronic tension-type headache with continuous symptoms or headaches that

Chapter 135 The diagnosis and management of facial pain I

Tender spots

Bilateral pressure/aching

_?-~~~~\,

Q( fdif>, ~

.

1725

,1j//1444~,

,~"

(--. ~)

Figure 135.5

Occipital

are continuous or last for more than 15 days a month. It does not worsen with routine physical activity, and rarely interferes with the patient getting to sleep. Hyperaesthesia of the skin or muscles of the forehead often arises, giving the patient the impression they have rhinosinusitis, as they know their sinuses lie under the forehead. It most often responds to low-dose amitriptyline, but propranolol, sodium valproate, gabapentin or a change in lifestyle may bring successful relief of symptoms. Amitriptyline should be given for six weeks before judging its effect, and should be continued for six months if it has helped. 3s , 36, 37 [****] The starting dose is 10 mg, and after six weeks if pain is not controlled this can be increased to 20 mg (and rarely 50 mg are needed). Patients need to be warned of the sedative effects even at this low dose, but they can be reassured that tolerance usually develops after the first few days. It is our practice to inform patients that amitriptyline is also used in higher doses for other conditions such as depression, but that it is not being given for this reason and its effect is unrelated to its analgesic properties, which would take effect much more quickly and normally require 75 mg. It is often reassuring for patients to know that the dose used for depression is some seven or more times the dose used in tension-type headache and that other antidepressants do not help this condition. In a proportion of patients there are migrainous features, and a trip tan may help acute exacerbations as up to 50 percent of patients have an overlap between tension-type headache and migraine. 1 It may be that the underlying pathology is similar. The aetiology of this type of pain is uncertain but Olesen's theory,I outlined at the beginning of this chapter (see above under Background), provides one of the best models and integrates the effects of myofascial afferents, the activation of peripheral nocioceptors and their convergence on the caudal nucleus of trigeminal, along with qualitative changes in the central nervous

The characteristics of tension-type

headache.

system. 38 ,39 There is also a suggestion that there is a downregulation of central inhibition from supraspinal impulses due to psychological stress and emotional disturbances. Another factor in those who have had a peripheral injury or inflammation is that these may induce neuroplastic changes in the trigeminal brainstem sensory nuclear complex and produce central sensitization. 40 This theory can also account for the superadded potentiation by nociceptors on top of the peripheral/ central sensitization that may be happening in this condition.

MIDFACIAl SEGMENT PAIN Many patients previously labelled as having atypical facial pain appear to have a specific clinical syndrome that is very similar to tension-type headache except that their symmetrical facial pain involves the midface and retroorbital region (Figure 135.6).41 It is not unusual for them also to have tension-type headache although they often fail to volunteer that they have symptoms involving the forehead as well. The quality of their pain and all its characteristics are identical to those mentioned in tension-type headache including the hyperaesthesia of the skin and soft tissues in the area affected. It is not uncommon for patients to say that their cheeks or periorbital skin is swollen although no abnormality can be seen. In these patients psychological factors are far less apparent than in atypical facial pain yet they have a relatively high prevalence of irritable bowel syndrome and fibromyalgia. Various names have been given to this condition including tension-type facial pain and midfacial segment pain. The authors favour the term 'midfacial segment pain' as it avoids having to explain to the patient why the term 'tension' is being used!

1726 I PART

Figure 135.6

13 THE NOSE AND PARANASAL SINUSES

Facial map of the patterns of distribution of midfacial segment pain.

Midfacial segment pain is a common cause of facial pain, and in the experience of the authors, the commonest single category of patient presenting to an otorhinolaryngology clinic. Midfacial segment pain may be considered as the facial version of tension-type headache and the two conditions frequently overlap.29,42 These patients are often misdiagnosed as having sino genic pain and may have undergone previous sinus surgery. Unlike many patients with atypical facial pain, although rather demoralized with their pain, patients with midfacial segment pain are generally functioning well in their everyday lives. There is rarely a history of depression. Typically, there is a bilateral band -like ache or pressure that can be frontal, periorbital or maxillary. The pain lasts

for many hours and on a daily basis. It is often present on waking, although it does not interfere with the patient getting to sleep. The pain can reduce during periods away from work such as weekends or while on holiday. To complicate matters, although not typically accompanied by rhinological symptoms, this type of pain can begin with a genuine episode of sinusitis. An upper respiratory tract infection or hayfever with rhinological symptoms can exacerbate the pain. A few patients also experience episodes of migrainous-type pain against a background of a more constant band-like pain. Many patients describe a gradual reduction in efficacy of simple analgesics. These drugs rna)' have been initially helpful but are eventually of no benefit.

Chapter 135 The diagnosis and management of facial pain

Examination often relieves a degree of tenderness or hyperaesthesia over the skin and soft tissues of the affected area. Nasendoscopy is performed and it is helpful, and by showing normal mucosa helps to reassure the patient they do not have sinusitis. After explanation and reassurance many patients are happy to 'live' with their symptoms rather than take medication with a possible side-effect profile. Lifestyle changes, including alterations to work routines or taking exercise can help break the cycle of pain. Some patients find alternative approaches, such as acupuncture, helpful. As described above the first-line prophylactic treatment of tension headache is low-dose amitriptyline43 ,44 and a similar regime will often help patients with midfacial segment pain. Assuming there are no contraindications to prescription, 10 mg of amitriptyline should be taken at night for six weeks. If the patient fails to respond, it is reasonable to double the dose for a further six weeks. Patients are generally advised to take medication for a full six months after their symptoms have been controlled. Second-line treatment includes propanolol, particularly if the pain has occasional migrainous features or carbamazepine if the pain has a neuropathic quality, possibly after injury or surgery. Recently, gabapentin has also proved useful as a second-line drug. [***1*]

ATYPICAL FACIAL PAIN This is very much a diagnosis of exclusion and care must be taken in reaching this conclusion, even when the patient has received previous opinions and no pathology has been identified. The history is often vague and inconsistent with widespread pain extending from the face onto other areas of the head and neck. The pain may move from one part of the face to another between different consultations, and other symptoms, such as 'mucus moving' in the sinuses, are often described. A number of patients have such completely fixed ideas about their condition that they will not be convinced otherwise whatever the weight of evidence to the contrary. Pain is often described in dramatic terms in conjunction with an excess of other unpleasant life events. Many of these patients have a history of other pain syndromes and their extensive records show minimal progress despite various medications. They have often undergone previous sinus or dental surgery and may be resentful about their treatment. It is not uncommon for such patients to give a history of nasal trauma. Many patients with atypical facial pain exhibit significant psychological disturbance or a history of depression and are unable to function normally as a result of their pain. Some project a pessimistic view of treatment, almost giving the impression they do not wish to be rid of the pain that plays such a central role in their lives. A comprehensive examination (including nasendoscopy) is essential to identify significant pathology before the patient is labelled as having atypical pain. The

I 1727

management of such patients is challenging and confrontation is nearly always counterproductive. A good starting point is to reassure the patient that you recognize that they have genuine pain and an empathetic consultation with an explanation should be conducted. Drug treatment revolves around a gradual build-up to the higher analgesic and antidepressant levels of amitriptyline (75-100 mg) at night. The second-line treatment includes gabapentin and carbamazepine. Patients should sympathetically be made aware that psychological factors may playa role in their condition and referral to a clinical psychologist or psychiatrist may be helpful. Referral to a pain clinic is often appropriate. [**/*]

KEY. POINTS • History taking istlie.keytoaccurate diagnosis ill patients with fa.cial pain. • Sinusitis isariue cause of chronic facial pain and mostpatienisWithfacial pain.' aonot . wardllitsllrgery. • Sinogenicpain Ismtermittel1tahdassociated with rhin()logiCaY symptoms. • SinUS'lnUcusa1thi~~el1ingoIiac=Tsc~hldoes

n?t indicate.thatpainiscsi~og~ni~.

• Vascular 'pain,'.s\Jcl1 • asmi?rain~.andc1uster • ·. headaches, can cause rhinoirhoeaa1.1dnasal congestion. • Many patients with. chronic facial pain benefit from the apprQpriate'n~l,lrological' medication~

A carefully taken history is key to accurate diagnosis, with recognition that the majority of patients will not have sinogenic pain. Examination should include nasendoscopy, which is particularly useful if the patient is currently in pain. In such circumstances, a normal nasendoscopy makes the diagnosis of sinogenic pain extremely unlikely. Sinus x-rays are not helpful in the diagnosis and management of chronic facial pain and sinusitis. Computed tomography scans should not be routinely performed because sinus mucosal thickening is common in asymptomatic patients and management based only on scan findings will result in unnecessary surgery. Given that the majority of patients presenting with facial pain will not have sinusitis, surgery is very rarely indicated in the treatment of chronic facial pain.

1728 I PART 13 THE NOSE AND PARANASAL SINUSES ./ Familiarity with the various medications available to treat nonsinogenic facial pain and liaison with other specialties, such as neurology, maxillofacial surgery and clinical psychology will benefit patients.

7. 8.

9.

10.

Deficiencies in current knowledge and areas for future research Further basic science research into the fundamental pathogenesis of rhinosinusitis is needed to increase our understanding of mucosal disease, including the potential of such inflammation to cause pain. Continued research into the neurophysiology of headache and facial pain is required, in particular, tension-type pain. Credible models have been proposed which help our understanding, but are they correct? ,.. Low-dose amitriptyline has been shown to help tension-type headache and midfacial segment pain. A randomized controlled study is required to test the efficacy of this treatment in midfacial segment pain. ,.. Psychological aspects of facial pain are well recognized. Well-designed studies are required into the usefulness of various psychotherapeutic techniques, particularly with regard to the selection of patients who will benefit.

11.

* 12. 13. 14.

15. 16.

17.

18.

19.

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Olesen J. Clinical and pathophysiological observations in migraine and tension type headache explained by integration of vascular, supraspinal and myofascial inputs. Pain. 1991; 46: 125-32. 2. Bateman NO, Woolford TJ. Facial pain: diagnosis and management. CME Bulletin Otorhinolaryngology and Head and Neck Surgery. 2001; 5: 50-3. 3. The Royal College of Radiologists Working Party. Making the best use of a department of clinical radiology: Guidelines for doctors. London: Royal College of Radiologists, 1993. 4. Marshall AH, Jones NS. The utility of radiologic studies in the diagnosis and management of rhinosinusitis. Current Infectious Disease Reports. 2003; 5: 199-204. 5. Lloyd GAS. CT of the paranasal sinuses: A study of a control series in relation to endoscopic sinus surgery. Journal of Laryngology and Otology. 1990; 104: 447-81. 6. Jones NS. A review of the CT staging systems, the prevalence of anatomical variations, incidence of mucosal findings and their correlation with symptoms, surgical and pathological findings. Clinical Otolaryngology. 2002; 27: 11-7.

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Fahy C, Jones NS. Nasal polyposis and facial pain. Clinical Otolaryngology. 2001; 26: 510-3. Khan 0, Majumdar S, Jones NS. Facial pain after sinus surgery and trauma. Clinical Otolaryngology. 2002; 27: 171-4. Jones NS, Cooney TR. Facial pain and sinonasal surgery. Rhinology. 2003; 41: 193-200. Bumann A, Lotzmann U. TMJ disorders and oro facial pain. New York: Thieme, 2002: 1-378. Lipton RB, Stewart WF. Migraine in the United States: A review of epidemiology and health care use. Neurology. 1993; 43: 6-10. Goadsby PJ, Oelson J. Diagnosis and management of migraine. British Medical Journal. 1996; 312: 1279-83. Daudia AT, Jones NS. Facial migraine in a rhinological setting. Clinical Otolaryngology. 2002; 27: 251-5. Silberstein SO, Goadsby PJ, Lipton RB. Management of migraine: An algorithmic approach. Neurology. 2000; 55: 46-52. Goadsby PJ. Cluster headaches: new perspectives. Cephalgia. 1999; 19: 39-41. Fuad F, Jones NS. Paroxysmal hemicrania and cluster headache: two disrete entities or is there an overlap? Clinical Otolaryngology. 2002; 27: 472-9. Antonaci F, Sjaastad O. Chronic paroxysmal hemicrania. A review of clinical manifestations. Headache. 1989; 29: 648-56. Pascual J. A case of chronic paroxysmal hemicrania responding to subcutaneous sumatriptan. Letters, correspondence, book reviews. Journal of Neurology Neurosurgery and Psychiatry. 1998; 65: 407. Evers S, Husstedt I. Efficacy of sumatriptan in chronic paroxysmal hemicrania. Letter to the editor. Headache. 1998; 38: 630-1. Sluder G. The role of the sphenopalatine (or Meckel's) ganglion in nasal headaches. New York Medical Journal. 1908; 87: 989-90. Sluder G. Etiology, diagnosis, prognosis and treatment of sphenopalatine ganglion neuralgia. Journal of the American Medical Association. 1913; LXI: 1202-06. Puig CM, Driscoll CLW, Kern EB. Sluder's sphenopalatine ganglion neuralgia. Treatment with 88% phenol. American Journal of Rhinology. 1998; 12: 113-8. Pollock BE, Kondzioloka D. Stereotactic radiosurgical treatment of sphenopalatine neuralgia. Journal of Neurosurgery. 1997; 87: 450-3. Cepero R, Miller RH, Bressler KL. Long term results of sphenopalatine ganglion neuronectomy for facial pain. American Journal of Otolaryngology. 1987; 8: 171-4. Abu-Bakra M, Jones NS. The prevalence of nasal contact points in a population with facial pain and .a control population. Journal of Laryngology and Otology. 2001; 115: 629-32. Abu-Bakra M, Jones NS. Does stimulation of the nasal muoosa cause referred pain to the face? Clinical Otolaryngology. 2001; 26: 403-32.

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29. Jones NS. The classification and diagnosis of facial pain. Hospital Medicine. 2001; 62: 598-606. 30. Hooge CF, Redekop WP. Trigeminal neuralgia in multiple sclerosis. Neurology. 1995; 45: 1294-6. 31. Cheng TMW, Cascino TL, Onofrio BM. Comprehensive

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Cerbo R, Barbanti P, Fabbrini G, Pascali MP, Catarci T. Amitriptyline is effective in chronic but not episodic tension-type headache. Headache. 1998; 38: 453-7.

PJ, Silberstein SD (eds). Headache. Blue books of practical

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acyclovir therapy accelerates pain resolution in patients

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136 Medical negligence In rhinology MAURICE HAWTHORNE

Introduction Inadequate standard of care prior to referral to an otolaryngologist Inadequate presurgical management Interpretation of radiological investigations interpretation of histological investigations Failure to consider conservative treatment Consent

1730 1730 1731 1731 1731 1732 1732

Incompetent surgical technique Intraoperative monitoring Postoperative monitoring Inadequate conservative treatment Key points Best clinical practice References

1732 1734 1735 1735 1735 1736 1736

This data in chapter are supported by a PubMed search using the key words medical negligence, rhinology, sinus surgery, complications and risk. In addition, a manual search of Clinical Risk and the Journal of the Medical Defence Union was undertaken. There are virtually no published series of medical negligence cases in the field of rhinology. At best, the practice that might be considered acceptable by the profession is determined by expert opinion. Therefore, the evidence base of this chapter is level 4.

INTRODUCTION In the field of rhinology, damage caused by the surgeon has the potential of being considerably greater than any possible damage caused by leaving disease untreated. Damage occurs primarily to orbital structures, cranial nerves, the cavernous sinus and the brain. Delayed diagnosis or inadequate surgery for malignant skin lesions can lead to excessive and unnecessary cosmetic deformity. Failure to identify a patient with dysmorphophobia can lead to the surgeon himself being physically attacked and possibly even murdered.

INADEQUATE STANDARD OF CARE PRIOR TO REFERRAL TO AN OTOLARYNGOLOGIST Negligence in general practice in relation to rhinological disease is unusual. It appears to occur mainly due to a

failure in recognizing the significance of certain symptoms or signs. One of the most common areas is in failure to arrange timely treatment for a nasal fracture. In most practices, the best opportunity in effectively manipulating a nasal fracture is within the first 21 days of the injury. Failure to undertake timely treatment may lead to the necessity of performing a formal septorhinoplasty. The consequence of this is a longer period off work than would be necessary, as well as additional pain and suffering such as periorbital bruising. A delayed referral can rarely be defended and usually the best outcome is to seek a speedy settlement. There are exceptions and one of the most important is the cartilaginous injury of the nasal tip or septum when simple manipulation does not give the best results and a septorhinoplasty is indicated as first-line treatment. A delay in referral of sinusitis is also a common area that leads' to litigation. The general practitioner ( GP) usually fails to realize when a sinus infection has spread

Chapter 136 Medical negligence in rhinology

beyond the sinus and is involving the orbit, bone, meninges or soft tissues. The most common examples of this are orbital and forehead swelling. It is only the onset of symptoms in relation to brain abscess, meningitis, blindness or severe orbital swelling that leads to a speedy referraL The significance of unilateral nasal symptoms, such as discharge, may be ignored by the GP and so a delay in diagnosis of a tumour or foreign body occurs. In general practice, common errors that lead to litigation include: • delay in referral of nasal fractures; • delay in referral of complicated sinusitis; • failure to realize the significance of unilateral nasal obstruction. Sadly, it is not just the GP who may fall into the situation of delaying a diagnosis. Inexperienced senior house officers within the specialty, accident and emergency doctors, and occasionally other specialists within hospital practice, may all find themselves at the centre of litigation arising from these relatively common areas of negligence.

INADEQUATE PRESURGICAL MANAGEMENT Failure to investigate significant symptoms arises from time to time. Wegener's granulomatosis is a condition that regularly goes unrecognized. Often it may be mistaken for atrophic rhinitis or even industrial rhinitis, and the patient treated symptomatically, without adequate investigation. It is only when other systems are involved, such as the renal or respiratory system, that adequate investigations are triggered and the condition identified. Clearly if the condition has progressed to frank renal failure requiring dialysis before diagnosis has been made, then the patient may have a valuable claim. It is common to find that other conditions, such as scleritis, flitting arthropathy or skin lesions are not linked to the nasal symptoms. However, occasionally circumstances arise in which the investigations for this condition, such as a raised plasma viscosity or erythrocyte sedimentation rate (ESR) , which are usually positive, are within the normal range. Occasionally even the AN CA test is negative, and where adequate investigations have been undertaken but the diagnosis has been delayed, a reasonable defence can usually be mounted. From time to time, a post-nasal space carcinoma may present as a neck lump to another specialist. Although the malignant nature of the neck lump is identified, occasionally the primary site is missed. The ear, nose and throat (ENT) expert may be asked to comment on such a delay. It is of course inappropriate for him to deal with issues of liability concerning delay in diagnosis by another specialist, but it would not be unreasonable for him to comment on how the delay may affect the prognosis of the patient. Similarly, a restriction in eye movement may indicate early involvement of orbital contents or a cranial nerve by a sinus malignancy. Clinical examination may not reveal

I 1731

an obvious restriction in movement, due to the partial nature of the malignant process. Sudden onset of double vision, without an obvious cause such as head injury, or orbital displacement should never be ignored, and requires thorough investigation to exclude a pathological cause requiring treatment.

INTERPRETATION OF RADIOLOGICAL INVESTIGATIONS In many small district general hospitals, there is no radiologist with a special interest in ENT radiology. Consequently, the experienced consultant ENT surgeon may be just as competent at reading radiological films as the specialist radiologist. In all departments it is wise that both the clinician and the radiologist assess the radiological films within the clinical context of the patient's disease. From time to time, a difference of opinion may arise, and a failure to address this difference of opinion can lead to a delay in diagnosis, with a significant impact on prognosis for the patient. Where films have been discussed, and a difference of opinion continues, then it is advisable that a further opinion is sought from a radiologist with a special interest in the field. Nowadays, films can often be easily transferred electronically and the vagaries of the postal system do not have to be endured. Where there is a difference of opinion between the clinician and the radiologist, most patients would expect the radiological investigation to be repeated after some weeks. Although the radiologist, and indeed the clinician, may have some concerns about the increased dosage of x-rays necessitated by repeat films, where there is a possibility of malignancy, the patient will see this risk as being trivial, and would be much more concerned about obtaining peace of mind about what they see as a real and existing problem, compared to some theoretical risk of developing a malignancy in the future. Therefore, the wise clinician should argue for a repeat investigation. In the field of endoscopic sinus surgery, whether it is functional, diagnostic or treating an iatrogenic complication, it is essential to have a complete demonstration of the radiological anatomy by computed tomography (CT) scan. The nose should be scanned from its rostral extremity as far back as the basilar artery. In addition, it is essential that the window setting is such as to maximize the bony detail and also a second series should be saved with window settings to give some soft tissue information.

INTERPRETATION OF HISTOLOGICAL INVESTIGATIONS Much of what has been said above on radiological investigations also applies to histopathological investigations. The wise otolaryngologist will be well aware of the pitfalls that can arise. These are especially true in salivary

1732 I PART 13 THE NOSE AND PARANASAL SINUSES gland and nasal neoplasia. Also, fine needle aspiration cytology needs to be carefully considered in the light of the clinical picture and if a lesion deemed benign on cytological examination does not appear to be behaving clinically as such, rebiopsy should be undertaken speedily.

FAILURE TO CONSIDER CONSERVATIVE TREATMENT This issue appears to arise more commonly in private practice. It is not uncommon for the patient who has had a mishap following sinus surgery to state that conservative options for treatment, such as oral steroids and antibiotics in the case of nasal polyposis, had never been discussed, but that an immediate recommendation for surgery had been made by the practitioner. In the case of simple, benign, nasal polyposis in a fit individual, there is rarely any defence for not discussing the possibility of a course of oral steroids, with or without antibiotics. If there is doubt as to the nature of the polyps, a simple biopsy can be undertaken, followed by conservative treatment, rather than an attempt to radically clear the disease, as first-line treatment. In those patients with nasal obstruction due to enlarged inferior turbinates, first-line treatment should normally be conservative. Adequate investigation to identify the presence of any allergy and then treatment either by allergen avoidance or topical steroid treatment would usually be first-line treatment. If the general practitioner has already undertaken this in an adequate and thorough manner, it may be reasonable to proceed with an offer of surgery. However, a significant complication occurring following turbinate surgery in the patient who has not been offered an initial trial of conservative treatment can be very difficult to defend.

CONSENT Issues of consent on their own are rare in relation to sinus surgery and they are much more common when coupled with allegations of incompetent surgical technique. However, it is in the field of turbinectomy, particularly in the adolescent and young adult, that issues of consent arise. Torrential haemorrhage following inferior turbinectomy is not uncommon, probably happening in most surgeons' practice, with an incidence of between 1 in 60 and 1 in 200 cases. It does appear to be more common where a posterior turbinectomy has been undertaken, rather than removal of the anterior end of the interior turbinates. Nevertheless, torrential haemorrhage can happen in any form of turbinate surgery. When this occurs, and the patient has undergone the usual treatment to arrest the haemorrhage and correct the blood loss, an allegation of permanent psychological damage may arise, accompanied by the allegation that they were not warned of the risk and had they been, then they w~uld have refused consent for the surgery. In taking consent, the

wise surgeon must assess the personality of the patient and ask himself whether they would have the mental fortitude to withstand the psychological trauma which can accompany a severe nasal haemorrhage, particularly as it may appear life-threatening to the patient and their family. If the patient, despite warning of such haemorrhage, still wishes to proceed with the surgery, then it should be carefully annotated in the notes that the warning has been given and understood. Since the introduction of functional endoscopic sinus surgery on a wide basis, between 1988 and 1992, consent for these procedures seems, on the whole, to be well annotated in the notes, particularly from the point of view of the risks of injury to orbital contents and breach of the dura. However, where contention may arise, it is not that the patient was warned of these anatomical risks, but that they did not understand the consequences of these risks on their day-to-day life, or that should such risks occur, that secondary complications may supervene. One patient, an intelligent banker, freely admitted that he had been warned of the risk of cerebrospinal fluid leaking from his nose, but was adamant that he had been given no warning that this could lead to meningitis and subsequent intellectual impairment, which had occurred in his particular circumstances. However, the claimant may find it difficult to establish in court that, had they been fully warned, they would not have gone on to subject themselves to the surgical procedure. The landmark Australian case, Rogers v Whitaker, l in which it was felt the risk of sympathetic ophthalmitis, with a risk of approximately 1 in 30,000, should have been explained to a patient, has not found favour in the English courts. The risk of blindness as the result of submucosal diathermy, or the use of adrenalin-containing local anaesthetic in rhinological surgery, is somewhat similar. In one case, a young woman woke up following a general anaesthetic for a submucosal diathermy and had totally lost sight in one eye. Thorough investigation did not reveal any evidence of direct trauma to orbital or retroorbital structures, and a literature search revealed that this can happen despite competent surgical technique. On the Bolam principle, as virtually no ear, nose and throat surgeon practising in the United Kingdom would warn of this risk, her case would fail and therefore her solicitor advised that she should not pursue the issue. A Legal Aid Certificate was not granted to test the principle that such a loss was so catastrophic that, despite its rarity, it should have been warned of and that any reasonable patient would expect that this issue be mentioned.

INCOMPETENT SURGICAL TECHNIQUE Just because a recognized complication happens, it does not automatically mean that surgical technique has been below a 'satisfactory standard. A complication such as damage to the medial rectus muscle in endoscopic sinus

Chapter 136 Medical negligence in rhinology

surgery2 may occur, despite all care being taken by the surgeon. On the other hand, it may occur as a result of a 'gung ho' or slapdash technique, in which due respect and identification of the relevant surgical landmarks has not been attempted. For the claimant, it can be difficult, if not impossible, to prove that the damage has occurred because of the latter surgical technique, rather than the former. It would indeed be rare for a claimant to successfully identify a witness, present at the time of the surgery, who was willing to testify in court as to incompetent technique practised by the surgeon. Increasingly, video is used in theatre and, in the case of endoscopic sinus surgery, operating via camera is a common technique. If the surgery is recorded and the tapes kept until it is established there is no significant postoperative complication, the recording may be available to both parties in which an adverse outcome to the surgery has developed. For example, in one case, a trainee undertook a difficult nasal polypectomy endoscopically. The procedure was recorded and a breach of the anterior cranial fossa, and how it was acquired, could be clearly seen on the video. In this particular instance there was a dehiscence of the cribriform plate, with absence of bone in the anterior cranial fossa. The repair was also filmed. An explanation was given to the patient, who was quite satisfied. However his children, who lived some distance away, attended the hospital some days later. One child, a nurse in a maxillofacial surgical department, clearly felt that causing a cerebrospinal fluid (CSF) leak had to be negligent. Fortunately the video material was available, which was immediately shown to the family and carefully explained. This diffused the aggression and was successful in preventing litigation. Occasionally, massive injury can be sustained in which the degree of damage is so gross that basic surgical technique has to have been ignored, or not understood. For example, in one case where the patient failed to regain consciousness from the anaesthetic following intranasal surgery. On arrival at the intensive care unit, a 2 x 1 x 1 cm piece of cerebral cortex was retrieved from the oropharynx and subsequently an extensive resection of the anterior cranial fossa was identified, and the nose contained a huge herniation of the brain. The neuroradiological expert in the case felt that there was clear evidence of instrument tracks within the substance of the frontal lobes of the brain, suggesting that instruments had been passed repeatedly into this brain substance. In this particular case, preoperative CT scans did not reveal any evidence of a congenital abnormality of the anterior skull base. Not surprisingly the surgeon's insurers sought a settlement early on in the process of the case. Where the evidence is strong that the lack of standard of care would suggest a flagrant disregard for safety, or recklessness in performing the surgical technique, then the public may need to be protected by requesting the General Medical Council for a competency assessment.

I 1733

Frontal sinus trephine may lead to supratrochlear nerve damage and indeed supraorbital nerve damage. Where there is significant oedema in the surgical area, most surgeons could understand how the supraorbital nerve might be stretched by retractors, or indeed cut. However, in a simple drainage procedure, there can be no excuse for extending the incision so far laterally as to divide the supraorbital nerve. The Lynch-Howarth procedure gives wider access to the frontal and ethmoid sinuses. In extensive disease it may be necessary to sacrifice the supraorbital and the supratrochlear nerve. This should be anticipated before the surgery and the patient warned. In addition, the patient should be warned of the possibility of double vision postoperatively due to the detachment of the trochlea. Resting cheek retractors against the supraorbital rim in a Caldwell-Luc procedure, or similar surgery, can lead to a neuropraxia of the supraorbital nerve. This usually recovers, however, it is a well-recognized risk and adequate precautions can usually be taken to prevent it happening. On the other hand, infraorbital nerve neuropraxia in Caldwell-Luc surgery can be inflicted despite all care being taken, and does not indicate de facto substandard technique. A major saddle deformity of the supra tip area, following septal surgery, causes such a deformity that it can be difficult to prevent the angry and embittered patient from seeking redress. If the complication has occurred as the result of a postoperative septal abscess, and the patient has been warned of the possibility when consent was taken, then sympathetic handling can usually prevent the patient seeking redress through the help of a solicitor. A careful explanation that infection had destroyed the septal cartilage may suffice. However, most patients will ask immediately why they were not given antibiotics at the time of the original surgery. An explanation such as, 'It is not usual practice', will frequently earn the retort from the patient 'Well don't you think it should be'. A sympathetic explanation, couched in easy to understand terms, about bacterial resistance and the natural ability of the nasal area to defend itself against infection, is much more likely to be successfully accepted than a patronising comment such as 'We don't routinely give antibiotics: The patient clearly will require reassurance that an effective attempt to repair the damage will be undertaken. Rarely the saddle could be due to a failure to recognize and diagnose disorders such as Wegener's granulomatosis, sarcoidosis or relapsing polychondritis. 3 The wise surgeon, when presented with such an unexpected outcome, should take the appropriate tests to exclude such disorders. Clearly the presence of an alternative explanation for a septal collapse or perforation may present an opportunity for a defence. Septal perforation is another common complication, especially in submucous resection of the nasal septum that causes such symptoms that a patient may seek compensation. It should always be mentioned when septal surgery is being offered. The presence of a perforation

j

1734 I PART 13 THE NOSE AND PARANASAL SINUSES does not automatically imply a substandard technique; it is more likely to be an indicator of a severely fractured nasal septum or the presence of another disease, such as atrophic rhinitis or a granulomatous disease. The circumstances of a submucosal resection, in which the surgeon has made an error of judgement and resected too much cartilage, are likely to be much more difficult for the patient to accept. Once more, recognition of the patient's concern is the key to good management. In addition to offering surgery to correct the deformity, it is important to recognize that the patient may no longer have confidence in the surgeon, or indeed even in the hospital in which they have been treated, and so a prompt offer of transfer to another department and another surgeon may assuage the patient's anger. An allegation of inadequate primary resection of a tumour, either malignant or benign, usually follows the unexpected diagnosis of a recurrence. This may seem surprising to the profession, but sadly a combination of belief in modern surgical techniques, plus the confident statement from the operating surgeon that 'all the tumour has been removed', can lead to an accusation of incompetent technique. Clearly, a confident statement on tumour resection is meant to reassure the patient and improve their psychological well-being. However, there is no excuse to take on a patronising role, supported by frank lies. It is important that the patient is told the exact situation, as best as can be determined, and if the outcome for the future appears to be bleak, then such bad news needs to be adequately supported with appropriate psychological therapy. It is possible that any psychological damage may be looked upon leniently by the courts, and appropriately rewarded, especially when the major defence to the bulk of the claim is that 'the patient would have died anyway'. On the subject of failure to diagnose a recurrence, a claim may arise where a patient has been lost to follow up and only represents with an extensive recurrence. Such cases may have a prospect of being successful where the patient can establish that had there been a regular examination and inspection of the nose and sinuses, with the draining lymph nodes, that a recurrence would have been easily identified at an early stage. It is therefore important that health trusts have a robust system of tracking down those patients who fail to attend for their expected appointment. Trusts should be seen to communicate with the GP in order to establish whether the address that they have is correct, and furthermore to inform the GP that the patient has not attended and request the GP to investigate the reasons why. Failure to do so may be difficult to defend, unless the trust can establish that they have emphasized to the patient the importance of regular follow up and examination following treatment for the tumour. There is considerable merit in a letter being sent to the patient, giving details of the diagnosis, treatment and the planned follow-up care, for the purposes of identifying early

recurrence of disease. It is not uncommon for an expert to examine a claimant's notes and identify frequent nonattendance in out-patient departments of various specialties. However, the claimant usually alleges that they failed to receive the appointment in the mail. Although the treating physician may have a healthy scepticism of such allegations, it must be borne in mind that many people live in multiple occupancy dwellings in which security of mail may indeed be in doubt. As such, where follow up is important, it is advisable that the GP is asked to investigate for repeated failures to attend. In circumstances where resection margins are being monitored by frozen section examination during the surgery, it is vital that all specimens are sent. Thus, when a report says that the tumour is at the margin of resection, on one specimen it is useful to have a subsequent specimen indicating that there is a clearance. Obviously, where the tumour is up to, but not invading, a nerve sheath, the surgeon may have to exercise clinical judgement and decide whether to resect such a nerve sheath or not. These points should be carefully noted in the records. Thus, a histological report which indicates that the margin of resection is up to the edge of the tumour can be seen to have been acted upon appropriately. Prior to the 1980s, it was very common practice to undertake a 'proof puncture' of the maxillary antra. This would often be done at the time of a tonsillectomy or adenotonsillectomy, in older children and young adults. It was considered a legitimate investigation to detect the possibility of chronic sinus infection. Many surgeons advocated its routine use, even in the absence of any sinus or nasal symptoms. Such an invasive procedure, which carries the risk of damage to unerupted secondary dentition, the inferior orbital nerve and the orbital contents, in the absence of sinus symptoms, can no longer be justified without taking a formal consent for the procedure and offering alternative methods of investigation. In addition, several cases have arisen due to misunderstanding of the term 'examination under anaesthesia'. Some claimants who have sustained complications as a result of such procedures have asserted that they only gave consent for the surgeon to look, but not to biopsy. A typical examination would be obtaining consent for examination of the post-nasal space in a child or adult with glue ear, and then proceeding to perform an adenoidectomy having identified a significant pad of lymphoid tissue in the post-nasal space, the damage occurring usually being a secondary haemorrhage. It is important therefore, in taking consent, to include the potential risks that may arise for any additional procedure, usually a biopsy of an excisional or incisional nature.

INTRAOPERATIVE MONITORING This term usually invokes thoughts of nerve monitoring in the context of ENT surgery, but it is still particularly

-_----.i

Chapter 136 Medical negligence in rhinology

important in rhinological surgery, although the monitoring may be purely visual in nature. In endoscopic sinus surgery, it is the orbital contents which are at risk. Clues to a significant intraorbital incursion can be obtained by careful observation of both eyes during the procedure. This may not be possible by the operating surgeon, as it is likely that he will be observing the operating field either indirectly through a camera or directly through an eyepiece. However, an operating assistant, such as a scrub nurse or other colleague, should be in a position to observe both orbits and watch for the tell-tale signs of a complication arising. These may include a sudden movement of the globe of the eye, the development of proptosis or subconjunctival haemorrhage. Although in the early parts of the procedure it is important to protect the cornea from damage, at points in the surgery in which the eye may be in a critical, vulnerable, state, it is permissible for the eyelid to be opened and the globe observed for brief periods. Early identification of an incursion into the orbit is essential to reduce the risk of damage. Equally, in the recovery area, postoperative observation of the orbits and eyes is essential, particularly if a significant retrobulbar haemorrhage is to be identified, monitored and, if necessary, treated before permanent damage to the eyesight occurs.

POSTOPERATIVE MONITORING Even though frontal sinus infection has been adequately drained at surgery, the patient still has the potential of developing an intracranial complication, though this is now much less. It is likely that the infection has spread through the venous system prior to the actual drainage of the sinuses and so the intracranial symptoms usually commence 24-48 hours after surgery. Complaint of severe headache which is progressive, along with drowsiness, confusion or disinhibition, should alert the wary to the onset of a frontal lobe infection. Usually the patient should be on an antibiotic with a high ability to cross the blood-brain barrier, but if this is not the case, then with the onset of these symptoms the antibiotic will need to be changed. Inadequate monitoring in the postoperative period may also lead to delay in detection of a cerebrospinal fluid leak. Although these may be blatantly obvious when there is a striking flow of clear fluid from the nose and the patient has an accompanying headache, the occasional case may be intermittent. In one case, the patient reattended the hospital on three occasions complaining of watery nasal discharge, which was ignored. No investigations were undertaken, other than admission and observation on the ward, on one occasion only for 48 hours. On the final attendance at hospital the patient presente-d with a small bottle containing a sample of the CSF and symptoms of frank meningitis. The confusing issue is that nasal discharge which may be watery in nature is not uncommon following sinus surgery, or indeed may

I 1735

be related to the underlying sinus disorder. It is quite likely that minor CSF leaks are probably more common than is actually realized, but fortunately they close spontaneously without any secondary damage.

INADEQUATE CONSERVATIVE TREATMENT The usual practice is to treat the frontal and ethmoidal sinusitis with decongestants and antibiotics which is often successful. However, if there is a failure to respond or there is evidence of the infection beyond the sinus, then drainage of the sinus is required. Failure to recognize that the infection has spread beyond the sinus can lead to orbital abscess and intracranial infection. Within the hospital setting, spread of the infection should be recognized within 24 hours of the first signs or symptoms of this complication developing. If the infection spreads beyond the frontal sinus, then infection of the bony walls of the sinus can be inferred, as well as thrombosis of the venous channels through the bone. This infection is difficult to eradicate and requires prolonged use of antibiotics in the order of 8-12 weeks. When a patient presents with marked swelling of the soft tissues of the forehead, perhaps with a subperiosteal abscess, there may be an initial settling of the symptoms with a two-week course of antibiotics. The dramatic improvement in the patient's condition may lull either doctor and/or patient into a false sense of security and the antibiotic course is terminated as a result. Then, weeks later, the features of chronic osteomyelitis develop or even secondary intracranial sepsis. It is indefensible not to give a prolonged course of antibiotics where there is a significant risk of osteomyelitis. 4

KEY POINTS • Wegener's granulomatosis is a condition that regularly goes unrecognized. • Issues of consent on their own are rare in relation to sinus surgery. • Where contention may arise, is not that the patient was warned of anatomical risks, but, that they did not understand the consequences of these risks on their day-today life, or that, should such risks occur, secondary complications may supervene. • Just because a recognized complication occurs, it does not automatically mean that surgical technique has been below a satisfactory standard. • Infraorbital nerve neuropraxia in Caldwell-Luc surgery can occur despite all care being taken and does not indicate de facto substandard technique.

1736 I PART 13

THE NOSE AND PARANASAL SINUSES

REFERENCES if Sudden onset of double vision should never be ignored. It is wise that both the clinician and the radiologist assess the radiological films, within the clinical context of the patient's disease.

1. 2.

anatomy by CT scan. If a lesion deemed benign on cytology does not appear to be behaving clinically as such, rebiopsy should be undertaken speedily. In the case of simple, benign, nasal polyposis, there is rarely any defence for not discussing the possibility of a course of oral steroids, with or without antibiotics. Where there is evidence of a flagrant disregard for safety or recklessness in performing a surgical technique, then the public may need to be protected by requesting the General Medical Council for a competency assessment. A postoperative saddle could, rarely, be due to a failure to recognize and diagnose disorders such as Wegener's granulomatosis or sarcoidosis. In such an event, the appropriate tests to exclude such disorders should be taken. Where an adverse outcome of treatment has occurred, prompt offer of transfer to another department and another surgeon may assuage the patient's anger. Trusts shou Id have a robust system of tracki ng down those patients who fail to attend for their expected appointment.

Johnston MN, Jones NS. The complications of endoscopic sinus surgery. Clinical Risk. 2002; 8: 133-6.

3.

Morrison AW 'Silence in court': Twenty one years of otolaryngology litigation. Journal of Laryngology and

In the field of endoscopic sinus surgery, it is essential to have a complete demonstration of the radiological

Rogers v. Whitaker [1993] 4 Medical Law Reports, High Court of Australia 79.

Otology. 1990; 104: 162-5. 4.

Hawthorne M. Clinical negligence in rhinological surgery.

Clinical Risk. 2003; 9: 44-8.

PART

14

THE NECK EDITED BY JOHN HIBBERT

137 Surgical anatomy of the neck

1739

Chris R Jennings

138 Examination and imaging of the neck Sheila C Rankin and John Hibbert

1754

139 Neck trauma

1766

Johannes J Fagan and Andrew J Nicol

140 Benign neck disease: infections and swellings Peter Clarke

1777

137 Surgical anatomy of the neck CHRIS R JENNINGS Introduction Developmental anatomy Surface anatomy Triangles Fascia I layers Neck spaces Muscles Cervi~al

lymphatics

1739 1739 1740 1741 1743 1743 1745 1746

Major blood vessels Nerves Summary Key points Deficiencies in current knowledge and areas for future research Further reading

1749 1750 1752 1752 1753 1753

This chapter is based on anatomical and surgical texts and is supported by a Medline search using the key words head and neck anatomy. The anatomical evidence is supported by cadaver dissection studies. The clinical evidence is also observational.

INTRODUCTION Surgeons should master surgical anatomy of the neck before embarking on any neck dissection. The aim of this chapter is to outline the clinically important areas of neck anatomy. The upper border of the neck is the floor of the mouth anteriorly and the skull base posteriorly. The lower border is the upper border of the first rib and body of the first thoracic vertebra. The development of the neck helps in understanding the anatomy and will be discussed, while an appreciation of surface anatomy is important for neck examination and will be included in this chapter. The triangles of the neck, fascial boundaries, neck spaces, cervical lymphatics, muscles, vessels and nerves will be discussed in turn with particular points of surgical importance emphasized. It is not the intention of this chapter to be an operative surgery text and operations will not be discussed in detail.

DEVELOPMENTAL ANATOMY The ~~f the neck is derived from cervical dermatomes, Whic;se from the second to the sixth cervical

~

'-

segments. The sternocleidomastoid, strap muscles and trapezius originate from cervical myotomes. The viscera in the neck and structures innervated by the cranial nerves have their embryological origins in the branchial apparatus. The six branchial arches form at four weeks gestation and each has a principal nerve and vessel. The vascular anatomy changes unrecognizably from the embryo. The neural anatomy forms in an identifiable pattern. Each arch has a principal or trematic nerve. The branches to the previous arch are called pretrematic and to the following arch are post-trematic. Between the arches are the pharyngeal pouches. The contribution of each to the neck structures will be considered in turn. The first arch structures form the upper border of the neck. The mandible is derived from Meckel's cartilage which is the cartilage formed by the chondrofication of the first arch mesenchyme (embryological connective tissue). The mylohyoid, anterior belly of digastric are also first arch derivatives and the trematic nerve is the mandibular branch of the trigeminal nerve. The first pouch forms ear structures only. The bone structures of the second arch are formed from Reichart's cartilage, which represents chondrofication of the second arch mesenchyme. They are the styloid

1740 I PART 14 THE NECK

Anterior to this is the angle and ascending ramus of the mandible and between these two lies the parotid gland.

process, lesser cornu and upper body of the hyoid bone. The muscles are the platysma, posterior belly of digastric and stylohyoid. The trematic nerve is the facial nerve. The second pouch contributes only to ear-related structures. The third arch contributes the greater cornu and the inferior body of the hyoid bone and contributes to the hypobranchial eminence, which forms the cartilage of the epiglottis. It forms only one muscle in the neck, the stylopharyngeus. The trematic nerve of the third arch is the glossopharyngeal nerve. The third pouch gives origin to the thymus gland from its ventral aspect and the inferior parathyroid (parathyroid III) gland from the posterior aspect. The descent of the thymus draws down the parathyroid III so that it lies inferior to parathyroid IV. Arches four, five and six merge to form one, with the fifth arch being completely absorbed. The third and fourth arches fuse forming a transitory sinus between the two, called the cervical sinus. If this persists it runs in the lower neck, between the internal and external carotid arteries to the apex of the piriform fossa. If such a tract is found in the lower neck, a cervical sinus must be excluded by a sino gram. The fourth and six arches form the cartilages and muscles of the larynx and the muscles of the pharynx. The trematic nerves are the superior laryngeal branch and the recurrent laryngeal nerves of the vagus. The fourth pouch gives rise to the superior parathyroid gland (parathyroid IV), while the fifth pouch forms the ultimobranchial body from which the parafollicular cells or C-cells develop. The sixth pouch gives the intrinsic muscles of the larynx. The branchial arch derivatives are summarized in Table 137.1, and the brancial pouches in Table 137.2.

Mandible This represents the upper border of the neck. The superficial lobe of the submandibular gland can be palpated just inferior to the lower border of the inferior ramus of the mandible.

Hyoid The body and greater cornu of the hyoid bone are important bony landmarks in the neck. The body is in the midline and the greater cornu is just inferior to the angle of the mandible. The greater cornu acts as a guide to the lower extent of the course of the marginal mandibular branch of the facial nerve and it divides node levels II and III.

Thyroid cartilage Just inferior to the body of the hyoid is the thyroid cartilage. The thyrohyoid membrane links the two. This cartilage can be moved over the cricoid cartilage when the resulting crepitus is a sign of normality.

Cricoid cartilage This can be palpated inferior to the thyroid cartilage in the midline. Between the two is the cricothyroid membrane. This has a spring-like feel and is the site of emergency tracheotomy. The cricoid is at the level of the sixth cervical vertebra and represents the boundary between larynx and trachea, and the pharynx and oesophagus.

SURFACE ANATOMY Mastoid

Trachea

When examining the neck, this is as good a place to start as any, as it represents the origin of the sternocleidomastoid muscle. Anterior and inferior to the mastoid process, the transverse process of the atlas can be palpated.

Table 137.1

The cervical trachea can be palpated just inferior to the cricoid cartilage.

Summary of branchial arch derivatives.

Arch First Second Third Fourth and sixth

Muscles

Nerves

Skeletal structures

Mylohyoid digastric (anterior belly) Stylohyoid digastric (posterior belly) platysma Stylopharyngeus

V1 VII

Cricothyroid constrictors of the pharynx Intrinsic muscles of the larynx

x (Superior laryngeal)

Mandible Styloid process Hyoid (lesser cornu + upper body) Hyoid (greater cornu + lower body) epiglottis Laryngeal cartilages

IX

(Recurrent laryngeal)

Chapter 137 Surgical anatomy of the neck I 1741

Table 137.2 Pouch

Summary of branchial pouches.

Contribution to the neck stn.lCture

First and second

No neck structures

Third

Inferior parathyroid gland (parathyroid III) thymus Superior parathyroid gland (parathyroid IV)

Fourth

Accessory nerve

Thyroid gland The isthmus can be palpated overlying the trachea between the second and fourth rings. The thyroid lobes lie deep to the sternocleidomastoid muscles and cannot be palpated unless enlarged.

Sternocleidomastoid This muscle arises from the mastoid process and divides into a tendinous sternal head and a fan-shaped clavicular head. Between and deep to the two heads is the internal jugular vein. It divides the neck into anterior and posterior triangles, the posterior border is the anterior border of the posterior triangle and the anterior border is the posterior border of the anterior triangle. On its deep surface is the carotid sheath, which includes the carotid arteries, the internal jugular vein, the vagus nerve and the jugu ar chai 0 lymph nodes. This accounts for 80 perce of lymph nodes in the neck. The jugular chain of nodes is more easily palpated by tilting the head to the side that is being examined. This relieves the tension on the muscle and allows the deep surface to be accessed.

Trapezius This very large muscle can be palpated posterior to the sternocleidomastoid. Elevating the shoulder against resistance makes it more prominent. It should be remembered that the muscle extends out of the neck and into the chest down to the level of the 12th thoracic vertebra.

Marginal mandibular branch of the facial nerve This has a variable course, but it can be accurately located at the point where it crosses the mandible. It crosses with the facial artery, which can be palpated anterior to the masseter muscle. The lower limit of the nerve is the greater cornu of the hyoid, so that incisions below this should not damage the nerve.

This nerve runs through the sternocleidomastoid and in this part of its course is well protected. It exits the posterior border of the sternocleidomastoid at the junction of the upper and middle third about 1 cm above Erb's point. This is the point at which the cervical plexus emerges from the posterior border of the muscle. The nerve then courses across the roof of the posterior triangle and enters the trapezius at the junction of its middle and lower thirds in the neck.

Carotid artery This can be best palpated at the carotid bifurcation. This is between the angle of the mandible and the greater cornu of the hyoid bone. It follows a line from the sternoclavicular joint to midway between the angle of the mandible and mastoid tip.

Root of the neck The manubrium, sternoclavicular joints and the clavicles form the surface landmarks of the root of the neck. The superior surface of the manubrium is the jugular notch and above this is the suprasternal fossa or Burn's space. Laterally, the clavicles articulate with the acromium forming the anterior boundary of the root of the neck.

TRIANGLES It is difficult to readily compartmentalize anatomically all of the structures in the neck. To make this easier, the neck has been classically divided into triangles (see Figure 137.1). The sternocleidomastoid divides the neck into anterior and posterior triangles; the muscle itself is in neither triangle. The submental, submandibular, carotid and muscular triangles divide the anterior triangles.

Anterior triangle The boundaries of this triangle are sternocleidomastoid, the inferior ramus of the mandible and the midline. The contents of the triangle are summarized in Table 137.3.

SUBMENTAL

The boundaries are the anterior belly of the digastric, midline and hyoid bone. It contains lymph nodes and the submental salivary gland.

1742 I PART 14 THE NECK

Stylohyoid muscle Digastric muscle (posterior belly)

Sternocleidomastoid ------'- 12 hours for iatrogenic oesophageal injury has a mortality rate of 40 percent as opposed to 9 percent for < 12 hours.13 In a retrospective multicentre study, Asensio et al.14 compared the outcome of patients undergoing diagnostic studies, with patients taken straight to the operating theatre. The study revealed a significantly increased oesophageal complication rate (41 versus 19 percent) and length of intensive care unit (lCU) stay with patients having diagnostic studies. They concluded that time delays in instituting active manage­ ment incurred by investigations associated with selective exploration can lead to increased morbidity and mortal­ ity. This report does not distinguish between the cervical and thoracic oesophagus. Whether their conclusion applies to isolated cervical oesophageal injury is therefore open to question, particularly in view of reports of the successful outcome of small cervical oesophageal injuries treated conservatively. However, centres practising selec­ tive management of penetrating neck injuries should make rapid diagnosis and definitive repair a priority. [***] [Grade C]

11771

SURGICAL TECHNIQUE

The majority of oesophageal Injuries can be repaired primarily. The remainder can be repaired by resection and anastomosis, or managed by drainage alone. Intravenous antibiotics and enteral or parenteral nutrition should be instituted. Adequate drainage, suction or dependent, is important as even with a technically sound repair, 13 percent develop fistulae.9 If part of the oesophagus has been blown away, if mediastinitis or sepsis is already present, or if oesophageal injury extends into the chest, then a lateral cervical oesophagostomy may be required. [Grade C] There is no evidence to favour a double or single layer repair. Local muscle flaps, such as strap or sternocleidomastoid muscle, can be used to buttress the repair or used as an interposition between an oesophageal injury and a vascular or tracheal repair.25 Barium swallow is undertaken on day 5-7, as 50 percent of postoperative oesophageal fistulae are asymptomatic and detected only on contrast study.9 Should a fistula or sinus be present, the drain is retained. [**]

COMPLICATIONS OF OESOPHAGEAL INJURY

Oesophagocutaneous fistulae develop in 9-28 percent of oesophageal trauma.25 They are more common with gunshot wounds, but generally close spontaneously. Oesophagotracheal fistulae need to be repaired with interposition of a muscle flap. More serious complica­ tions include abscess formation, mediastinitis, septicae­ mia and death. [**]

DO ALL OESOPHAGEAL I"'JURIES NEED TO BE EXPLORED?

Recommended treatment of oesophageal injuries varies from observation to simple repair of the wall, with or without drainage of deep neck spaces, to primary diversion of flow of saliva to the skin by means of partial or total exteriorization procedures. Wound site, size, mechanism of injury, time delay until presentation, associated injuries, availability of and expertise with diagnostic tests such as oesophagography and oesophago­ scopy, and availability of theatre time all play a role in determining the management of oesophageal injuries. All high velocity injuries should be explored. The manage­ ment of stab wounds and low velocity GSW s is more contentious. Generally it is recommended that if barium swallow is positive or equivocal, treatment should proceed to rigid oesophagoscopy; if positive, then the oesophagus is explored. [Grade D] However, Ngakane et al.12 concluded from a prospective study of penetrating visceral injuries of the neck, that should oesophagography reveal minimal leakage of contrast, then patients can be managed conservatively; oesophagography is repeated on day 5, before commencing oral feeding. Similar favour­ able outcomes of small cervical oesophageal perforations treated conservatively have been reported by MandaI et al.24 and others. [***]

�--

Tracheal injury Cervical tracheal injuries are relatively uncommon and are frequently associated with oesophageal, vascular or spinal injuries. The initial priority is to secure an airway. The trachea can sometimes be readily intubated through a blowing wound in the neck. Tracheotomy is appropriate in the presence of laryngeal trauma to protect the injured larynx, when it is not possible to safely pass an endotracheal tube, or in the presence of quadriplegia necessitating ventilatory support. Earlier teaching that nasotracheal or orotracheal intubation should be avoided as it may aggravate an existing tracheal injury or cause a false passage, appears to have been overcautious.26 Distal tracheobronchial disruptions can be bypassed under vision ,with an introducer passed through a rigid bronchoscope, or by intubating over a flexible bronchoscope. Symptoms of tracheal injury include a blowing wound, surgical emphysema, haemoptysis and hoarseness. CXR may reveal surgical emphysema and pneumomediasti­ num. Tracheobronchoscopy can be useful to assess the injury, but the diagnosis is usually readily apparent on exploring the neck for associated injuries.

1772 I

PART 14 THE NECK

Minor tracheal injuries in patients not otherwise requiring cervical exploration can be managed expec­ tantly. In cases of marked surgical emphysema, a tracheotomy might expedite recovery. Tracheal repair is achieved with interrupted sutures. When there is an associated oesophageal or vascular injury, then the repair can be bolstered with a muscle flap. Tracheotomy or an endotracheal tube may, in selected cases, be used to protect the tracheal repair. [*] [Grade C]

Vascular injury The common carotid artery is the most frequently injured vessel, accounting for 22 percent of vascular injuries?7 Hard signs of vascular injury are an expanding haema­ toma, external haemorrhage, absent or diminished distal pulses, ischaemia, neurological deficit or coma. Current debate centres on evaluation of the asymptomatic patient and optimum treatment of vascular trauma. The majority of published series are small, retrospective and from single centres.

CLINICAL EVALUATION

The reported accuracy of clinical evaluation varies widely. Sclafani et al.28 reported a specificity of 80 percent and sensitivity of 61 percent, and Meyer et al?9 reported an accuracy of 68 percent.7 Demetriades et al.30 reported a sensitivity of 100 percent for clinical detection of significant vascular injury. The widely disparate results may reflect shortcomings of retrospective data collection and the lack of emphasis placed on clinical decision making in centres with a bias towards routine angio­ graphy or exploration.

INVESTIGATIONS

The gold standard investigation is four-vessel arch angiography with selective catheterization. Angiography has the following benefits: identification of the site of injury; identification of subclinical vascular injury, including vertebral artery trauma; provision of a road map for the surgeon; delineation of the extent of crossover circulation through the Circle of Willis; and identification of injuries amenable to endovascular intervention. Vascular injuries in zone II are readily exposed and with zone II penetrating injuries, the chance of detecting an asymptomatic arterial injury by angio­ graphy is less than 1 percent, which approaches the complication rate of angiography? With penetrating injuries in zone II, angiography may therefore be employed selectively for patients, with clinical suspicion of a vascular injury or when the tract of a gunshot wound passes close to vascular structures. Vascular injuries in zones I and III can be difficult to assess clinically. Surgical

exposure and control are more challenging. Hence, preoperative angiography of suspected vascular injuries in zones I and III is generally recommended. Demetriades et al.30 reported that colour-flow duplex Doppler (CFD) imaging, when combined with clinical examination, had a sensitivity of 91 percent and specificity of 99 percent to detect vascular injury. CFD only missed an intimal tear, which did not require treatment.30 CFD, however, requires trained personnel, is not always readily available and is best avoided if there is a suspected cervical column injury. The origins of cervical arteries from the aortic arch and the arteries close to the skull base, as well as the vertebral arteries, may not be well demonstrated and it is difficult to identify individual branches of the external carotid artery.

CAROTID REVASCULARIZATION VERSUS LIGATION

With the introduction of vascular surgical techniques during the Korean War, primary repair of carotid artery injuries was recommended. In 1973, Bradley31 concluded from two autopsy studies which revealed haemorrhagic infarction of the brain, that revascularization should not be attempted in patients with severe neurological deficits. Fear about converting an ischaemic into a haemorrhagic infarct by reperfusion and worsening the neurological outcome has since been a major concern to vascular surgeons, and has fuelled the dilemma of ligation versus reperfusion. In 1978, Liekwig and Greenfield32 showed that patients with severe neurological deficits just short of coma had significantly better results with reperfusion. This was supported by Brown et al.33 in 1982 who showed that revascularization in patients with preoperative coma (lack of meaningful response to verbal or noxious stimuli) was indicated when ischaemia had only been present for a short period of time prior to surgery (Figure 139.3).

PSEUDOANEURYSMS AND INTIMAL INJURY

Pseudoaneurysms (focal, eccentric widening of the lumen extending beyond the arterial wall), intimal defects and intimal flaps, have been managed conservatively.34, 35 Studies are small and the safety of a conservative approach, particularly with respect to the carotid artery, has not been confirmed. Clearly, the risk of surgery should not exceed the risk of complications developing from the arterial defect. Patients managed conservatively should be followed with arteriography to confirm that the lesion does not increase in size.

Vertebral artery injury With the increased availability of angiography, vertebral artery injury is being recognized more frequently.36 Patients may present with acute bleeding or with late

Chapter 139 Neck trauma I

1773

Suspected carotid injury

Unstable patient

Stable patient

Active bleeding

Pulse deficit

Expanding haematoma

Bruit

Unresponsive shock

Pulsatile haematoma

Angiogram

Reperfuse

Carotid artery injury

Reperfuse

Ligate

Coma 4--6 hours

No ischaernic infarct

Ischaemic infarct

Distal flow

Brain oedema Complete distal occlusion Inaccessible high injury

Figure 139.3

Recommended protocol for carotid ligation versus reperfusion.

complications of thrombosis, false aneurysm formation, bleeding,

arteriovenous fistula

and

stroke.

Torrential

bleeding may be encountered from a lacerated vertebral artery. Arteriovenous

fistulae may present with a bruit,

thrill, haematoma, neurological deficits or cardiac failure. A neurological deficit is

seldom acquired in the presence

of a normal contralateral vertebral artery and intact collateral circulation.37 Mortality from isolated vertebral artery

trauma ranges from 5 to 17 percent, but increases associated inju ry of the carotid artery.38 The majority of vertebral artery injuries can be managed by angiographic embolization. If the injury is discovered at emergency surgery, the vessel should be ligated. This is fairly simple if the injury is located at a point before the vertebral artery enters the foramen to 50 percent if there is an

transversarium at the level of the sixth cervical vertebra. Once inside the vertebral canal, ligation may be achieved

Figure 139.4

with ligaclips. Alternatively, bone

caroticojugular fistula following stab wound at skull base.

wax:

should be

used to

False a neurysm of internal carotid artery, with

tamponade bleeding and followed with angiographic . embolization. [Grade C]

and

139.5), Bleeding from branches of the external

carotid artery can be controlled by embolization. ENDOVASCULAR TECHNIQUE

Endovascular techniques

are playing an increasing role in the treatment of both ac u te and late manifestations of vascular trauma, particularly at the skull base. False aneurysms and arteriovenous fistulae can be treated with covered stents, or by trapping the lesion proximally and distally with balloons or coils, once good crosstlow in the Circle of Willis has been demonstrated (Figures 139.4

VENOUS INJURlES

The external

jugular vein may be ligated if injured. The vein may be repaired by lateral venor­

internal jugula r

rhaphy or ligated. Should both internal jugular veins have been injured, then at least one ve i n should be repaired to prevent sequelae of raised intracranial pressure such as blindness, syndrome of inappropriate antidiuretic

1774

I PART

14 THE

NECK

•

Exposure: - chest and face for zones I and III injuries, to permit additional surgical exposure if required; - contralateral groin and lower leg to permit harvesting of saphenous vein for grafting.

•

Approach: - localized injury: horizontal skin crease incision, subplatymal flaps; - wider exploration: long incision along anterior border of sternocleidomastoid muscle.

•

Additional exposure: - zone I: divide omohyoid muscle; bilateral exploration: apron flap; - zone III: anterior dislocation of the mandible.

SUMMARY Figure

139.4),

139.5

Trapped false aneurysm and fistula (Figure

Management of penetrating injuries of the neck remains

wi th coils inserted by endovascular technique.

controversial for the following reasons: paucity of well­ designed,

large,

prospective

studies;

the

uncommon

hormone secretion (SIADH) and even death. A remnant

occurrence of oesophageal injuries; the variable levels of

of the contralateral internal jugular vein or saphenous

radiological and surgical expertise at trauma centres confronted with cervical injuries; the wide spectrum of

vein can be used should a graft be required. [H 1

the site and severity of injuries and of aetiological agents; and the lack of a uniform classification system when

Chylous injury Patients with

zone

reporting neck injuries. I

injuries

can

rarely

develop

a

chylothorax due to injury to the thoracic duct or right lymphatic duct. Although one can attempt a diet of medium-chain triglycerides, Worthington et

al.39 re­

ported that conservative treatment is uniformly unsuc­ cessful and advocate early ligation via thoracotomy. [H]

Neurological injury

......

-_

......

-... .... . -.. ... .... _ ...

.......

_----

_-----

•

Properly executed, se)cctive management of penetrating inj uries of the neck is as safe as

•

Trauma

mandatory exploration.

centr�s need

to design their

in accordance with their investigative and therapeutic ca pabili t ies.

manag,ement protocols Significant brachial

nerve injury should be repaired

electively within 24-72 hours unless the neck is explored

" '\'

for other reasons. [Grade D)

EXPLORATION OF THE NECK: GENERAL PRINCIPLES •

Intravenous infusion lines, suction, blood in theatre.

•

General anaesthesia: Crash induction with cricoid

•

Airway:

pressure. - nasotracheal/orotracheal intubation; _

•

cricothyroidotomy or tracheotomy.

Position: _

_

supine; neck extended, turned to opposite side) if no C­ spine injury.

Deficiencies in curre nt knowledge and areas for future research » Significance of delayed repair of cervical oesophageal inj ury.

).- CT scan for initial evaluation of penetrating cervical inj ury.

> MRI scan for initial evaluation of penetrating cervical injury.

>- Endbvascular intervention for vascular trauma.

; '.. -

Chapter 139 Neck trauma

REFERENCES

I 1775

hypopharyngeal-cervical esophageal funnel. Journal of Trauma. 1997; 42: 675-9.

1.

2.

Roon AJ, Christensen 1\1. Evaluation and treatment of

19.

penetrating cervical injuries. Journal of Trauma. 1979; 19:

Flanigan DP. The necessity of mandatory exploration of

391-7.

penetrating zone II neck injuries. Surgery. 1986; 100:

Atta HM, Walker NIL. Penetrating neck trauma: Lack of universal reporting guidelines. American Surgeon. 1998;

655-60. 20.

64: 222-5. 3.

5. 6.

1984; 50: 198-204. 21.

7.

Bailey H (ed.). Surgery of modern warfare, 3rd edn.

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Fogelman IVlJ, Stewart RD. Penetrating wounds of the

22.

A, Gens DR et 01. Flexible endoscopy for the dianosis

Stone HH, Callahan GS. Soft tissue injuries of the neck.

of esophageal trauma. Journal of Trauma. 1996; 40:

Demetriades D, Charalambides D, Lakhoo M. Physical

261-5. 23.

RS, Krevsky B. Role of flexible endoscopy in the evaluation of possible esophageal trauma after

Journal of Surgery. 1993; 80: 1534-6.

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Metzdorff MT, Lowe DK. Operation or observation for 24.

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treatment of penetrating injuries to the cervical esophagus. Laryngoscope. 1983; 93: 801-4.

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25.

Narrod JA, Moore EE. Selective management of

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26.

Kantarovsky A, John KD et 01. lVIanagement of penetrating

Surgery. 1984; 119: 574-8.

injuries of the cervical trachea. Annals of the Royal

Campbell FC, Robbs N. Penetrating injuries of the neck: A

College of Surgeons of England. 1997; 79: 195-7.

27.

America. 1996; 76: 661-83.

Ngakane H, Muckart DJJ, Luvuno FM. Penetrating visceral injuries of the neck: Results of a conservative

28.

The role of angiography in penetrating neck trauma.

908-10.

Journal of Trauma. 1991; 31: 557-63.

Weigelt JA, Thai ER, Snyder III WHo Diagnosis of

29.

Asensio JA, Chahwan S, Forno W, MacKersie R, Wall M,

Archives of Surgery. 1987; 122: 592-7.

30.

Lake J et 01. Penetrating esophageal injuries: Multicenter study of the American Association for the Surgery of

in patients in stable condition. Physical examination, angiography, or color flow Doppler imaging. Archives of

Demetriades D, Theodorou D, Cornwall E, Asensio J,

Surgery. 1995; 130: 971-5.

31.

injuries: Mandatory operation is not necessary. Journal of Gracias VH, Reilly PM, Philpott J, Klein WP, Lee SY, Singer

248-54. 32.

M et 01. Computed tomography in the evaluation of

587-92. 33.

Surgery. 1982; 144: 748-53.

DH. Management of traumatic hypopharyngeal injuries. 18.

Brown IVlF, Graham JM, Feliciano DV, Mattox KL, Beall AC, De Bakey ME. Carotid artery injuries. American Journal of

Fetterman BL, Shindo ML, Stanley RB, Armstrong WB, Rice Laryngoscope. 1995; 105: 8-13.

Liekwig WG, Greenfield U. Management of penetrating carotid artery injury. Annals of Surgery. 1978; 188:

penetrating neck trauma. A preliminary study. Archives of Surgery. 2001; 136: 1231-5.

Bradley EL. Management of penetrating carotid injuries: An alternative approach. Journal of Trauma. 1973; 13:

Trauma. 1996; 40: 758-60.

17.

Demetriades E, Theodorou D, Cornwell III E, Weaver F, Yellin A, Velhamos G et 01. Penetrating injuries of the neck

Trauma. Journal of Trauma. 2001; 50: 289-96. Belzberg H, Velhamos G et 01. Transcervical gunshot

16.

Meyer JP, Barret JA, Schuler JJ, Glanigan DP. Mandatory vs selective exploration for penetrating neck trauma.

Journal of Surgery. 1987; 154: 619-22.

15.

Sclafani SJ, Cavaliere G, Atweh N, Duncan AO, Scalea T.

management policy. British Journal of Surgery. 1990; 77:

penetrating cervical oesophageal injuries. American

* 14.

Demetriades D, Asensio JA, Thai E. Complex problems in penetrating neck trauma. Surgical Clinics of North

Surgery. 1980; 67: 582-6.

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Levy RD, Degiannis E, Hatzitheophilou C, Maberti P,

penetrating neck injuries. A prospective study. Archives of

prospective study of 108 patients. British Journal of

* 12.

Winter RP, Weigelt JA. Cervical esophageal trauma. Incidence and cause of esophageal fistulas. Archives of

America. 1991; 71: 267-96.

* 11.

Mandai AK, Bui HD, Oparah SS. Surgical and nonsurgical

Asensio JA, Valenziano CP, Falcone RE, Grosch JD. surrounding zone II injuries. Surgical Clinics of North

* 10.

Srinivasan R, Haywood T, Horwitz B, Buckman RF, Fisher

patients with penetrating injuries of the neck. British

penetrating neck wounds? A retrospective analysis. 9.

Flowers JL, Graham SM, Ugarte IVIA, Sartor WM, Rodriguez

neck. American Journal of Surgery. 1956; 91: 391-3.

examination and selective conservative management in

8.

Wood J, Fabian TC, Mangiante EC. Penetrating neck

Baltimore: Williams 8: Wilkins, 1944: 674.

Surgery, Gynecology and Obstetrics. 1963; 117: 745.

*

Dunbar L, Adkins RB, Waterhouse G. Penetrating injuries to the neck. Selective management. American Surgeon.

Hall JR, Reyes HM, Meller JL. Penetrating zone-II neck injuries in children. Journal of Trauma. 1991; 31: 1614-7.

4.

Bishara RA, Pasch AR, Douglas DD, Schuler JJ, Lim LT,

34.

Stain S, Yellin AE, Weaver FA, Pentecost MJ. Selective

Stanley RB, Armstrong WB, Fetterman BL, Shindo ML.

management of nonocclusive arterial injuries. Archives of

Management of external penetrating injuries into the

Surgery. 1989; 124: 1136-41.

1776 I 35.

PART 14 THE NECK

Frykberg ER, Crump JM, Vines FS, McLellan GL. Dennis JW,

and management. Journal of Trauma. 1987;

Brunner RG et al. A reassessment of the role of

856-63.

arteriography in penetrating proximity extremity trauma.

36.

McLaughlin OJ, Modic M, Masaryk T, Pratt 0, Huang D. A

A prospective study. Journal of Trauma. 1989; 29:

new approach to the treatment of penetrating injuries

1041-52.

to the vertebral artery. Vascular Surgery. 1998;

Meier DE, Brink BE, Fry WJ. Vertebral artery trauma -

639-46.

acute recognition and treatment. Archives of Surgery. 37.

38.

27:

39.

32:

Worthington MG, de Groot M, Gunning AJ, Von Oppel UO.

1981; 116: 236-9.

Isolated thoracic duct injury after penetrating

Golueke PJ, Sclafani E, Phillips T, Goldstein A, Scalea T,

chest trauma. Annals of Thoracic Surgery. 1995; 60:

Duncan A. Vertebral artery injury - diagnosis

272-4.

140 Benign neck disease: infections and swellings PETER CLARKE

Introduction Cystic lesions Lateral cervical cysts and branchial abnormalities Benign tumours Infections Deep neck space abscesses Conclusion

1777 1777 1779 1782 1783 1785 1786

Key points Best clinical practice Deficiencies in current knowledge and areas for future research References Further reading

1787 1787 1787 1787 1788

A PubMed search revealed only reviews and case reports. Searches for the following were undertaken: branchial cysts, branchiogenic carcinoma, lateral cervical cysts, thyroglossal cysts, dermoid cysts, bronchial cysts, lymphangiomata, cystic hygroma and thymic cysts. A search combining 'neck' and 'cervical' and the following was also undertaken: TB, HIV, AIDS, infectious mononucleosis, cat scratch disease, toxoplasmosis, brucellosis, Kikuchi's disease, actinomycosis, necrotizing fasciitis, abscess and infection. There are no Cochrane Reviews, and no level II or I evidence for the management of these conditions. Management recommendations are supported only by retrospective reviews and expert opinion. [Grade D]

INTRODUCTION

i

\

I

L_

Benign neck swellings may be nodal or cystic, lateral or central, neoplastic, inflammatory or congenital. Thyroid and salivary gland swelling will be considered elsewhere. Maisel 1 suggests that 80 percent of nonthyroid neck masses are neoplastic. Of these, 80 percent are metastatic and three-quarters are from primaries above the clavicle. , Although the statement above might be prefaced by a suggestion that this applies to masses over 2 em in diameter in those over 35 years of age, it is a useful rule of thumb - especially for those not regularly seeing patients with neck masses. This compares with 90 percent of neck masses in children representing benign conditions of which 55 percent were congenital. 2 [**/*] Neck masses are often enlarged lymph nodes, which may be reactive to either local or generalized infection. Nodal swellings may be suppurative or nonsuppurative. Pus may also spread from a local focus of suppuration related to, for example, the tonsil or a tooth. Swellings

may also be related to cystic structures, the most common of which are branchial cysts and thyroglossal cysts. These may present as infections or become infected. Benign tumours, particularly neurogenic tumours may present as neck swellings. Initial investigation should include history, physical examination and fine needle aspiration for cytology or microbiology and culture. This, with knowledge of the anatomy and physiology should allow an accurate diagnosis in most cases. Ultrasound scanning, computed tomography (CT) and magnetic resonance (MR) images will often be helpful in difficult cases.

CYSTIC LESIONS Thyroglossal cysts Most authors suggest these are the most common cystic lesions in the neck. Up to 50 percent will present in

1778

I PART 14 THE NECK

30 percent presenting before ten

present suprahyoid and just over 10 percent related to the

years of age. There is an equal male-to-female incidence

thyroid. Because of the hyoid attachments, these masses

(Figure 140.1).

move both on swallowing and protrusion of the tongue.

Embryology

may result in the development of a sinus from the cyst to

adulthood, with over

Cysts may become infected and incision and drainage the skin.

During the development of the branchial apparatus in a four- to five-week-old embryo, the thyroid enJage arises in an invagination of endoderm in the floor of the pharynx and develops caudally, with the descent of the great vessels. It enJarges and becomes bilobed, coming to rest at the root of the neck as the thyroid gland by the end of the seventh week. T he tract that is left usually atrophies and disappears, although the caudal end often remains as a

Pathology Thyroglossal cysts are usually lined by columnar epithe­ lium with small glands frequently containing thyroid colloid.

Decalcification of the hyoid bone

will often

confirm that the tract passes through it. 4

pyramidal lobe. Failure of the tract to involute may leave epithelial remnants or an open area of duct, which may expand into a cyst

because

of an accumulation

of

secretions. The hyoid bone, developing later, may entrap a portion of the thyroid duct or draw it caudally, leaving the duct dorsal to the bone.3

The diagnosis is clinical and may be confirmed with a fine needle

aspirate

if

the

mass

is

suprahyoid

to

help

differentiate it from a dermoid cyst or submental lymph node.

Clinical features

Further

investigations

are

not

necessary

for

diagnosis, but should be undertaken to ensure that a

Most present as midline masses 2-3 cm in diameter just inferior to the hyoid bone

Management

(Figure 140.1).

A quarter

normal thyroid gland is present. Ultrasound scanning is accurate,

cost-effective

investigation

of

and

choice

would

and

is

seem

the

to

most

be

the

common

investigation undertaken in the UK. Radionuclide scan­ ning may be reserved for patients with normal thyroid glands that cannot be localized. Thyroglossal cysts should be treated surgically. An infected cyst may be aspirated for microbiological culture and to avoid incision into the cyst. A horiwntal skin crease incision is made over the cyst and skin flaps raised superiorly and inferiorly. Care should be taken through­ out the dissection to avoid rupturing the cyst.

For

infrahyoid cysts, the sternohyoid and thyrohyoid muscles must be reflected laterally and stripped off the hyoid to reveal the cyst. The cyst is dissected from surrounding tissues up to the body of the hyoid. The hyoid is then divided with heavy scissors just medial to the lesser horns on both sides. A cuff of suprahyoid and intrinsic tongue muscles is then taken with the body of the hyoid. A specific tract is often not found, but if a convincing tract is seen, this should be followed into the musculature of the tongue base and removed. There is no need to resect epithelium of the tongue base. Sistrunk described this operation in

1920.5 Recurrence rates are significantly less

after Sistrunk's operation than after simple cyst excision. If a sinus is present, an ellipse of skin around the sinus is removed in continuity. Surgery for recurrent cysts should include excision of the hyoid and a cuff of tongue base muscle if the hyoid has not previously been excised, or excision of the cyst with a surrounding cuff of muscle if Sistrunk's operation has previously been performed. Figure 140.1

large thyroglossal cyst presenting in late

There have been a large number of case reports and

adulthood with symptoms of obstructive sleep apnoea. Elevation

small

of cyst on tongue protrusion demonstrated.

thyroglossal cyst. Up to

S'eries

of

thyroid

carcinoma

presenting

in

a

90 percent of these are papillary.

2-8% Vs 85%

1779

Chapter 140 Benign neck disease: infections and swellings I

6

A recent review from Slone Kettering suggests treatment

should be with Sistrunk's operation and thyroid suppres­ sion wit h thyroxine. Thyroidectomy and postoperative

Pharyngeal clefts

�.,-'

Ph�ry.,geat

Pharyngeal pouches

a rch es

radioactive iodine, although revealing malignancy in a quarter of patients, did not confer a survival advantage and they conclude that this should be reserved for high­ risk groups. [Grade D]

--="'-"---

1. Eustachian tube and middle ear cleft

LATERAL CERVICAL CYSTS AND BRANCHIAL ABNORMALITIES Lateral cervical or branchial cysts occur equally

in the

2'----1---.,.,

sexes and present most commonly in young adults,

3:----+-1---1

although they can occasionally present at any age. There is

3. Inferior parathyroid thymus

4----r+-�=:OO'�

debate about the precise aetiology of the cysts and fistulae and there may be a number of different aetiologies resulting in

r-----

.

�-

a similar clinical presentation.

4. Superior parathyroid u ttimobran chial body

Branchial fistulae Branchial

fistulae usually present in childhood as a

weeping defect along the anterior border of sternoclei­ domastoid, or occasionally as an acute infection.

Figure 140.2

Development of the pharyngeal ( branchial)

apparatus. The second arch grows over the third and fourth pharyngeal arches so burying the second, third and fourth pharyngeal clefts. The remnants of these clefts form the cervical sinus of His.

EMBRYOLOGY

ultimobranchial body

During the fourth week of intrauterine life, (or pharyngeal)

six branchial

(Figure 140.2) and hence extend

from the pharynx to the thyroid. The fact that fourth

arches develop as neural crest cells

branchial fistulae only seem to occur on the left would

fifth

suggest that the timing of the descent of the aorta allows a

migrate into the head and neck region. During the

week, the second branchial arch grows over the third and fourth branchial clefts which form a cervical sinus

(Figure

fistula to remain on the

left whilst descent of the

subclavian artery may obliterate potential fistulae on the

140.2). Failure of the cervical sinus to close may therefore

right. Although fistulae undoubtedly exist running from

potentially

the piriform fossa to the skin and are presumably related

communicate

with

the

second

branchial

pouch (and therefore the tonsil fossa), the third branchial

to the branchial apparatus, the exact mechanism of their

pouch in the area of the larynx or the fourth branchial

formation cannot be certain. Often described in the literature as fourth branchial pouch fistulae, they present

pouch opening in the piriform fossa.

on the left, often involve the thyroid gland and may present as a suppurative thyroiditis PRESENTATION

(Figure 140.4). They

usually present in children though may be asymptomatic

Clinically, second branchial cleft fistulae are the most

until adult life.

[** /*]

common. They have a cutaneous opening along the anterior border of the sternocleidomastoid, usually at the junction of the middle and lower thirds, and track up

MANAGEMENT

through the neck to rW1 between the internal and external

Fistulae may be investigated with a sinogram. Pharyngo­

(Figure

scopy with careful examination of the tonsil fossa and

carotid arteries and end in the tonsillar fossa

140.3). Third and fourth branchial fistulae are more rare,

piriform

fossa

mucosa,

depending

on

presentation,

should be undertaken prior to excision.

opening low in the neck and ending in the piriform fossa.

Surgical excision should be started with an elliptical

The third arch fistula should pass over the hypoglossal

excision of the fistula opening. Dissection of the track can

nerve and the fourth arch fistula should run down into

be helped by careful injection of dye into the opening

the chest, running below the arch of the aorta on the left

before commencement. A second branchial fistula

or subclavian archway on the ri gh t. It is doubtful whether

need one or more further skin incisions to allow safe

these exist as clinical entities, however. A recent pape?

dissection to the carotid. Excision of the tonsil fossa

suggests that 'fourth branchial cleft fistulae' arise from the

opening or dissection in the fossa will allow excision in

_.�

will

-1 .�_.__-.

:.

-

1780

I PART 14 THE NECK

Figure 140.3

Second branchial cleft fistula.

between internal and external carotid.

(b)

(a)

Dissection followed up through neck and second incision to allow safe dissection

Opening of fistula in tonsil fossa demonstrated by appearance of dye.

Lateral cervical (or branchial) cysts The cervical sinus normally closes by six weeks leaving a cervical vesicle and it is this trapped ectoderm which) it is po stulated, forms the lateral cervical or branchial cysts commonly seen in young adults. There are a number of theories of origin for these lateral cervical cysts, which have a constant clinical pr esentation anterior to the upper third of sternocleido­ mastoid in young adults. They usuaHy appear fairly quickly often in relation to an upper respiratory tract infection. There is a slight left-sided predominance

.

All

three main theories of their aetiology have some merit. 8 Figure 140.4

Fourth branchial arch fistula. Young adult

presenting with anterior neck abscess and fistula

1.

Origin from a branchial pouch remnant:

A tract

from lateral cervical cysts running between the

communicating with left pyriform fossa. Lifetime history of

internal and external carotid would bolster this

intermittent weeping from small punctum in left neck.

theory and is sometimes described but often not proven histologically and may be a surgically

continuity

(Figure 140.3).

Removal of a fourth branchial

produced

arch fistula should include excision of the thyroid tissue

common

a g gregati on of tissue. Certainly, in with several eminent surgeons, the

as necessary to allow removal of the whole tract. Ligation

author has never seen a tract from a branchial

of the tract at the pharyngeal mucosa can then be

cyst. 'Detailed histological mapping of the

undertaken. [Grade D 1

cytokeratin profile of lateral cervical cysts and

Chapter mucosa from the tonsil fossa does, however, suggest an origin from this area. 9 2. Origin from the cervical sinus: This theory proposes the branchial cleft rather than pouch forms cysts. Overgrowth of the second arch produces the cervical sinus of His (Figure 140.2) which closes to leave the cervical vesicle, which, it is postulated, results in the lateral cervical cyst. This is an attractive theory embryologically, although does not fully explain why cysts usually do not appear clinically until the third or fourth decade and it might be expected that more cysts are found with tracts or sinuses running to the skin. 3. Origin from lymph node degeneration: Originally postulated in the nineteenth century, Kingl0 in 1949 studied a large number of cysts and concluded that cystic transformation of lymph nodes was the likely origin of lateral cervical cysts. Certainly most cysts have lymphoid tissue similar to lymph nodes in their walls and few, if any, have tracts to the skin or pharynx. It might be expected, however, if this theory were true that the position in the neck may be more variable.

140 Benign neck disease: infections and swellings

I 1781

infection, attempts should be made to aspirate the contents and treat the patient with intravenous antibiotics, though incision and drainage may be necessary. Imaging is not usually necessary to make the diagnosis. Lateral cervical cysts should be surgically removed. After a transverse skin crease incision over the cyst is made, dissection is followed along the anterior border of the sternocleidomastoid. Blunt dissection close to the cyst is usually simple and allows removal of the cyst. If there has been previous infection or the cyst is very adherent to local tissues, dissection should continue as for a neck dissection with identification of the spinal accessory nerve, carotid sheath and hypoglossal nerve with resection of the cyst and surrounding nodes and fibrous tissue. Proponents of a branchial sinus origin suggest looking for a cord or tract extending between the internal and external carotid and possibly examination of the tonsil fossa prior to excision of the cyst. If cysts have presented with acute infection and been treated with antibiotics, the cyst will occasionally not be apparent after resolution and unless radiology - either ultrasound scan or CT scan shows an obvious cyst, these cases can be treated expectantly. [Grade D]

Branchiogenic carcinoma PRESENTATION

In early adulthood, a cystic, often tender oval mass 4-6 cm in diameter anterior to the upper third of the sternocleidomastoid develops fairly quickly. A recent upper respiratory tract infection is often described. Swelling may be intermittent and occasionally the cyst is overtly infected with overlying erythema and pain at presentation. The main differential diagnosis is from reactive lymphadenopathy. In children, a dermoid cyst or rhabdomyosarcoma should also be considered. In young adults, lymphoma, tuberculosis (TB) or nerve sheath tumours need to be excluded. In patients over 35 years of age, a cervical metastasis from a head and neck primary must be excluded.

PATHOLOGY

Lateral cervical cysts are usually lined with squamous . epithelium with transitional type pseudo stratified epithelium. Cysts presenting in the younger age group are more likely to be lined with respiratory epithelium.

MANAGEMENT

The differential diagnosis can be simplified considerably by fine needle aspiration. Occasionally straw-coloured fluid is obtained, though more often, greasy yellow thick mucoid material is aspirated, probably representing recent inflammation. Should they present with acute

Opinion on the existence of this entity varies from the suggestion that it is underdiagnosed to denial of its existence. Following initial descriptions and the coining of the term 'branchiogenic carcinoma', it became clear that the majority of these cases were actually metastatic deposits from primary squamous cell carcinomas in the head and neck. In 1950, Martin et al. 11 therefore suggested that to diagnose a branchiogenic carcinoma, four criteria had to be met. 1. The cervical tumour occurs along the line extending from the tragus to the clavicle, along the anterior border of sternocleidomastoid. 2. The histological appearance must be consistent with an origin from tissue known to be present in branchial vestiges. 3. No primary source of the carcinoma should be discovered after at least five years of follow-up. 4. There is histologic demonstration of cancer arising in the wall of an epithelial-lined cyst. Even apparent fulfilment of these criteria, however, cannot exclude a metastatic squamous cell carcinoma. A cystic metastasis may have many similar features, histologically, to a branchial cyst and the addition of radiotherapy to the excision of these masses will treat small tonsil or tongue base tumours, so making even 12 adherence to Martin's criteria meaningless. Most metastatic nodes 'with unknown primary' are solid and most cystic nodes have primary tumours that

1782 I

PART 14 THE NECK

can be demonstrated in the tongue base or tonsil. Several 136

cases

of

cystic

squamous

cell

and about a

in the neck. They are uncommon

with only five cases per 3000 admissions to a pa(�(ll;atfllc

carcinoma,13 without an obvious primary site of origin

hospital and only account for four of 152

at initial diagnosis, have shown that in the vast majority a

of the neck. These lesions are COJngt�nltal, lyn1prlalllC stasis caused by congenital blockage

tumour can be found. Most are in but other sites found include

of a

There is no innate reason

and

lateral cervical cyst should not become

certainly there are case reports of tumours that fit Martin's criteria, but estimates

from

budding that arises from the lymphatic sacs. Capillary or simple

1.

lymphangiomas are comprised of capillary-like

that the incidence

if they

lyn1Pl1atllc vasculature and are usually

is in the

.... ,..,,' ...... .__ t-"rY'O'.b,..

of all head and neck carcinomas. 14

of 0.3

or

sequestration of the primitive embryonic endothelial

palate, sinuses and salivary

of

lymphatic

thyroid,

or oral

skin

and confined to the They are pale, small vesicle-like

lesions and no treatment is necessary. Cavernous

2. Cavernous

of dilated

lymphangiomas are lymphatic channels and These originate from ectoderm and mesoderm, and the head and neck is the most common site to find them. can

at any age, but the majority present in

patients aged under six years. Sex distribution is equal and

occur in the

tongue, cheeks and lips,

DERMOID CYSTS

fusion of the

along lines of

in the midline or lateral

facial processes to the submandibular

They occur in the sub-

cutaneous tissues and contain skin aplJenlaa.ges hair follicles, sebaceous

and sweat

wounds.

These account for 40 percent of lymphangiomas. hygromas are

3.

similar to cavernous lymphangiomas with thin-walled sinuses and cysts that are lined by flat endothelial cells and contain eosinophilic, acellular

fluid. In cystic hygromas, there

are larger

masses which may communicate

or be isolated.

so

differentiating them from epidermoid cysts. Dermoid cysts may also occur from implantation

diffuse swelling.

to

surgical excision is required

PRESENTATION Lymphangiomas occur predominately in the neck, often triangle where tissue planes are looser

in the

and is usually straightforward.

than in the lips, tongue and cheek. As these enlarge, they may involve the cheek,

BRONCHIAL CYSTS These are uncommon congenital lesions that can occur in the suprasternal notch, but are more common within the thoracic

or mediastinum. They are more common in

males and one-third are associated with a sinus. The cysts are lined by ciliated pseudostratified and tpe sinus with stratified squa-

columnar mous epithelium.

eXClSIOn is curative.

Cervical or plunging ranulae are considered glands

with

147, Non-neoplastic

gland diseases) and laryngocoeles are considered with the larynx

oral cavity and media­

stinum or axina. In the neck they are fluctuant, soft,

88, Congenital disorders of the larynx,

trachea and bronchi).

they

diffuse masses with indistinct margins. transilluminateo The majority of lymphangiomas are may now be

at birth and

prenatally. It is extremely rare for

these to present after the age of two years, but recurrence after

treatment may occur after many years.

are usually asymptomatic, but are a distressing cosmetic problem.

hygroma and cavernous lymphangioma

either grow

or remain static and there have

also been many reports of involution. may, however, cause or airway largest

or infection

growth and symptoms of which may be

The

may also cause airway problems

without infection or bleeding.14[**I*]

MANAGEMENT Magnetic resonance imaging

(MRl) delineates the extent of

the lesion well and will facilitate accurate diagnosis and These benign lymphatic lesions can be divided into three morphological types:

cavernous and

One-third of cases are found in the oral cavity and cheek

pretreatment pi arming. Prenatal diagnosis may be made with ultra�ound assessed with

and potential airway compromise

MRl. There are case reports of

Chapter 140 Benign neck disease: infections and swellings

infants being treated ex utero to avoid airway problems at birth. In this country, the majority are not diagnosed until birth and treatment depends on the size and anatomical location of the lesion. Treatments used for lymphangioma include surgical excision and intralesional injections of such sclerosants as bleomycin, tetracycline and alcohol. Surgery is challenging and should be undertaken in specialist centres. With time and expertise, results can be excellent and surgery remains the treatment of choice. Resection is often limited to the neck initially and recurrence rates can be high and may occur rapidly or after many years. Surgical excision may be helped by the injection of tissue blue into the lymphatic spaces. A staging system has been used to predict surgical outcome (Table 140.1).15,16 Injection of sclerosants may lead to scarring and make subsequent surgery more difficult, but a new intralesional injection of OK-432 (picinabil) has shown promising results. It is thought to produce an inflammatory reaction leading to adhesions or to increased permeability of the endothelial lining, increasing the rate of drainage from the lesion. Pooled results from a group of series show marked shrinkage in 72 out of 116 children. It may be less effective in cavernous lymphangioma and lymphangioma with haemangiomatous elements. 17 [**1*]

I 1783

develop from lymph nodes, may develop in any of the cystic masses described above or may spread from local abscess formation around teeth, tonsil or from trauma. Tuberculosis, infectious mononucleosis, human immunodeficiency virus (HIV) infection, cat scratch fever and toxoplasmosis may all produce generalized cervical lymphadenopathy.

Tuberculosis Cervical lymphadenopathy is the most common head and neck manifestation ofTB and can occur in any age group, though most series suggest the most common presentation is in young adults. Most series suggest only 10-20 percent to have associated pulmonary disease or to have had a history of contact with TB. Up to half of these patients will have systemic symptoms. It has been suggested that the bacillus enters via the tonsils as tonsillectomy specimens will often show evidence of TB infection in these patients. Nodes tend to be tender and may have overlying erythema or occasionally a discharging sinus. Suspicion should be raised in immunocompromised patients, those from regions with high levels of TB, such as the Indian subcontinent and parts of the former Soviet Union, as well as parts of Africa. IS, 19 [**1*]

Paragangliomas and nerve sheath tumours These neoplasms of neural crest origin arise from the paraganglionic cells in the carotid body, vagal ganglia and jugulotympanum and, less commonly, other areas including the larynx, nose and orbit. Most present in the parapharyngeal space with swelling of the pharynx and fullness in the upper neck. These are considered in more detail in Chapter 191, Tumours of the parapharyngeal space.

INFECTIONS Infections may be suppurative or nonsuppurative and related to lymph nodes or neck spaces. Suppuration may

Stage 1 Stage 2 Stage 3 Stage 4 Stage 5

Unilateral infrahyoid disease Unilateral suprahyoid disease Unilateral infrahyoid and suprahyoid disease Bilateral suprahyoid disease Bilateral supra and infrahyoid disease

17 41 67 80 100

MANAGEMENT Ultrasound scanning of the nodes will often show multiple matted nodes most commonly in the upper deep jugular chain or supraclavicular region. A fine needle aspiration (FNA) should be undertaken and will often reveal mycobacteria. With an increased number of multiresistant strains of TB, however, an excision biopsy is often needed to establish sensitivities. 20 This may become less necessary as the application of DNA probes and polymerase chain reaction (PCR) becomes more widespread in the future. When FNA and PCR were used together, a specificity of 84 percent and sensitivity of 100 percent were found. 21 If possible, total excision of the node or nodes is suggested rather than incisional biopsy to reduce the chance of developing a discharging sinus. This may mean utilizing neck dissection techniques to undertake a selective neck dissection often with some excision of the sternocleidomastoid, which is commonly involved if the nodes are very inflamed and matted. Antituberculous chemotherapy should be started immediately after surgery and adjusted according to sensitivities. [**1*]

Infectious mononucleosis Although generally diagnosed and seen in the ear, nose and throat (ENT) setting due to the acute tonsillitis, generalized lymphadenopathy is often present. EpsteinBarr virus infection often occurs in childhood and usually

1784

I PART 14 THE NECK

results in a mild self-limiting illness characterized by

syndrome (AIDS) or a poor prognosis. Enlarging or

fever, pharyngitis, tonsillitis and lymphadenopathy. How­

tender nodes are not typical of PGL and in a series of HIV

ever, in teenagers and young adults, it may present with

patients with enlarging or tender nodes none had PGL

significant tonsillar enlargement causing airway compro­

and a diagnosis of TB was made in eight, opportunistic

mise and rarely death. Suppuration of lymph nodes can

nocardial

occasionally occur and awareness of the possibility of

metastatic carcinoma in one.25

infection

in

two,

lymphoma

in

one

and

splenic rupture is important. The enlarged tonsils have a characteristic grey fibrinous exudate and patients often have a typical 'hot potato' voice.

This is an infection caused by

MANAGEMENT

Bartonella henselae, a

fastidious Gram-negative bacillus acquired from exposure

Nasopharyngeal endoscopy may be a useful diagnostic tool showing the typical fibrinous membrane in the nasophar­ yngeal cavity of over 90 percent of patients with infectious mononucleosis

Cat scratch disease

and

none

of

patients

24

with

acute

tonsillitis?2 Diagnosis is made by Paul Bunnell monospot serology, detection of a viral capsid antigen-IgM or the presence of lymphocytosis with characteristic apoptotic lymphocytes present in 89 percent of blood smears of patients with acute infectious mononucleosis and only 4 percent of controls. Elevated liver function tests are usual. More recent quantitative polymerase chain reaction may allow prediction of fulminant infections. Treatment is supportive with hydration, if swallowing is a significant

to an infected kitten or cat, either directly or via cat fleas. Symptoms are of fever, arthralgia and lymphadenopathy. The presence of cat scratches or a history of a cat scratch should guide the diagnosis which is confirmed by indirect immunofluorescence antibody assay with high antibody titres

which

settle

over

several

weeks.

polymerase chain reaction RNA of

More

recent

Bartonella henselae

RNA has been used to confirm the diagnosis. The organism is sensitive to many antibiotics and so it is likely that many cases are never diagnosed. There is some evidence

that

azithromycin is

associated

with rapid

resolution, but many infections will settle without the 6 need for antibiotic therapy. 2

problem, and pain relief. Nonampicillin antibiotics are usually prescribed to prevent secondary bacterial infection. Upper airway compromise should be treated early with steroids and there are case reports of antiviral treatment with acyclovir or famcyclovir which may be of help. Tracheostomy should be avoided by the prompt use of steroids. Acute tonsillectomy has been advocated for upper airway obstruction. Only a small proportion of patients go on to have recurrent tonsillitis and so interval tonsillect­ omy is not indicated. A prospective cohort of 250 primary care patients with infectious mononucleosis or ordinary upper

respiratory

fatigue

syndrome

tract at

infections

two

months

suggested was

postviral

most

reliably

predicted by cervical lymphadenopathy and initial bed rest and at six months was predicted by a positive monospot test and lower physical fitness.23

Toxoplasmosis This is a parasitic infection with

Toxoplasma gondii which

may be asymptomatic or produce cervical lymphadeno­ pathy,

fever

and

malaise.

The

main

reservoir

for

toxoplasma in the UK is the cat, but transmission may also occur from cysts in undercooked meat. A fine needle aspiration will often show the microorganism on micro­ scopic examination.

Diagnosis can be confirmed by

serology. A retrospective review of 731 patients who had 'reactive

lymph

node

hyperplasia'

revealed

features

supporting a diagnosis of toxoplasmosis in 15 percent of cases.27 Treatment is not usually indicated.

[***1**] Brucellosis

HIV infection Persistent

The brucella group of pathogens occur in domesticated

generalized

lymphadenopathy

(PGL)

is

a

syndrome of diffuse lymphadenopathy involving two or more extrainguinal sites for greater than three months. It may be an early sign of HIV infection with up to 70 percent of patients infected developing diffuse lympha­ denopathy within the first few months after seroconver­ sion. Fine needle aspiration will usually show a reactive picture, so. excision biopsy of a node or panendoscopy is usually

not

undertaken,

indicated. will

Histology,

usually

show

if a

the

excision

reactive

is

in

patients

with

acquired

meat or dairy products or via direct contact through broken skin. An undulating fever, malaise and cervical lymphadenopathy in 20 percent of patients is typical. The diagnosis is confirmed serologically and treatment is with doxycycline and rifampicin for six weeks. Prevention by animal vaccination is the ideal.

Kikuchi's disease

follicular

hyperplasia.24 A lymphocyte depletion pattern may be seen

animals and brucellosis is caught from contaminated

immunodeficiency

Kikuchi's disease, previously known as subacute necrotiz­ ing lymphadenitis, has been increasingly recognized in the

Chapter 140 Beni gn neck disease: infections and swellings'

1785

West, having been described more commonly in Asia. It is

a histiocytic necrotizing lymphadenit is without granular cell

infiltration,

mainly

affecting

young

women.

It

presents with malaise, mild fever and painless posterior triangle cervical ly mphadenopathy The course is benign .

and resolves in weeks or months. There may be an association with systemic lupus erythematosus

(SLE)

which may develop subsequently up to three years later

.

Monitoring should therefore be considered. Fine needle

aspiration will usually be nonspecific, but diagnosis has been made with cytology. Excisional biopsy is usually required to rule out a lymphoma or TB. Histology shows areas of patchy necrosis and a paucity of neutrophils

.

Cervical necrotizing fasciitis This is a rare, but life-threatening, infection that causes progressive necrosis of the subcutaneous fat and fasciae and secondary necrosis of the overlying skin. It may complicate deep neck space infections or result directly from odontogenic or tonsillar infection. Streptococcus miIIeri or S. viridans and mixed anaerobes are commonly

found. The clinical picture is of cellulitis with dispropor­ tionate pain Reduced skin sensation may be a useful early .

sign of affected areas. CT may show oedema and air pockets in the deep and superficial neck spaces and necrotic

patches

of

skin

are

diagnos tic.

Untreated,

necrotizing fasciitis can be rapidly fatal and so early diagnosis and

aggressive

t reatment

with

intravenous

penicillin and metronidazole and surgical debridement of all necrotic areas are the keys to success

(Figure 140.5).

Skin that has not been affected should be preserved to reduce cosmetic deformity.

Actinomycosis Actinomycosis species are facultative anaerobes whose normal habitat is the oral cavity but they can cause a chronic lesion mimicking malignancy or TB, usually following surgery or trauma to the mouth. The anterior cervical triangle is most often affected and they present as a slow growing mass or as an abscess or group of abscesses with sinus tracts to the skin

(Figure 140.6). These

manifestations are probably polymicrobi al The organism .

is difficult to culture but the characteristic colonies,

Figure 140.5

NecrotiZing fasditis.

(a)

Gross oedema and

diagnostic collections of subcutaneous gas on CT. (b) Outcome following debridement and seconda ry skin grafting.

known as sulphur granules, are often seen histologically. Fine needle aspiration may also show sulphur granules. A

DEEP NECK SPACE ABSCESSES

two-month course of penicillin or cephalosporin and removal of carious teeth is usually curative. courses of

antibiotics

may be

necessary

Longer

in resistant

These

include

parapharyngeal

or

lateral

pharyngeal,

retropharyngeal and submandibular space abscesses and

cases. Infection with Nocardia species may give a similar

although less common than in the past, partially because

picture,

.

of better oral care and antibiotic treatment, may still

especially

in

immunocompromised

patients

Growth of the organism may therefore be important as

present as life threatening infections. Some series suggest

tr eatment should be with imipenem or sulphonamides for

there are slightly more men than women presenting with

Nocardia.28

these infections and the peak ages are in the third and

-

1786 I

PART 14 THE NECK

anterior to the superior half of the sternocleidomastoid. Submandibular space infections

will have localized swelling

below the mandible and may be associated with signifi cant oedema of the floor of the mouth, with elevation of the floor of mouth and tongue when it is described as Ludwig's angina. In this situation, airway compromise is significant and in one series, 75 percent of these patients required a 3o Airway co m promise is the most common

tracheostomy.

complication of deep neck space abscess, but internal jugular vein thrombosis characterized by spiking fevers, tenderness along the sternocleidomastoid and prostration, or mediastinitis are potential complications which should be anticipated.

[H/*]

MANAGEMENT

Antibiotics should be commenced before culture and sensitivities are available. Intravenous penicillin or amox­ ycillin with the addition of clavulanic acid or metronida­ zole to treat anaerobic bacteria should be the first choice. The history and examination should highlight the possibility of an abscess but investigations should include the assessment of the white cell count, inflammatory markers and radiologic assessment of the neck. A plain soft tissue lateral x-ray

will oft e n show a retropharyngeal

abscess but should be interpreted with caution in children and an orthopantomogram infection.

Ultrasound

(OPG) may show a tooth root or CT scanning will

scanning

usually delineate between cellulitis and abscess fonnation, Figure 140.6

Actinomycosis. Note multiple weeping punctae.

fourth decade. There is a dental origin in 40 percent of patients and other aetiologies are

tonsillitis with

or

without peritonsillar abscess, TB, intravenous drug abuse following injection into the internal jugular vein, man­ dibular fracture and foreign body ingestion. In up to 20 29 percent of cases, no obvious aetiological factor is found. Most bacterial cultures are polymicrobial and Streptococcus viridans is the most common organism isolated. Sta phy­ lococcus aureus and

Staphylococcus epidermidis are also

important. Anaerobes probably play a significant role in these abscesses, but are not always cultured.

but the possibility of false-negatives with either technique must be considered.

Needle aspiration under radiological Small loculated

control with either should be considered.

abscesses may be treated with intravenous antibiotics for 12-24 hours and drainage reserved for those that are 3l 32 , A signifi­

not improving or with large collections.

cant abscess that cannot be substantially drained under imaging

control should be treated with surgical 33 Retropharyngeal abscesses or parapharyngeal

drainage.

abscesses medial to the carotid sheath may be drained intraorally though care must be taken not to rupture the abscess before the endotracheal tube is safely in the trachea.

Submandibular

and

most

parapharyngeal

abscesses are best drained externally.

If there is significant airway compromise, endotracheal incubation with gas induction is usually possible� Twenty­

Clinical features

four to 48 hours of intubation may be necessary or the insertion of a tracheostomy following drainage will allow

in the

a return to the ward. Local anaesthetic tracheotomy

throat and neck, fever, a raised white cell count and raised

should be undertaken if the airway cannot be maintained

inflammatory markers. The clinical picture depends on the

for a gaseous induction.

T hese patients present acutely unwell with pain

[Hj*]

space affected. Retropharyngeal abscesses are more com­ mon in children and in adults are most commonly caused by TB of the spine. These may cause no neck swelling but

CONCLUSION

present with dysphagia, odynophagia and airway compro­ mise. The

m os t

common deep neck abscess is a lateral

pharyngeal (parapharyngeal) space infection which causes a diffuse, tender, indurated swelling which is usually

Any chapter on neck swellings should probably conclude with the advice to 'put a needle in' if there is any doubt or potential doubt about a diagnosis. In the presence of

Chapter 140 Benign neck disease: infections and swellings I

infection, this may also

be therapeutic, The addition of needle a pir a te is pr obably be t practice and the availability of this and a cyt ol ogist in a

s

ultrasound to the fine

one -stop clinic for neck

.I For deep neck space infections:

s

- start intravenous antibiotics immediately; - image and consider draining under ultrasound or

swellings is ideal and should be

CT control;

becoming commonplace,

- consider endotracheal intubation or tracheostomy

neck lump in the over 40-year-old must be treated metastatic squamous cell carcinoma from a head and

Any

as a

neck so

1787

early.

urce until another definite diagnosis is made. The

of branchial cys t in the over 40-year-old should be treated with caution and the pathologist asked to do m ultiple sections. Similarly, the diagnosis of carcinoma in a branchial cyst even if suppo rte d by histology should be treated with he al thy scepticism and a search for the

diagnosis

Deficiencies in current knowledge and areas for future research

primary.

Many of the conditions in this chapter are relatively rare and any prospective data collection or randomized trial of management options would therefore be very difficult unless multicentred or even national. For the majority of

'�i_ Thyro'glossal cysts may

�

'

,

"

-

present

at

·swallbwing. Excision I11l1st

i

th�'hYOtd. Branchial Cysts

of '� .

r

,

,(

-

"

"

•

are

any age,

author would hope to see the tertiary referral of cystic

midline, '�ssocjated "'lith the flyoid bone and

S9, move on - tons;ue protnlsion and ,

these conditions this is probably not practical. The

,

in the

oveI

include

3S

hygromas to a small number of surgeons so that progress in their management can be focused and

the body ','

controlled trials may be possible.

age group should /', .

be invesrigalted and treated as metastat ic ' ::"�' L � squamqw cell carc�f1��a:.�xcision should be ".' , undert�en',Qn�y �r f:ihe�n�d1e aspiration.� , panend��copY'�W:lth\ �Jopitd;, of the tongue ba�e-,,-�,,:.:,-:" :,' postn�al : space and: 'b iiatetabto'ilsillectomy. .-:�� ',, ' Inj'ectl9D-.'of bK�f!3ris',a'�prdm)sing new

..

It would be encouraging if a database of all cystic neck nodes with squamous carcinoma and no identifiable

I

primary could be developed to plan a trial of positron emission tomography (PET) scanning and bilateral tonsillectomy versus tonsil biopsy to prove best practice. A current European Organisation for Research and Treatment of Cancer (EORTC) trial may help decide whether the mucosal sites should be included in

",.: \

tre;1tinent for';Jymp�a-r�:gi0f!1�':' Where possibh�,;, ; :>:; s�rgical -resecti'6:n rema!h$"the_':;trcatment of�·l . ' , ' ,:",chpt�e. .,.

'fB

-lymphadenopathy

may be

atypical .J\1.ycobac/eria and

biopsy

radiotherapy fields when treating these tumours. A randomized trial of ultrasound-guided aspiration of neck abscesses versus open drainage versus antibiotics

c.aused by

therefore

alone may struggle to recruit sufficient patients as the

excision

author would predict there would be a large number of

-' I�

of the node may be required [or ,",", � - ,: 'I' ' culture and antimicrobial sensi-tivi!jes. '-. Many ea rly or sma Jll: deep neck space - infec ti ons cap be·treated with aspiration " ,.-i:"'-' under -ultrasound or CT c.ohtrol. together "'�'�.: ' , ' with intravenous antibiotics. " • Ludwig's angina has a high-1ncidence of "' · airway c�mpromise and sho.�� =

exclusions and would certainly need to be multicentred. A study looking at CT and ultrasound accuracy in the detection of pus in deep neck abscesses would be easy

"

Lr l

be considered

ea r ...

ly.

' •

,

•

\

__ � .

and expertise.

--0

tia£��ostomy .• ,< �

to plan, but choice will often depend on local availability

_,_ .�-

_

......:;..:..\'....�.:.. .:: __.-�. " .

Best clinical practice

t

_:"_

REFERENCES 1.

Maisel RH. When your patient complains of a neck mass.

2.

Torsiglieri Jr AJ, Tom.

Geriatrics. 1980; 2: 103-8.

*

SD, Potsic WP. Paediatric neck masses: guidelines for

.I If history and examination does not lead to a clear

evaluation. International Journal of Pediatric

diagnOSiS, neck swellings should have a fine needle aspirate for cytology and microbiology ideally with ultrasound imaging as part of a one-stop clinic.

Otorhinolaryngology. 1988; 16: 199-210.

3.

orientated embryology, 5th edn. Philadelphia: WB

,/ Lymphangiomas should be treated surgically in a specialist unit.

.I Fine needle aspiration and polymerase chain reaction should be utilized for the diagnosis of lB.

b

Moore KL, Persaud TVN. The developing human, clinically Saunders, 1993.

4.

Ellis PDM, Van Nostrand AWP. The applied anatomy of thyrog I ossa I tract remnants. Laryngoscope. 1977; 87: 765-70,

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-

---.:..---

1788 I 5. 6.

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Sistrunk WE. The surgical treatment of cysts of the

22.

Keerl R. Nasopharyngeal endoscopy adds to reliability of

Patel SG, Escrig M, Shaha AR, Singh B, Shah JP.

clinical diagnosis of infectious mononucleosis. Journal of Laryngology and Otology. 2001; 115:792-5.

Management of well-differentiated thyroid carcinoma presenting within a thyroglossal duct cyst. Journal of 7.

23.

Surgical Oncology. 2002; 79: 134-9; discussion 140-1.

mononucleosis tonsillitis and upper airway obstruction. Journal of Laryngology and Otology. 2001; 115:973-7. 24.

Otorhinolaryngology. 1998; 44: 5-10.

fatigue syndromes and mood disorders that occur after

Golledge J, Ellis H. The aetiology of lateral cervical

infectious mononucleosis. Lancet. 2001; 358: 1946-54.

(branchial) cysts: past and present theories. Journal of

25.

Regauer S, Gogg-Kamerer M, Braun H, Beham A. Lateral

generalised lymphadenopathy in homosexual men:

neck cysts - the branchial theory revisited. A critical

relation to acquired immunodeficiency syndrome. Lancet. 1984; 1: 1033-8.

review and clinicopathological study of 97 cases with special emphasis on cytokeratin expression. APMIS: Acta

26.

Scandinavica. 1997; 105: 623-30.

Otolaryngology and Head and Neck Surgery. 1992; 107:

King ESJ. The lateral Iympho-epithelial cyst of the neck

367-9.

(branchial cyst). Australian and New Zealand Journal of

27.

Conrad DA. Treatment of cat-scratch disease. Current

28.

Tuzuner N, Dogusoy G, Demirkesen C. Ozkan F, Altas K.

Opinion in Pediatrics. 2001; 13: 56-9.

Surgery. 1949; 19: 109.

12.

Martin H, Morfit HM. Erlich H. The case for branchiogenic cancer (malignant branchioma). Annals of Surgery. 1950;

Value of lymph node biopsy in the diagnosis of acquired

132: 867-87.

toxoplasmosis. Journal of Laryngology and Otology. 1996; 110: 348-52.

Soh KB. Branchiogenic carcinomas: do they exist? Journal of the Royal College of Surgeons of Edinburgh. 1998; 43: 1-5.

* 13.

29.

Thompson LD, Heffner DK. The clinical importance of

Actinomycosis of the posterior triangle: a case report and review of the literature. Journal of Laryngology and

Singh B, Balwally AN, Sundaram K, Har-EI G, Krgin B.

Otology. 1997; 111: 1082-5. 30.

Branchial cleft cyst carcinoma: myth or reality? Annals of Orvidas U, Kasperbauer JL. Pediatric lymphangiomas of the

Laryngology. 2001; 110: 1051-4.

* 31.

Laryngology. 2000; 109: 411-2. Hamoir M. Plouin-Gaudon I, Rombaux P, Francois G, Cornu

and Head and Neck Surgery. 1997; 116: 16-22. 32.

AS, Desuter G et al. Lymphatic malformations of the head

17.

Plaza Mayor G, Martinez-San Millan .I, Martinez-Vidal A. Is conservative treatment of deep neck space infections appropriate? Head and Neck. 2001; 23: 126-33.

and neck: a retrospective review and a support for staging. Head and Neck. 2001; 23: 326-37.

Gidley PW, Ghorayeb BY, Steirnberg CM. Contemporary management of deep neck space infections. Otolaryngology

head and neck. Annals of Otology, Rhinology, and 16.

Parhiscar A, Har-EI G. Deep neck abscess: a retrospective review of 210 cases. Annals of Otology, Rhinology, and

Otology, Rhinology, and Laryngology. 1998; 107: 519-24. 15.

Burns BV. al-Ayoubi A, Ray .I, Schofield JB, Shotton JC.

cystic squamous cell carcinomas in the neck: a study of 136 cases. Cancer. 1998; 82: 944-56. 14.

Burton F, Patete ML, Goodwin Jr WJ. Indications for open cervical node biopsy in HIV-positive patients.

Pathologica, Microbiologica, et Immunologica

11.

Mathur-Wagh U, Enlow RW. Spigland I, Winchester RJ, Sacks HS, Rorat E et al. Longitudinal study of persistent

Laryngology and Otology. 1994; 108: 653-9.

10.

White PD, Thomas .1M, Kangro HO, Bruce-Jones WD. Amess

.I, Crawford DH et al. Predictions and associations of

of the literature. International Journal of Pediatric

9.

Chan SC, Dawes PJ. The management of severe infectious

Nicollas R, Ducroz V, Garabedian EN, Triglia .1M. Fourth branchial pouch anomalies: a study of six cases and review

8.

Weber R, Hegenbarth V, Kaftan H, Krupe H, Jaspersen D.

thyroglossal tract. Annals of Surgery. 1920; 7 1: 121-2.

33.

Poe LB, Petro GR, Matta I. Percutaneous CT-guided

De Serres LM, Sie KCY. Richardson MA. Lymphatic

aspiration of deep neck abscess. AJNR American Journal of

malformations of the head and neck: a proposal for

Neuroradiology. 1996; 17: 1359-63.

staging. Archives of Otolaryngology - Head and Neck Surgery. 1995; 121: 577-82. 18.

Brewis C, Pracy JP, Albert DM. Treatment of lymphangiomas of the head and neck in children by

FURTHER READING

intralesional injection of OK-432 (Picibanil). Clinical Otolaryngology. 2000; 25: 130-4. 19.

neck. Facial Plastic Surgery Clinics of North America. 2001 ;

tuberculosis of the head and neck. British Journal of Oral

9: 131-45.

and Maxillofacial Surgery. 1996; 34:508-10.

* 20.

AI-Serhani AM. Mycobacterial infection of the head and neck: presentation and diagnosis. Laryngoscope. 2001; 111:2012-6.

21.

Enepekides DJ. Management of congenital anomalies of the

Penfold CN, Revington PJ. A review of 23 patients with

Morad NA. Tuberculous cervical lymphadenopathy; should antituberculous therapy be preceded by histological proof? Tropical Doctor. 2000; 30: 18-20.

Gidley PW, Ghorayeb BY, Stiernberg CM. Contemporary management of deep neck space infections. Otolaryngology and Head and Neck Surgery. 1997; 116: 16-22. McGuirt WF. The necK mass. Medical Clinics of North America. 1999; 83: 219-34. Soh KB. Branchiogenic carcinomas: do they exist? Journal of the Royal College of Surgeons of Edinburgh. 1998; 43: 1-5.

PART

15

THE UPPER DIGESTIVE TRACT EDITED BY JOHN HIBBERT

141 Anatomy of the mouth and dentition

1791

Barry KB Berkovitz

142 Benign oral and dental disease

1816

Crispian Scully and Jose-V Sebastian Bagan

143 Abnormalities of taste Steven M Bromley and Richard L Doty

1840

144 Salivary gland anatomy Graham J Cox

1852

145 Physiology of the salivary glands

1858

Roderick Cawsont and Michael Gleeson

146 lmaging of the salivary glands Nicole JM Freling

1871

147 Non-neoplastic salivary gland diseases

1898

Stephen R Porter

148 Cysts and tumours in and around the jaws, including sarcoma

1921

Mark McGurk, Anna Cassoni and Lisa Pitkin

149 Anatomy of the pharynx and oesophagus

1942

Nigel Beasley

150 Physiology of swallowing

1954

Paula Leslie and Stephen McHan well

151 Functional investigations of the upper gastrointestinal tract Lisa J Hirst

1964

152 Acute and chronic pharyngeal infection

1981

Marcelle Macnamara

153 Causes of dysphagia

2025

Elfy B Chevretton

154 Globus pharyngeus Petros D Karkos and Janet A Wilson

2037

155 Pharyngeal pouch

2043

Grant Bates

156 Oesophagal diseases Robert C Mason

2062

157 Neurological disease of the pharynx

2074

Robert J Sandersont, Paul Tierney and Richard Adamson

158 Dysphagia: management and intervention

2084

Alison Perry

159 Management and treatment of intractable aspiration Elfy B Chevretton

2094

141 Anatomy of the mouth and dentition

BARRY KB BERKOVITZ

I n troduct ion Palate Cheeks Lips Floor of the mouth Oropharynx Tongue Oral mucosa Muscles of the oral cavity

1 791 1 7 91 1 792 1 793 1 7 93 1 7 93 1 794 1 7 94 1 7 95

INTRODUCTION

Vascular supply and lymphatic drainage of the oral cavity Teeth Tissue spaces around the jaws The salivary glands Temporomandibular jo i nt Development of the face and palate Key points References Further reading

1 7 98 1 803 1 805 1 807 1 8 08 18 1 0 1 814 1 815 1 815

occupied mainly b y the tongue. The lateral walls of the mouth are defined by the cheeks and retromolar regions.

The importance of the mouth to the otolaryngologist is

Opening into the mouth are three pairs of major salivary

self-evident. It is the portal of entry for visual examina­

glands

tion of the oropharynx, larynx and the opening of the

numerous minor salivary glands (labial, buccal, palatal,

Eustachian tube in the nasopharynx. It is also the portal

lingual).

(parotid, submandibular and sublingual) and

of entry for many of the surgical procedures undertaken in these regions. Malformations in the mouth, such as cleft palate, may have important consequences for the

PALATE

healthy functioning of the system. Infection or tumours from the mouth may spread to involve the ear, nose and

The palate is divisible into two regions: the hard palate in

throat. As an example, infection from maxillary teeth may

front and soft palate behind.

give referred pain to the nasal region, while infection from mandibular teeth may spread to the neck and result in severe (and sometimes fatal) respiratory embarrassment.

H a rd palate

Due to its close physical (and also evolutionary) relation­ ship to the ear, clinical conditions affecting the jaw joint

The skeleton of the hard palate is formed by the palatine

(temporomandibular joint) may need to be differentiated

processes of the maxillae and the horizontal plates of the

from those directly involving the ear.

palatine bones. The oral mucosa is bound tightly to the

The mouth can be subdivided into the vestibule

underlying periosteum. In its more lateral regions it also

(external to the teeth) and the oral cavity proper (internal

possesses a submucosa where the main neurovascular

to the teeth). The mouth extends from the lips and cheeks

bundles lie

(Figure 141.1).

externally to the anterior pillars of the fauces (palato­

The periphery of the hard palate surrounding the

glossal arches) internally, where it continues into the

necks of the teeth is termed the gingiva and a zone

oropharynx:. The roof of the mouth is the palate and

similarly lacking submucosa runs anteroposteriorly in the

separates the oral and nasal cavities. The floor of the

midline as a narrow, low ridge, the palatine raphe. At the

mouth is formed by the mylohyoid muscles and is

anterior extremity of the raphe behind the incisor teeth is

_ . ..

..1_"--:'_

1792

I PART 15 THE UPPER DIGESTIVE TRACT

Fig ure 141.1

The hard palate. A, I ncisive papilla; B, palatine

raphe; C, palatine rugae; D, alveolus. Reprinted from Ref. 1 by permission from Elsevier.

Figure 141.2

Th e soft palate and oropharyngeal isthmus. A,

Palatoglossal fold; B, palatopharyngeal fold; C, palatine to n si l ; D,

a small prominence) the incisive papilla) that covers the

uvula. Reprinted from Ref. 1 by permission from Elsevier.

incisive fossa at the oral opening of the incisive canal. Radiating outwards from the palatine raphe in the anterior half of the hard palate are irregular transverse ridges or rugae. The pattern of rugae is unique for the individual. The submucosa in the posterior half of the hard palate contains minor mucous salivary glands. These secrete through numerous small ducts although) bilaterally) larger ducts collecting from many of these glands often open at the

paired

palatine

foveae.

These

are two sagittally

elongated depressions) sometimes a few millimetres deep) which flank the midline raphe at the posterior border of the hard palate. The upper nasal surface of the hard palate is the floor

The soft palate contains an aponeurosis) muscular tissue)

vessels)

nerves)

lymphoid tissue and

mucous

glands) while some taste buds are situated on its oral aspect. A thin) fibrous palatine aponeurosis is attached to the

posterior border of the hard palate. It represents the expanded tendons of the tensor veli palatini muscles and provides the fibrous skeleton of the soft palate that supports the palatine musculature. The aponeurosis is thick in the anterior two-thirds of the soft palate but very thin further back. All the other palatine muscles are attached to the aponeurosis.

of the nasal cavity and is covered by ciliated respiratory epithelium. The lower oral masticatory surface is covered by keratinized epithelium.

CHE EKS The cheeks are covered externally by skin and internally by

S o ft palate

mucous membrane (buccal mucosa) and have a muscular skeleton) the buccinator. Internally) the pink mucosa of the cheek adheres firmly to the buccinator muscle.

Few

The soft palate is a mobile flap suspended from the back

structural landmarks are visible. The parotid duct drains

of the hard palate) sloping down between the oral and

in the region of a small parotid papilla opposite the

nasal parts of the pharynx (Figure 141.2). The boundary

maxillary second molar tooth. A whitish line (the linea

between the hard and soft palate may be distinguished by

alba) may be seen at a position related to the occlusal

a change in colour) the soft palate being a darker red with

plane of the teeth) this hyperkeratinized line is presumably

a yellowish tint. In its relaxed and pendant position) its

the result of continuous mild trauma during biting. Yellow

anterior (oral) surface is concave) with a median raphe. Its

patches (Fordyce's spots) representing ectopic sebaceous

posterior aspect is convex and continuous with the nasal

glands may be evident on the internal surface of the cheek.

floor. A median conical process) the uvula, projects

Their numbers increase in puberty and in later life. Behind

downwards from its posterior border. Just behind and

the molar teeth, a fold of mucosa can be seen extending

medial to each upper alveolar process) in the lateral region

from the upper to the lower alveolus) especially when the

of the anterior part of the soft palate) a small bony

mouth is opened widely. This fold covers the pterygo­

prominence can be felt. This is produced by the pterygoid

mandibular

hamulus) an extension of the medial pterygoid plate of

hamulus to the back of the mylohyoid line. The raphe

the sphenoid bone.

gives origin to the buccinator muscle from its anterior

raphe

that

extends

from

the

pterygoid

Ch apter

141

1793

An atomy of the mouth and dentition I

surface and the superior constrictor muscle from its posterior surface. The entrance to the pterygomandibular space lies lateral to the pterygomandibular raphe and medial to the ridge produced by the anterior border of the ramus of the mandible. Between the lips or cheeks and the teeth lies a slit-like space, the oral vestibule. Where the mucosa covering the alveolus of the jaw is reflected onto the lips and cheeks, a trough or sulcus is formed which is called the fornix vestibuli. With the teeth in occlusion, a space still exists behind the last molar tooth (retromolar region), allowing for the passage of fluids when the jaws are wired together following fractures. A variable number of sickle-shaped folds containing loose connective tissue run across the fornix vestibuli. The upper and lower labial frena (or frenula) are constant folds in the midline. Other folds may traverse the fornix near the canines or premolars.

Fi 9 u re

141.3

The ventral surface of the tong u e, rel ated to the

fl oor of the mouth. 1 , Ti p of tongue; 2, l ingual frenum; 3, s u bl i ngual p a p i l la; 4, s u bl ingual fold; 5, deep l ingual vein; 6, fim briated fold. Reprinted from Ref. 1 by per m ission from Elsevier.

The aperture of communication between the mouth

LIPS

and pharynx, the oropharyngeal isthmus, is situated

Like the cheeks, the lips are covered externally by skin and

between the soft palate above and the dorsum of the

internally by mucous membrane (labial mucosa) that is

tongue below, bounded on both sides by the palatoglossal

smooth and shiny and shows small elevations caused by underlying mucous glands. The lips have a muscular skeleton, the orbicularis oris muscle. The red zone of the lip (the vermilion) is a feature characteristic of man. In the upper lip, the vermilion

arches. Each palatoglossal arch runs downwards, laterally and forwards and contains the palatoglossus muscle with its covering mucous membrane. The approximation of the arches, to shut off the mouth from the oropharynx, is essential to deglutition.

protrudes externally in the midline to form the tubercle, above which is situated a grooved region termed the philtrum.

The

lower

lip

shows

a

slight

depression

corresponding to the tubercle.

Palatine tonsil The palatine tonsil is a mass of lymphoid tissue situated in the lateral wall of the oropharynx, where it lies within

FLOOR OF THE MOUTH

the tonsillar fossa between the diverging palatopharyngeal

This comprises a small horseshoe-shaped region beneath the tongue

(Figure 141.3). Near the base of the tongue in

the midline, a fold of tissue called the lingual frenum is seen to extend onto the inferior surface of the tongue. The sublingual papilla is a conspicuous centrally positioned protuberance at the base of the tongue. The submandib­ ular salivary ducts open into the mouth at this papilla. On either side of the sublingual papilla are the sublingual folds, beneath which lie the submandibular ducts and sublingual salivary glands. The muscle forming the floor of the mouth is the mylohyoid muscle.

and palatoglossal arches. It forms the anteroinferior part of

Waldeyer's

ring

of

lymphoid

tissue.

This

ring

surrounds the openings into the digestive and respiratory tracts and consists of the palatine and tubal tonsils laterally,

the

nasopharyngeal

tonsil

(adenoids)

and

smaller collections of lymphoid tissue in the intertonsillar intervals

posterosuperiorly,

and

the

lingual

tonsil

inferiorly. The medial (free) surface of the palatine tonsil projects into

the

oropharynx

and

usually

presents

a

pitted

appearance. These pits, 10-15 in number, lead into a system of blind-ending, often highly branching crypts, which extend through the whole thickness of the tonsil.

OROPHARYNX

The deep (lateral) aspect of the tonsil is covered by the

The oropharynx extends from the soft palate to the upper

easily separated throughout most of its extent from the

Figure 141.2). Its lateral wall

underlying superior constrictor muscle. An important

fibrous tissue of the tonsillar hemicapsule that can be border of the epiglottis (see

consists of the palatopharyngeal arch, with the palatine

and

tonsil lying between this arch and the palatoglossal arch

paratonsillar vein) descends from the soft palate lateral

sometimes

large

vein

(the

external palatine

or

anteriorly. The two arches are also known as the pillars of

to the tonsillar hemicapsule before piercing the phar­

the fauces. The floor of the oropharynx is occupied by the

yngeal wall. Haemorrhage from this vessel can complicate

pharyngeal aspect of the tongue.

tonsillectomy.

1794

I PART

15 THE UPPER DIGESTIVE TRACT

The size of the tonsil varies greatly and at puberty may

palates above and has a tapered tip or apex touching the

be approximately 10-15 mm in transverse diameter and

incisor teeth, and a margin in contact with the gums and

approximately 20-25 mm in the vertical dimension. The

teeth. On each side, in front of the palatoglossal arch,

tonsil starts to atrophy at puberty so that, by old age, only

are four or five vertical folds, the foliate papillae.

a little tonsillar lymphoid tissue remains.

The dorsum has a longitudinal median sulcus and is papillated, the three remaining types of papillae repre­ senting the filiform, fungiform and circumvallate papil­

TONGUE

lae. Of the papillae, all except the filiform papillae bear

The tongue is a highly muscular organ of deglutition, taste and speech. It is partly oral and partly pharyngeal in position. It has dorsal and ventral surfaces and a root and an apex. The curved dorsum of the tongue (Figure 141.4) shows an anterior, oral part facing upwards and a posterior, pharyngeal part facing posteriorly, the two being separated by a V-shaped groove, the sulcus terminalis. The sulcus terminalis runs anterolaterally towards the palatoglossal arches from a median depression, the foramen caecum, that indicates the site of the embryonic thyroid diverti­ culum that gives origin to the thyroid gland. The anterior two-thirds of the tongue anterior to the sulcus terminalis is related to the hard and soft

taste buds. The keratinized filiform papillae are the most numer­ ous. They are minute, conical or cylindrical projections, arranged

in diagonal rows extending

anterolaterally,

parallel with the sulcus terminalis, except at the lingual apex where they are transverse. They have a masticatory function, appearing to increase the friction between the tongue and food, facilitating the movement of particles by the tongue within the oral cavity. The fungiform papillae appear scattered on the lingual margin but also irregularly on the dorsal surface, where they may occasionally be numerous. They differ from filiform papillae by their larger size, rounded (mushroom) shape and deep red colour (due to their thin, nonkeratinized epithelium and highly vascular connective tissue core). The circumvallate papillae are cylindrical structures, 1-2 mm in diameter, varying in number from about 8 to 12 and form a V-shaped

row

immediately

in

front

of

the

sulcus

terminalis. Each papilla is circumscribed by a groove. The pharyngeal (postsulcal) third of the tongue lying behind the sulcus terminalis forms the base of the tongue and lies posterior to the palatoglossal arches. It forms the anterior wall of the oropharynx and its mucosa is 2

reflected on to the epiglottis posteriorly by a median and two lateral glossoepiglottic folds. The folds surround two depressions or valleculae. The pharyngeal part of the tongue has underlying lymphoid nodules that produce low surface elevations collectively termed the lingual tonsil. The ducts of small seromucous glands open on the apices of these elevations. The ventral inferior surface of the tongue (see Figure 141.3) is smooth, purplish and reflected on to the oral floor and gums. Anteriorly is a median mucosal fold, the

7

lingual frenum. Lateral to this fold on either side, a deep lingual vein is visible, and lateral to the vein is a fringed mucosal ridge, the plica fimbriata.

ORAL MUCOSA The lining of the mouth, the oral mucosa, is continuous with the skin at the vermilion of the lip and with the pharyngeal mucosa at the oropharyngeal isthmus. It Figure 141.4

The dorsum of the tongue. " Sulcus terminalis; posterior third (pharyngeal part); 3, lingual follicles; 4, pillars of fauces; 5, epiglottis; 6, vallecula defined by median and lateral glosso-epiglottic folds; 7, anterior two-thirds (palatine part); 8, circumvallate papillae; 9 , foramen caecum. Reprinted from Ref. 2 by kind permission of Informa Healthcare. 2,

varies in structure, appearance and function in different regions of the oral cavity. 1 It can be classified into masticatory, lining and specialized mucosae. Masticatory mucosa covers the gingivae (gums) and hard palate. Its epitheliu� is keratinized (often parakeratinized) and has a dense fibrous lamina propria. It is pink in colour. A

Chapter 1 41

Anatomy of the mouth and dentition I 1795

submucosa is absent from the gingivae and the midline

alveolar processes of the maxilla and mandible by the side

palatine ra phe, but is present over the rest of the hard

of the molar teeth. Behind, it arises from the anterior

palate ,

margin of the pterygomandibular r aphe (a thin band of

especially where

it contains m ucous

salivary

glands, and also the greater palatine nerves and vessels.

tendinous fibres passing between the hamulus of the

The masticatory mucosa is bo u nd firmly to underlying

med ial pter ygoid plate down to the posterior end of the

bone or to the necks of the teeth, forming in the gingivae

mylohyoid line of the mandible). Additionally, a few

and palatine raphe a mucoperiosteum.

fibres arise from a fine tendinous band that bridges the

internal surfaces of the lips

interval between the maxilla and the pterygoid hamulus.

and cheeks, floor of the mouth, soft palate, ventral surface

From these origins the fibres of buccinator pass forwards

of the tongue and the alveolar processes (excluding the

towards the angle of the mouth. Here,

gingivae). It is red in colour, having a nonkeratinized

decussate, those from below crossing to the upper part of

Lining mucosa covers the

the central fibres

stratified squamous epithelium overl ying a loosely fibrous

the mouth, those from above crossing to the lower part.

and elastic la m ina propria, and the submucosa conta ins

The highest and lowest fibres of buccinator continue

some fat deposits and collections of minor mucous glands. Gustatory mucosa covers the anterior two-thirds of the

forward

to

enter

their

corresponding

lips

without

decussation.

dorsum of the tongue. The vermilion (red zone) of the lip

The buccinator muscle compresses the cheek against

separates the skin from lini ng mucosa has been

the teeth and gums during mastication and assists the

that

regarded by some as being specialized, as it shares features

tongue in directing food between the teeth. When the

of bo th l ining and masticatory mucosa. The epithelium

cheeks have been distended

that attaches the tooth to the gingiva, the j unctional

it between the lips, for example when playing wind

epithelium, also has features that dis tinguish

it from all

inst ruments . The buccinator muscle is innervated by the

other stratified squamous epithelia (for example, internal

buccal branch of the facial nerve. Its arterial supply is

and external basal lamina) .

with air, the buccinators expel

derived from the facial and maxillary (buccal branch ) arteries .

MUSCLES OF THE ORAL CAVITY Mylohyoid muscle Buccinator muscle The main muscle forming the floor of the mouth is the

141.7). Immedi­

The muscle of the cheek is the buccinator (Figure 141.5).

mylohyoi d muscle (Figures 1 41.6 and

Above and below it is attached to the outer surfaces of the

ately above it is the geniohyoid muscle. The mylohyoid

Figure 141 . 5 The buccinator muscle and the infratemporal fossa. '/ Lateral pterygoid muscle (two heads) ; 2/ bu ccal nerve; 3/ lingual nerve with accompanying branch from maxillary artery; 4, inferior alveolar nerve and artery; 5, mylohyoid nerve; 6, medial pterygoid muscle ( two heads) ; 7, maxillary artery; 8, buccinator muscle; 9, depressor anguli oris muscle. Reprinted from Ref. 2 by kind permission of Informa Healthcare.

_

.... .J _�_.

1796

I PART 15 THE U PPER DIGESTIVE TRACT

muscle

is

a

flat,

triangular

sheet

attached

to

the

mylohyoid line of the mandible. The posterior fibres pass medially and slightly downwards to the front of the body of the hyoid bone near its lower border. The middle and anterior fibres from each side decussate in a median fibrous raphe that stretches from the symphysis menti to the hyoid bone. The mylohyoid muscle elevates the floor of the mouth in the first stage of swallowing. It also aids in elevating the hyoid bone or depressing the mandible when the hyoid bone is fixed. The muscle is supplied by the mylohyoid branch of the inferior alveolar nerve and derives its blood supply from three sources: the lingual artery (sublingual branch), the maxillary artery (the mylohyoid branch of the

inferior

alveolar

artery)

and

the

facial

artery

(submental branch).

Ge niohyoid This narrow muscle lies above the medial part of the

(Figure 141.8

mylohyoid

and see also

Figure 141.6).

It

arises from the inferior genial tubercle (mental spine) on the back of the symphysis menti. It runs backwards and Figure 141.6

The floor of the mouth as seen in a median

sagittal section through the head. 1, Hard palate; 2, soft palate; 3, upper lip and superior part of orbiculari oris muscle; 4,

slightly downwards to attach to the anterior surface of the body of the hyoid bone. The geniohyoid muscle elevates the hyoid bone and

edentulous maxillary alveolar ridge; 5, lower lip with inferior

draws it forwards. When the hyoid bone is fixed, the

part of orbicularis oris muscle; 6, body of mandible; 7 , tongue;

geniohyoid muscle depresses the mandible. The muscle is

8, genioglossus muscle; 9, geniohyoid muscle; 10, mylohyoid muscle; 11, hyoid bone; 12, epiglottis: 13, valeculla. Reprinted

supplied by the first cervical spinal nerve, through the hypoglossal nerve. The blood supply is derived from the

from Ref. 2 by kind permission of Informa Healthcare.

lingual artery (sublingual branch).

Fig u re 141.7

Med ial view of the floor of mouth

showing deep part of the submandibular gland

(A)

passing around the free border of the mylohyoid muscle

(8). C,

Superficial part of submandibular

gland; D, submandibular duct wrapping around the lingual nerve (E); F, sublingual strand; G, bristle in opening of submandibular duct at the subling ua I pa pilla; H, branches of the lingua I nerve to the sublingual gland carrying •

parasympathetic nerve fibres. Reprinted from Ref. 1 by permission from Elsevier.

Chapter 1 41

Anatomy of the mouth and de ntition I 1797

Figure 1 41 .8 Muscles of the tongue and structures on the hyoglossus m uscle in the floor of the mouth. 1 , Li ng ua I nerve; 2, hypog lossa I nerve ; 3, submandibular duct (cutl ; 4, external carotid artery; 5, lingual artery; 6, glossopharyngeal nerve (cut) ; 7 , hyoglossus muscle; 8, styloglossus muscle; 9, geniohyoid muscle: 1 0, g enioglossus muscle. Reprinted from Ref. 1 by permission from Elsevier.

Muscles of the tongue

runs up to insert into the side of the tongue between the inferior longitudinal muscle medially and styloglossus

The muscles of the tongue comprise both extrinsic and intrins ic muscles,

the

former

extending

outside

the

laterally. The hyoglossus m uscle depresses the tongue. It is

tongue and moving it bodily, the latter lying who lly

innervated by the hypoglossal nerve. Its arterial supply is

within it and altering its shape.

derived fro m th e lingual (sublingual branch) and the

The extrinsic musculature is composed of fo u r pairs of muscles:

geniogloss us,

hyoglossus,

styloglossus

facial arteries (s ubmental branch).

and

palatogloss us.

STYLOGLOSSUS This muscle arises from near the apex of the styloid

GENIOGLOSSUS MUSCLE

process (an terolate ral aspect) and from the styloid end of

The genioglossus m uscle lies near the midline ( see

Figures

the stylomandibular ligament (a fibrous cord extending

141.6 and 141.8). It arises from the superior genial

from the tip of the styloid process to the lesser cornu of

tubercle ( men tal spine) behind the mandibular symphysis

the hyoid bone)

and above the origin of the geniohyoid muscle. From its

m uscle

origin, the fibres fan out as they pass backwards and

s u bstance of the tongue,

upwards. The inferior fibres are attached to the upper

hyoglossus.

anterior s u rface of the hyoid body near the midline.

The

runs

(Figure 141.8). From this origin, the

downwards an d forwards

styloglossus

muscle

to enter

the

decussating with fibres of acts

to pull

the

tongue

r ntermed iate fibres pass backwards into the posterior part

upwards and backwards. The muscle is supplied by the

of the tongue, and superior fibres ascend forwards to enter

hypoglossal nerve and derives its blood supply from the

the whole length of the ventral surface of the tongue from

sublingual branch of the lingual artery.

root to apex, intermingling with the intrinsic muscles. The genioglossus muscle brings abo ut the forward traction of the to ngue to protrude its apex from the mouth. Acting bilaterally, the two muscles depress the central part of the tongue, making it concave from s ide to side.

Acting unilaterally,

the muscle helps move the

tongue to the opposite side.

Genioglossus receives its

motor innervation from the hypoglossal nerve. Its blood supply is de rived from the lingual artery (sublingual branch) and the facial artery (submental branch).

PALATOGLOSSUS Although th i s muscle is most closely associated with the soft palate in function and innervation, it is described here with the other tongue muscles. The palatoglossus arises from the palatine aponeurosis and passes to the side of

the

tongue

within

the

palatoglossal arch. Acting

bilaterally, the muscles narrow the oropharyngeal isthmus and can also help raise the s i des of the tongue. Unlike the other tongue muscles, the palatoglossus is innervated, not

HYOGLOSSUS

by the hypoglossal nerve, but by the cranial portion of the accessory nerve through the pharyngeal plexus. Its blood

Thi s muscle arises from both the greater cornu and from

supply is derived from the ascending pharyngeal and

the front of the body of the hyoid bone (Figure 141.8). It

facial (ascending palatine) arteries.

1798 I

PART 1 5 TH E U PPER D I G ESTIVE TRACT

I N TRI N S I C MUSCLES OF THE TON G U E

These

comprise

four

inferior longitudinal,

groups:

the

superior

and

the transverse and the vertical

muscles.

VASCULAR SUPPLY AND LYMPHATIC DRAINAGE OF THE ORAL CAVITY Arteries

The superior longitudinal muscle forms a thin stratum of longitudinal and oblique fibres lying beneath the lining of the dorsum of the tongue. The inferior longitudinal muscle is a narrow band of muscle beneath the inferior surface between the genioglossus and hyoglossus muscles. The transverse muscle fibres pass laterally from the median fibrous septum to the submucous fibrous tissue at the lingual margin. The vertical muscle fibres extend from the dorsal to the ventral aspects of the tongue in the borders of its anterior part. When the superior and inferior longitudinal muscles contract, the tongue tends to shorten. In addition, the superior longitudinal fibres also pulls the apex and sides upwards to

make

the

dorsum concave,

while

the inferior longitudinal fibres pull the apex down to make

the

dorsum

convex.

The

transverse

contracts to narrow and elongate the

muscle

tongue

while

contraction of the vertical muscle makes the tongue flatter and wider. Acting alone or in pairs and in endless combination,

the

intrinsic

muscles

give

the

tongue

precise and highly varied mobility, important not only in alimentary function but also in speech. All intrinsic lingual muscles are supplied by the hypoglossal nerve and

derive

their

blood

supply

from

the

lingual

artery.

The main arteries to the teeth, palate and cheeks are derived chiefly from the maxillary artery, a terminal branch of the external carotid. The lips are mainly supplied by the superior and inferior labial branches of the facial artery. The floor of the mouth and tongue are supplied by the lingual arteries. The cheek is supplied by the buccal branch of the maxillary artery. Teeth in the lower jaw are supplied by the inferior alveolar (dental) artery, a branch of the maxillary artery (see

Figure 14l.5). It lies in the infratemporal fossa where

it gives off a mylohyoid branch before entering the mandibular foramen. The inferior alveolar artery then traverses the mandibular canal

(accompanied by the

inferior alveolar nerve) to supply the mandibular molars and premolars and divides into the incisive and mental branches below the premolar teeth. The incisive branch continues below the incisor teeth (which it supplies) to the midline. The mental artery leaves the mental foramen to supply the chin. Teeth in the upper jaw are supplied by the posterior, middle and anterior superior alveolar (dental) arteries that form a plexus of vessels. The posterior superior alveolar artery usually arises from the third part of the maxillary artery in the pterygopalatine fossa. It descends on the infratemporal surface of the maxilla to supply molar

MUSCLE OF THE LIPS

and

premolar

teeth,

adjacent

bone

and

the

maxillary sinus, and provides superficial branches that

The muscles surrounding the mouth and contributing to

continue over the alveolar process to supply the gingivae.

the lips are derived from two different sources. Neigh­

The anterior superior alveolar artery arises from the

bouring muscles of facial expression converge on the

infraorbital

angle of the mouth and pass through into the lips. For

through

the upper lip, these are chiefly from the buccinator and

infraorbital groove and canal with the infraorbital nerve.

depressor

the

It curves through a fine canal (canalis sinuosus) to supply

buccinator, levator anguli oris and zygomaticus major.

the upper incisor and canine teeth and the mucous

anguli

oris,

and

for

the

lower

lip,

artery

the

that

inferior

enters

orbital

the

orbit

fissure

to

posteriorly run

in

the

In addition, an accessory muscle, incisivus labii super­

membrane in the maxillary sinus. A middle superior

ioris, is present in the upper lip, arising from the floor of

alveolar artery is often present and supplies the premolar

the incisive fossa of the maxilla above the eminence of the

teeth.

lateral incisor tooth and running laterally within the

The gingival tissues derive their blood supply from the

upper lip to the modiolus (a zone of muscle intersection

maxillary and lingual arteries. In the lower jaw, the buccal

just beyond the corner of the mouth). A similar strip of

gingiva posteriorly is supplied by the buccal artery (a

muscle

is

present

in

the

lower

lip,

incisivus

labii

branch of the maxillary artery as it crosses the lateral

inferioris, arising from the floor of the incisive fossa of

pterygoId muscle) and by perforating branches from the

the mandible and running laterally within the lip to the

inferior alveolar artery. Anteriorly, the labial gingiva is

modiolus.

supplied by the mental artery and by perforating branches

The muscles of the mouth are capable of various

of the incisive artery. The lingual gingiva is supplied by

movements, including closing, protrusion and pursing of

perforating branches from the inferior alveolar artery and

the lips. The muscles are innervated by the facial nerve

by the lingual artery of the external carotid.

(buccal and mandibular branches) and derive their blood

In the upper jaw, the buccal gingiva around the molar

supply from the facial (superior and inferior labial),

and

maxillary (mental and infraorbital branches) and super­

perforating branches from the posterior and middle

ficial temporal arteries.

superior alveolar arteries and by the buccal artery. The

premolar

teeth

are

supplied

by

gingival

and

Chapter 141

Anatomy of the mouth and dentition I 1799

labial gingiva of the anterior teeth is supplied by labial

The cheek is drained by the buccal vein that leads to

branches of the infraorbital artery and by perforating

the pterygoid venous plexus in the infratemporal fossa.

branches of the anterior superior alveolar artery. The

Venous blood from the lips is collected by the superior

palatal gingiva around the maxillary teeth is supplied

and inferior labial veins and drains into the facial vein.

primarily by branches of the greater palatine artery, a

The veins of the hard palate accompany the arteries and

branch of the third part of the maxillary artery in the

drain largely to the pterygoid plexus. Veins accompanying the superior

pterygopalatine fossa. The hard palate derives its blood supply principally from the greater palatine artery, a branch of the third part

alveolar

arteries

drain the upper jaw and teeth anteriorly into the facial vein, or posteriorly into the pterygoid venous plexus.

artery

Veins from the lower jaw and teeth collect into several

descends (with its accompanying nerve) in the palatine

inferior alveolar veins. Some of these veins drain through

canal. In the canal, it gives off two or three lesser palatine

the mental foramen to the facial vein, others via the

arteries that are transmitted through lesser palatine canals

mandibular foramen to the pterygoid venous plexus.

of the maxillary

artery.

The

greater

palatine

to supply the soft palate and tonsil (anastomosing with

No accurate description is available concerning the

the ascending palatine branch of the facial artery). The

venous drainage of the gingivae, although it may be

greater palatine artery emerges on to the oral surface of

assumed

the palate at the greater palatine foramen and runs in a

nasopalatine veins are involved. These veins run into

that

buccal,

lingual,

greater

palatine

and

curved groove near the alveolar border of the hard palate

the pterygoid plexuses (apart from the lingual veins,

to the incisive canal. It ascends this canal and anasto­

which pass directly into the internal jugular veins).

moses with septal branches of the nasopalatine artery.

There are two sets of veins draining the tongue. The

The tongue and floor of mouth are supplied mainly by

deep lingual vein begins near the apex of the tongue and

the lingual artery that arises in the neck from the external

runs back on its ventral surface (see

carotid artery (see

a sublingual vein from the sublingual salivary gland, to

Figure 141.8). It reaches the floor of

Figure 141.3). It joins

the mouth by passing between the hyoglossus muscle and

form the vena comitans nervi hypoglossi. This then passes

the middle constrictor of the pharynx. Near the tip of the

backwards with the hypoglossal nerve and joins the

tongue, the lingual artery anastomoses with its fellow. The

lingual, facial or internal jugular vein. Dorsal lingual veins

branches of the lingual artery in the floor of the mouth

drain the dorsum and sides of the tongue and join the

are the dorsal lingual branches, the sublingual artery and

lingual veins accompanying the lingual artery. They drain

the deep lingual artery.

into the internal jugular in the region of the hyoid bone.

The dorsal lingual arteries (usually two or three) arise medial to the hyoglossus muscle and ascend to the posterior part of the dorsum of the tongue. They supply

Lymph drainage

the mucous membrane of the dorsum of the tongue, the palatoglossal arch, soft palate, tonsil and epiglottis.

The principal sites of drainage of lymphatic vessels from

The sublingual artery arises from the lingual artery at

orodental tissues are the submental, submandibular and

the anterior margin of hyoglossus. It passes forward

jugulodigastric lymph nodes. However, the manner of

between the genioglossus and mylohyoid muscles to the

drainage is so variable that there is no precise and

sublingual gland. It also provides a branch that enters a

accurate description.

small

foramen

(lingual

foramen)

on

the

mandible,

The cheek, upper lip and lateral parts of the lower lip

situated in the midline on the posterior aspect of the

drain to the submandibular nodes. The central part of the

symphysis, immediately above the genial tubercles.

lower lip drains to the submental nodes.

The deep lingual artery is the terminal part of the

The lymph vessels from the teeth usually run directly

lingual artery and is found on the inferior surface of the

into the submandibular lymph nodes on the same side.

tongue near the lingual frenum.

Lymph from the mandibular incisors, however, drains

In addition to the lingual artery, the tonsillar and

into the submental lymph nodes. Occasionally, lymph

ascending palatine branches of the facial and ascending

from the molars may pass directly into the jugulodigastric

pharyngeal arteries also supply tissue in the root of the

group of nodes.

tongue. In the region of the valleculae, epiglottic branches

The

lingual

and

palatal

group

of

gingivae

nodes,

drain

either

into

the

of the superior laryngeal artery anastomose with the

jugulodigastric

directly

inferior dorsal branches of the lingual artery.

indirectly through the submandibular nodes. Lymphatics

or

from the bulk of the palate terminate in the jugulodigas­ tric group of nodes.

Veins

The lymphatics from the tongue drain to three main regions: marginal, central and dorsal. The anterior region

As with veins elsewhere in the body, the pattern of

of the tongue drains into the marginal and central vessels

IS

and, behind the circumvallate papillae, the posterior part

drainage for the mouth and associated structures variable and only a generalized account will be given.

of the tongue drains into the dorsal lymph vessels. Of

_

1

______..•__

1800 I

PART 1 5 THE UPPER DIGESTIVE TRACT

Innervation

particular clinical importance is appreciating that more central

regions

may

drain

both

ipsilaterally

and

contralaterally. Marginal lymph vessels of the tongue that arise from

The

the apex of the tongue and the lingual frenum area descend under the mucosa to widely distributed nodes. •

derives

its

sensory

mandibular, facial and hypoglossal nerves. The salivary the facial and glossopharyngeal nerves. The innervation of the oral musculature is shown in Table 141.1.

pass to both sides. Some vessels drain to submandibular nodes.

mouth

glands are supplied by secretomotor fibres derived from

efferent vessels of submental nodes that are median

Some vessels end in jugulodigastric nodes, one vessel

the

supply the tongue, including special taste sensation. The

under the frenulum to end in the contralateral nodes,

•

lining

muscles of the mouth are supplied chiefly by the

Some vessels enter the submental nodes and also pass to the jugulo-omohyoid nodes. Vessels may cross

•

mucosa

innervation chiefly from the maxillary and mandibular divisions of the trigeminal nerve. Additional nerves

SEN SORY S U PPLY

often reaching the jugulo-omohyoid node.

Cheeks, lips and palate The sensory supply to the cheek is derived from the buccal branch of the mandibular nerve (see Figure

Vessels from the lateral margin of the tongue may drain into the submandibular nodes while others end in the jugulodigastric or jugulo-omohyoid nodes. Vessels from

141.5). This nerve arises from the anterior division of

the posterior part of the lingual margin traverse the

the mandibular nerve within the infratemporal fossa. Initially deep to the lateral pterygoid muscle, it emerges

pharyngeal wall to the jugulodigastric lymph nodes.

between the two heads of the muscle and runs forwards to

Vessels draining the central region of the tongue may

supply the mucosa of the cheeks.

drain to either or both sides into the deep cervical nodes, jugulo-omohyoid

The upper lip is supplied by the infraorbital branch of

nodes. Some pierce the mylohyoid muscle to enter the

the maxillary nerve (Figure 141.9). Running along the

submandibular nodes.

floor of the orbit in the infraorbital canal, it enters the

especially

the

jugdlodigastric

and

Vessels draining the dorsum of the tongue in the

face at the infraorbital foramen, where its labial branch

region of the circumvallate papillae and behind them run

runs downwards to supply the upper lip. The mental

posteroinferiorly. Those near the median plane may pass

nerve is a terminal branch of the inferior alveolar nerve

to either or both sides, draining to the jugdlodigastric and

and exits the mandible at the mental foramen to supply

jugulo-omohyoid lymph nodes.

the lower lip (see Figure 141.9).

Table 1 41.1

'Begion-" '. t

-.

Innervation of the oral musculature.

i;�"

Nerve

��

} }

Orbicularis oris

Facial

Cheeks

Buccinator

Facial

Tongue (intrinsic musculature)

Transverse

Lips

Longitudinal Vertical Tongue (extrinsic musculature)

GeniOglOSSUS Hyoglossus

Hypoglossal

Hypog lossa I

Styloglossus

Floor of mouth

Palatoglossus

Accessory (cranial part)

Mylohyoid

Mandibular division of trigeminal

Geniohyoid Palate

Tensor veli palatini Levator veli palatini Palatoglossus Palatopha ryngeus Musculus uvulae

Reprinted from Ref. 1 by permission from Elsevier.

)

Hypoglossal (CI fibres) Mandibular division of trigeminal

Accessory (cranial part)

Chapter 1 41 Ana tomy of the mouth and dentition I

1801

The sensory nerves to the palate are derived from the

palatine nerve descends through the greater palatine

greater and lesser palatine and nasopalatine branches of

canal, entering the hard palate at the greater palatine

the maxillary nerve (Figure 141.10) These nerves pass

foramen. It then passes forwards on the bony palate

through

towards the canine tooth) supplying the gums and the

the

pterygopalatine

ganglion.

The

greater

mucosa and glands of the hard

p alate (excluding the

anterior teeth). As it leaves the greater palatine canal, palatine branches are also distributed to the soft palate. The smaller lesser palatine nerves descend thro ugh the greater palatine canal to emerge through the inconspic­ uous lesser palatine foramina and give branches to the uvula) tonsil and soft palate.

Fibres conveying taste

impulses from the palate probably pass via the palatine nerves

to

the

pterygopalatine

ganglion

(see

Figure

141.10) and through it to t he nerve of the pterygoid canal and then the greater pet rosal nerve to reach the facial ganglion, where their somata are situated. Para­ sym p athetic

p ostganglionic secretomotor fibres run in the

facial nerve through its greater petrosal nerve to reach the pterygopalatine ganglion to be distributed in the palatine nerves) thereby reaching palatine mucous glands. The nasopalatine nerves (Figure 141.10) enter the palate at the incisive foramen to supply the anterior part of the hard palate behind the incisor teeth. There is an additional sensory component in the region of the pillars of the fauces derived from the glossopharyngeal nerve which is involved in the gag reflex (see Figure 141.8). The floor of the mouth is supplied by the lingual nerve (see Figures 141.5 and 141.9). This nerve arises from the posterior division

of

the

mandibular

nerve

in

the

infratemporal fossa.

Teeth and gingivae The regional supply to the teeth and gingivae is shown in Table 141.2. The posterior superior alveolar nerves supply Figure 1 4 1 .9

Infratemporal fossa showing mandi bula r nerve

branches . 1 , Auric ulotemporal nerve surro und ing middle meningeal artery ; 2, buccal nerve; 3, lingual nerve

G oined

the teeth in the upper jaw, while the inferior alveolar nerve supplies those in the lower jaw.

by

chorda tympani nerve); 4, inferior alveolar nerve; 5, mylohyoid

There are three superior alveolar (dental) nerves that arise from the maxillary nerve in the pterygopalatine fossa

nerve; 6, mental nerve; 7, sphenomandibular ligament; 8, medial

or in the infraorbital groove (canal) and these form a

pterygoid muscle; 9. infraorbital nerve. Reprinted from Ref. 1 by

plexus of nerves supplying all the upper teeth. The

permission from Elsevier.

posterior superior alveolar (dental) nerve leaves the maxillary nerve in the pterygopalatine fossa and runs

Figure 141.1 0

The pterygopalatine ganglion. 1,

I nternal carotid artery; 2, maxillary nerve; 3, nerve of pterygoid ca na I; 4, pterygopalati ne gang I ion; 5, greater palatine nerve; 6, lesser palatine nerve; 7, nasopalatine nerve d issected off from nasal septum ; 8, nasopalatine nerve at incisive fossa. Reprinted from Ref. 1 by permission from Elsevier.

I

;'

L·_ ..i..

""", ,,, , _____ __ _ _

.

1802 I

PART 1 5 THE UPPER D I GESTIVE TRACT

Nerve su p ply to the teeth and gingiva.

Table 1 41 .2

Maxilla

Nasopalatine nerve

Greater palatine nerve

Anterior su perior al veolar nerve

Middle su perior al veolar

Palatal gingiva Teeth

Posterior superior al veolar nerve

nerve I nfraorbital nerve

1

4

3

5

7

6

8

Tooth position

Buccal nerve and perforating branches of inferior alveolar nerve

Menta I nerve

Mandible

Buccal gingiva

Posterior superior alveolar nerve and buccal nerve

2

Buccal gingiva Teeth

Inferior alveolar nerve

I ncisive nerve

Lingual gingiva

Lingual nerve and perforating branches of inferior alveolar nerve Repri nted from Ref. 1 by permission from Elsevier.

anteroinferiorly to pierce the infratemporal surface of the

premolar teeth to supply the skin of the lower lip and

maxilla, descending under the mucosa of the maxillary

chin (see

Figure 141 .9).

sinus. After supplying the lining of the sinus, the nerve

The nerves supplying the gingiva in the upper jaw

divides into small branches that link up as the molar part

come from the maxillary nerve via its greater palatine,

of the superior dental plexus, supplying twigs to the

nasopalatine and anterior, middle and posterior superior

molar teeth. It also supplies a branch to the upper gingiva

alveolar branches. The mandibular nerve innervates the

and the adjoining part of the cheek.

gingiva in the lower jaw by its inferior alveolar, lingual

The variable middle superior alveolar (dental) nerve arises from the infraorbital nerve as it runs in the infraorbital groove, and passes downwards and forwards in the lateral wall of the maxillary sinus. It ends in small

and buccal branches (see

Table 141 .2).

The tong ue and floor of the mouth The sensory supply to the tongue involves both general

branches that unite with the superior dental plexus,

and taste afferents and reflects the embryological devel­

supplying small rami to the upper premolar teeth.

opment of the structure. Thus, it can be considered in two

The anterior superior alveolar (dental) nerve leaves the

portions,

the

presulcal

(anterior

two-thirds)

and

a

lateral side of the infraorbital nerve near the midpoint of

postsulcal (posterior one-third). The nerve of general

its canal and traverses the canalis sinuosus in the anterior

sensation to the presulcal part is the lingual nerve,

wall of the maxillary sinus. Curving first under the

indicating the embryological derivation of this part from

infraorbital foramen, it passes medially towards the nose,

the first branchial arch. The lingual nerve also carries taste

turns downwards and divides into branches supplying the

sensation derived from the chorda tympani branch of the

incisor and canine teeth. It assists in the formation of the

facial nerve. The main nerve supplying general (and taste )

superior dental plexus.

sensation to the postsulcal part is the glossop haryngeal

The inferior alveolar (dental) nerve arises from the posterior

division

infratemporal

fossa

of

the

(see

mandibular

nerve

in

nerve, while an additional area in the region of the

the

valleculae is supplied by the internal laryngeal branch of

Figures 141 .5 and 141 .9).

the vagus nerve, indicating the embryological derivation

Emerging from beneath the lower head of the lateral

of the postsulcal part from the third and fourth branchial

pterygoid muscle, it descends to enter the mandibular

arches.

canal via the mandibular foramen (accompanied by the

The lingual nerve originates from the posterior trunk

inferior alveolar artery). Just before entering the man­

of the mandibular nerve in the infratemporal fossa (see

dibular canal, the inferior alveolar nerve gives off a small

Figures 141.5, 141 . 8 and 141 .9. Here, it is joined by the

mylohyoid branch to supply the mylohyoid muscle and

chorda tympani branch of the facial nerve that supplies

the anterior belly of digastric.

taste to the

anterior two-thirds of the

tongue

and

In the mandibular canal, the inferior alveolar nerve

parasympathetic fibres that supply the submandibular

runs downward and forward, generally below the apices

and sublingual glands via the submandibular ganglion

of the cheek teeth until, below premolar teeth, it divides

(Figure 141 .9). Emerging from beneath the lower head of (Figure 141 .5), the lingual

into terminal incisive and mental branches. The incisive

the lateral pterygoid muscle

branch continues forward in a bony canal or in a

nerve passes forwards medial to the inferior alveolar nerve

plexiform arrangement, giving off branches to the first

and is closely applied to the periosteum of the medial

premolar, canine and incisor teeth, and the associated

surface of the mandible by the side of the roots of the

labial gingivae. The lower central incisor teeth may receive

third molar tooth

a bilateral

is at great risk during surgical removal of (impacted)

innervation,

fibres probably crossing

the

(Figure 141 .7). Here, the lingual nerve

midline within the periosteum to re-enter the bone via

lower third molars using a lingual approach. The lingual

numerous canals in the labial cortical plate.

nerve then passes medial to the mandibular origin of the

. The

mental

nerve

passes

upward,

backward

and

mylohyoid muscle, which carries it progressively away

outward to emerge from the mandible via the mental

from the' mandible. It passes downward and forward on

foramen between and just below the apices of the

the deep surface of the mylohyoid muscle and then runs

Chapter 1 41 A n a t o my of t h e m o u t h a n d d e nt iti o n I

below the subma nd ib u l a r du ct, which crosses it from medial to lateral ( F ig ure

141 .7). The nerve next cu rves

upward, forward and media]]y to en te r the tongue. The

lingual nerve is connected to

the submand ib ular

g ang l io n

1 803

a n d for w a rds between t h e mylohyoid a n d hyoglossus

m usc l es . Here, the hypoglo ssal nerve is situated below the

deep p art of the su bm a n d ib ular gl and , the submandibular duct and the l ingu al nerve. It then passes on to the lateral

by two or three branches and, at the anter i o r margin o f

aspect of the ge n i ogloss u s muscle, continuing fO lWa rds in

the hyoglossus , it forms co nnecting loo ps wi th twigs o f

i ts substance as fa r as the t ip of the tongue. The muscular branches from the hyp oglo ss al nerve are

the hypoglossal nerve. l i ngual

d is tr ib u ted to styloglo ssus , hyoglossus and genioglossus.

gingivae, the m ucosa of the floor of the mouth and the

Numerous slender rami ascend into the tongue to supply

anterior

its intri ns i c muscles .

Branches

of the lingual nerve supply the

two - thirds

(pres ulcal

part)

of

the

tongu e

( excluding the circ umvallate papillae, which a re suppl ied The glossopharyngeal nerve is present i n the neck where it passes around

posterior

the

The nerve to geniohyoid ( and to thyrohyo id) arises near th e posterior border of the hyo gloss us . It is derive d

by the gloss opharyngeal nerve ) .

styJ opharyngeus muscle, which it s u pplies

fro m fi bres of the

fi rst cervical sp inal

nerve .

border o f th e

( Figure 14 1.8).

It runs forwards on this m uscle and ei ther pierces the

TEETH

lowe r fibres of the s uperior cons t ri ct o r m uscle or passes between it and the middle constrictor to be d istrib u ted to the

p ostsuJcal

part

of

circumvallate p ap illa e) . It

the

( i n cl u d ing

tongue

the

co mm un i cates with the l i ng ual

nerve.

There

are

( p ri mary)

two

genera tio ns

dentit i o n

and

of teeth:

the

decid uous

the p e rm anen t

(secondary)

d entition. In the complete deciduous denti tion there are

20 teeth - five in each j aw qu a dra nt. In the co mplete

The senso ry sup ply to the valle cu l ae of the tongue ( t h e

perma nent de n t i tion there are 32 teeth - eight in each jaw

recess between t h e m edia n a nd lateral glossoepiglottic

q u a d r a nt . In both dentitions, there are three basic to oth

folds ) is derived from the internal laryngeal nerve. This

fo rms: incisiform, caninifonn and molarifo nn . I nc isi form

nerve is a branch of the s u p erior l aryngeal nerve and ,

teeth ( i nc isors ) are

p assing between the superi o r an d i n fer i o r constrictor

crowns.

muscles, pierces the thyro hyoid membrane. Fro m here,

tea ri ng teeth, having a single, s tout, p o inte d , cone-s h a p ed

branches ascend to supply the valleculae.

is

The p arasympathetic innervation o f the

li n g ual

nerve (Figure ] 4 1 .9) and

synapses in the submandib u l ar gangl i o n . Postgangl ionic fibres re-enter the lingual nerve symp athetic s upply to l i n g ual the to ngue thro u gh plexuses

to

teeth, having thin, blade-l i ke ( canines)

are

piercing

or

grinding teeth possessing

a num b er

of cusps separated by

fiss ures . Premolars are bicuspid teeth th at are pecuJ i ar to the

perma n en t

dentition

and

replace

the

deciduous

molars .

the gla nd s. The

g lands and vessels en ters

aro und

teeth

crown. Molari fo nn teeth ( mol ars and premolars ) are glands

derived from the chorda tympani b ranch o f the facial

nerve that joins th e lingual

c utting

Caniniform

its arteries, ari s ing

Pe r manent teeth

from the carotid plexus. There are two incisors, a cen tra! and a lateral, in each half jaw or qua drant

(Figures 141. 1 1 and 1 4 1 . 1 2). Viewed

from the front, the crowns are tra pezo id , the m axillary

MOTOR SU PPLY

incisors (particularly

The nerves sup ply i ng the b u cci na to r a nd o rbic u la ri s o ris

mandibular. They are surmo unted by the b i ting or incisal

muscles are derived fro m the facial nerve t hat exits the

edges. In side view their labial pro files are co nvex wh ile

the central) being larger than the

skull at the stylomasto id fo ramen and then passes into the

their lingual surfaces are concavo -co nvex (the co nvexity

substance o f the p arotid g l an d , where it divides into its

near th e cervical m a rgin being due to a low

term inal bran ches. The b uccinator is s upplied by its

ci ngu lum , pro m inent only on upper inciso rs ) . The roots

buccal bra n c h ( es)

of incisors are single and ro u nded in maxillary teeth, but

and the lips fro m the b uccal and

rid ge o r

mandibular branches. The nerve supplying the mylohyo id

flattened mesio distally in m andib ul ar teeth . The upper

nerve a nd is seen bra nch i ng fro m

lateral incisor may be congenitally absent or may have a

muscle is the mylohyoid

the inferior alveolar nerve j us t above the mandibular foramen The

m uscl es

reduced Conn (peg-shaped lateral inci so r ) . Behind each lateral inciso r is a canine tooth with a

(Table 1 41 . 1 ) . o f t h e t ongue

a re

supplied b y the

single cusp ( hence the American term cusp i d ) instead o f

hypoglo ssal nerve. These m uscles develop pri marily from

a n incisal edge . The maxillary canine is stou ter an d more

occipital so mites that migrate into the d evelo ping tongue

carrying their nerve s up p ly, t he hypoglossal nerve, with them . The hypoglossal nerve is seen i n the neck crossing

over the inte rnal and ex terna l ca rotid and then the loop of

the l ingual artery ( Fig ure

] 41 .8). It th en p asses

u pwards

po inted than the m and ib ul ar cani ne. The canine roo t is single and is the longes t o f any too th . Behind the canines are t w o premo lars, e a c h with a -buccal and lingual cusp (hence the term bicusp i d ). The o cclusal surfaces o f the maxillary p rem o l a rs are oval ( t he

1804 I

PART 1 5 T H E U PPER D I G ESTIVE TRACT

seco nd premolar, the ling ual cusp is more substantial co mpared with the first, and frequently presents as two cusps. Each lower premolar tooth generally has one root. Posterior to the p remolars are three molars whose size

decreases as one p asses backwards. Each has a large rhomboid (upper jaw) or rectang ular (lower jaw) occlusal surface with four or five cusps. The maxillary first molar has a cusp at each corner of its o cclusal surface and the mesiop alatal cusp is connected to the distobuccal by an obli que r i d ge.

A smaller cusplet or tubercle ( cusplet of

Carabelli) usually appears on the mesi op alatal cusp. The

to o th has three wi dely separated roots, two buccal an d one palatal. The smaller maxi l lary second molar has a re d uced distopalatal cusp. Its three roots are less divergent and two of them may be fuse d . The maxillary third m olar, the smallest, is very variable in fo rm. I t us u ally has three cusps ( the disto p alatal being absent ) and co mmonly the three r o o ts are fused. The mandibular first molar has three buccal and two lingual cusps on

i ts rectang ular o cclusal surface, the

smallest cusp being distal. It has two widely separate d

roots, one mesial and one distal . The smaller mandibular second molar is like the first but has o nly four cusps (lacking the distal cusp of the first molar) and its two Figure 1 41 . 1 1

Pe rm a n e n t d entition upper jaw. Re pri n ted from

Re f. 1 by permission from El sevier.

roots are closer to gether . The mandibular third molar is smaller still and, like the upper third mo lar, is var i able in form. I ts crown may resemble that of the lower first or second molar and its ro ots are frequently fused. The third molar is often imp acted against the second molar, with

p acki ng and i nflammation. Third molars

resultant fo o d

are frequently congenitally absent.

Deci d u o u s teeth The incisors, canine and premolars of the permanent dentition rep lace two deci d uo us incisors, a deciduous

canine and two deciduous molars in each j aw quadrant. The deciduous incisors and canin e are shaped like their successors

but

are

smaller

an d

whiter

and

become

extremely worn in older children. The deciduous second molars

resemble

permanent

ones

rather

than

their

successors, the p remolars . Each second deciduous molar F i g ure 1 41 . 1 2

Perm a n e n t dentition lower j aw. Repri n ted from

Ref. 1 by perm i ssion from Elsevier.

has a crown almost identical to that of their respective, adjacent first permanent molar. The upper first decidu ous

molar has a triangular occlusal surface (its roun ded ' ap ex '

being p ala t al) awl a fissure separates a d o uble buccal cusp

from the p alatal cusp. The lower first deciduous m o l ar is a mesiodistal fissure

long and narrow; its two bu ccal cusps are separated fro m

separati ng the two cusps. The maxillary first premolar

the two lingual cusps by a zigzagging mesiod istal fissure.

us u ally has two ro ots ( one buccat one palatal) . The

Like permanent molars, u pper deciduous molars have

long axis is buccopalatal)

with

maxillary second premolar usually has one root. The

three ro ots and lower deciduous m olars have two ro ots.

o cclusal surfaces of the man dib ular premolars are more

These roots diverge m o re than those of perm anent teeth,

circular or squarer than those of the uppers. The buccal cusp of the mandibular first p r emo l ar towers above the

very much reduce d li ngual cusp. In the mandib ular

as each developing - premolar tooth crown is accommo­ dated

dire ctly

p re decesso r.

under

The

the

roots

crown of

of

its

deciduous

deciduous teeth

are

Cha pter progressively resorbed by osteoclast-like cells ( odonto ­

1 805

1 4 1 Ana tomy o f the m outil a n d den titi o n I

TISS U E S PACES ARO U N D TH E JAWS

clasts) p rior to being shed. The most common cause of infection of the tissues associated with the mouth is dental caries ( tooth decay) .

As infection may spread from the teeth to the neck and

Eru pti o n

become life- threatening, a knowledge o f the tissue spaces

The first

d eciduous teeth t o erupt into

the mouth

appear at approximately six months a ft er birth and, by aro und the age o f three years, all the decid uous teeth have erupted. By six years of age, the first permanent teeth appear (lower incisors and first molars) and thence the deciduous teeth are exfoliated one by one as they are replaced

by their permanent successors.

A complete

permanent dentition is present when the third molars erupt at around the age of 18-2 1 years. Information on the sequence of development and eruption of teeth into the oral cavity may b e important in fo rensic medicine and archaeology in helping to age individuals. The data provided

in

Tables

1 4 1 .3

and

1 4 1 .4

concerning

the

chronology of tooth development are largely based on Euro p ean-derived populations and there is evidence o f

ethnic

variation.

When

a

permanent

tooth

erupts,

approximately two-thirds o f the root is formed and it takes approximately another three years for the ro ot to be completed. For deciduous teeth, root completion is more rapid . The developmental stages of initial calcification and crown completion are less affected by environmental influences than eruption, the timing of which may be mod ified by several factors such as early tooth loss and severe malnutrition.

Figures

141.13

and

1 4 1 . 14

show

the

panoramic

appearance of the dentition seen with orthopantomo­ grams at 5 and 1 1 years of age. For detailed info rmation concerning the structure and function o f teeth

the

reader is

referred

to

standard

textbooks. 1 Ta ble 1 41 . 3

Tooth

associated with the j aws is of considerable clinical , , ' 3 4 5 6 . unpor t ance. ' , , Th e tissue spaces are only potential spaces that are normally occupied by loose connective tissue. It is only when inflammatory p roducts destroy the loose connective tissue that a d efinable space is pro duced. The tissue spaces associated with the mouth are p rimarily defined by muscles (principally the mylohyoid, buccina­ tor, masseter, medial p terygoid, superior constrictor and orb icularis

oris

muscles).

Knowledge

of the

normal

position of the root apices in relation to some of these muscles is important fo r an appreciation of the treatment 7 of d ental abscesses. Because of the potential of inflammation associ ated with the tissues surround ing partially erupte d , impacted third molars (pericoronitis) , the spaces associated with this region are particularly important and, as they are interconnected, inflammation can spread to involve the tissue spaces of the neck. The submental and subman­ d ibular tissue spaces are located below the inferior bo rder of the mandible, beneath the mylohyoid muscle,

in the

suprahyoid region of the neck. The submental space lies beneath the chin in the midline, between the mylohyoid muscles and the investing layer of deep cervical fascia. It is bounded laterally by the two anterior bellies of th e digastric muscles. The submental space communicates posteriorly with

the two

submandibular spaces.

The

sub mand ibular space is situated between the anteri or and posterior bellies of the digastric muscle. It co mmunicates with the sublingual space around the posterior free border of the mylohyoid muscle .

Chro nology of the d ecid u o us d e n ti tions.

Crow'��-,C�lT!pl�ted . (mo ll �bl( '

First evidence of

calcification

f,�,

(months IU)

.{; �

' If

�ru p�j�n (mo nths)

R90t co,mp}eted (years)

_ .

-;:5?.t (,

Maxillary

�

A

3 -4

4

7

1 -2

B

41

5

8

1 1 over2-2

C

5

9

1 6-20

2 -3

D

5

6

1 2- 1 6

2-2

E

6-7

1 0- 1 2

21 -30

3

A

41

4

B

41

4J-2

6b2 7

C

5

9

1 6-20

D

5

6

1 2- 1 6

� � 2�- 3 2-2�

E

6

1 0- 1 2

21 -30

3

2

t

Mandibular 2 2

-

' U n le ss otherw i se i n d i ca ted a l l d a tes a re po st pa r t u m . l U, in utero. Th e teeth are i d e n t i fi ed a . Repri n ted from Ref. 1 by perm i ssio n from Elsevier.

!

1 -2 1 -2

cc o rd i ng

to the Zsigmo ndy System.

I • 1

.

' . _..._.__ ._·

1806 Table

I PART

1 5 THE UPPER DIG ESTIVE TRACT

1 41 .4

(years)

Root completed (years)

4-5 4-5 6-7 5-6 6-7 2r-3 7-8 1 2- 1 6

7-8 8-9 11-12 1 0- 1 1 1 0-1 2 6-7 1 2- 1 3 1 7-21

10 11 1 3- 1 5 1 2- 1 3 1 2-1 4 9-10 1 4- 1 6 1 8-25

4-5 4-5 6-7 5-6 6-7 2r-3 7-8 1 2- 1 6

6-7 7-8 9-10 1 0- 1 2 11 -1 2 6-7 1 2- 1 3 1 7-21

9 10 1 2- 1 4 1 2- 1 3 1 3- 1 4 9- 1 0 1 4- 1 5 1 8-25

Eruption

Tooth

Moxiffory

1 2 3 4 5 6

3-4 mon ths 1 0- 1 2 months 4- 5 months 1 t- 1 � years 2-2! yea rs

7

2r-3 years 7-9 years

B i rth

8 Mandibular

1 2 3 4 5 6 7 8

3 -4 months 3-4 months 4-5 months 1�-2 years 2t-2t years Bi rth

2!-3 years 8- 1 0 years

All d a tes a re post- partum. T h e teeth are i d en tified acco rd i ng t o the Zsigmo ndy Syste m . Rep r i n ted from Ref. 1 b y permission from Elsevier.

Teeth erupted

Max Mand.

ABCDE ABCDE

Teeth unerupted

Max Mand .

1 23 4 5 6 7

Fig u re 1 41 . 1 3 Orthopantomogram from patient aged five years. Reprinted from Ref. 1 by pe rm ission from Elsevier.

1 2 345 67

T h e subli ngua l space lies i n t h e flo or of the mouth,

because t h e fibres of the ma sseter muscle h ave multiple

above the mylohyo id muscles. It is continuous across the

i nsertions o n to mos t o f the lateral surface of the ramus.

midline

and

co mm unicates

with the subman dibular

Between the

med ial surface o f the

sp aces over the post eri or free borders of the mylohyoid

man d i b le

mu scles ( see Figure 1 4 1 .7) .

pterygomandib u l ar space.

The remaining tissue spaces in the region of the molar teeth a re illustra ted i n Figure 1 4 1 . 1 5 . The buccal space is loca ted i n the cheek, on the lateral s ide of the buccinator

th e

medial

p terygoid

ramus

muscle

o f the

lies the

Be h i nd the ramus o f the mand ible is located the paro t id space, in and arou nd t h e

p aro ti d gla nd .

The parapha ryngea l sp a ce is bo unded by the superior constrict o r of the pharynx a n d the medial surface of the

muscle. Between the

and

lateral surface of the

ramus

o f the

m ed i a l p terygoid mu scle. This space i s restricted to the

ma ndible and the masseter muscle is a series of sp aces

i n fra te m p o r al region of the head and the sup ra hyoi d

called the submasseteric spaces These sp aces are formed

region

'

.

of

th e

neck.

It

com m un i cates

wi th

the

.

-

C h a pter 1 41 A n atomy of the m o u th a n d dentition I

Teeth erupted

Teeth unerupted

Max Mand.

1 2 3 4 E6 1 2 34 E6

Figure 1 41 . 1 4

Max

5 78 578

aged 11 years. Repri n ted from Ref. 1 by

Mand.

1 807

Ortho pa ntom ogra m from patient

permission from E l sevier.

buccinator) and will have natural drainage. Where, for example, a cheek tooth drains b elow the buccinator (into the b u ccal space) or lingually b elow the mylohyoid ( into the submandibular space, especially when involving the second and third mandibular molars), surgical interven­ tion will be required to ensure drainage. As an example, direct spread may involve the submental space and the sublingual and submandibular spaces of both sides, giving rise to Ludwig's angina.

TH E SA LIVARY G LAN DS Salivary glands are compound, tubular, acinous, mero ­ crine glands whose ducts open into the oral cavity. They secrete a fluid, the saliva that, among its many functions,

Fig u re 1 41 , 1 5

aids in the mastication, digestion and deglutition of S 9, 1 0 fo o d. '

Diagra m s h o w i n g the tissue spaces i n the

There are three pairs of major salivary glands: the

retrom a n d i bular reg ion. 1 , Vestibular su l cus; 2, peritons i l l a r

paro tid, the submandibular and the sublingual glands.

space ; 3, bucca l s pace , fi l l ed w i th bucca l p a d of fa t ; 4 ,

Numero u s

subm asseteric spaces; 5, pterygo m a n d i b u l a r space ; 6, pa ra p haryngeal s pace ; 7 , parot i d s pa ce ; 8, bucci nato r m uscl e; 9, masseter m u scl e ; 1 0, ra m u s of mandib l e ; 1 1 , su perior constrictor of pharynx; 1 2, med i a l pte ryg oid m uscl e : 1 3 , mylohyoid m uscle ;

1 4, retro pha ryngea I space ; 1 5, pterygoma ndibu l a r ra phe.

retropharyngeal

space,

which

itself extends

minor

salivary

glands

are

also

scattered

throughout the o ral mucosa. App roximately 0 . 5 L of saliva is secreted per day. Salivary flow rates are approximately 0 . 3 mL/min when unstimulated, rising to 1 . 5-2 mL/min when stimulated. D u ring sleep, salivary flow rate is negligible. In the

into

unstimulated state, the paro tid gland contrib u tes ap­

the

proximately 2 0 percent, the submandibular gland ap­

The peritonsillar space lies around the palatine tonsil

salivary glands the remainder. When stimulated, the

retrovisceral space in the lower part of the neck. between

the pillars

of the

fauces.

It is part

of the

intrapharyngeal space and is bo unded by the me dial surface of the superior constrictor of the pharynx and its m ucosa.

proximately 65 percent and the su blingual and minor parotid contribution rises to 50 percent.

1I

Pa roti d g l a n d

Most dental abscesses from lower teeth will drain

.

either into the m o u th lingually above the mylohyoid

The paro tid gland is the largest salivary gland . I t is a

muscle (sublingual space) or into the vestibule (above the

serous gland. The paro tid gland is situated in front of the

--

/ �. . -

j

--�-.

� ,.. -

1808 I

PART 1 5 TH E LI PPER D I G ESTIVE TRACT

external ear on the face and consequently will not be described further. The parotid duct runs through the

The postganglionic fibres are distributed to the sub­ mandibular and sublingual salivary glands.

cheek and drains into the mouth opposite the maxillary second permanent molar tooth. Its parasympathetic innervation is derived from the lesser petrosal branch of

S u b lingual gland

the glossopharyngeal nerve via the otic ganglion. The sublingual gland, the smallest of the main salivary glands, lies beneath the oral mucosa, which is raised as a

Su bmandibu lar gland

sublingual fold (see Figure 141. 3). The gland lies in

The submandibular gland is irregular in shape and

the mandible, close to the mandibular symphysis. It is

approximately the size of a walnut. It consists of a larger

narrow, flat, shaped like an almond and weighs 3-4 g. The

superficial and a smaller deep part, continuous with each other around the posterior border of the mylohyoid

gland

contact with the sublingual fossa on the lingual aspect of

is

seromucous

(but

predominantly

mucous).

Beneath the gland is the mylohyoid muscle, whilst behind

muscle (see Figure 141.7). It is partially enclosed between

it lies the deep part of the submandibular gland (see

two layers of deep cervical fascia extending from the

Figure 141.7). The genioglossus muscle lies medial to the

greater cornu of the hyoid bone. It is a mixed, but

sublingual gland, separated from it by the lingual nerve

predominantly serous, gland. The superficial part of the

and submandibular duct. The sublingual gland has 8-20

submandibular gland is situated in the digastric triangle.

excretory ducts. From the posterior part of the gland,

Above, it extends medial to the body of the mandible.

smaller sublingual ducts mostly open separately on the

Below, it usually overlaps the intermediate tendon of the

summit of the sublingual fold. From the anterior part of

digastric muscle and the insertion of the stylohyoid

the gland small rami sometimes form a major sublingual

muscle.

duct (Bartholin's duct) , opening with or near to the

The inferior surface, covered by skin, platysma and

orifice of the submandibular duct.

deep fascia, is crossed by the facial vein and the cervical branch of the facial nerve. The lateral surface is in relation with the submandibular fossa on the medial surface of the

Minor salivary glands

body of the mandible and the mandibular attachment of the medial pterygoid muscle. The medial (deep) surface is

The minor salivary glands of the mouth include the

related anteriorly to the mylohyoid muscle and poster­

buccal, labial, lingual, palatal and palatoglossal glands.

iorly to the styloglossus muscle. In its intermediate part,

The buccal and labial glands contain both mucous and

the medial surface is related to the hyoglossus muscle,

serous elements. The palatal glands are mucous glands.

separated from it by the styloglossus muscle, the lingual

They are located in both the soft and hard palate. The

nerve, submandibular ganglion, hypoglossal nerve and

anterior and posterior lingual glands are mainly mucous.

deep lingual vein.

The anterior glands are embedded within muscle near the

The deep part of the submandibular gland lies between

ventral surface of the tongue and open by means of four

the mylohyoid muscle inferolaterally and the hyoglossus

or five ducts near the lingual frenum. The posterior

and styloglossus muscles medially. It extends forwards to

glands are located in the root of the tongue. Around the

the posterior end of the sublingual gland (see Figure 141.7).

circumvallate papillae are serous glands (of Von Ebner) .

The submandibular duct is approximately 5 cm long and emerges from the medial surface of the superficial

The palatoglossal glands are mucous glands and are located around the pharyngeal isthmus.

part of the gland behind the posterior border of the mylohyoid muscle. It passes through the deep part of the gland,

runs between the sublingual gland and the

TEMPOROMAND IBULAR JO INT

genioglossus muscle and opens in the floor of the mouth on the summit of the sublingual papilla at the side of the frenulum of the tongue. On the hyoglossus muscle it is

As the

temporomandibular

joint

is

closely

related

anatomically to the external acoustic meatus and clinical

crossed laterally by the lingual nerve, some of whose terminal branches ascend on its medial side (see Figures

that must be distinguished from those directly associated

141.7 and 141.8).

with the ear, the joint will be considered in this chapter.

The secretomotor supply to the submandibular gland

conditions of the joint may produce symptoms of pain

The

temporomandibular

(craniomandibular)

joint

is associated with the submandibular ganglion. This is a small, fusiform body that lies on the upper part of the

(glenoid) fossa articulating with the mandibular condyle.

hyoglossus muscle suspended from the lingual nerve. The

The shape of the mandibular fossa does not conform

motor, parasympathetic root conveys preganglionic fibres travelling in the facial, chorda tympani and lingual nerves

exactly to the shape of the mandibular condyle and this

(see Figure 141.9) to the ganglion, where they synapse.

divided by an articular disc into upper and lower

(TMJ) is a synovial joint. It is formed by the mandibular

may partly explain why, unusually, the joint cavity is

Ana to my of the mouth a n d dentiti on I 1 809

Ch apter 1 4 1

s u b a tm osp h e ri c, m asti ca t io n

1 4, 1 5

but

this

is

greatly

eleva ted

duri n g

.

Articu l a r disc The

art icul a r

disc

(meniscus)

is

of a

dense,

fib ro us

co nsisten cy and is moulded to the b ony j oint surfa ces above and below. Blood vessels are only evident a t the pe r i p hery of the articular disc, the bulk of its central p art

b ein g avascular. When viewed in sa gittal section, the upper su rface o f the disc is concavo-convex from before back­ wards and the lower surface i s concave. Viewed superiorly, the articuJ ar disc is somewhat rectangular or oval in o utl ine

Th e temporoma ndi b u l a r j o int s h o w i n g the a n d a ttachments of th e latera l pterygoid m uscle. A, Articu lar emine nce ; B, articu lar disc; C, m a nd ibular fossa ; D, condyle; E, u pper l a m i n a (fibro-e lastic) ; F, l ow e r l a m i n a (non­ elastic) ; G , ca psu l e of j o i n t ; H , l a t e ra l pteryg oid m uscle. Yel l ow layers indicate u pper a n d l ower joint cavities, black layers i n d icate u pper a n d l ower fi brous a rticular su rfaces. Redrawn by permission from Ref. 1 2. Fi g u re 1 4 1 . 1 6

a rt i c u l a r d i s c

compartments (Figure 1 4 1 . 1 6 ) . 6, 13 The temp orom a nd ib ular j o i n t a ll ows b oth glid ing ( upper j oint co mp ar tm en t ) ­

a nd h inge ( l ower jo int comp artment) m ovem en t s . The

c ondyle

joins th e ram us thro u gh a thi n bony

proj ecti on t er med the neck of the condyl e a frequent si te ,

for a fr a ct u re of the mandible. A small depres s i on ( the

p terygo i d fovea) situ a ted on th e anterior s u rface of th e neck marks part of the a ttachment of the la teral p terygoid muscle. The articular su rface s o f the con d yl e are the

a n te rior and superi or s urfaces. During the period o f growth u p t o early teens, a layer of hyaline cartilage (co ndyl a r cartila g e ) lies immedi ately b e n ea th the fib rou s ar ticu la ting surface of the condyle .

.

The overall sh a p e of the articular disc is tho ught to provide a

self centring mechanism, which automatically acts to -

maintai n its correct relationship to the articular surface of the mandibular condyle during mandibular movem ents.

Wh ereas s o me regar d the functions of the articu la r disc as he lpin g to sp read th e j oin t forces and to stabilize the condyle oth ers see its function as pri marily destabilizing ,

t he co ndyl e and permitting it to move more freely.

16

The central ( i n termediate) zone o f t h e disc is t he th innest a nd the co l la g en fibres are crimped, perhap s

evidence tha t the disc is subj ec t ed to tensional, as well as co mpressi o n a l , fo rces .

17

The ma rgin of the articu l a r d is c

merges peri p h e rally with the j oi nt ca p s ul e. An teriorly, fibrous ba nds connect the disc to the anterior m a rgin o f the

a r tic u lar

eminence a bove, a nd to the anterior ma rgi n

of the co ndyle b elow. Media l ly and la tera ll y, the articular disc is atta ch ed to th e j o int capsule and, just below the medial and l a tera l p oles of the co ndyle

,

by t riang u lar

zones of co nnec tive t iss ue . Pos teri orly, the dis c is a ttached to the c a psule by a looser connective tissue, the retrodiscal tiss ue

(pad)

tha t

has

a

bil aminar

appearance.

T he

su p e r io r l a m ina is lo ose a nd possesses numerous vascular elements and elastin fibres. It attaches to the ant erio r m argin

of the squam o tympanic fissure.

The inferior

l a m ina is rela t i vely avascular, less extensible ( as it has few

The joint caps u l e

elastin fibres) and is a ttache d to the p os te rio r m argin o f the condyl e. The volume of the retrodiscal tissu e a pp ears

The

.

o f th e

above t o

to i ncrease fo u r to five times as a result of venous

mandibular fossa, extending anterior ly to j us t in fro n t

engorgement as the jaw is opened and the co ndyle moves

of the crest of the articular eminence and pos teri o rly to

downwards a nd fo rwards. The return of the articu lar disc

the squ amo tymp anic and pet ro ty mp anic fissu res. Bel ow,

to i t s o r i gin al p osi tio n m ay p os s ibly be aided by the elastic

the capsu l e is attached to the neck of the co ndyle. Being a

recoil o f the superior la m ella .

slack

cuff,

the

TMJ

is a tt ached

the

t hin

capsule

capsule

itself

does

not

limit

N u mero us stu dies have been undertaken t o determine

mandi bular movements and is too weak t o p rovide m u ch

t he p recise attachment of the l atera l p t erygoid muscle

support for the joint. The inne r sur fa ce of the fibrous caps ule is l i ned by a

synovi al membrane that is reflected over the ma rgi ns o f

p redis p osit i on t o

temporom an d i bu lar j oint

syn­

dro me. Variations in the attachm en t a re seen . Th e

the ar ticular disc, but the membrane does not cover the

fi n dings indicate that, in the m aj ority of a r ticles (60

j oi n t .

p e rce nt ) , fibres from the superior head o f the l ateral p terygo id muscle are described as gaining a di rect

articu lar surfaces of the

The syn ovi a l me m b ran e

secre tes the synovial flui d , which occup ies the j o int

I·

with respect to the ar t icula r disc in t he hop e o f expla i n i ng any

cavities a nd lubricates the j o in t . Imp orta n t co mponents

att ach men t i n to the capsule of the

of the fluid are the p roteoglycans . At rest, th e hyd ros ta tic

medial a sp e ct of the

preSSure of the synovial fluid has been rep or ted as bei ng

( as well as to th e co ndyle) . In 30 p ercent

I· �- . .

� nterior

j oint and

to the

border o f the a rticular disc 0f

articles, only a

J

___ _

.-1 __ . _-

1810 I

PART 1 5 THE U PPER D I G ESTIVE TRACT

while in the remaining 10 percent of articles the superior

In nervation and vascu lature of the temporomandib u l ar joint

head of the lateral pterygoid muscle is attached only to 18 the condyle. Very rarely, some fibres from the inferior head of the lateral pterygoid may insert into the articular

Innervation for the joint is provided by the auriculotem­ poral, masseteric and deep temporal branches of the

few muscle fibres are described as inserting into the disc,

disc.

mandibular nerve. Of particular functional significance are the proprioceptive nerve endings important in the reflex control of mastication.

Ligaments of the temporomandibu lar joint The main ligament limiting lateral movement at the TMJ is the lateral ligament (temporomandibular ligament) , which cannot be readily separated from the capsule. It runs

downwards and

The vascular supply is derived from the superficial temporal artery and the

maxillary

artery

(anterior

tympanic and deep auricular branch). Other branches from neighbouring arteries may also contribute (e.g. deep temporal and transverse facial arteries) .

backwards from the articular

tubercle (a bony protrusion on the lateral surface of the articular eminence) to the lateral surface and posterior border of the neck of the mandibular condyle. 1 9 To help resist posterior movement of the mandibular condyle, the lateral ligament is reinforced by a horizontal band of fibres running from the articular eminence to the lateral surface of the condyle. As there is little evidence of a medial ligament, medial displacement of the TMJ is likely to be prevented by the lateral ligament of the opposite side. Accessory ligaments of the temporomandibular joint are the stylomandibular ligament, the sphenomandibular ligament and the pterygomandibular raphe. However, of

Condylar cartilage A secondary cartilage is present beneath the fibrous articular surface of the condyle until puberty. Unlike a primary cartilage, this secondary condylar cartilage has less extracellular matrix, the cartilage cells themselves do not undergo cell division and do not align themselves into columns. Although once thought to be a prime causative factor in controlling mandibular growth, the secondary condylar cartilage is now not thought to have any intrinsic

growth

potention.

The

condylar

cartilage

disappears around the age of 16 years.

these, the only one likely to have any significant influence upon mandibular movements is the sphenomandibular 0

ligament. 2

The stylomandibular ligament is merely a reinforced lamina of the deep cervical fascia that extends from the tip of the styloid process and from the stylohyoid ligament to the angle of the mandible. The pterygomandibular raphe is a thin band of tendinous fibres passing between the hamulus of the medial pterygoid plate and the posterior end of the mylohyoid line of the mandible. It gives origin anteriorly to the buccinator muscle and posteriorly to the superior constrictor muscle. The sphenomandibular ligament runs from the spine of the sphenoid bone to the lingula of the mandible. It represents the remnants of the perichondrium of the

DEVELOPMENT OF THE FACE AND PALATE As the neural plate of the embryo invaginates to form the neural tube, neural crest cells (ectomesenchyme) at the margins proliferate and migrate from this site to various parts of the body. There, interacting with the overlying epithelium

(epithelial/mesenchymal

interactions) ,

the

neural crest plays a significant role in normal develop­ ment, contributing to many systems, such as the nervous system, soft and hard connective tissues. In addition, the cells play a major role in tooth development and give rise to melanocytes.

cartilage of the embryonic first branchial arch. The sphenomandibular ligament is slack when the jaws are

Deve lopme nt of face

closed, but during jaw movement becomes tense at about the time when the condyle has passed in front of the lateral ligament. 2o

In a four-week-old embryo, the developing oral cavity

In addition to the above, an additional ligament, the retinacular ligament, has recently been described. This ligament is said to originate fom the articular eminence and insert into the fascia overlying the masseter muscle at the angle of the mandible. As the ligament is connected with the retrodiscal tissues, and contains an accompany­ ing vein, it may function in maintaining blood circulation during masticatory jaw movements.z1

(stomodeum) is present as a small, blind-ended pit bounded by five facial swellings (prominences) produced by proliferating zones of mesenchyme lying beneath the surface ectoderm. Much of the mesenchme is derived from neural crest cells. The five facial swellings are the single frontonasal and the paired maxillary and mandib­ ular processes (Figure 141. 17). The centrally positioned frontonasal process lies above, the two maxillary processes are located at the sides and the two mandibular processes lie below the mouth. The maxillary and mandibular

Ch a pter 1 41

Fig u re 1 41 . 1 7

Sca nn i ng electro n mic rog ra ph of the face at a n

equivalent fou r week stag e On t h e ra t). Frontal aspect. A, Fro ntonasa l process; B, mandibular processes ; C, maxil l a ry processes; 0, pericardial swe l ling. Repri nted from Ref. 1 by perm ission from Elsevier.

processes are derived from the first branchial arches. The facial processes are separated by grooves t ha t , in the co urse of normal development, become flattened out by the proliferative and migra tory activi ty of the underlying mesenchyme. Any disturba nce in this process affecting, for example, the number, migra tion a nd subsequent differentiation (or apoptosis) and pattern formation of these cells can produce congenital abnormalities such as cleft lip and palate.

An a t om y

of th e mouth a nd dentition . 1 8 1 1

At t h i s early stage of development, a membrane (the oropharyngeal membrane) separates the invagination representing the pri mitive oral cavity from the developing pharynx behind. The oropharyngeal membrane is bila­ minar, being composed of an outer ectodermal layer and an inner endodermal layer. This membrane soon breaks down to esta blish continu ity between the ectodermally lined oral cavity and the endodermally lined pharynx. Although not detectable in the adult, the demarcation zone between mucosa derived from ectoderm and endoderm corresponds to a region lying just behind the third permanent molar tooth. In a five-week-old embryo, localized thickenings of ectoderm on each side give rise to the optic and nasal placodes. These placodes wiJl invaginate to form the lens of the eye and the olfactory epithelium, respectively. The nasal placodes sink into the underlying mesenchyme, forming two blind-ended nasal pits. Proliferation of mesenchyme fro m the frontonasal process around the openings of the nasa l p its produces the medial and lateral nasal processes. The nasal pits continue to deepen until eventually they approach the roof o f the primitive oral cavity, being partitio ned from it by oronasal membranes (Figure 141.18). By the end of the fifth week, these membranes rupture to produce communications between the developing nasal and oral cavities. In the six-week-old embryo, the two mandibular processes fuse in t he midline to form the tissues of the lower jaw. Rarely, persistence of a midline groove in this region produces a mandibular cleft. The mandibular and maxillary p rocesses meet at the angle of the mouth {labial

(a) Sagitta l section through the deve l o p i n g n asa l A and o ra l B cavities to C. de�eloping ton gue 0, oronasa l mem bra ne; E. m a xill ary isthm us. Toluidi n e b l u e x 30. (b) Diagra m with similar labe l l ing. Repri nted from Ref. 1 by perm ission from Elsevier.

Fig u re 1 4 1 . 1 a

. I ----=---

1812 I

PART 1 5

THE UPPER D I GEST IVE TRACT

commissure ) , thus defining

its outline. Disturbances in

maxillary processes being sup plied by the maxillary nerve,

this develo p ment may give rise to macrostomia ( enlarged

the frontonasal process by the o phthalmic nerve. It is o f

oral orifice) or microstomia ( small oral orifice) , or rarely

interest t o no te that, in cases o f bilateral de ft lip, the

to an astomia (lack of an oral orifice) . From the corners of

iso l ated median segment of the lip is innervated by the

the mo uth, the maxillary processes grow inwards beneath

ophthalmic nerve, al t hough t h is dearly is a patho logi cal

the lateral nasal processes and to wards the medial nasal

condition. At presen t , too

p rocesses of the upper lip (Figure 1 4 1 . 1 9 ) . An apprecia­

behaviour and any subsequent m ig ra tio n o f the mesench­

l ittle is kn own abo ut the

tion of this arrangement helps explain the occurrence o f a

yme of t h e facial p rocesses a nd the acco mp anyi ng nerves

un ilateral or bila teral cleft lip (when the medial nasal and

a fter the i nitial fusion of th e maxillary and medial nasal

maxillary processes fail to merge successfully) and a

pro cesses. The muscles of the lip, like the other fa cial

median cle ft (when the two median nasal processes fail to

muscles, are derived from the mesenchyme of the second

me rge ) .

branchial arch, and are therefore innervated by th e facia l

T he severity of a cle ft may vary fro m a barely

imp erceptible groove to a co mplete cle ft. Between the

nerve .

merging maxillary and the lateral nasal process lies the naso - op t ic furrow. From each furrow a solid ectodermal rod of cells si nks below the s urface and can alizes to fo rm the n asolacrimal duct.

Persistence

Deve lopment of the pa late

o f the naso-o p t ic

furrow may produce an oblique facial cleft.

As we have seen at five weeks o f develo p ment, the nasal th e

p i t deepens and the nasal p lacode comes to lie in its roof

development of the upper lip. One interpretation is that

where it will form the olfacto ry epitheli um. The region of

th e m axillary processes meet the medial nasal processes,

t he fro nto nasal p rocess lying between the two nasal pits

Two

differin g

accounts

have

been

given

for

the latter thus forming the middle third of the up per l i p.

forms the primary nasal septu m , while the tissue beneath

is favoured by macroscopic observations o f

the p its a n d sep a rati ng them fro m the sto mo de u m fo rms

Thi s view

develo pment. The altern ative view states that t h e m ax­

the p r i m ary palate . At this stage, the nasal pits are

the medial nasal processes,

co n fined o nly to the anteri o r extremity of the fu ture oral

m ergin g in the m idline to contribute all the tiss ue fo r the upper lip. This interpretation is based on the innerva t io n

co mmon oronasal cavity (Figure 141 .20a) . S ubsequent

illary processes overgrow

o f the fully formed upper lip (i.e. t h e infraorbital branch of the

maxillary division of the trigeminal nerve ) , the

cavity, the large regio n behi nd at this s tage fo rming a develo p ment is

d esigned

to extend the p rimary nasal

septu m and p ri mary p alate backwards by means of a secon dary nasal sep t u m and secondary pala te. D u ring the sixth week of development, a secondary nasal

sep tum

sto modeum

grows

behi nd

d o wn the

from

the

p rimary nasal

roof

of the

sep tum,

thus

div i ding the nasal aspect of the comm on oron asal c avity i nto two. In ad dition, a lateral p alatal shel f grows out fro m each maxillary p rocess b u t, d u e to t h e comparat ively la rge size of t he developing to ngue, is prevented fro m growing ho ri w ntall y and beco mes o riented more verti­ ca ll y ( Figures 1 4 1 .20b and 1 4 1 .2 1 ) . D u ri ng the eighth week o f develop ment, the tongue 'drops' and the vertically i ncli ned palatal shelves can now occupy

a

horizontal

positi o n

(Figures

141 .20c

and

1 4 1 .22 ) . The reaso n fo r the displacement of the tongue is no t

known. As

the to ngu e is attached to the

mand ible,

it has been su ggested that the descent of the tongue is

related to m andibular growth. Co nversely, reflex move­ ments of th e tongue and / o r a change in its shape may be impl icate d . On beco ming horizo ntal, the p alatal shelves co n t act each other (and the secon dary nasal sep tu m) in the midline to fonn the secondary palate . The shelves contact the primary pala te anteriorly so that the oral and Fi g u re 1 41 . 1 9

Scanning electron microg rap h of developing ra t upper jaw and lip at an equivalent stage to six weeks i n the human showing the merging maxillary and medial nasa l processes. A, Mandibu lar p rocess; B, maxillary process; C, l atera l nasa l process ; D, medial nasa l process ; E, naso-optic furrow ; F, primitive nasa l cavity. Reprinted from Ref. 1 by permission from Elsevier.

nasal cavities beco me completely separ ated from each other. A fter shel f contact, the medial edge epithel ia of the two

shelves

fuse to form a midline epitheJial seam .

Subsequently, this seam (to gether with the epitheliu m sep a rating the p alatal shelves from the secondary nasal sept u m ) degenerates, allowing conti nuity of mesenchy m e

.

Chapte r 141 Anatomy of the mouth a n d den tition I

Fi g u re 1 41 .21

1813

Coro n a l section th rough t h e h e a d a t a n

eq u iva lent stage of seven weeks i n tra u teri ne development i n t h e h u m a n show i n g t h e vertica l ly i ncl i n ed pa l ata l shelves (A) . B, Developing tong ue. M asso n 's trich rome x 3 0. Reprinted from Ref. 1 by permission from E lsevier.

Fi g u re 141 .22

Coro n a l section throug h deve l o p i ng o ro- nasa l

regions following contact of the palata l s h e lves (A) a n d secon d a ry n a s a l s e p t u m

(8).

C, M i d l i n e e p i th e l i a l seam ; 0 ,

developing bone o f maxilla. Masson's trich ro m e x 30. R e p ri nted

Fi g u re 1 41 .20 pa l ate

(a) .

Diag ra m show ing the d eve l o p m e nt of the

from Ref. 1 by permission from E lsevier.

By sixth week of i n tra uterine life; ( b) d u ri ng s ixth

week of i n tra uteri n e l ife a n d

(c).

At n i nth week of i n traute rine

l ife. A, latera l palata l she lves of secondary pa l ate : B, primary

b one

pa l ate ; C, primary nasal se ptu m ; 0, seco n d a ry n asa l sep tu m ;

perpendicular plate and appears in th e eighth week of

is

situated

in

the

region

fo rming

the

future

d evelopment. Incomplete ossification of the palate from

E , pri m itive n asa l cavities.

these centres defines the median and transverse palatine sutures. There does not appear to be a separate centre of 'to be established across the now intact and horizontal

ossification for the primary palate in man ( in other

secondary palate. Fusion

species

of the palatal processes is

complete by the 1 2th week of d evelopment. B ehind th e secondary nasal septum, the palatal shelves fuse to form the soft palate and uvula.

is

such

a

centre,

formin g

a

separate

The muscles of the palate are derived fro m the lower branchial

arches,

hence

their

nerve

supply

by

the

The hard palate ossifies intramembranously from fo ur

pharyngeal plexus. However. tensor veli palatini develops

centres of ossification, one in each developing maxilla and

from the first branchial arch, explaining its innervation by

one in each developing palatine b one. The maxillary

th e mandibular nerve.

ossification centre lies above the developing d eciduous

hr·

there

'premaxilla') .

As is

evident

from

the

description

above,

palate

canine tooth germ and appears in the eighth week of

formation is a complicated process. The palatal shelves

development. The centre of ossification for the palatine

must grow to the appropriate size; the palatal shelves

18 14 I

PART 1 5 TH E U PPER D I G ESTIVE TRACT

must elevate at the appropriate time on both sides; the

tissues across the midline. The signals that are responsible

medial edge epithelia of the palatal shelves must adhere to

for such a breakdown are not yet fully understood. As a

form a midline epithelial seam: the midline epithelial

breakdown of medial edge epithelium occurs in single

seam must degenerate to allow for the establishment of

isolated palatal shelves, the process does not depend

mesenchymal continuity across the midline. Any inter­

specifically upon shelf contact.

ference or mistiming of these processes may contribute

Factors that may contribute to thinning and then

towards the formation of a cleft palate. Clefts of the

removing the epithelial seam include: growth of the palate

palate, like those of the lip, are multifactorial malforma­

(in terms of oronasal height) and epithelial cell migration

tions, involving both genetic (polygenic) and environ­

from the region of the seam on to the oral and nasal

mental factors. Recent research on palatogenesis has

aspects of the palate; programmed epithelial cell death

concentrated on two main events: palatal shelf elevation

(apoptosis); migration of epithelial cells from the epithelial

and the initial stage of fusion of the shelves.

seam

into

the

palatal

shelf mesenchyme

and

their

subsequent differentiation into mesenchymal cells (epithe­ 23 lial/mesenchymal transformation). As in other systems, it

PALATAL S H ELF ELEVATION

is thought that signals

This process, during which the palatal shelves move from

( e.g.

growth factors such as

epidermal growth factor and transforming growth factors)

the vertical to the horizontal position, is thought to occur

emanating from the underlying mesenchyme are ultimately

very rapidly (in terms of minutes and hours rather than

responsible for controlling epithelial degeneration.

days). Although it was once thought that extrinsic forces might be responsible (e.g. forces derived from the tongue or jaw movements), recent research has primarily focused

CLI N I CAL AN D AETIOLOG I CAL CO N S I D ERATI O N S

on the search for a force intrinsic to the palatal shelf.

Malformations

Systems which

appearance of clefts. The mildest form of cleft is that

have

at

one

time

or

another

been

o f palatogenesis

may

result

in

the

implicated include differential mitosis, contraction of

affecting the uvula, such a disturbance occurring relatively

connective tissue elements such as collagen or mesench­

late

ymal cells, changes in vascularity or tissue fluid pressures.

occurring during the early phases of palatal fusion can

in

the

process

of palatal

fusion.

Disturbances

Particular attention has focused on the proposition that

result in a more extensive cleft involving most of the

the intrinsic shelf elevation force might develop as a result

secondary palate. Should the cleft involve the primary

of hydration of ground substance components (princi­ 22 In this

palate, it may extend to the right and/or left of the incisive

pally hyaluronan) in the shelf mesenchyme.

foramen to include the alveolus, passing between the

context, hyaluronan is a highly electrostatically charged,

lateral incisor and canine teeth. Cleft palate may be

open coil molecule that is capable of binding up to ten

associated with cleft lip, though the two conditions are

times its own weight in water. In support of the possible

independently determined.

Dental

malformations are

importance of hyaluronan in palatogenesis, research using

commonly associated with a cleft involving the alveolus.

organ culture systems have shown that the presence of

A submucous cleft describes a condition where the palatal

agents either leading to an alteration in hyaluronan

mucosa is intact, but the bone/musculature of the palate

content

is deficient beneath the mucosa.

or

size,

or

that

disrupt

glycosaminoglycan

substitution on proteoglycans, or that alter the balance of matrix molecules secreted via the Golgi complex and hyaluronan produced at the cell surface, all detrimentally 1 affect normal palatogenesis. In addition to hyaluronan, however, other matrix components (including proteogly­ cans) may also be important during shelf elevation.

KEY POI NTS •

mouth and dentition . is mandatory . for · all head and neck surgeons operating in this

FUSION OF TH E PALATAL S H ELVES

Following palatal shelf elevation, contact is made initially in the middle third of the palate and then extends both anteriorly and posteriorly. The two adjacent medial edge epithelium contact each other and adhere by means of a 'sticky' glycoprotein coating their surfaces. In addition, the epithelial cells develop desmosomes that form a

A detailed knowledge of the anatomy of the

area. •

the general ENT surgeon, the anatomy of: - the floor of mouth is irnportant when For

dealing with submandibular duct pathology or submandibular duct rerouting, . tongue tie, treatment of a ranula and biopsying suspicious . lesions;

medial epithelial seam. The adherence of the medial edge

the buccal mucosa is irnportant wheri

epithelia is site-specific as palatal epithelia will not fuse 22 with epithelia from other sites (e.g. the tongue).

biopsyil}g suspicious lesions;

For normal palatogenesis to proceed, the epithelial seam must be removed for consolidation of mesenchymal

dealing with parotid duct pathology and

- the hard palate is important when biopsying suspicious lesions;

C h a pte r 1 4 1 Anatomy of the mouth and dentition I

".' 1' -,' ' �':"

�e. �ft, p;alate is ,import,J: : .

For t he genera l ENT surgeo n ,

the development of t he

is

' The.anatomyofthe .• fascial . . capsules'()£the salivaryglandsinfluenc~s thespreadQf salivary gland. djsease.

Davis RA, Anson BJ, Budinger JM, Kurth RE. Surgical anatomy of the facial nerve and parotid gland based on a study of 350 cervicofacial halves. Surgery, Gynaecology and Obstetrics. 1956; 102: 385-412.

3.

Katz AD, Catalano P. The clinical significance of the various anastamotic branches of the facial nerve. Archives

c.

of Otolaryngology - Head and Neck Surgery. 1987; 113: 959-62. 4.

Ragbir M, Dunaway OJ, Chippindale AJ, Latimer J, Mohammed F, McLean NR. Prediction and position of the intraparotid portion of the facial nerve on MRI and CT. British Journal of Plastic Surgery. 2002; 55: 376-9.

5.

McKean ME, Lee K, McGregor IA. The distribution of lymph nodes in and around the parotid gland: an anatomical study. British Journal of Plastic Surgery. 1985; 38: 1-5.

-

145 Physiology of the salivary glands RODERICK CAWSONt AND MICHAEL GLEESON

Introduction

Salivary

Collection of

1858

Salivary assays Key poin ts

1861

References

1858

secretion

saliva

Sia lochemistry

in diagnos is

1867 1869 1869

1863

The data in this chapter are supported by a Medline search using the term saliva and focusing on chemistry, drug effects, immunology, physiology and secretion.

INTRODUCTION

both viruses and bacteria can be detected and monitored from salivary samples as can drug and hormone levels.

It would not be unreasonable to say that in the past most

There is little doubt that our knowledge of saliva, its

surgeons' interest in salivary glands has been limited to

function, production and modulation

will grow (Table

145.1).

The next generation of surgeons will require a

recurrent infections. Yet disturbances of salivary gland

much

more

function or inability to cope with normal salivary flow

physiology which this chapter attempts to address.

the treatment of primary tumours, duct obstruction and

thorough

knowledge

of

salivary

gland

can have a devastating effect on the quality of life, not only for our patients but that of their relatives. For example,

the

dry

mouth

of patients with

Sjogren's

SALIVARY SECRETION

syndrome can lead them to despair, while drooling secondary to cerebral palsy may make them a social outcast and precipitate placement in institutional care. Over the past decade there has been an explosion of

The amount of saliva secreted and, to a large extent, its composition, is normally controlled by the autonomic nervous system. Other factors can also interfere with

the biotechnology of diagnostic tests. Clinical practice is

glandular

in the process of change. A wide variety of tests, once

autonomic responses, dehydration and disease or destruc­

function,

for example,

drugs which affect

almost exclusively confined to the examination of blood

tion of salivary gland parenchyma.

and urine, can now be undertaken as precisely and accurately on other body fluids, such as sweat, tears and

saliva. While the collection of saliva may lack the drama

Autonomic control of salivary secretion

of venepuncture, the sincerity of sweat or the emotional appeal of tears, it is, at the least, readily accessible. 1

Samples can be reliably collected by the patient at home or in the workplace and, unlike blood, samples do not clot or carry the risk of needle-stick injury. Antibodies to

Extensive animal experimentation has been undertaken to establish the effects of autonomic stimulation on salivary flow.2 Normal reflex secretion of saliva depends on centrally coordinated parasympathetic and sympathetic

-

�

,

Chapte r 145 Physiology of the salivary glands I

Table 145.1

1859

The major functions of saliva.

Function Protective functions Lu b rication

M ucins, proline-rich g l ycoproteins, water

Antimicrobial

Amylase, complement, defensins, lysozyme, lactoferrin, lactoperoxidase, mucins, cystatins, histatins, pro l ine-rich glycoproteins, secretory leukocyte protease inhibitor, statherin, th rombospondin

Growth factors

EGF, TGF -ct, TGF-�, F GF, I GF-l and I GF - 2 , N GF

M ucosal integrity

M u cins, el ect rolytes and water

Lavage/cleansing

Water

Buffering

Bicarbonate, phosphate ions, proteins

Reminera I ization

Calcium, phosphate, statherin, anionic proline-rich proteins

Food

and

speech-related functions

Food preparation

Water, mucins

Digestion

Amyl ase, lipase, ribonuclease, proteases, water mucins

Taste

Water, g u stin

Speech

Water, mucins

EGF, epidermal growth factor; FGF, fibroblast growth factor; IGFl and IGF2, insulin-like growth factor; NGF, nerve growth factor; TGF-Cl, transforming growth factor-alpha; TGF-�, transforming growth factor-beta.

activity. Parasympathetic activity evokes most of the fluid

secreted by causing variable degrees of exocytosis from the

secretory cells and vasodilatation within the gland. It also induces contraction of the myoepithelial cells. Sympa­

thetic stimulation of the salivary glands tends to be more

binding and probably conveys the functional specificity to

a G protein. Binding of a neurotransmitter to a receptor greatly strengthens the ability of a receptor to associate

with a G protein. The association with a G protein

stimulates replacement of GDP by GTP at the nucleotide

intermittent and is also directed at the cells that receive

binding site and promotes dissociation of the hetero­

composition of saliva by increasing exocytosis from

subunit

parasympathetic impulses. certain cells.

It tends to modulate the

Some sympathetic

fibres exercise tonic

effects on blood vessels; these fibres are likely to be under

separate central control and not involved directly in the

reflex secretory pathway.

trimer into a free can

cr

then

subunit and a �'Y complex. The

activate

the

appropriate

cr

receptor

molecule. Activation continues until the GTP is broken down

into

GDP

activity in the

cr

by endogenous

enzymic

(GTPase)

subunit. The GDP bound to the

cr

subunit then unites with the �'Y complex to regenerate the

heterotrimeric molecule. There is also some evidence to suggest that the �'Y complex, or even the � subunit alone,

Mechanisms of salivary secretion

may be able to activate some receptor molecules directly.

The best recognized signal transduction processes in The mechanisms of saliva secretion have been reviewed in 3 detail by Baum, who emphasized that much of the

information presented below comes from the study of rat salivary glands. Little is known of the mechanisms of

salivary glands are first, generation of cAMP as a result of

� adrenergic receptor stimulation and second, formation

of 1,4,5-inositol triphosphate (IP3 ) after acetylcholine receptor stimulation. The latter leads to calcium ion

human salivary secretion, but such data as exist generally

mobilization and, as a consequence, fluid secretion.

described,

intermediary

.conform to the broad principles discussed here. As Baum saliva

is

produced

only

in

response

to

neurotransmitter stimulation. Neurotransmitters prob­

ably bind to specific receptors in the basolateral region of

the acini.

Noradrenaline binds

to both

cr-

and

�­

The specific mechanism by which cAMP acts as an

Typically,

in

cAMP

protein elicits

exocytosis

a

remains

response by

unclear.

activation

of

cAMP-dependent protein kinase and protein phosphor­

ylation.

In rat salivary glands,

�-adrenergic receptor

adrenergic receptors, while acetylcholine binds to choli­

stimulation leads to a rise in cAMP levels, activation of

nucleotide-binding regulatory protein (G protein) for

several cellular proteins and thus, amylase or glycoprotein

nergic receptors. These receptors depend on guanine transduction of the neurotransmitter stimuli. The G

the A kinase, phosphorylation or dephosphorylation of secretion

by

the

parotid

leads to protein secretion. Kinetic analyses suggest that

cr

of

cr,

�

and

'Y

subunit is the site of guanine nucleotide

of

rat

glands,

subunits. The

molecules consisting

treatment

submandibular

respectively.

heterotrimeric

Indeed,

or

proteins that carry the stimuli into the acinar cells are

parotid

and

submandibular gland cells with cAMP analogues also

1860 I

PART 1 5 TH E U P P ER DIGESTIVE TRACT

the 24-26 kDa species protein is the most probable

contains. The acinar cells are water permeable and derive

protein exocytosis is unknown. Moreover, the role of a

carried by the duct system which is impermeable to water.

protein secretion has become somewhat controversial. ++ It is well established that Ca plays a central role in

electrolytes. Most of the sodium and chloride ions are

candidate for phosphorylation although its function in

kinase-dependent phosphorylation in rat parotid gland

fluid secretion by acinar cells in response to acetylcholine

fluid from the surrounding blood vessels. The fluid is During passage through the ducts, there is exchange of

removed, but some potassium and bicarbonate ions as well as a little protein, are added.

receptor stimulation. Activation of this receptor leads to

fluid-related signal transduction via coupling to a G protein often designated G • The latter is probably a p member of the Gq/ll family which is known to couple to phospholipase C. Activation of phospholipase C results in hydrolysis of a minor membrane phospholipid, phospha­

tidylinositol 4,5-bisphosphate, and formation of second

Mechanisms of primary fluid secretion in salivary acini As in all secretory epithelia, fluid transport in salivary

gland cells is

thought to be

driven osmotically by

messengers

transepithelial salt gradients. Turner4 has explained that

but diacylglycerol can promote activation of protein kinase

three mechanisms for primary salivary fluid secretion.

IP3 and diacylglycerol. IP3 is the main ++ mediator of Ca mobilization and thus of fluid secretion, C and lead to stimulation of a minor exocytic pathway.

IP3 binds to a receptor protein located on an ++ intracellular Ca storage pool that is probably related

to, or part of, the endoplasmic reticulum. The IP3 ++ receptor also functions as a Ca release channel and ++ allows stored Ca to move down a concentration ++ gradient into the cytoplasm. These Ca levels quicldy rise approximately ten-fold as a consequence of acet­

ylcholine receptor stimulation. This reaction triggers a ++ cascade of events which include sustained Ca entry and

activation of specific ion transport pathways. Generation

of fluid in the acinar lumen is the final result, by mechanisms discussed in detail by Turner.4 ++ ++ Extracellular Ca and entry of Ca sustain high

levels of salivary fluid secretion over long periods. However, ++ mechanisms of Ca entry into acinar cells which are

studies on rat and rabbit salivary glands have suggested

Unexpectedly, these mechanisms do not appear to be

alternative explanations for salivary fluid secretion, but

appear to operate concurrently within the same gland or

possibly within the same acinar cells.

The first mechanism, of which the other two can be

regarded as variations, is that fluid secretion depends on the

combined

systems, namely: 1. an Na

+

-K

+

action

of

four

membrane

transport

-2CI- cotransporter that is located in

the basolateral membrane of the acinar cells; ++ + 2. a basolateral Ca -activated K channel;

3. an apical conductive pathway for CI- which is ++ presumably a Ca -activated CI - channel; + + 4. the Na IK ATPase.

+ In the resting state, both K and CI- are concentrated in

nonexcitable and not voltage activated, are poorly under­ ++ stood. Possible mechanisms for Ca entry into acinar cells ++ include a direct receptor-gated Ca channel, a G protein­ ++ activated Ca channel and a second messenger-activated ++ Ca channel. However, there is little evidence for the

the acinar cell above electrochemical equilibrium, with + + + being concentrated by Na IK ATPase and CI - by + + Na -K -2CI- cotransporter. As discussed under Me­

activated by the synergistic action of IP3 and its metabolite 1, 3, 4,5-inositol tetrakisphosphate (IP4)'

and CI- conductance allow KCI to flow out of the. cell

involvement of the first two of these mechanisms and only ++ a few reports have suggested that Ca entry may be

Currently,

the

most favoured explanation of the ++ mechanism of Ca entry is one termed 'capacitative ++ ++ Ca entry'. This suggests that depletion of the Ca

storage pool provides the driving force for sustained ++ Ca entry. Support for this theory comes from studies ++ that have shown that graded depletion of the Ca store ++ have led to similarly graded Ca entry.

Use of nonreceptor activation by, for example ++ thapsigargin, to deplete the Ca store also leads to ++ entry. Although the exact mechanism by which Ca ++ ++ depIetl· On 0 f Ca stores causes Ca entry IS . uncertam, .

it is clear that it can be modulated by extracellular pH and ++ . cytopIasml. C Ca Acinar cells are responsible for production of the fluid

component of saliva and most of the proteins that it

K

chanisms of salivary secretion, secretagogue stimulation ++ leads to a rise in intracellular Ca concentration and, in ++ + turn, opening of the basolateral Ca -activated K + channel and the apical CI- channel. Increases in K and this results in accumulation of CI- ions and their

associated negative electrical charge in the acinar lumen. + As a consequence, electrical attraction causes Na to leak

from the interstitium through the tight junctions to

follow Cl. The resulting osmotic gradient for NaCI causes

transepithelial movement of water from the interstitium to the lumen. Continued influence of an agonist results in

a transepithelial chloride flux and concomitant fluid + + secretion. This is sustained by CI- entry via the Na -K 2CI- cotransporter and exit via the apical CI - channel.

Removal

of

intracellular

the

stimulus

is

followed

by

a

fall

in

calcium concentration to resting levels, + closure of the K and CI- channels and return of the

cell to its resting state.

The second mechanism is similar except that the + + basolateral Na -K -2CI- cotransporter is replaced by a

Chapter 145 Physiology of the salivary glands.

CI- IHC03 exchanger acting in parallel with an Na++ I H+ exchanger. A faU in intraceUular chloride concentra­ tion as a result of secretagogue KCI loss, thus leads to entry of more CI- in exchange for HC03 -. Acidification

1861

Depression and anxiety states are often associated with

a

dry mouth.

Busfield and Wechsler6 measured the rate of

salivary secretion in 42 untreated depressed patients and

found it to be decreased in comparison with nonde­

of the cytoplasm that results from this bicarbonate loss is

pressed

buffered by the Na

correlation was found between the degree of xerostomia

++ IH+

exchanger, which uses the

hospital

patients

and

healthy

controls.

No

extracellular-to-intracellular sodium gradient generated

and the assessed severity of depression, nor was there any

by

difference found in secretion rates in

+ Na+ IK

ATPase, to drive protons out of the cell.

Unlike the first two mechanisms in which chloride is

Busfield

et

al?

between the

a

different

later study by categories

of

the secreted ion, the third involves acinar bicarbonate

depression or between those who complained of dry

secretion. In this last mechanism, CO2 enters the acinar

mouth and those who do not. The fact that decreased

cell across the basolateral membrane and is converted to

salivary flow is associated with sympathetic overactivity,

HC03 - plus a proton, by intracell ular carbonic anhy­ drase. HC03 - is lost across the apical membrane via an

as in anxiety states, or caused by sympathomimetic drugs

anion channel which is possibly the same as that involved

that the sympathetic supply to the salivary glands is

in chloride secretion. The proton is expelled by the

inhibitory in humans. However, sympathetic inhibitory

basolateral

fibres have not been found and, until they are, the

+ Na ++ IH

exchanger.

and can be relieved by �-blockers would seem to suggest

generally

accepted

hypothesis for the

mechanism

of

anxiety-induced xerostomia is that of central inhibition from higher centres which act on the salivary nuclei in the

Factors affecting salivary flow

brainstem and suppress reflex activity.

Even in the absence of parenchymal salivary gland disease, function can be abnonnal and, when diminished, result in

xerostomia. Although xerostomia is usually a complaint of late middle-aged women, it should not be assumed that ageing itself causes deterioration of salivary gland function. Several studies have been carried out and have yielded somewhat conflicting results. Ship et aI.s found that nom1al

postmenopausal women, the main group thought to be at

risk, had no deterioration of salivary gland function. In clinical practice, drugs are the most common cause of decreased salivary gland function. Tricyclic antidepres­ sants and phenothiazine neuroleptics are among the most

Drying of the mouth is virtually inevitable in dehydra­ tion and, as such, is a consequence of haemorrhage, diarrhoea, chronic vomiting, polyuria secondary to diabetes mellitus, restricted fluid intake or overdose of diuretics. A detailed discussion of the salivary gland diseases that cause xerostomia is outside the scope of this chapter. Nevertheless, it is

worth noting some of the more

common conditions, if only for further reference, namely: autoimmune sialadenitis, human immunodeficiency virus

(HIV)

infection,

radiation

damage,

graft-versus-host

disease, sarcoidosis, iron overload, amyloidosis and type

V

hyperlipoproteinaemia.

troublesome as they have particularly strong antimus­ carinic effects and are generally used over long periods. Formerly, ganglion-blocking drugs had the same effect,

COLLECTION OF SALIVA

but are now generally regarded as obsolete for the routine management of hypertension. A list of commonly used drugs that affect salivary flow is shown in

Table 145.2

Table

145.2.

Saliva may need to be collected to determine the flow rate, to confirm or discard a clinical complaint of xerostomia

Drugs liable ,to 53use xerost?mia.

Drugs with antimu5carini,c activi,ty Atropine and analogues (hyoscine, ipratropium, etc . )

Tricyclic antidepressives

'Cold cures' and decongestants containing ephedrine or phenylpropylamine

Monoamine oxidase inhibitors

B ronchod ilators (isoprenaline, orci prenaline, etc.)

Phenothiazines and related neuroleptics

Appetite suppressants, particularly amphetamines

Orphenadrine, benzhexol and related anti-Parkinsonian agents Antihistamines Ganglion blockers and clonidine Antiemetics (antihistamines, hyoscine and phenothiazin es)

and diethylpropion

1862 I

PART 15 TH E LI P P E R DIGESTIVE TRACT

or to provide a sample for assays of any type. Many

methods have been devised and may be summarized as follows: •

unstimulated flow of whole (mixed) saliva;

•

stimulated flow of whole saliva;

•

SPITIING AND DRAINAGE METHODS

The patient is put into a comfortable sitting position with the head inclined forward and encouraged to spit at one­

minute intervals or to allow saliva to drain out of the mouth into a funnel draining into a sterile collecting vessel.

stimulated or unstimulated flow from individual glands.

SUCTION

This method requires the equipment associated with a

dental chair. The patient is put into a similar position as

Salivary stimuli

before to allow saliva to collect into the anterior floor of Over the years, a variety of stimulants to salivary secretion

have been used.

These are either systemic or local

sialagogues. The main systemic sialagogue that has been

the mouth. A saliva ejector is placed behind the lower

incisor teeth and the secretion is trapped in a bottle

intervening between the ejector and the drainage system.

used is the parasympathomimetic, pilocarpine. Although

effective, pilocarpine does not precisely reproduce the

balance of

sympathetic and parasympathetic activity

ABSORBENT DEVICES

responsible for normal secretion. One consequence is

Preweighed cotton wool rolls are placed under the tongue

ents, particularly sodium and potassium. Pilocarpine can also cause systemic cholinergic effects such as colic,

This method allows quantitation only. More recently, ® proprietary devices such as OraSure have been intro­

troublesome.

analysis. Its use has been reported by Thieme et

that it can alter the concentrations of normal constitu­

diarrhoea, bradycardia and sweating,

which may be

Local sialagogues are convenient and generally more

satisfactory. A good example is 5 percent citric acid

for a two-minute period then taken out and reweighed.

duced to simplify saliva collection and preserve it for

a1.9 who

collected saliva for determination of measles, mumps and

rubella immunization status. OraSure is a cotton-fibre

solution, which is a potent sialagogue and does not

pad which can absorb 1 mL of oral fluid. In manufacture,

Five drops of this solution can be dropped from a pipette

mounted on a plastic handle. In clinical practice, it is

interfere with the composition of the final specimen.

or disposable syringe onto the dorsum of the tongue.

This may be used as a preliminary measure before collecting unstimulated saliva.

The purpose of

this

it is saturated with hypertonic saline solution, dried and

placed between the gingiva and buccal mucosa for two

minutes, then withdrawn and placed in an antiseptic

transport medium. In the laboratory, the saliva specimen

manoeuvre is to flush out stagnant secretions that

is recovered by centrifugation.

tongue and saliva is collected for 15 minutes to eliminate

system for gathering oral fluid, it must be appreciated that

confuse the analysis. Thus, citric acid is applied to the

rest transients.

Stimulated or unstimulated saliva can then be collected

While there appear to be advantages to the OraSure

by virtue of the filtering effect of the cotton-fibre pad, it does not collect whole saliva. Cordeiro et

al.10 found that

for periods of 15-30 minutes as necessary. The results

the levels of IgG in OraSure oral fluid were three to four

necessarily comparable because individuals with a vigor­

two to four times higher. They suggested that the cotton­

obtained

by

different

sialometric

methods

are

not

ous secretory response to one stimulus do not necessarily have a similar response to another.

times higher than those of saliva and amylase levels were

fibre pad might promote passage of crevicular gingival exudate and stimulate secretion of amylase as a result of the pad's buffer solution. However, North et

Collection of mixed whole saliva There is a variety of methods for collecting saliva from individual glands or collecting mixed whole saliva. Many

al.ll found

that the OraSure system provided a reliable index of tobacco usage by means of continuous salivary assay.

Collection of parotid gland saliva

of these methods require specially made equipment and 8 are mainly suitable for research purposes. Navesh has

Pure parotid saliva can be collected by cannulating the

follows:

cup.

reviewed the methods for collecting saliva, which are as

parotid duct with a polythene catheter or using a suction Catheterization of the duct allows collection of

uncontaminated saliva, but is uncomfortable for the

•

spitting;

•

drainage;

the most widely used devices are the Carlson-Crittenden

cotton wool rolls.

parotid papilla. Each cup consists of a central chamber

• •

suction;

patient and the tube has to be held in place. For suction,

or Lashley cups, the centre of which are placed over the

Chapter 145 Physiology of the salivary glands I

into which the saliva flows and an outer chamber to which suction is applied to cause the cup to adhere to the buccal mucosa

(Figure 145.1).

all salivary glands, including the minor

glands, make a greater contribution to the amount of saliva

produced under normal conditions. Stimulated parotid flow is unsatisfactory because of difficulties that some

patients have with the collection devices and because the

Collection of submandibular gland saliva

variety of stimulants used have caused confusion as to what

Particularly in the past, submandibular gland saliva was collected by catheterizing the submandibular duct after dilatation.

the total activity of

1863

This technique is neither very simple nor

comfortable for the patient. Segregator appliances must be purpose-made to fit the patient's mouth and function in the same way as the Carlson-Crittenden cup. The device, which fits the anterior floor of the mouth, has a central collecting chamber, isolated by a surrounding ridge from the outer suction chambers.

is meant by abnormal flow rates. Speight et

al.13 found that

a whole unstimulated salivary flow rate of 0.1 mLlmin or

less was 81 percent predictive of Sjogren's syndrome if other causes of xerostomia could be excluded. Saliva was collected

by encouraging the patients to spit gently or drool into a beaker for 15 minutes. Although controversy persists about

methods of saliva collection and whether or not sialagogues should be used, there is a growing body of opinion that

collection of unstimulated mixed saliva best reflects the normal resting state. The European Community Study Group on Diagnostic Criteria for Sjogren's Syndrome14 have

Advantages and disadvantages of the different methods The choice of method depends on the type of investiga­ tion being carried out. If, for example, the aim is to investigate viral shedding from the parotid gland then catheterization of the duct has to be carried out or a Carlson-Crittenden cup used. Mandel12 listed the variations in concentrations of sodium, potassium, calcium, magnesium, bicarbonate and phosphate in the secretions of individual glands and whole saliva. However, overall, his findings suggest that collection of whole saliva is satisfactory for such chemical measurements. For straightforward measurement of salivary flow rate for confirmation of the diagnosis of Sjogren's syndrome, for example, the simple spitting method for unstimulated saliva

also concluded that collection of whole unstimulated saliva is the best method for confirming the degree of xerostomia.

Radioisotope salivary function tests Various isotopes have been used to visualize a number of organs and their ability to concentrate a particular isotope can be used to indicate function. 99mTc pertechnicate has been used for the study of salivary gland function and, with scintigraphy, an objective measure of its uptake, concentration and excretion can be made

(Figure 145.2).

This method of investigation has the advantage that both the parotids and submandibular glands can be studied at the same time. Although available for some time, this method of investigation has met with little clinical enthusiasm.

over a period of 10 or 15 minutes, requires minimal equip­

ment and is satisfactory. In the past it was thought that since the parotid glands were predominantly affected by this disease, it was necessary to collect parotid saliva. However,

SIALOCHEMISTRY Normal The composition of saliva changes in disease states, particularly those causing xerostomia, and these changes have been reviewed by Mandel.l It must be emphasized that most studies on the composition of saliva have been based on relatively few subjects and may therefore be biased. Differences in laboratory methods may also result in discrepancies in the results which are not in fact real. Other complications are the presence of enzymes which are probably of bacterial origin. The composition of saliva in health provides a baseline for variations resulting from disease or drug administration. Unfortunately, even in healthy people, many variables, such

Figure 145.1

A Lashly collecting cup. The cup consists of

a

central chamber into which the saliva flows and an outer

as those listed in

Ta b le s 145.2 and 145.3, can affect the

composition of saliva. The major constituents of saliva are

Table 145.4,

chamber to which suction is applied so that it adheres to the

shown in

buccal mucosa.

made, these figures should be accepted with caution.

but in view of the comments already

1864

I PART' 5 THE UPPER DIGESTIVE TRACT

=

80 Lemon given here

J,

60

u c 0 0 C) (/)

I J

(j)

C

\ ......

.---'

\ ...

I

Q. (/)

Left parotid .\

40

::J

8 Right submandibular

20

o�------�--� 3.75

(b) Figure'45.2

7.5

11.3

15.0

Minutes

(a) 99rrrrc pertechnicate s tudy undertaken in a pat ient with early Sjogren's syndrome. There is poor tracer accumulat ion

in the right parot i d gland (arrowed) when compared to the left parotid (b) Following the administration of lemon drops. there is a brisk .

discharge of ac tivity from the left parotid gland and the right submandibular gland, but less response from the left submandibular gland.

Diseased states

of the most valuable diagnostic tests. Neither involves either exposure to x-rays or scintigraphy. A low resting

Changes in the concentration of inorganic ions have been 12 documented in the conditions listed in Table 145.5. Changes in the concentration of the organic components

have been found in the conditions listed in Three

diseases that

have

been

studied

Table in

145.6.

terms

of

sialochemistry in particular detail are Sjogren's syndrome, cystic fibrosis and coeliac disease.

SJOGREN'S SYNDROME

salivary flow rate and stimulated flow rate are typical of Sjogren's syndrome, cut off values of 0. 1 mLimin for resting whole

saliva and 0.5 mLimin for stimu­

lated saliva have been considered diagnostic of gland hypofunction.

IS

Mandell has noted that salivary function changes, in addition to a lowered secretion rate include: •

raised sodium and chloride but lowered phosphate;

•

raised titres of immunoglobulin (Ig)A, IgG,

firming the diagnosis of Sjogren's syndrome because of

•

raised

the great variability in the abnormalities that may be

•

raised kallikrein concentrations;

detected. The problem is well illustrated by the neces­

•

a 20-fold elevation in the concentration of

•

increased concentrations of inflammatory mediators,

lactoferrin and albumin;

Considerable difficulties are sometimes found in con­

group concluded in its report that unstimulated whole saliva flow rate and minor salivary gland biopsy are two

microglobulin;

phospholipids;

sity to institute the European Community Study Group on Diagnostic Criteria for Sjogren's Syndrome.14 The

P2

e.g. eic6sanoids, PGE2, thromboxane B2 and interleukin.

Chapter 145 Physiology of the salivary glands I

Table 145.3

1865

Variables affecting the composition of saliva.

Flow rate Saliva specifically collected from the major glands differs in composition from whole saliva which includes that

Source

secreted by the minor glands Diurnal variation Duration and type of stimulus Rest transients

The concentration of a molecule. such as potassium. may vary according to whether the saliva sample is taken

Age and gender differences

Findings on differences in salivary flow and hence salivary composition, according to the age or gender of the

shortly after stimulation or after the Aow has been allowed to continue for several minutes subjects have been conflicting. Findings that, for example, salivary flow rates are lower in older women than men could conceivably be biased by the presence of unsuspected Sjogren's disease among women Plasma levels

The concentration of many molecules in saliva is related to and varies with their plasma levels

Diet

Findings suggesting that a predominantly carbohydrate diet, for example, leads to higher concentrations of salivary amylase or that high protein diets lead to higher concentrations of salivary amylase have not been widely confirmed or are conflicting

Drugs

Any drug that affects salivary flow rates can affect the concentration of salivary constituents that are Aow

Hormonal effects

Mineralocorticosteroids affect both plasma and salivary concentrations of molecules such as sodium and

dependent. Important drugs that affect salivary now rates are shown in Table 145.2 bicarbonate. It has also been suggested that the concentration of some salivary constituents such as calcium and sodium may fall and potassium levels rise at the time of ovulation

Table 145.4

Composition o � mixed saliva.

.Subs'��nc� '.

� � -: -�-.-'

.

.

�

:"-t::

'_Unstimulat�d ; �

. �-.

Stimulated

r

. � � . ....

.of:..

Mean ..±.s;d.:

Protein (giL)

Range 1.4-6.4

'. Mean.±. 2.8

1.8-4.2

Lysozyme (mg/Ll

108.9 ± 29.1

3.7-625

Carbonic anhydrase (KILl

2100

IgA (mg/L)

194.0

IgG (mg/Ll

14.4

IgM (mg/Ll

2.1

Amylase (giLl

0.38 ±0.08

Histamine (mg/Ll

0.15

Glucose (mmol/LJ

0.55±0.048

Urea (mmol/Ll

3.22 ± 2.5

2.33-12.5

Creatinine (mg/Lj

0.09

0.04-0.18

Cholesterol (mg/L)

0.2

0.07-1.3

Sodium (mmol/Ll

6.2 ± 0.46

26.4± 11.8

Potassium (mmol/Ll

21.6± 1.2

19.7 ± 3.9

0.11-0.18 0.056

0.02-0.17

2.17

0.1-4.8

Calcium (mmoI/L)

1.56± 0.06.

1.48 ± 0.04

Magnesium (mmoI/L)

0.21 ±0.01

0.15±0.04

Phosphate (mmol/L)

6.2

Chloride (mmol/L)

17.4± 1.4

Iodide (IlmoI/L)

0.8

0-3

Fluoride (Ilmol/Ll

15±0.68

0.5-3

29.0 ±8.8 1-3 0.56± 0.25

0.25-1.2

Parotid gland lysozyme was also found to be raised in

CYSTIC FIBROSIS

co'uld indicate whether labial gland biopsy or other tests

clinical effects on salivary gland function are

primary but not in secondary Sj ogren's syndrome. Such changes could be useful for screening.16, 17 These indices were

indicated but) perhaps more importantly) be used

for monitoring disease progress.

is affected the minimal, b ut there are signifi c an t changes in salivary composition. In particular, there are dramatic elevations in sa l ivar y Although all exocrine gland function

,

1866

•

PART 15 THE UPPER DIGESTIVE TRACT

Table 145.5

Changes in the concentration of ��organic ions, ,

Changes in the concentration of inorganic ions.

Condition

Raised Na +, K + , Ca++ and p+ levels Raised Na + , Ca++ , Mg + + and CI- levels Raised Na + , CI- and P + in parotid gland saliva Raised Na + , Ca ++ and P + levels. Mandel12 suggested that the combined Ca++ and P + concentrations formed a useful diagnostic index Depressed Na + but raised K + levels. Mandel12 suggested that the product Na + /K + could form a useful diagnostic index Depressed Na + levels Raised K + levels Raised Ca++ levels Raised Ca++ levels Depressed HC03 - levels Possibly raised Na + levels Raised Na + and K + levels, Mandel12 suggested that the product, Na + x K + , could form a useful diagnostic index

Sialadenitis Radiation damage Sjogren's syndrome Cystic fibrosis Aldosteronism Hypertension Alcoholic cirrhosis Hyperparathyroidism Diabetes mellitus Chro nie pancreatitis Psychiatric illness Digitalis intoxication

Table 145.6

Conditions affecting the composition of saliva. -

Conditi n o

�

�,,; ;:.

Sjogren's syndrome Cystic fibrosis Cirrhosis Hyperparathyroidism Diabetes mellitus Sarcoidosis

· >�,o.:"

��ct on composition of

.. �'

S��-li��!:�:"

-'"',

; .�;,

Raised total protein and �2 microglobulin levels in parotid gland saliva Raised total proteins, amylase, lysozyme in submandibular gland saliva and glycoproteins in parotid gland saliva Raised total protein and amylase in parotid gland saliva Raised total protein Raised total protein, IgA, IgG and IgM and raised glucose levels Depressed amylase and lysozyme levels

protein and cakium concentrations. The complexmg of

Antibacterial substances in saliva

these substances leads to obvious turbidity of t he saliva. This is such

as

to obstruct the excretory ducts of the

minor salivary glands and to cause the build up of calculus around teeth. Their greatly depressed secretion rate can be measured in the accessible labial glands with a

capillary tube.

Unfortunately, saliva cannot be used to detect or diagnose cystic fibrosis in affected patients or carriers of the disease. Screening for carriers can only be detected by

DNA analysis and even this is hampered by the number of mutations in the cystic fibrosis gene.

The

function

of

substances

such

as

lysozyme

and

lactoferrin secreted by the salivary epithelium and which have antibacterial act iv it y in vitro is unclear. Molecules such

as

these can be shown to have antibacterial activity

in vitro. Other factors which might affect bacterial activit y in

t he oral cavity are the pH and buffering capacity of

saliva and its content of immunoglobulins. Despite the presence of these substances, t he oral cavity supports a flourishing population of microbes including a

great variety of pathogens. Dental caries is usually also active on a high sugar diet and periodontal disease is likely to progress unless a high standard of oral hygiene is

COELIAC DISEASE

m ain t ai ne d. Despite great efforts to show protection

This condition'

is characterized by the malabsorption of gluten in the small intestine. Antibodies (IgA-AGA) to

against these diseases by saliva, the findings have been

gliadin) a major component of gluten, are produced and

have harmful effects by promoting adhesion of bacteria to

detectable in both serum and saliva. Measurement of this

the

antibody in saliva has been used as a screening test for

substances are difficult to substantiate in

coeliac disease but lacks the sensitivity of its serological counterpart.

18

Salivary tests are probably better employed

to monitor compliance with a gluten-free diet.19,

20

unconvincing. Moreover) salivary mucins for example, may teeth.

That

aside)

putative

effects

of

protective

h umans because

of the difficulty of performing the critical experiments. It is clear that the oral cavity has a high level of local

immunity.

The

large

bony

wounds

resulting

from

Chapter 145 Ph ysiology of the salivary g l ands I

extraction of teeth normally heal rapidly despite con­

tamination

by

the

vast

and

pathogenic

flora

that

1867

3 4 discussed by Ferguson.2 Read2 has discussed the current status of salivary oestrogen and androgen measurement.

proliferates in periodontal pockets, but this fortunate

An

outcome is likely to result from local tissue immunity

hormone measurements is cast by the problems of salivary

Another factor which may affect the nature of the

salivary concentration of this hormone is only about 0.1

rather than from salivary components.

bacterial flora of the mouth is bacterial competition for

nutrients in their individual, ecological niches. However, the bacterial flora of the mouth is undoubtedly affected by

informative

light

on

the

limitations

dehydroepiandrosterone sulphate assay

of

salivary

(DHA-S).

The

percent of that in plasma and is a poor predictor of plasma levels in an individual or a particular plasma sample. The

inconsistencies arise from the fact that the concentration

low salivary flow rates which, in particular, promote

in parotid fluid is particularly low. Most salivary DHA-S

species. Nevertheless, xerostomia does not appear to

The fall in DHA-S concentrations with high salivary flow

The only aspect of saliva production that can be

concentrations of DHA-S in saliva depend largely on the

cariogenic

activity

and the

proliferation

of

Candida

promote periodontal disease.

probably therefore comes from blood in gingival exudate.

rates helps to confirm this possibility. If this is the case,

reliably related to protection against infection is that of

degree of contamination and, as has been shown, are

Candida albicans

and the method of saliva collection. Read concluded that

the overall flow rate. In conditions of xerostomia, the oral bacterial flora changes and in particular

and staphylococci are likely to flourish. Dental caries

activity and mucosal infections are thus promoted. From

the clinical viewpoint, therefore, the main contribution of

affected by the oral conditions in the person being tested

salivary assays for testosterone in men, androstenedione

and oestriol in particular, were valuable, but considered

that the value of salivary testosterone and oestradiol in

saliva to defence against infection appears to be the largely

women remained unproven.

the gastric acid.

salivary progesterone assay and their interpretation, and

mechanical effect of its flow washing down microbes into

reviewed the utility of these assays for clinical purposes,

particularly for the diagnosis and treatment of infertility.

Salivary progesterone levels are valid indicators of plasma

SALIVARY ASSAYS IN DIAGNOSIS Saliva is undoubtedly a valid medium for many diagnostic assays. Collection of saliva is noninvasive, painless and

obviates the risk of needle-stick injuries. Nevertheless,

most

Ellison25 has considered certain technical aspects of

clinicians are

unused

to

practised in collecting blood,

collecting

saliva

but

which can usually be

achieved more quickly. Moreover, most laboratories use

equipment for handling blood and are unused to dealing with saliva with its mucins and other constituents or

levels

and

Ellison

concluded

that

their

assay

was

particularly valuable because of the ease of obtaining

serial samples from the same individual. On a shorter

timescale, salivary monitoring could provide samples at short intervals for characterization of pulsatile progester­

one patterns without the inconvenience and expense of

hospitalization. The ability to adapt salivary progesterone

monitoring under field conditions has also made possible

basic research on a wide scale into human reproductive

contaminants which may affect the assays. With regard to

biology. Ellison's concerns were the need for standardized

mention the possibility of using saliva.

analysis, recognized reference ranges and statistics on

drug

assays,

few

current

pharmacology

texts

even

laboratory procedures, methods of data reduction and

diagnostic efficiency. Furthermore, the low absolute levels

of steroids in saliva placed a premium on an unusually

Malignancy screening

high level, of quality control in the laboratory.

In recent years peptides and proteins associated with

social behaviour. Assay of testosterone in saliva provides a

Testosterone is a hormone that may, in addition, affect

malignancy have been detected in saliva. p53, a tumour

valuable method for field studies. Dabbs26 has summar­

defensin-1 can be detected in the saliva of patients with oral

testosterone levels both between and within individuals.

suppressor protein, and increased levels of the peptide squamous cell carcinoma.21 Similarly, raised levels of the tumour marker c-erbB-2 and cancer antigen 15-3 (CA153) found in the saliva of women with breast carcinoma

offer the potential of relatively simple screening tests.22

ized the preliminary findings on differences in salivary Studies on individual differences are being carried out in

relation to violent and antisocial behaviour, occupational

achievement and everyday behaviour. Studies on chan­

ging testosterone levels have been undertaken on winning sports contests,

Hormone monitoring Lipid-soluble, unconjugated steroids pass readily into

saliva and their concentrations are proportional to the concentrations of free, unbound steroids in plasma as

winning

nonathletic

events,

winning

political contests, 'winning' games of sex and vicarious

winning (sports spectators). Broadly speaking, it appeared

that salivary testosterone levels fell in losers and were unchanged or rose in winners according to the impor­

tance of the victory. While animal studies confirmed

elevations of testosterone with real or anticipated sexual

1 868

• PART 1 5 TH E U PPER D I G ESTIVE TRACT

activity, there were considerable difficulties in obtaining

samples at appropriate times in humans. However, it

appeared that testosterone levels were higher on evenings when there was sexual activity, particularly in women, and low in its absence. Studies o n salivary testosterone levels und er conditions of abuse, depression and suicide are also in pro gress.

D rug m o n ito ri n g Jusko and Milsap27 have discussed the p harmacokinetics of drug distribution in saliva. They showed that the primary properties of a drug which determined its entry into saliva were molecular size, lipid so lubility, pKa and protein binding. However, salivary flow rates, the time o f sampling and disease states which altered saliva composi­ tion could also affect the results. Drugs that are not ionizable or not ionized within the pH range o f saliva are most suited to salivary mo nitoring. Haeckef8 has summarized some of the reasons fo r the failure to use saliva to any great extent for therapeutic drug monitoring as follows. •

Ta ble 1 45,7

D rugs that ca n be mon itored by sal ivary a n a lysi s, Recreationsl d rugs

Therapeutic drugs

Am phe tami nes

Antipyrine Caffei ne

B a r bitu rates

Ca rba mazepine

B e nzod iaze p i n es

Cisplatin

Coca ine

Cyclospori n e

Ethanol

Diazepam

Marij u a n a

Digoxon

N ico ti ne

Ethosux i m i d e

Opioids

Irinoteca n

Phencycl i d i n e

Lithium M ethadone Oxyprenalol Paraceta mol Phe nytoin Prim idone Proca i n a m i d e Quinine

Su I p h a n i l a m i d e Th eophyl l i n e

To l b utam ide

Blo o d h as to be sampled for other electrolytes anyway.

•

There are technical difficulties with sampling saliva.

•

There are existing di fficulties with the interpretation of salivary drug concentrations.

The remaining

applications for saliva sampling were

therefore:

VI RAL H E PATITIS

In view o f the hazard to surgeons, a simple method of detecting car riers o f hep atitis viruses has o bvious benefits.

•

when sampling at home is required;

•

special cases where the sample is taken only fo r the monitoring o f a par ticular drug;

•

M i cro b i a l a nti gens and a ntibod i es

circumstances where the sample volume is critical, as for example in newborns.

The

presence

of h epatitis

B

antigen

in

saliva

was

dem onstrated by Bro dersen et al. 3 1 Shikata et al.32 were

able to detect the hepatitis B surface antigen (HBsAg) and the core antigen in hepatocytes, but could not detect it in parotid gland parenchymal cells. However, in the patient

Nevertheless, if a constant saliva/plasma ratio can be established, use o f saliva for therapeutic drug monitoring becomes a clinically useful possibility and Siegel29 has pointe d out that the saliva/plasm a ratios

for at least

1 70

drugs have been established experimentally. Drugs which can be m o nitored in saliva include digitalis, phenytoin, primidone, ethosuximide, carbamazepine, theophylline,

with the highest serum titre of H BsAg, immu noreactivity was detected in the vascular wall and luminal fluid of the p arotid gland. P iacentini et al. 33 have reported high

sensitivity and specificity in the diagnosis of hepatlt,is

A, B

and C using the OraSure collection system, and that the samples had titres of antibodies to viral hepatitis that were similar to those of the serum.

caffeine, lithium, methadone, cyclosporine, marij uana, cocaine and alcohol A

related

(Table 145.7 ) .

u s e for salivary drug monitoring i s fo r

H IV I N FECTI O N

detection of drugs of abuse as described by Cone,30 who

M alamud34 has made a strong plea for u s e o f saliva a s a

reviewed the findings for alco hol , amphetamines, b a rbi­

diagnostic fluid for the detection of antibodies to HIV,

turates, benzo diazepines, caffeine, cocaine, inhalants s uch

among other purposes. For the detection of carriage of

as general anaesthetic agents, as well as solvents, LSD,

antib o dies t o

marijuana, op io ids, phencyclidine and tob acco . The use

method and presents enorm o us advantages. It removes

of saliva for the detection of illicit drug use is particularly

the

attractive. The commo n recreational drugs appear in th e saliva at the sam e time as serum.

From

a forensic

standpoint it is not the concentration of these drugs that matters, it is their presence that is important.

risk

HIY, saliva testing is a simple noninvasive

of needle-stick

injuries

and

the

emotional

connotations o f blo o d sampling in this alarming disease. Salivary sampling has special advantages when investi ­ gating

�h i1dren

Archibald

et

because of the great ease of collection. aI.35

have

described

the

p ractical

Chapter

applica tions fo r sal iva tes t i ng fo r p er ina tal HIV dia g

­

n osis. Only a m i no rity of infants

born to

HIV- p o sitive

mothers d evelop

but

the neona tal

H I V in fection,

in

R E FERE N CES *

1.

perio d usually carry pass ive ly tra ns ferred a n t ibo d ies to 2.

.

the value of an IgA-specific Wes tern blot assay, A rch ibald et al. 36 collected blo o d and saliva fro m 95 i nfan t s and

4.

24-35. 5.

percen t for infants over 1 2 mo n t hs T h e earliest a g e fo r .

detection of ser u m

IgA

antibod ies to HIV is

b elieved to

be two m onths. Sal ivary I gA an t ibod ies were det ect ed by

al. 3 6 in an infected infant a t six months, but had been nega t ive a t fo ur months . Relia b l e sa li va ry

Archibald et

6.

detection of HIV infection in the i m me d i a te neonatal perio d awai ts more sens itive metho ds, However, i f t hese can be fo u n d

,

saliva s amp l ing fo r antibod ies to HIV,

does not re q uire skille d personnel, avoids the need fo r re pea ted vene­ metho d .

punctures co u nt ries

and

Saliva

is

collectio n

ideal

fo r

studies

in

develop i ng

Ship JA, Patton LL. Tylenda CA. An assessment of salivary gland function in healthy premenopausal and postmenopausal females. Journal of Gerontology. 1 991 ; 4 6 : M11-3. Busfield BJ, Wechsler H. Studies of salivation in depression. Archives of General Psych iatry. 1 961 ; 4 : 1 0-5.

7.

part icularly for infants and chil dren, is a pote n t ia l ly valuable

Baum J B . Principles of saliva secretion. Annals of the Ne w 694: 1 7-23. Turner RJ. Mechanisms of secretion by saliva ry glands. Annals of the Ne w York Academy of Sciences. 1993; 694 :

for de tecti ng antibo dies to HIV gp 1 60 a nt igens was 50 1 2 mon ths old and 9 7 . 3

Humphrey SP, Williamson Rl A review of saliva : normal composition, flow a nd function. Journal of Pros thetic

York Academy of Sciences. 1993;

buccal sulcus. The total se nsit ivi ty o f the sal ivary assay percent of infants less than

Journal of Oral

Den tistry. 2001 ; 8 5 : 162 -9. 3.

children born to HIV-infected women. Sal iva sa mples

fro m infa nts were co l lected by ge nt le aspi ra t io n from t h e

Mandel ID. The diagnostiC uses of saliva.

Pathology and Medicine. 1990; 1 9 : 119-25.

,

HN from t he moth er. However, it is believed th a t IgA and fgM ant ibo d ies do no t cross t h e pla cent a To assess

1 45 Physiology o f t h e saliva ry glands . 1869

Busfield BJ, Wechsler H, Ba rnum WJ. Stu dies of salivation in de pression. Archives of General Psychiatry. 1961 ; 5 : 7 6-81.

8.

Navesh M. Methods for collecting saliva.

Annals of the

Ne w York Academy of Sciences. 1 993 ; 694 : 7 2-7. 9.

.

Thieme TR, Piacentini 5, Davidson S, Steingart K. Determi nation of measles, mumps and rubella immunization status using oral flu i d samples. Journal

of

th e A merican Medical Association. 1994 ; 2 7 2 : 219-2"

MONITORING OF I M M U N IZATION STATUS Thieme et

mumps

1 0.

al.9 h ave used sa liva ry sa mp l i ng fo r measles

and

,

rubella immunization st atus . SaJ iva was

collected using the OraSure system. Antibo dies in oral fluid

specimens

correla ted

with

and

sp ecificity to

the following

an d

1 00

respective ly;

percent,

levels degree: mum ps,

Academy of Sciences. 1993; 694 : 330-1. 11.

o f sensi t ivi ty measles,

97

94

94

an d

percent, resp ectively; and rubella , 9 6 a n d 98 percent,

13.

KEY PO I NTS

•

Ii

•

I

499-502.

Drug thera p y is t he most common cause o f decreased sal i vary tlow. Dry mo u t h is a com mon man i festatio n of a u ro i m m u n e d isease. l·B V infec t ron, sa rcoidos i s i ron overload, .,--17

19 ....J.-=o.,..,..,...,-N--,~---20 ~~~~#m~-----21

'--""'=+------22

10

28

12 13

29

14

30

~m\\\1~----23

11

24 ----1--------25

15

16 12-----------~~~~~~~~---------26

17

Figure 149.6

The pharynx from behind. 1, Pharyngotympanic

13-------------~

tube; 2, tensor palati; 3, levator palati; 4, stylohyoid ligament;

14 -----------tH-+-H+ttI11'r>-r.,.,....,.,.,.,....,.,crr(

5, stylopharyngeus muscle; 6, styloglossus muscle; 7, superior

15 _ _ _ _ _ _ _ _---JH+1I1111--....u.!.J.llJ.~

constrictor muscle; 8, medial pterygoid muscle; 8a, stylohyoid muscle; 9, posterior belly of the digastric; 10, greater horn of hyoid bone; 11, middle constrictor; 11 a, palatopharyngeus muscle; 12, superior horn of thyroid muscle; 13, inferior constrictor muscle - thyropharyngeus; 14, Killian's dehiscence; 15, inferior constrictor muscle - cricopharyngeus; 16, circular oesophageal muscle coat; 17, longitudinal oesophageal muscle coat; 18, facial nerve; 19, hypoglossal nerve; 20, pharyngobasilar fascia; 21, accessory nerve; 22, inferior ganglion of vagus nerve; 23, external carotid artery; 24, superior cervical sympathetic ganglion; 25, ascending pharyngeal artery; 26, internal jugular vein; 27, middle cervical sympathetic ganglion; 28, inferior thyroid artery; 29, inferior cervical ganglion; 30, right recurrent laryngeal nerve.

Figure 149.7

Lateral wall of the pharynx to show the

constrictor muscles and associated structures. 1, Tensor palati; 2, spine of sphenoid bone; 3, levator palati; 4, pterygoidhamulus; 5, superior constrictor; 6, stylopharyngeus; 7, glossopharyngeal nerve; 8, middle constrictor; 9, greater horn of hyoid bone; 10, lateral thyrohyoid ligament; 11, thyropharyngeal part of inferior constrictor; 12, Killian's dehiscence; 13, cricopharyngeus part of inferior constrictor; 14, right recurrent laryngeal nerve; 15, oesophagus; 16, buccinator; 17, pterygomandibular raphe; 18, styloglossus; 19, geniohyoid; 20, stylohyoid ligament; 21, lesser horn of hyoid bone; 22, thyrohyoid membrane; 23, internal laryngeal nerve; 24, superior laryngeal vessels; 25, oblique line on thyroid cartilage; 26, fascial bridge overcricothyroid muscle.

artery, ascending palatine branch of the facial artery, stylohyoid ligament, styloglossus and stylopharyngeus muscles and, more laterally, the medial pterygoid muscle inside the angle of the mandible (Figure 149.8). On the outer surface of the middle constrictor lie the lingual artery, hyoglossus muscle, hypoglossal nerve and the tendon of the posterior belly of the digastric. On the outer surface of the inferior constrictor lie the external laryngeal nerve, thyroid gland, sternothyroid, sternohyoid and omohyoid muscles. STRUCTURES PIERCING THE PHARYNX

The cartilaginous portion of the Eustachian tube and the tensor and levator palati pass through the pharyngobasilar

fascia to reach the lateral wall of the pharynx and palate, respectively. The palatine branch of the ascending pharyngeal artery curls over the upper edge of the superior constrictor. The stylopharyngeus enters the pharynx between the middle and superior constrictors, it then blends with the fibres of the palatopharyngeus. The stylopharyngeus is accompanied by the glossopharyngeal nerve which supplies it before passing forwards to the tongue. The internal laryngeal nerve and superior laryngeal nerve pierce the thyrohyoid membrane between the middle and inferior constrictors and come to lie submucosally on the lateral wall of the piriform fossa. The recurrent laryngeal nerve and inferior laryngeal artery

1950 I PART

15 THE UPPER DIGESTIVE TRACT

Figure 149.8 2--------/

Transverse section at the level of C2

showing the relations of the oropharynx including the parapharyngeal and retropharyngeal space. 1, Tongue; 2, cavity of oropharynx; 3, palatoglossus muscle; 4,

3----4 _ _ _ __

palatine tonsil; 5, palatopharyngeus muscle; 6, retropharyngeal space; 7, cervical sympathetic

5--------'

ganglion; 8, internal carotid artery; 9, vagus nerve; 10, hypog lossal nerve; 11, g lossopharyngea I nerve; 12, internal jugular vein; 13, accessory nerve; 14, external 1--+---15

carotid artery; 15, sternocleidomastoid muscle; 16, posterior belly of digrastic; 17, parotid gland; 18, stylohyoid muscle; 19, stylopharyngeus muscle; 20, styloglossus muscle; 21, parapharyngeal space; 22, medial pterygoid muscle; 23, masseter muscle.

pass between the cricopharyngeal part of the inferior constrictor and the oesophagus behind the articulation of the inferior horn of the thyroid cartilage with the cricoid cartilage. Understanding the position of the internal laryngeal nerve and superior laryngeal vessels is crucial to the safe surgical dissection of the upper pole of the thyroid gland. The close anatomical relationship of the recurrent laryngeal nerves, the carotid artery and the inferior thyroid artery, is described by an inverted rightangled triangle with the carotid artery forming the vertical side laterally, the inferior thyroid artery forming the horizontal base at the top passing from medial to lateral, and the recurrent laryngeal nerve forming the hypotenuse (or longest side) travelling from medial to lateral at a relatively shallow angle on the left and a more acute angle on the right. The nerves may sometimes pass superficially or deep to the inferior thyroid artery but the triangular relationship remains constant and is the only safe way of identifying the nerve.

POTENTIAL SPACES AROUND THE LARYNX

The retropharyngeal space lies between the prevertebral fascia and the buccopharyngeal fascia (Figure 149.8). The space is closed above by the base of the skull and on each side by the carotid sheath. Inferiorly, it is continuous with the superior mediastinum. The parapharyngeal space is lateral to the pharynx on each side and can be visualized as an inverted cone with its base under the temporal bone and its apex in the neck at the hyoid bone (Figure 149.8). Laterally, it is bounded by the fascia overlying the pterygoid muscles, the medial aspect of the deep lobe of the parotid gland and the ascending ramus of the mandible. Medially, the space abuts the fascia overlying the pharyngeal constrictors and the tensor and levator palatini. Anteriorly, it is bounded by the pterygomandibular raphe and the posterior wall is

formed by the posterior part of the carotid sheath and prevertebral fascia. The retropharyngeal and parapharyngeal space are filled with loose areolar tissue, fat and a number of lymph nodes. They communicate with each other and the submandibular spaces, which allows the spread of infection and tumour along fascial planes with little resistance.

Nerve supply of the pharynx The pharyngeal plexus is formed by the pharyngeal branches of the glossopharyngeal and vagus nerves with sympathetic fibres from the superior cervical ganglion. Many of the vagal fibres come from the cranial root of the accessory, which joins the vagus at its superior ganglion. The pharyngeal branches of the vagus supply all the muscles of the pharynx via the pharyngeal plexus, except the stylopharyngeus which is supplied by the glossopharyngeal nerve. The cricopharyngeus has an additional supply from the external laryngeal nerve and receives parasympathetic vagal fibres from the recurrent laryngeal nerve (relaxation) and postganglionic sympathetic fibres from the superior cervical ganglion (contraction). Sensation to the pharynx is provided by the pharyngeal plexus with the glossopharyngeal nerve supplying sensation to the upper part of the pharynx including the surface of the tonsil, which is also supplied by the lesser palatine branch of the maxillary nerve and posterior third of the tongue. The tongue in front of the valleculae and the valleculae themselves are supplied by the internal laryngeal nerve, a branch of the superior laryngeal nerve of the vagus. Sympathetic fibres reach the pharynx on the blood vessels supplying it and are derived from the superior cervical ganglion. Parasympathetic secretomotor supply

Chapter 149 Anatomy of the pharynx and oesophagus

to the minor salivary glands of the pharynx comes by way of the nasal palatine and pharyngeal branches of the pterygopalatine ganglion. These are derived from the nervus intermedius and pass through the facial nerve to the greater petrosal nerve and pterygopalatine ganglion.

Pharyngeal vasculature The ascending pharyngeal artery arises from the medial aspect of the external carotid artery just above its origin. It passes upwards behind the carotid sheath against the pharyngeal walL Branches go to the pharynx and the tonsiL Its palatine branch passes over the upper free edge of the superior constrictor to supply the inner aspect of the pharynx and soft palate. The pharynx receives an extra supply from the ascending palatine and tonsillar branches of the facial artery and the greater palatine and pterygoid branches of the maxillary artery. The veins of the pharynx are arranged in an internal submucous and external pharyngeal plexus with numerous communicating branches between the two plexuses and veins on the dorsum of the tongue, the superior laryngeal veins and the oesophageal veins. The pharyngeal plexus drains to the internal jugular vein and anterior facial veins, it also communicates with the pterygoid plexus. Lymphatics from the upper part of the pharynx drain first to the retropharyngeallymph nodes and then join the oropharynx in draining to the upper deep cervical nodes. The hypopharynx drains to the inferior deep cervical group and paratracheal nodes.

I 1951

which is separated from the wall of the oropharynx by loose areolar tissue. Laterally, the floor of the tonsillar fossa is formed by the pharyngobasilar fascia overlying in its upper part the superior constrictor and below the styloglossus muscle passing forward into the tongue. The glossopharyngeal nerve and stylohyoid ligament pass obliquely downwards and forwards beneath the lower edge of the superior constrictor in the lower part of the tonsillar fossa.

ANATOMY OF THE OESOPHAGUS General description The oesophagus extends from the lower border of the cricoid cartilage at the level of C6 to the cardiac orifice of the stomach at T11, a total distance of approximately 25 cm in adults. The diameter varies between 20 and 30 mm, stretching to allow the passage of a food bolus. In its course, it follows the curvature of the spine posteriorly and deviates to the left initially until it returns to the midline in the posterior mediastinum. A second bend to the left occurs as the oesophagus crosses the descending thoracic aorta to pierce the diaphragm. The oesophagus is the narrowest region in the digestive tract. It has three constrictions: at 15 cm from the incisors the cricopharyngeal sphincter, at 23 cm it is crossed by the aortic arch and left main bronchus and at 40 cm it pierces the diaphragm.

Oesophageal wall Lymphoid tissue of the pharynx The wall of the upper aero digestive tract contains a large amount of unencapsulated lymphoid tissue in the lamina propria, the so-called gut-associated lymphoid tissue (GALT). This is particularly prominent in the pharynx at the entrance to the upper aero digestive tract. The nasopharyngeal, tubal, palatine and lingual tonsils form a ring of GALT at the level of the oropharyngeal and nasopharyngeal isthmus, known as Waldeyer's ring. Lymphatic efferents leave this tissue and drain to regional lymph nodes.

THE PALATINE TONSIL

The palatine tonsil is located between the diverging palatopharyngeal and palatoglossal folds. A sulcus usually separates the tonsil from the base of the tongue, the tonsillolingual sulcus. The medial surface of the tonsil is characterized by numerous tonsillar crypts, which may penetrate nearly the whole thickness of the tonsiL The deep surface of the tonsil is covered by a fibrous capsule,

The oesophagus has four layers, from inside out: the mucous membrane, submucosa, muscular coat and an outer fibrous layer. It is lined by a nonkeratinizing stratified squamous epithelium continuous with that of the pharynx. Beneath this is the loose connective tissue of the lamina propria containing elastic fibres and GALT. The muscularis mucosa lies deep to this, becoming thicker as it descends. The submucosa loosely connects the mucous membrane and the muscular coat. It contains blood vessels, lymphatics, Meissner's plexus of postganglionic parasympathetic nerve fibres and minor mucous glands, which lubricate the oesophagus. The muscular coat has an outer longitudinal layer which is complete apart from a small dehiscence at the upper end where the fibres diverge from the midline posteriorly to form two longitudinal bundles which come around anteriorly and attach to the posterior lamina of the cricoid cartilage. The inner circular layer is continuous superiorly with the fibres of the cricopharyngeus and inferiorly with the oblique fibres of the stomach. The muscles of the upper third of the oesophagus are striated, while in the lower third they are smooth. There is a

1952 I PART 15 THE UPPER DIGESTIVE TRACT transitional zone in the middle third. The fibres of cricopharyngeus make up the upper oesophageal sphincter while at the lower end there is no anatomical sphincter. It is thought that a number of factors contribute to closure of the lower oesophagus including intrinsic muscle activity, the encircling fibres of the right crus of the diaphragm and oblique fibres of the stomach, the thoracoabdominal pressure gradient and the angle of the oesophagogastric junction. There is an external adventitia of dense connective tissue overlying the oesophagus. A condensation of this, the phreno-oesophageal ligament, attaches the oesophagus to the diaphragmatic opening.

Nerve supply to the oesophagus The striated muscle in the upper third of the oesophagus is supplied by the recurrent laryngeal nerve. The smooth muscle is supplied by parasympathetic fibres from the oesophageal branches of the vagus and recurrent laryngeal nerves. These fibres synapse in the ganglia of the submucosa (Meissner's plexus) and myenteric (Auerbach's) plexus, between the longitudinal and circular muscle layers. Sympathetic fibres are derived from the sympathetic trunk and come either directly from the cardiac plexus or around the blood vessels supplying the oesophagus. Afferent fibres run with the branches of the vagus and sympathetic nerves. The oesophageal mucosa is sensitive to heat and cold but insensitive to touch. Pain is poorly localized and probably caused by muscle spasm rather than direct stimuli.

Vasculature of the oesophagus The cervical oesophagus is supplied by the inferior thyroid artery and left subclavian artery. The thoracic part has a segmental supply directly from the descending aorta and branches of the bronchial and upper posterior intercostal arteries. The abdominal part is supplied by the left gastric artery, a branch of the coeliac artery, and the left inferior phrenic artery directly from the abdominal aorta. An extensive venous plexus lies on the outside of the oesophagus and drains in a segmental way to the inferior thyroid veins (systemic), azygos and hemiazygos veins (systemic) and left gastric vein (portal). The lower end of the oesophagus is an important area of portal systemic venous anastomosis. At the upper end of the oesophagus, longitudinal submucosal veins enter the pharyngeal and laryngeal plexuses. ~ Lymphatic plexuses lie in the mucous membrane and in the muscular coat. They drain by ascending or descending beneath the mucosa or by piercing the oesophagus. The cervical lymphatics drain to the lower

deep cervical and paratracheal nodes, the thoracic ones to posterior mediastinal and tracheobronchial nodes and abdominal ones to the left gastric nodes.

Relationships of the oesophagus The cervical oesophagus lies posterior to the trachea and anterior to the prevertebral fascia. The recurrent laryngeal nerves ascend on each side in the tracheo-oesophageal groove. The thoracic duct passes up and behind the left side of the oesophagus. In the superior mediastinum, the oesophagus lies slightly to the left of the midline and passes behind and to the right of the aortic arch. The left subclavian artery is immediately to the left of the oesophagus as it arises from the aortic arch. On the right is the azygos vein arching from posterior to anterior over the lung root to enter the superior vena cava. The mediastinal pleura is in contact with the oesophagus, separated on the right by the azygos vein and on the left by the aortic arch and left subclavian artery. At the level of the tracheal bifurcation the oesophagus is crossed anteriorly by the left main bronchus and immediately below by the right pulmonary artery. The fibrous pericardium overlying the left atrium comes into contact with the anterior surface of the oesophagus further down. The inferior tracheobronchial nodes lie between the bifurcation of the trachea and the oesophagus. The thoracic duct enters the thorax through the right side of the aortic opening in the diaphragm and runs up behind the right margin of the oesophagus until it crosses obliquely to lie behind the left side. The two hemiazygos veins lie between the oesophagus and the prevertebral fascia and then pass to join the azygos vein on the right. Inferiorly, near the diaphragm, the aorta passes behind the oesophagus as the latter curves toward the left and turns forwards to pass through the diaphragm. The left and right vagus nerves, having branched to form the cardiac and pulmonary plexuses, come together as the oesophageal plexus and form multiple nerve trunks that descend with the oesophagus through the diaphragm. The left vagal fibres usually lie on the anterior surface and those on the right posteriorly. After the oesophagus emerges from the right crus of the diaphragm slightly to the left of the midline, it lies in the oesophageal groove on the posterior surface of the left lobe of the liver. It curves sharply to the left to join the stomach at the cardia. This short abdominal section of the oesophagus is covered with peritoneum.

C

KE'(eOINTS ~

-

-

-

-

-- -

- - :- -

'- -

-- --

:--

--

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Chapter 149 Anatomy of the pharynx and oesophagus

.• ·Tll:~~• it~·gapbJt~:ewcri~eHharyrrigeiI~' . . ,

>"·'~~c:thYJ."~rh~IJTg~J1s:calle(h~~IHal1's ..~~i~isc~gg���, �·�:I)ctionaHnvest igati ons . using . ...", . , : :;' -..' �- , :, . ,: - . i '" -.

•

no n-Ho dgkin's lymphoma;

antibo dies (ELISA and confirmatory Western blot anti­

•

opportunistic infections involving head and neck sites

body

including TB due to

complex

influenced by the severity of the symptoms in primary

in non-TB en demic areas a nd CMV, toxoplasmosis,

HIV infection , the duration of illness, the presence of

Mycobacterium avium

Cryptococcus neoformans with

Ta b l e 1 52 .4

;

neurological

copies/mL)

testing) .

in

the

Progression

absence to

of HIV-specific

late-stage

disease

is

neurological symptoms and that of oral candidiasis.

Pha ryngeal a nd other head a n d neck m a n ifesta tions of H IV i n fectio n .

Pha ryn gea l

Ot her head and neck

Acute seroconversion i l l n ess

O ra l cavity disease

O p po rtun istic infections, espec ially with

Sinonasal d isease. R h initis and rh inos i n u sitis a re extrem ely com mon. End osco pic s i n us s u rg ery can be helpfu l

candida O ra l hairy leu kop l a k i a

Cervical lympha denopathy m a i nly d u e to lym phoid hyperplasia b u t TB or fu ngal infection (cryptococcus or h i stoplasmosis) may be responsib l e. N eoplasms were least common a n d

i

Lym phoid tissue hyperp l asia, parti c u l a rly n the post-n asal s pa ce

i ncl uded N H L a n d SCC Sal ivary g l a nd disease : be n i g n Iym phoe p i th e l i a l cyst d i sease ; Iymphopro l i ferative swe l l i ngs may regress w i th a nti retroviral therapy, doxycyc l i n e sclerotherapy injection a n d low­ dose external beam rad i othera py

Neop lastic l esions

Paroti tis d u e to infecti o n of i n traparotid nodes with C MV a n d mycobacteria Diffuse CDS lym phocytosis syndro m e ; sa l iva ry stones N H L (natura l killer type) ; m a l i g n ant transform ation of ben i g n Iymphoepithelia l l esion i nto B­ cel l N H L; KS of i ntra pa rotid lym p h nodes Ea r d isease: Be l l 's pa lsy; sensori neu ra l heari ng possi bly related to a n tiretrov ira l thereapy ototoxici ty, and co nductive heari n g losses seconda ry to Eu stach ian tube obstru cti o n from l y m p h o i d hyperplasia o r chronic s u p p u rative otis media (CSO M) ; vesti b u l a r fa i l u re ; K S o f t h e external a u ditory meatus ; B-cell Iym poma o f the external a u d itory m eatu s ; CS O M with intracerebral com p l ications; re lapsing polychond riti s ; Pneumocystis carin ii i nfection causing a u ra l polyps

Chapter 1 52 Acute and chronic p h a ryngea l infection I 20 1 3

Currently, the experimental evidence is insufficient to recommend whether or not those diagnosed with primary HIV infection should routinely receive antiretroviral therapy although it is routinely given in the USA. Treatment of primary infection with highly active antiretroviral therapy does not prevent establishment of chronic infection. Very early therapy could, potentially, decrease the viral set point, prevent viral diversification, preserve immune function, improve clinical outcome and decrease secondary transmission. ORAL AND OROPHARYNGEAL LESIONS DUE TO OPPORTUNISTIC INFECTION

Oral candidiasis has been reported as the most prevalent oral lesion. Candida forms other than Candida albicans are more prevalent in HIV disease including Candida glabrata, Candida krusei and Candida dubliniensis. Fluconazole is the mainstay of therapy, although resis­ tance is developing. Penicillium marneffei, a newly described fungal infection, has been identified in South East Asia. Oral lesions common in the early years of the AIDS epidemic including KS, oral aphthous ulceration, AIDS-associated oral lymphoma and oral hairy leukopla­ kia ( OHL) all contain EBV DNA. Abundant viral replication only occurs in OHL and is due to a previously unknown mechanism involving concurrent expression of prolific, replicative and transforming proteins of the gamma herpes virus EBV. Kaposi's sarcoma has also been shown to contain a different herpes virus specific to this lesion which is now known as KS­ associated virus. Oral lesions, especially pseudomembranous and/or erythematous candidiasis and OHL, which are highly suggestive of HIV infection in individuals of unknown HIV status, and in those known to be HIV infected, indicate that the battle lines between HIV virion production and destruction of immunologically impor­ tant cells have been drawn. These observations have led to the almost universal inclusion of oral lesions in HIV staging. Recently, lower frequencies of oral disease due to highly active antiretroviral therapy (HAART) have been noted, except that oral warts may become more common as the viral load falls and the CD4 count rises. Human papilloma v;iruses (HPV) are associated with oral warts in HIV-positive individuals, a diagnosis that is increasing, but currently there is no evidence for an HPV-mediated increase in oral cancer. Types include verruca vulgaris, condyloma acuminatum and focal epithelial hyperplasia. In addition to HPV, herpes simplex virus infection is increasing in this group and may run an atypical course including presentation as an exudative erythema multi­ forme. In children with HIV infection, additional oral lesions include angular cheilitis, ulcerative gingivitis/ periodontitis and enamel hypoplasia, salivary gland disease, linear gingival erythema over retention and

delayed primary eruption o f teeth. OHL common in children than adults.

IS

much less

LYMPHOI D TISSUE HYPERPLASIA IN THE PHARYNX

Lymphoid tissue hyperplasia in the pharynx commonly involves all the tissues of Waldeyer's ring including adenoidal, palatine and lingual tonsils. Adenoidal hyper­ plasia and hypertrophy may cause Eustachian tube obstruction and otitis media with effusion. Biopsy of this tissue to exclude nasopharyngeal carcinoma and lympho­ ma is mandatory but radical adeno-tonsillectomy is to be avoided because of the bleeding risks. HIV viral RNA can be identified in this tissue but no other microorganisms. The complex immunological changes suggest that HIV may be disseminated through the upper aerodigestive tract via target cells. Subsequent presentation of viral antigens to the tonsillar and adenoidal lymphoid tissue results in simulated neoplastic proliferation of these structures, which are highly suspicious of HIV even in asymptomatic HIV-positive patients.

PHARYNGEAL NEOPLASMS

Pharyngeal neoplasms include KS, NHL and Scc. The outlook for patients with these malignancies has improved significantly with the use of HAART and more aggressive cytotoxic therapies. The relative risk of acquiring these conditions compared with non-HIV-infected individuals is for KS greater than 1 0,000, B-cell NHL greater than 1 00, NHL eight and multiple myeloma five. Children also have a high risk of leiomyosarcoma (RR 10,000). Kaposi's sarcoma appears to result from an uncontrolled expres­ sion of latency genes of human herpes virus-S. It is exquisitely sensitive to immune deficiency and its incidence has declined rapidly with the use of HAART while that of NHL has declined much less so. Patients with head and neck squamous carcinoma who are HIV positive in the absence of AIDS, presented at a younger age, more frequently experienced treatment-related complications and had a poorer outcome, suggesting that head and neck SCC may be an AIDS-defining diagnosis. Oral cavity and oropharyngeal tumours in HIV-positive patients respond to radiation therapy but there is a marked difference in the degree of acute reactions to treatment between patients with and without KS. Breakthroughs in technology have produced tests for HIV antibody that are highly accurate and easy to use and can give a preliminary result in 20 minutes or less. These will be used increasingly in a variety of health care settings, such as the management of medical emergencies, health care worker exposure, labour wards, military operations, disaster management and in the developing world. Work on HIV vaccine development is refocusing on passive vaccination with monoclonal neutralizing antibodies as these seem to provide impress­ ive protection in primates. =

201 2 I

PART 1 5 TH E UPPER D I G ESTIVE TRACT

p resentatio ns, herpes simplex infections and oral

Pharyngeal and o ther head and neck manifestations of HlV infection are listed in

Table

1 52.4. • •

TUMOU RS OF THE H EAD AND N ECK

The

most

frequent

non-AI D S

•

defining

cancers

are

primary skin malignancies inclu ding melanoma, basal

40 years, of HlV infection and a h istory of

and SCCs. Risk facto rs included age greater than longer duration opp o rtunistic

infection.

The

use

of

hi ghly

active

antiretroviral therapy is protective. Aerod igestive t ract (lung and head and neck) malignancies are becoming

histo plasmosis; Al DS- defining dementia; p ro gressive multi focal leukoencephalopathy; wastin g.

The type

o f AIDS- defining

illness

is

an

impo rtant

determinant of survival, for examp le encephalopathy and wasting are associated with median survivals of less than three months, while others, for example extrap ul ­ monary

TB

and herp es simplex infection, are associated

with median survival greater than two years.

increasingly common with continued imp rovements in HN therapy. These neoplasms are associated with a younger age at di agnosis, cigarette smo king, advanced stage

at

presentation

and

more

aggressive

clinical

course. The reasons for this are not clear. Altho ugh these neo plasms are not AlDS de finin g,

fac tors that

may contribute to risk include HIV-related immuno­ suppression and co- infection with high-risk papillo ma subtypes.

PRI MARY HIV INFECTION/ACUTE SEROCONVERSION I LLNESS

Primary H IV can be asymptomatic or result in severe symp tomatic illness. Co mmon symptoms are pyrexia, pharyngitis, malaise, lethargy, maculopapular rash, mu­ co us membrane ulceration, cervical lymphadenopathy and headache. Non-head primary

and

neck manifestations of

HN infectio n might include gastr o intestinal

tract transit disturbances, weight loss, anorexia, ab dom­ inal pain, myalgia arthralgia and neurolo gical features.

AIDS D E F I NING H EAD AN D NEC K PRESENTATIONS

AIDS defining head and neck p resentations include: • • •

The differential diagnosis is with glandular fever-type syndromes. A careful sexual history may provide a clue. It can be diagnosed by virolo gy testing (HN- l RNA level

50,000 copies/mL)

absence

of HIV-specific

>

non- Ho dgkin's lympho ma;

antibo dies ( ELI SA and confirmatory Western blot anti­

opportunistic infections involving head and neck sites

body

TB due to Mycobacterium avium complex in no n- TB endemic areas and CMV, toxoplasmosis, Cryptococcus neoformans with neurological

in flu enced by the severity of the symptoms in primary

including

Ta ble 1 52.4

testi ng) .

in

the

Kaposi's sarcoma (KS ) ;

P rogression

to

late-stage

disease

is

HN infection, the duration of illness, the presence of neurological symptoms a n d that o f oral candidiasis.

Pharyn g e a l and o t h e r head and n eck m a n i festa t i o ns of H IV i nfect i o n .

Pharyngeal

Oth e r head and neck

Acute s e roconve rs i o n i l l n ess

O ra l cavity d ise a se

O p po rt u n i stic i nfectio ns, especia l ly with

S i n o n asa l d isease. R h i n i t i s a n d rh inos i n u sitis a re extre m e ly com m o n . End oscopic s i n u s

candida Ora l h a i ry l e u k o p l a k i a

su rg e ry ca n be h e l pfu l Ce rvica l lym p h a d e n o p a th y m a i nly d u e to l y m p h o i d hyp e rp l a s i a b u t TB or fu n g a l i nfection (cryptococcus o r h istop lasm osis) may be responsi b l e. Ne o p lasms we re l east co m m o n a n d i nc l u d e d N H L a n d SCC

Lym p h o id tiss u e hyperplas i a , particu l a rly i n the post-nasal space

Sa l ivary g l a n d d i sease : be n i g n Iym p h oe pit h e l i a l cyst d i sea s e ; Iym p h o p ro l iferative s w e l l i n gs may reg ress w i t h a n tire t rovira l t h e rapy, d oxycycl i n e scl eroth e rapy i nj ection a n d l ow ­ dose exte r n a l b e a m ra diothera py

N e o p las tic lesi o n s

Pa roti t i s d u e to i n fecti on of i n trapa rot i d n od es w i t h CMV a nd mycobacte ri a Diffuse C O B lym p h ocytosis syn d ro m e ; sa l iva ry stones NHL (na t u r a l k i l l e r type) ; m a l i g n a nt t ransfo r m a t i o n of b e n i g n Iym p h oe p i t h e l i a l l es i o n i nto B­ ce l l N H L ; KS of i nt ra p a ro t i d lymph n od es Ea r d i sease : B e l l's pa lsy ; se nsorineu ra l h ea ri n g possi b ly re lated to a nt i retrovi ra l th e re a py ototoxicity, a n d c o n d uctive hearing losses seco ndary to Eusta c h i a n t u b e o bst ructi o n from lym p h o i d hyp e r p l a s i a or ch ron ic su p p u rative o t i s m e d i a (CSOM) ; vesti bu l a r fa i l u re ; K S of t h e ext e r n a l a u d itory m eatus ; B -ce l l Iym po m a o f t h e exte rna l a u d i tory m ea t u s ; CSOM w i t h i nt race r e bra l complicq t i o ns; re l a ps i n g polyc h o n d ri t i s ; Pn e umocystis carinij i nfection c a u si n g a u ra l polyps

Ch apter 1 52 Acute a n d chro n ic pharyngeal i nfection I 20 1 3

Currently, the experimental evidence is insufficient to recommend whether or not those diagnosed with primary HIV infection should routinely receive antiretroviral therapy although it is routinely given in the USA. Treatment of primary infection with highly active antiretroviral therapy does not prevent establishment of chronic infection. Very early therapy could, potentially, decrease the viral set point, prevent viral diversification, preserve immune function, improve clinical outcome and decrease secondary transmission. ORAL AND OROPHARYNGEAL LESIONS DU E TO OPPORTUNISTIC I NFECTION

Oral candidiasis has been reported as the most prevalent oral lesion. Candida forms other than Candida albicans are more prevalent in HIV disease including Candida glabrata, Candida krusei and Candida dubliniensis. Fluconazole is the mainstay of therapy, although resis­ tance is developing. Penicillium marneffei, a newly described fungal infection, has been identified in South East Asia. Oral lesions common in the early years of the AIDS epidemic including KS, oral aphthous ulceration, AIDS-associated oral lymphoma and oral hairy leukopla­ kia ( OHL) all contain EBV DNA. Abundant viral replication only occurs in OHL and is due to a previously unknown mechanism involving concurrent expression of prolific, replicative and transforming proteins of the gamma herpes virus EBY. Kaposi's sarcoma has also been shown to contain a different herpes virus specific to this lesion which is now known as KS­ associated virus. Oral lesions, especially pseudomembranous and/or erythematous candidiasis and OHL, which are highly suggestive of HIV infection in individuals of unknown HIV status, and in those known to be HIV infected, indicate that the battle lines between HIV virion production and destruction of immunologically impor­ tant cells have been drawn. These observations have led to the almost universal inclusion of oral lesions in HIV staging. Recently, lower frequencies of oral disease due to highly active antiretroviral therapy (HAART) have been noted, except that oral warts may become more common as the viral load falls and the CD4 count rises. Human papilloma viruses (HPV) are associated with oral warts in HIV-positive individuals, a diagnosis that is increasing, but currently there is no evidence for an HPV-mediated increase in oral cancer. Types include verruca vulgaris, condyloma acuminatum and focal epithelial hyperplasia. In addition to HPV, herpes simplex virus infection is increasing in this group and may run an atypical course including presentation as an exudative erythema multi­ forme. In children with HN infection, additional oral lesions include angular cheilitis, ulcerative gingivitis/ periodontitis and enamel hypoplasia, salivary gland disease, linear gingival erythema over retention and

delayed primary eruption o f teeth. OHL common in children than adults.

IS

much less

LYMPHO I D TISSU E HYPERPLASIA IN THE PHARYNX

Lymphoid tissue hyperplasia in the pharynx commonly involves all the tissues of Waldeyer's ring including adenoidal, palatine and lingual tonsils. Adenoidal hyper­ plasia and hypertrophy may cause Eustachian tube obstruction and otitis media with effusion. Biopsy of this tissue to exclude nasopharyngeal carcinoma and lympho­ ma is mandatory but radical adeno-tonsillectomy is to be avoided because of the bleeding risks. HIV viral RNA can be identified in this tissue but no other microorganisms. The complex immunological changes suggest that HIV may be disseminated through the upper aerodigestive tract via target cells. Subsequent presentation of viral antigens to the tonsillar and adenoidal lymphoid tissue results in simulated neoplastic proliferation of these structures, which are highly suspicious of HIV even in asymptomatic HIV-positive patients.

PHARYNGEAL NEOPLASMS

Pharyngeal neoplasms include KS, NHL and Scc. The outlook for patients with these malignancies has improved significantly with the use of HAART and more aggressive cytotoxic therapies. The relative risk of acquiring these conditions compared with non-HIV-infected individuals is for KS greater than 10,000, B-cell NHL greater than 1 00, NHL eight and multiple myeloma five. Children also have a high risk of leiomyosarcoma (RR = 10,000). Kaposi's sarcoma appears to result from an uncontrolled expres­ sion of latency genes of human herpes virus-So It is exquisitely sensitive to immune deficiency and its incidence has declined rapidly with the use of HAART while that of NHL has declined much less so. Patients with head and neck squamous carcinoma who are HIV positive in the absence of AIDS, presented at a younger age, more frequently experienced treatment-related complications and had a poorer outcome, suggesting that head and neck SCC may be an AIDS-defining diagnosis. Oral cavity and oropharyngeal tumours in HN-positive patients respond to radiation therapy but there is a marked difference in the degree of acute reactions to treatment between patients with and without KS. Breakthroughs in technology have produced tests for HN antibody that are highly accurate and easy to use and can give a preliminary result in 20 minutes or less. These will be used increasingly in a variety of health care settings, such as the management of medical emergencies, health care worker exposure, labour wards, military operations, disaster management and in the developing world. Work on HN vaccine development is refocusing on passive vaccination with monoclonal neutralizing antibodies as these seem to provide impress­ ive protection in primates.

2016 I PART

)-

)-

>-

)-

>-

>-

15

THE U PPER D I G ESTIVE TRACT

adu lts a n d c h i l d ren to a ssess the efficacy, i n c l u d i ng dosage a n d d u ration , of antibiotic thera py with penici l l i n versus placebo. These trials would h ave to be ca rried out i n the pri m a ry ca re setting and the main outcomes would be d u ration a n d severity of sym ptoms, i n cl uding pain, fever, inabi l ity to eat and ca rry out usual a ctivities. Further tri a l s of a lternative anti biotics cou ld then va l idly be com pa red with the effectiveness of pen ici l l i n . A longitud i n a l cohort study is req u i red i n both a d u lts a n d c h i l d ren of the natu ra l h i story of recu rrent episodes of sore throat. The size of the cohorts shou ld be sufficient to a l low pre pa ration of a m u l tifactorial a n a lysis of those factors, wh ich m i g ht predict resolution / n o n resol ution. This is l i kely to i n cl u d e age, sex, n u m ber of s i b l i ng s/a d u lts in the home, deg ree of contact with peers (school, u n iversity, etc. ) , va rious i ndications of deprivation i n c l u d i n g socioeconomic g roup and exposure to ciga rette smoke. There is sti l l confl icting evidence on the role of perioperative loca l anaesth esia i njection and on postoperative a ntibiotics for ton si l l ectomy. Outcomes resea rch fo l l owing tonsi l l ectomy is a developing field. RCTs a re req u i red of tonsi l l ectomy versus nonsurg ica l m a nagement with d isease-specific o u tcom es, genera l h ealth outcomes a n d costings. Such tri a l s shou ld be suffi ciently powered to assess ben efit depen ding on frequency and severity of sym ptoms prior to operation. Stud ies re porting the prescri bing behaviour of G Ps for sore throat sym ptoms in rel ation to workload, time constrai nts and socioeconomic factors a re needed. La rge m u lticentre prospective tri a l s a re req u i red for different tonsi l lectomy techn iques and outcomes ; treatment mod a l ities for deep neck space infections; antivi ra l agents and stero ids in severe, atypica l g l a n d u l a r fever a n d defi n ing the risk of splenic ru ptu re with contact sport.

*

4.

*

5.

U ppa l K, Bais AS. Tons i l l a r m icroflora - su perfici a l su rface vs deep. Journal of Laryngology and Otology. 1 98 9 ; 1 03 :

6.

Schachtel BP, Fi l l i n g i m JM, Beiter OJ , La ne AC, Schwa rtz LA. Subjective and objective featu res of sore t h roat.

7.

D e l Mar C . Man aging sore throat: a l iterature review. I : Making the d i ag nosis. Medical Journal o f Australia. 1 99 2 ;

8.

Bu rke P, Bain J , Lowes A, Athersuch R . Rational decisions in managing sore throat: eva l u ation of a ra pid test. British

Brook I , Yocu m P, Shah K. Su rface vs core-tonsi l la r aerobic a n d anaerobic flo ra in recu rrent tonsi l l itis. Journal of the American Medical Association. 1 98 0 ; 2 4 4 : 1 696-8.

1 7 5-7.

Archives of Internal Medicine. 1 984; 1 44 : 49 7- 500.

1 5 6 : 5 7 2- 5 .

Medical Journal. 1 98 8 ; 2 9 6 : 1 646-9. 9.

La ing MR, McKerrow WS. Adu lt tonsi l l ectomy. Clinical Otolaryngology and Allied Sciences. 1 99 1 ; 1 6 : 2 1 -4.

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2020 I

PART 1 5

THE LI PPER D I G ESTIVE TRACT

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a rare example of metaanalysis in ENT with a significant positive outcome.

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weakn esses of the current evidence base.

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Steward D L, We lge JA, Myer CM. Steroids for i m proving recovery fol l ow i ng tonsi l l ectomy i n ch i l d ren. Cochrane Database Systematic Revie ws. 2003 ; CD003997. This unpublished trials.

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PART

1 5 TH E U PPER D I G ESTIVE TRACT

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n n '.lr\/rH1 P Ol I

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