Raymond W Clarke - Diseases of The Ear, Nose & Throat in Children - An Introduction and Practical Guide-CRC Press (2022)

DISEASES O F T H E EAR,
NOSE & THROAT I N

CHILDREN
AN INTRODUCTION AND PRACTICAL GUIDE
RAYMOND W CLARKE
CRC Press
Taylor & Francis Group
Diseases of the Ear, Nose
& Throat in Children
Diseases of the
Ear, Nose &
Throat in Children
AN INTRODUCTION AND PR ACTICAL GUIDE
Raymond W Clarke BA, BSc., DCH, FRCS, FRCS(ORL)

Paediatric Otolaryngologist
Alder Hey and University of Liverpool
Liverpool, UK
Boca Raton London New York
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Library of Congress Cataloging-in-Publication Data
Names: Clarke, Ray (Raymond) author.

Title: Diseases of the ear, nose & throat in children : an introduction and practical guide / Raymond W. Clarke.
Other titles: Diseases of the ear, nose and throat in children
Description: First edition. | Boca Raton : CRC Press, 2022. | Includes bibliographical references and index. |
Summary: “An essential introduction to the clinical examination, treatment and surgical procedures for children
with diseases of the ear, nose and throat. This book encompasses the conditions most commonly encountered in the
emergency setting, on the ward and in the outpatient clinic. With its highly practical approach and step-by-step guidance,
this book will be invaluable for all surgical trainees studying for higher postgraduate examinations in ENT, as well as an
essential guide for otorhinolaryngologists, primary care practitioners and specialist ENT nurses in the early years of
training”— Provided by publisher.
Identifiers: LCCN 2021062701 (print) | LCCN 2021062702 (ebook) | ISBN 9781138579422 (hardback) |
ISBN 9781138579347 (paperback) | ISBN 9780429019128 (ebook)
Subjects: MESH: Otorhinolaryngologic Diseases | Child | Emergencies | Otorhinolaryngologic Surgical Procedures
Classification: LCC RF32 (print) | LCC RF32 (ebook) | NLM WV 140 | DDC 617.5/1083—dc23/eng/20220328
LC record available at https://lccn.loc.gov/2021062701
LC ebook record available at https://lccn.loc.gov/2021062702
ISBN: 978-1-138-57942-2 (hbk)

ISBN: 978-1-138-57934-7 (pbk)
ISBN: 978-0-429-01912-8 (ebk)
DOI: 10.1201/9780429019128
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CONTENTS
Preface vii
Acknowledgements ix
Abbreviations xi
1 Introduction 1
2 The paediatric consultation 7
3 Children with special needs 11
4 ENT foreign bodies 15
5 Sepsis in the head and neck 19
6 The external ear 25
7 Hearing loss in childhood 31
8 Hearing tests 37
9 Otitis media with effusion 41
10 Acute otitis media 45
11 Chronic otitis media 49
12 Surgery for childhood hearing loss 53
13 Balance disorders 59
14 Facial palsy 63
15 The pharynx and oral cavity 67
16 Obstructive sleep apnoea 73
17 Congenital nasal disorders 77
18 Acute rhinosinusitis 83
19 Chronic rhinosinusitis 87
20 Allergic rhinosinusitis 91
21 Non-infammatory acquired sinonasal disorders 95
22 The obstructed airway 99
23 Congenital laryngeal disease 103
24 Acquired laryngotracheal disease 109
25 Tracheostomy 115
26 Congenital disorders of the neck 121
27 Acquired disorders of the neck 127
28 The salivary glands 133
29 COVID-19 in children’s ORL 139
Index 143
Contents v
PREFACE
Paediatric otolaryngology is a growing discipline day-to-day work looking afer children, and that the
with a large and increasingly complex body of newcomer to ORL will be motivated to learn more
knowledge to guide specialist practitioners. Te about paediatric aspects of our specialty.
subspecialty is already well covered by some excel-
lent textbooks and reference works. Te general ORL I have deliberately kept the chapters short, with
doctor, with a mixed adult and child practice, is less summaries of the main points, and a very small
well served and ofen bemoans the lack of a short, number of references as readers now have near-
easy-to-read account of the main disorders in chil- universal recourse to multiple up-to-date online
dren. I hope this little book flls that gap. sources of knowledge. Troughout, I have tried to
give sound advice that will be of use in the clini-
I have focused in the main on common conditions cal situation, much of it based on long personal
unique to children or where the presentation and experience.
management are diferent in children than in their
adult counterparts. Tis has meant leaving much Working with children, families, colleagues and
material out, for example in otology – including trainees in paediatric ORL has been a lifelong plea-
implantation otology, now a very large part of pae- sure and I hope this short text will stimulate others
diatric practice – where there is already very good to share that joy.
material available in the standard adult texts.
R.W. Clarke
I hope candidates for the main postgraduate Paediatric ORL Dept.
examinations (e.g. the UEMS Boards, and the UK Royal Liverpool Children’s Hospital
Intercollegiate fellowship) will have enough revision Alder Hey
material to satisfy the curriculum, that the estab- Liverpool, UK
lished generalist in ORL will fnd enough in the way
of advice regarding investigation and management Honorary Professor
of the common conditions to support them in their University of Liverpool
Preface vii
ACKNOWLEDGEMENTS
Tanks to Miranda Bromage and her team at Taylor on the audiology content, and Ms Ann-Louise
& Francis for seeing this book to completion. Becky McDermott helped with the chapter on COVID-19.
Freeman, Daina Habdankaite and Nora Naughton
provided immense support and patience as the Many of the fgures are borrowed from Scott Brown’s
manuscript developed. Sue Tyler drew several new Otolaryngology Head and Neck Surgery, 8th Edition,
illustrations, and Dr Shiv Avula helped with the Taylor & Francis 2018, and I am grateful to the chap-
radiological images. Dr Sudhira Ratnayake advised ter authors for providing high-quality images.
Acknowledgements ix
ABBREVIATIONS
ABC aspiration biopsy cytology CF cystic fbrosis
ABI auditory brainstem implant CHAOS congenital high airway obstruction
ABR auditory brainstem response syndrome
ABRS acute bacterial rhinosinusitis CI cochlear implant
AC air conduction CMV cytomegalovirus
AD auditory dysynchrony COM chronic otitis media
ADD attention defcit disorder CPAP continuous positive airway pressure
ADHD attention defcit hyperactivity CRP C-reactive protein
disorder CRS chronic rhinosinusitis
AGP aerosol-generating procedure CSOM chronic suppurative otitis media
AHI apnoea/hypopnoea index CT computed tomography
AIDS acquired immunodefciency CTR cricotracheal resection
syndrome CWD canal wall down
ALTB acute laryngotracheobronchitis CWU canal wall up
AMT appropriate medical treatment CYP children and young people
ANSD auditory neuropathy spectrum DISE drug-induced sleep endoscopy
disorder DNS deep neck space
AOM acute otitis media DSA Down’s Syndrome Association
APAGBI Association of Paediatric EBV Epstein Barr virus
Anaesthetists of Great Britain and ED emergency department
Ireland ENT ear, nose and throat
APLS Advanced Paediatric Life Support™ EPOS2020 European Position Paper on
AR allergic rhinitis / allergic Rhinosinusitis and Nasal Polyps
rhinoconjunctivitis / allergic 2020
rhinosinusitis ET endotracheal
ARS acute rhinosinusitis EXIT ex utero intrapartum treatment
ASD autistic spectrum disorder FESS functional endoscopic sinus surgery
ATM atypical mycobacteria FFP fltering face-piece
BAHA bone-anchored hearing aid FII Fabricated or Induced Illness
BAPO British Association for Paediatric FNA fne-needle aspiration
Otolaryngology GABHS group A beta-haemolytic
BC bone conduction Streptococcus pyogenes
BCHD bone-conducting hearing device GI gastrointestinal
BOA behavioural observation audiometry GMC General Medical Council
BPAP bilevel positive airway pressure HCW healthcare workers
BPCHI bilateral permanent childhood HDU high-dependency unit
hearing impairment Hib Haemophilus infuenza B
BPPV benign paroxysmal positional HIV human immunodefciency virus
vertigo HL hearing loss
BPVC benign paroxysmal vertigo of HPV human papillomavirus
childhood IgE immunoglobulin E
Abbreviations xi
INCS intranasal corticosteroid PPE personal protective equipment
IV intravenous PSA pleomorphic salivary adenoma
JOF juvenile ossifying fbroma PSG polysomnography
JORRP juvenile-onset recurrent respiratory PTA pure-tone audiometry
papillomatosis RAOM recurrent acute otitis media
LCH Langerhans cell histiocytosis RARS recurrent acute rhinosinusitis
LMA laryngeal mask airway RCoA Royal College of Anaesthetists
LTR laryngotracheal reconstruction RCPCH Royal College of Paediatrics and
MDT multidisciplinary team Child Health
MEI middle ear implant REM rapid eye movement
MMR mumps, measles, rubella RRP recurrent respiratory
MRI magnetic resonance imaging papillomatosis
NAI non-accidental injury RSV respiratory syncytial virus
NF2 neurofbromatosis type 2 SALT speech and language therapist
NO nitrous oxide SARS-CoV-2 severe acute respiratory syndrome
NORD National Organization for Rare coronavirus 2
Disorders SCBU special care baby unit
NPA nasopharyngeal airway SCIT subcutaneous immunotherapy
NSAID non-steroidal anti-infammatory drug SDB sleep-disordered breathing
NTM non-tuberculous mycobacteria SGS subglottic stenosis
OAE otoacoustic emission SIGN Scottish Intercollegiate Guidelines
OME otitis media with efusion Network
OR operating room SLIT sublingual immunotherapy
ORL otorhinolaryngology SSD single-sided deafness
OSA obstructive sleep apnoea THRIVE trans-nasal humidifed rapid
PCD primary ciliary dyskinesia insufation ventilatory exchange
PCHI permanent childhood hearing TOF tracheo-oesophageal fstula
impairment hearing TSH thyroid stimulating hormone
PCHI permanent congenital hearing UK United Kingdom
impairment VACTERL vertebral, anal, cardiac, tracheal,
PCHR Personal Child Health Record renal and limb
PCR polymerase chain reaction VRA visual reinforcement audiometry
PEWS paediatric early warning signs WHO/REAL World Health Organization/
PICU paediatric intensive care unit Revised European American
PONV postoperative nausea and vomiting Lymphoma
xii Abbreviations
1 INTRODUCTION
Specialists in otorhinolaryngology (ORL) have disorders, benign and malignant neck disease and
looked afer children since the beginnings of the ORL issues in children with complex medical con-
specialty. Children’s hospitals and dedicated pae- ditions. Most general ORL specialists will also see
diatricians came to the fore from the early twenti- large numbers of children with tonsil and adenoid
eth century onwards. It was clear that ORL surgeons disease, obstructive sleep apnoea, otitis media and
were essential to the care of children and the sub- congenital and acquired hearing loss. Whatever
specialty of paediatric otolaryngology gradually healthcare setting you work in, it is important to
came into being. Paediatric ORL specialists work have a good grounding in the basics of ear, nose and
mainly in children’s hospitals – or in the children’s throat (ENT) pathologies in children and in the gen-
sections of general hospitals – and focus on problems eral principles that make for optimum care of sick
such as airway pathology, congenital head and neck children and their families.
ORL SERVICES FOR CHILDREN

Paediatric otolaryngologists look afer ‘special prob- sick children. Some of the larger general hospitals
lems or special children, or both, in a special institu- have paediatric sections which include these facili-
tion’ (Bluestone, 1995). ties, and most hospitals where ORL specialists work
will look afer children with the more common ORL
Services for children and young people (CYP) can pathologies, but typically not the very young or very
be delivered in multiple ways in diferent health sys- sick children who may need PICU.
tems. In the UK and much of Europe and beyond,
most ORL specialists have a mixed adult and pae- ORL doctors perform more surgical interventions in
diatric practice. A small, but increasing, number children than any other surgical discipline. We can
devote most or all of their professional time to look- be powerful advocates for improved care of children
ing afer children. Tertiary and advanced care is pro- and should be to the fore when services are planned
vided in designated children’s hospitals (Figure 1.1) or reconfgured.
with ready access to other specialists, to paediatric
intensive care units (PICUs) and to anaesthesiolo- Children are best seen in dedicated clinics where
gists who work exclusively with children and have there are no adult patients, and with good audiol-
a great deal of expertise in the perioperative care of ogy testing facilities. Tis is easily accomplished in
DOI: 10.1201/9780429019128-1 Introduction 1

Figure 1.2 A ‘child-friendly’ clinic waiting room.
ideally with a trained and registered children’s nurse

in charge (Figure 1.2).
Children are best cared for as close to home as possi-

ble, in an environment suited to their needs. Despite
the desirability of looking afer children and families
in a local setting, children with complex conditions,
or who need highly specialised interventions, are
better managed in a regional centre where resources
and skills are concentrated. Tis will necessitate
good communication and liaison strategies between
diferent hospitals with carefully planned arrange-
Figure 1.1 The foyer, Alder Hey Children’s Hospital,
Liverpool. ments for transfer and for safe transport, particularly
in emergency situations. Many hospitals now have a
‘retrieval’ service whereby a sick child can be resus-
children’s hospitals but needs more careful planning citated and stabilised, including endotracheal (ET)
in a general hospital. Clinics and waiting areas need intubation where needed, by a skilled team before
to be ‘child-friendly’ with suitable toys, paper and being transferred for defnitive care to a specialist
pens, and facilities for siblings and nursing mothers, centre.
PERIOPERATIVE CARE
Surgical lists staf need to be suitably trained; the anaesthesiology
and recovery teams in particular should have exper-
Operating lists for children ideally should be for tise and training in looking afer children. Diferent
children only, i.e. dedicated children’s lists. Tis national societies and organisations will have their
has become the norm in many health systems, but own guidelines. Where it is deemed safe, and the
it can be difcult to schedule in a mixed adult and surgical and anaesthetic teams have agreed arrange-
children’s hospital and is dependent on local fund- ments, ‘day-case’ or ‘same-day’ surgery is generally
ing arrangements and resources. Operating room preferred, but clearly this depends on issues such as
2 DISEASES OF THE EAR, NOSE & THROAT IN CHILDREN

transport, home facilities, and local customs and laryngeal mask airway (LMA) permits easy adminis-
expectation. Children who need an overnight stay tration of vapours, including air and oxygen, during
or admission to wards are best looked afer in a chil- anaesthesia without the use of an ET tube or a cum-
dren’s rather than a mixed adult and child ward. bersome hand-held face mask. Tis has made simple
anaesthesia more widely available for many ‘minor’
Anaesthesia ORL procedures such as myringotomy and grommet
insertion. Some surgeons and anaesthetists now use
Good rapport and mutual understanding between the LMA for adenotonsillectomy (Figure 1.3).
surgeon and anaesthetist are especially important in
children’s ORL procedures. Both surgeon and anaes- Te widespread use of oil-based local anaesthetic
thetist ‘share’ the airway, which is ofen narrow and agents – ‘magic cream’ such as Ametop® (Smith &
already compromised, especially in a very young Nephew) or EMLA® (AstraZeneca) – applied to
child. Te two methods of providing an anaesthetic the skin over a suitable vein before venepuncture
for a child are essentially simple anaesthesia and bal- has made IV injections to induce anaesthesia far
anced anaesthesia. less frightening. A small number of children will
need a ‘pre-med’, typically a benzodiazepine such
‘Simple’ anaesthesia involves administering a single as Midazolam, but this may prolong the recovery
agent to induce unconsciousness and lack of move- period (‘hangover’) and is best avoided if possible.
ment in response to surgical stimulation. Te agent
is typically a vapour (e.g. halothane, or more ofen ‘Balanced’ anaesthesia involves the use of separate
sevofurane), but intravenous (IV) agents such as drugs to induce loss of consciousness, paralysis, and
Propofol and the benzodiazepines (Midazolam) cardiovascular control and analgesia. An anaesthetic
are increasingly popular. Te widespread use of the agent, a muscle relaxant and an opiate are usually
Figure 1.3 Equipment for paediatric anaesthesia: (1) Magill forceps, (2) Magill laryngoscopes, (3) ventilating
facemask, (4) Guedel (oropharyngeal) airways, (5) LMA, (6) ET tube, (7) bougie.
Introduction 3
combined. Te paralysed patient will need to be ven- very unhappy experience for both parent and child.
tilated, either by the anaesthetist by hand or via a Again, protocols vary but many units use prophylactic
mechanical ventilator. More sophisticated monitor- agents such as ondansetron (a serotonin antagonist) to
ing and awareness of the possibility of hypoxia are reduce this troublesome complication. Tere is now
required, but balanced anaesthesia permits longer good evidence that one single intraoperative dose of a
and more invasive procedures in younger and frailer corticosteroid (dexamethasone) improves PONV.
patients, with reduced postoperative morbidity.
Codeine is no longer appropriate in young chil-
Anaesthesia and perioperative care of children dren due to the risk of catastrophic idiosyncratic
undergoing laryngo-tracheoscopy, ofen with some reactions, especially in children with obstructive
form of open or endoscopic surgery to the larynx sleep apnoea (OSA) and should only be used with
or trachea, require particularly close cooperation extreme caution in older children and adolescents.
between surgeon and anaesthetist. Usually, the Non-steroidal anti-infammatory drugs (NSAIDs,
child will need to be breathing spontaneously, ideally e.g. diclofenac, ibuprofen) are now widely used and
without an ET tube obstructing the surgeon’s access are safe and efective despite some concerns that they
to the glottis and subglottis. Te ET tube can rest in may increase the incidence of postoperative bleeding.
the pharynx and, if the surgeon requires access to the
trachea and bronchi for a longer period, a ventilating
bronchoscope permits safe and prolonged adminis- Consent in children
tration of oxygen and anaesthetic vapours. Topical
anaesthesia (Xylocaine spray) to the mucosa of the Every medical intervention requires the full agree-
glottis makes for reduced laryngospasm, but gentle ment of the patient, but children may not have the
and controlled introduction of telescopes and instru- capacity and understanding (‘competence’) to evalu-
ments also helps in this regard. Occasionally, a short ate the benefts and risks of a procedure, and ‘con-
period of apnoea may be needed and the anaesthe- sent’ will need to be given on their behalf. Tis is a
tist will want to ‘pre-oxygenate’ the child. Tere has complex ethical and legal area. Clinicians will need
been increased interest of late in some centres in the to be familiar with the guidelines and arrangements
technique known as THRIVE (trans-nasal humidi- in their jurisdiction and as outlined by their national
fed rapid insufation ventilatory exchange), which medical regulatory bodies. It is, of course, wise to
involves the delivery of oxygen via high-fow nasal involve the child at all times wherever possible.
cannulas combined with IV anaesthesia to permit
an extended ‘window’ of apnoea without the need In the UK, a person with ‘parental responsibility’ –
for an ET tube. typically but not always the parent – can give con-
sent on behalf of a child. A ‘child’ in this context in
England and Wales is a young person up to the age of
Analgesia 16 years, but this defnition varies across healthcare
systems.
Adequate and well-chosen analgesia greatly improves
the outcome in children’s surgery. Paracetamol (acet- A child under the age of 16 years may well be able to
aminophen) is sufcient for most minor procedures. understand the implications of a treatment strategy.
Intraoperative opiates supplemented by one or two In UK law, a child who has ‘sufcient understanding
postoperative doses may be used for tonsillectomy, and intelligence to enable him or her to understand
but local protocols vary and, with improved mod- fully what is proposed’ is termed ‘Gillick competent’
ern surgical techniques, very little analgesia may be or ‘Fraser competent’. Te decision as to whether the
required. child is ‘Gillick competent’ rests with the clinician, so
you could decide that a teenager, for example, under-
Postoperative nausea and vomiting (PONV) may be going a tonsillectomy can give their own ‘consent’.
worsened by the use of opiates and can make for a It is best practice to involve the parents at all times.

Te ‘Montgomery’ legal judgment in the UK has In other words, the discussion regarding treatment
clarifed the requirements for consent even further. options needs to be open, frank and customised to
Essentially, the judgment of the courts places a duty that individual patient’s needs and priorities. An
on the clinician to explore in detail the patient’s (or example might be the possible impact of a change
in the case of a child, the responsible adult’s) likely in voice, which could be of great importance to a
assessment of the impact of the benefts and risks of child who was a keen singer but of less importance
an intervention in his or her particular circumstances. to another child.
ORGANISATIONS AND SOCIETIES

As paediatric ORL progressed, it became clear that
ORL and allied specialists needed to meet, exchange
ideas, foster education and advocate for children, their KEY POINTS
families and the professionals who look afer them.
Tere are now several national and international ● Children are best treated in dedicated clin-
societies focusing on paediatric ORL, with excellent ics, with appropriately trained staf.
educational and information resources. Te Royal ● Audiology staf, soundproof testing rooms
College of Paediatrics and Child Health (RCPCH) and facilities for tympanometry are an
in the United Kingdom (UK) provides a wealth integral part of the ORL clinic.
of resources – many available to non-members – ● Children’s surgery should be performed
and most of the various national ORL societies now during ‘children’s’ theatre lists, with an
have a section focusing on ORL issues in children experienced children’s anaesthetist and
(Box 1.1) support staf.
● Codeine is no longer appropriate as an
analgesic agent in young children.
● ‘Parental responsibility’ varies in diferent
Box 1.1 Organisations and societies jurisdictions but, in the UK, it essentially
American Society of Pediatric includes the child’s mother, and the child’s
Otolaryngology: https://aspo.us father if he is married to the child’s mother
British Association for Paediatric or named on the birth certifcate.
Otolaryngology: https://www.bapo. ● Check local arrangements for ‘looked afer’
co.uk or ‘in-care’ children who may be under the
Confederation of European care of social services.
Otorhinolaryngology – Head and Neck
Surgery: https://www.ceorlhns.org
European Society of Pediatric
Otorhinolaryngology: http://espo.eu.com
Royal College of Paediatrics and Child
Health: https://www.rcpch.ac.uk
Introduction 5
Head Neck Surg. 2019;160(2):187–205.
FURTHER READING doi: 10.1177/0194599818807917.
Bluestone CD. Paediatric otolaryngology: past Safe delivery of paediatric ENT surgery in the UK:
present and future. Arch Otolaryngol Head a national strategy. A report of a combined
Neck Surg. 1995;121:505–8. doi: 10.1001/ working party of the British Association for
archotol.1995.01890050005001. Paediatric Otolaryngology (BAPO), ENT UK,
General Medical Council (GMC). 0–18 years: guid- the Royal College of Anaesthetists (RCoA) and
ance for all doctors. Available at: https://www. the Association of Paediatric Anaesthetists of
gmc-uk.org/ethical-guidance/ethical-guidance- Great Britain and Ireland (APAGBI). Available
for-doctors/0-18-years (accessed 21 January at: https://www.bapo.co.uk/introducing-the-
2022). safe-delivery-of-paediatric-ent-surgery-in-the-
Mitchell RB, Archer SM, Ishman SL. Clinical uk-a-national-strategy/ (accessed 21 January
practice guideline: tonsillectomy in children 2022).
(update) – Executive summary. Otolaryngol

2 THE PAEDIATRIC
CONSULTATION
INTRODUCTION
Otolaryngologists are well trained in history tak- to see you will have typically come from the par-
ing and examination for adults with disorders of the ents (often the mother) and this makes for some
head and neck, but there are aspects of the paediatric important differences between the adult and pae-
consultation that set it apart. diatric consultation; the diagnosis, the discussion
of management options and the decision making
A good first meeting with a child is a unique are essentially ‘by proxy’ and they will usually
opportunity for you, the clinician, to establish a involve the parents or carers rather than the child.
rapport with them and their family that may per- The older child may be able to express their views,
sist well into the child’s adult life. The health and but with babies and young children, you need to
welfare of the child are paramount and must be at look after essentially two patients, the child and
the forefront of any plans made, but the decision the parent or parents.
HISTORY
Take time to read the case notes, including the results Te birth history, whether the child needed a stay on
of investigations if applicable, before the child enters a special care baby unit (SCBU), whether the baby
the room. Case records are ofen electronic, and it needed ET intubation or any form of airway support
can be disconcerting for the child and the parent or had feeding difculties are especially important
if the doctor is constantly turning to look at their considerations in the ORL clinic. If the child has
computer screen. Greet the child by name, make eye a chronic medical condition or a syndrome, read
contact, and introduce yourself and any other staf in up on it before you see the family if you can. Tis
the room. Establish who is with the child – it may be should be easy in most settings as so much infor-
a parent, a carer or a grandparent. Be clear on who mation is available online. Parent and child will
is going to give you the history and make sure the appreciate continuity, and if you are seeing a child
child is given the opportunity to speak if they are for repeat visits, it is ideal if the same doctor sees
old enough. them each time.
DOI: 10.1201/9780429019128-2 The Paediatric Consultation 7

Te worldwide COVID-19 pandemic has brought had to develop the skills to take detailed histories in
about an increased need for remote consultation – this way (see Chapter 29).
by video link or by telephone – and clinicians have
EXAMINATION
Begin your examination as soon as the child comes using a standard headlight as the child elevates the
into the room. Note the child’s gait, breathing pat- palate by saying ‘Ahhh’. Endoscopes, both fexible
tern and state of alertness. Once they have had a and rigid, are increasingly the norm in ORL clinics
little while to settle in the clinic room, most chil- and can make for an excellent view of the nose, naso-
dren are happy to be examined. Smaller children pharynx and laryngeal introitus in co-operative chil-
are best examined sitting on their mother’s knee. dren. It can be especially helpful to project the image
It is reasonable for the mother to gently hold the onto a large screen so the child and parent can see it.
child, but it is not appropriate to restrain a child
for a clinical examination; do not persist if they are
fractious or struggling. A preliminary nasal exami-
nation includes an assessment of the nasal airway,
and the ‘cold spatula test’ (placing a cold stainless-
steel instrument with a fat surface under the child’s
nostrils during quiet breathing to test for conden-
sation). Tis is especially useful in young children.
Children do not like the Tudicum’s speculum; get
a good view of the nasal cavities by gently elevating
the tip of the nose using your thumb and inspecting
the nose with a good light source such as a headlight
or an otoscope (Figure 2.1).
Tongue depressors are not popular with children Figure 2.1 Nasal examination. Use a good light
either; you can usually get a good view of the pharynx source and gently elevate the tip of the nose.
NORMAL GROWTH AND DEVELOPMENT

ORL doctors are not medical paediatricians, but develop. Parents will usually have a careful record
many conditions that are more properly the pre- of their child’s progress, ofen in the form of an
serve of paediatricians will present to the ORL ofcial health record (Figure 2.2). Te Personal
clinic. Delayed speech, hearing difculties and bal- Child Health Record (PCHR, ‘red book’) in the
ance disorders may be markers for global develop- UK contains a wealth of data which parents can
mental delay, autistic spectrum disorders (ASDs) refer to and presents an opportunity for them and
or neurodevelopmental conditions. It is wise to their health professionals to fll in details as the
be aware of some of the expected milestones in child progresses. Te ‘red book’, or its equivalent,
child growth and development so that you can can be a very useful source of information during
make appropriate referrals when there is con- a consultation. Electronic records that can easily
cern. Remember that there is great variation even be stored on a mobile phone are becoming more
within families as to how quickly diferent skills common.

Figure 2.2 Example growth chart from the ‘red book’: WHO weight-for-age, girls, birth to 2 years (percentiles).
CHILD PROTECTION
Looking afer the safety and welfare of children is well-founded but not handled with extreme delicacy.
everybody’s responsibility. It is a sad fact that chil- Around 75% of children who sufer physical abuse
dren are sometimes subject to physical, emotional have injuries to the head and neck (Box 2.1).
and sexual abuse. Tis may be perpetrated by fam-
ily members, friends and acquaintances, or even by
professionals who come in contact with children.
Box 2.1 Possible signs of abuse in ORL
Regulatory bodies such as the General Medical
Council (GMC) in the UK expect doctors to be con- • Tears to the lingual frenulum
versant with the tell-tale signs of abuse or neglect and • Bruises to the cheeks, lips, gums
to act quickly on any concerns they may have. Most • Nasal injuries
hospitals will arrange training for healthcare per- • Injuries to the pinna, especially ‘pinch’
sonnel to make them aware of the path to follow in marks
the event of any concerns, and will usually signpost • Auricular haematomas
clinicians to more experienced staf who have spe- • Traumatic perforation of the eardrum
cifc expertise in these matters. If you fnd yourself • Maxillofacial fractures
in a situation where you are worried, seek senior help • Dental trauma
according to the arrangements in place locally. Tis • Injuries to the palate, e.g. due to
is an area of great sensitivity. An accusation or suspi- forceful feeding
cion of abuse or neglect can cause great distress if it • Bruising to the neck
is unfounded, or prejudice appropriate action if it is
The Paediatric Consultation 9

A very small number of parents and carers deliber- by proxy’. Tis is an extreme and complex form of
ately cause or feign symptoms and signs of disease child abuse and may be evidence of a serious psy-
in their children. ORL examples include ear injuries, chiatric condition in the parent. FII requires urgent,
blocked tracheostomy tubes and deliberate smother- skilled and expert management. If you have any con-
ing. Te term ‘Fabricated or Induced Illness (FII)’ is cerns, admit the child for observation and seek the
preferred to the older term ‘Munchausen’s syndrome help and advice of an experienced paediatric team.
FUNCTIONAL ORL DISORDERS

Just as in the adult world, children present to the functional disorders seem to be on the increase.
ORL clinic with symptoms for which no organic Children do, of course, develop depression, severe
pathophysiological cause can be found despite a life-threatening eating disorders and very rarely
thorough examination and following extensive psychosis; in such cases, expert psychiatric help
investigations. The term ‘functional disorders’ will be needed.
emphasises that although there is no structural
or demonstrable anatomical abnormality, there
may be very real physiological dysfunction.
Terms such as ‘medically unexplained’, ‘psycho- KEY POINTS
genic’, ‘stress-related’, ‘psychosomatic’ and ‘hys-
terical’ were used in the past but are unhelpful ● Children with neurodevelopmental disor-
and became derogatory with the implication of ders may present to ORL. If in any doubt,
blame on the part of the patient. These symptoms seek a paediatric opinion.
are very real, cause immense distress and warrant ● Child protection and promoting good
thorough and sensitive investigation and manage- child health are everybody’s business.
ment. ORL symptoms include earache, tinnitus, ● ‘Functional’ disorders are not the same as
dysphagia, balance disorders, dysphonia and, feigned or factitious illness. Many are short-
very rarely, stridor. Functional or ‘non-organic’ lived and should not be ‘over-medicalised’.
hearing loss (see Chapter 7) is well recognised.
Parental disharmony, bullying – increasingly
‘online’ – and the physiological and psychologi-
cal changes of puberty can all have an impact on FURTHER READING
child health and somatic symptoms often come to Electronic ‘eredbook’. Available at: https://www.
the fore. A sensitive and thoughtful explanation eredbook.org.uk (accessed 21 January 2022).
of the symptoms, with reassurance that there is Royal College of Paediatrics and Child Health
no worrying progressive pathology and that the (RCPCH). Resources and advice on child pro-
prognosis is good, is often all that is required. The tection and safeguarding. Available at: https://
worldwide COVID pandemic has caused a great www.rcpch.ac.uk/key-topics/child-protection
deal of often unrecognised distress to both adults (accessed 21 January 2022).
and children, and somatic manifestations and

3 CHILDREN WITH
SPECIAL NEEDS
INTRODUCTION
All children are special. Some have unique medi- neurological impairment, attention defcit hyperac-
cal or developmental difculties which create needs tivity disorder (ADHD) and ASDs.
in addition to those of their age-matched peers.
Te term ‘special needs’ encompasses a huge range Children in the ORL clinic ofen have a ‘syndrome’ or
including children with mild learning disability, a series of medical issues that constitute a ‘sequence’
developmental delay, severe motor and sensory or an ‘association’.
SYNDROMES, SEQUENCES AND ASSOCIATIONS

A syndrome is a group of birth defects with a sin- Down syndrome (trisomy 21)
gle (usually genetic) cause, e.g. Down syndrome
(trisomy 21). Te genetic anomaly in Down syndrome is the pres-
ence of an extra chromosome 21, hence the term ‘tri-
In a sequence, the clinical features are due to a somy 21’ (Figure 3.1). Children with Down syndrome
single anatomical abnormality, e.g. in Pierre Robin are prone to a variety of ENT pathologies. Tey have a
sequence, it is the hypoplasia of the lower facial skel- high incidence of OSA due to muscle hypotonia, rela-
eton that causes micrognathia, tongue base prolapse tive macroglossia due to a mid-facial hypoplasia, and
and palatal defects. large obstructing tonsils and adenoids. Congenital
heart disease is more prevalent than in age-matched
An association is a group of defects that commonly peers, and OSA can precipitate pulmonary hyper-
occur together, without a specifc known cause, e.g. tension, a serious and potentially devastating fea-
VATER, CHARGE (see below). ture of trisomy 21. Airway problems are common
and include tracheo-oesophageal fstula (TOF), tra-
Some syndromes, sequences and associations with cheomalacia and subglottic stenosis (SGS). Other
ORL manifestations are listed in Box 3.1. ENT issues include deafness, both sensorineural
DOI: 10.1201/9780429019128-3 Children with Special Needs 11

Box 3.1 Some syndromes,
sequences and associations with ORL
manifestations
Syndromes
• Down syndrome (trisomy 21)
• Turner’s syndrome
• Treacher Collins syndrome
• Goldenhar’s syndrome
• Crouzon’s syndrome
• Apert’s syndrome
• 22Q11 deletion syndrome
Figure 3.1 A girl with Down syndrome (trisomy 21).
• CHARGE syndrome
• Syndromes causing congenital deafness
Sequences and associations

• Pierre Robin sequence
• VATER association
• CHARGE association
and conductive. A small external ear canal, a high

incidence of otitis media with efusion (OME) and
anatomical abnormalities of the ossicles, cochlea and
facial nerve are all well documented. Tyroid pathol-
ogy is also more common.
Figure 3.2 A baby with Treacher Collins syndrome.
Children with Down syndrome ofen feature on
Note she has microtia and micrognathia. Tracheotomy
ENT operating lists and the surgeon and anaesthetist was performed soon after birth.
need to be aware of some particular issues relating to
perioperative care. For example, a child with Down
syndrome will ofen need a smaller-sized ET tube
than their age-matched peer and have a tendency for Treacher Collins syndrome
atlantoaxial instability, so the child’s head has to be
manipulated with special care. Tis is now known to be caused by a gene defect,
most commonly in the TOCF1 gene which codes
for a protein known as ‘treacle’. It is inherited in
Turner’s syndrome an autosomal dominant pattern but with vary-
ing severity. ENT features include abnormalities
Girls with this syndrome have the sex chromosome of the pinna, microtia, conductive deafness, clef
make-up XO. ENT issues are common and include palate, choanal atresia, and mandibular and max-
both sensorineural deafness and conductive deaf- illary hypoplasia. Micrognathia can cause severe
ness, a high incidence of clef palate and abnormali- airway problems necessitating a tracheostomy
ties of the pinna. (Figure 3.2).

Craniofacial syndromes contrast with the usual advice to put babies on their
backs – ‘back to sleep’. Tey may need a nasopha-
Conditions such as Goldenhar’s, Crouzon’s, Pfeifer’s ryngeal airway but the airway improves as the baby
and Apert’s syndromes are characterised by cranio- develops and the mandible grows. Nowadays, trache-
facial dysmorphism, ofen with hemifacial microso- ostomy is very rarely needed.
mia, craniosynostosis (premature fusion of the skull
sutures), microtia, mandibular and maxillary hypo-
plasia, clef palate and ofen severe airway problems VATER (VACTERL) association
needing tracheostomy.
This association, characterised by vertebral, ano-
rectal, cardiac, tracheal, esophageal, renal and
Sensorineural hearing loss limb abnormalities, is not uncommon in paedi-
atric practice and some of these babies may also
A number of specifc syndromes (e.g. Waardenburg’s, have ENT conditions as well (e.g. tracheomalacia,
Pendred’s, Alport’s and Jervel/Lange Neilson syn- SGS).
drome) are known causes of profound sensorineural
hearing loss and are seen in cochlear implant clinics.
CHARGE
22Q11 deletion syndrome (formerly known as
CHARGE is characterised by coloboma (an ocular
DiGeorge syndrome) is increasingly recognised as a
anomaly), heart defects, atresia of the choanae,
cause of sensorineural deafness.
growth defects (often genital hypoplasia), renal
abnormalities and ear defects. ENT surgeons
Pierre Robin sequence often diagnose the condition as it presents with
severe airway obstruction in the newborn due
Tis is characterised by mandibular hypoplasia, clef to choanal atresia (see Chapter 22). CHARGE is
palate and micrognathia. Glossoptosis – a tendency now regarded as in some cases a syndrome rather
of the tongue to prolapse back into the airway – con- than an association, as a range of specific genetic
tributes to airway obstruction. Feeding problems are causes have been identified, notably a mutation of
common and these babies are best nursed prone in gene CHD7.
NEURODEVELOPMENTAL CONDITIONS
ORL specialists are seeing children in increasing become very upset if they have to wait for long peri-
numbers with ASD. Tis is a complex and, as yet, ods. Tere is no ‘typical’ pattern with ASD and each
incompletely understood spectrum of develop- child has diferent features, needs and responses.
mental conditions characterised by impaired social Close liaison with the anaesthetist, ward staf and
interaction and communication, ofen with repeti- theatre staf but especially the parents to plan admis-
tive behaviour patterns. It varies greatly in severity sion and discharge will make for a much happier
and is especially important in ORL as children may experience for all.
present with language delay and the diagnosis is ofen
delayed. Te reason for the greatly increased preva- ADHD is an umbrella term used to describe a series
lence is unclear, but greater awareness and wider of behavioural conditions associated with hyperac-
diagnostic criteria are probably important. Children tivity, impulsiveness, a poor attention span and ofen
with ASD need particularly sensitive management disruptive behaviour. Suspected or actual hearing
if they are scheduled for surgery as some may fnd loss and poor sleep patterns can be reasons for pre-
the company of other children distressing and may sentation to ORL. ASD and ADHD are very diferent
Children with Special Needs 13

KEY POINTS
● One of the paradoxes of looking afer children is that, despite their smaller size, they require more
clinic space than adults. Tis is especially true in children with special needs.
● ADHD is characterised by hyperactivity, impulsivity and inattention, but many children will
exhibit these characteristics in varying degrees.
● Children with ASD may present to the ORL clinic with delayed speech and apparent hearing loss.
● Be sensitive with your language when dealing with the parents of children with syndromes. Refer
to the child frst and the condition later. Parents prefer the term ‘a child with Down syndrome’ to
‘a Down syndrome child’. When comparing the progress of a child with a syndrome to another
child, avoid the use of terms such as ‘normal’. A child with Down syndrome, for example, is more
likely to have middle ear efusions than a ‘typically developing child’ rather than a ‘normal’ child.
Attention to such subtleties makes for a far better rapport with parents.
● Children with syndromes usually need intensive multidisciplinary input but are increasingly seen
in ENT clinics. Many will have cochlear implants (CIs), bone-anchored hearing aids (BAHAs) and
tracheostomies.
● Parents are usually very familiar with the features of their child’s syndrome and may be members
of one of a number of patient/parent support groups.
conditions but they may coexist, and children sus- Chin CJ, Khami MM, Husein M. A general review
pected of either will need referral to the appropriate of the otolaryngologic manifestations of Down
paediatric or psychology team for skilled assessment Syndrome. Int J Pediatr Otorhinolaryngol. 2014;
and diagnosis. 78(6):899–904. doi: 10.1016/j.ijporl.2014.03.012.
Down’s Syndrome Association (DSA). Available at:
http://www.downs-syndrome.org.uk/for-new-
parents/ (accessed 21 January 2022).
FURTHER READING National Organization for Rare Disorders (NORD).
British Association for Paediatric Otolaryngology Available at: https://rarediseases.org (accessed
(BAPO). Available at: https://www.bapo.co.uk/ 21 January 2022).
(accessed 21 January 2022).

4 ENT FOREIGN BODIES
INTRODUCTION
Children are curious. Tey like to explore and nursery friend may. Te child will not always volun-
experiment, ofen putting objects in places they teer or admit what has happened, hence the need for
shouldn’t – including in their ears, noses and throats. vigilance when there is any suspicion.
If they don’t do this themselves, a sibling or school or
EAR
Toys, pieces of crayon, beads, sponge, organic matter removal. Use a ‘grasping’ instrument, ‘crocodile’ or
such as food particles and even live insects can be ‘alligator’ forceps, under good lighting conditions
found in the ear canal. Te parent or carer may wit- and with the child relaxed and quiet if the object has
ness the child putting something in their ear, or an an irregular edge. A spherical object (e.g. a bead) is
object can be found incidentally on otoscopy. Ofen, better removed using a curved or hooked instrument
there is no pain or discharge, but organic matter can introduced to be able to get behind the object and
become infected. Wax tends to accumulate around gently withdraw it. If the child is fractious or very
the object and may become impacted. Gentle suction nervous, you may need to arrange a general anaes-
under vision (microscopy) or syringing with warm thetic. Live insects can cause intense distress; immo-
water may be enough, but if the object is impacted, or bilise the insect by flling the ear canal with a local
very deep in the ear canal, it will need instrumental anaesthetic (lidocaine) prior to removal.
NOSE
Nasal foreign bodies ofen pass into the pharynx sees the event or the child admits it, but ofen the
and are harmlessly swallowed. Impacted objects object stays impacted in the nose for days or weeks
include crayons, chalk, beads, sponge and small until it causes infection, a discharge (sometimes foul-
toys. Presentation can be early if the parent or carer smelling), with bleeding and excoriation of the skin
DOI: 10.1201/9780429019128-4 ENT Foreign Bodies 15

around the nostril (Figure 4.1). Remove the object if
you can in the outpatient setting using a good light
and suitable instrument. A hooked or curved instru-
ment is best, otherwise the object may be pushed fur-
ther back. A fractious or nervous child, especially if
the object has been in for some time and has become
crusted and adherent, will need a general anaes-
thetic. Tis is best arranged soon, ideally within a
day or so of seeing the child. If the child is otherwise
well and has no neurological issues (i.e. has a good
swallow and no tendency to aspiration), the risk of Figure 4.1 Excoriation of the skin around the nostril
inhalation is extremely low. caused by a retained nasal foreign body.
INGESTED FOREIGN BODIES

Coins, toys and small household objects including food bolus or a large coin can compress the trachea
most dangerously ‘button batteries’ are easily swal- and cause acute airway obstruction. A sharp object,
lowed especially by toddlers who are inclined to a pin or nail, can cause oesophageal perforation. An
explore and very ofen put objects into their mouths. impacted pharyngeal or oesophageal foreign body
Many are innocuous and pass into the stomach and will need to be removed under general anaesthe-
beyond with no ill-efects, but some may impact at sia, ideally within a few hours. Button batteries are
the level of the vallecula, cricopharyngeus or mid- especially destructive, with the capacity to erode the
oesophagus. Tere may be a defnite history, and the oesophageal mucosa and cause catastrophic bleeding
child will complain of difculty with swallowing, but in the mediastinal great vessels so need immediate
presentation can be delayed. Some objects such as a removal, even if the child has a full stomach.
INHALED FOREIGN BODIES

Babies and toddlers may inhale various objects they
put in their mouths, sometimes with devastating
consequences. Te event is not always witnessed, and
coughing, choking, shortness of breath and sudden
onset of stridor or ‘wheezing’ may be the frst signs
something is amiss. An object impacted in the lar-
ynx or trachea can be fatal. Immediate frst-aid mea-
sures at the time (e.g. a series of sharp blows to the
supine baby’s back or in an older child a ‘Heimlich’
manoeuvre) can be lifesaving.
A foreign body that passes into the bronchus

will cause some degree of oxygen desaturation,
but the child will usually breathe well using the
other lung. Not all foreign bodies are radiopaque, Figure 4.2 Chest X-ray showing obstructive
but a chest X-ray is nevertheless helpful and may emphysema.

Figure 4.3 Optical forceps for removal of a bronchial foreign body.
show hyperinfation of the lung on the side of the bronchoscopes, a series of optical ‘grasping’ forceps
obstruction due to reduced airfow in expiration (Figure 4.3) and postoperative recovery and care
(‘obstructive emphysema’, Figure 4.2). If there is facilities. Organic objects, such as peanuts and food
reasonable suspicion of a bronchial foreign body, particles, cause a local infammatory reaction and,
arrange airway endoscopy (tracheobronchoscopy) if any foreign body has been present for a prolonged
quickly, but with care to ensure that the surgeon, period, recovery may be complicated by the devel-
anaesthetist and nursing team have the appropri- opment of long-term bronchopulmonary disease
ate experience and equipment including ventilating (bronchiectasis).
CAUSTIC INJURIES
Caustic agents such as detergents, bleach, oven- tragic accidents still occur. If caustic ingestion is
cleaning fuid and various household cleaning sub- suspected, admit the child urgently and consider
stances may be ingested by toddlers. Devastating early and careful endoscopy. Complications include
injuries to the mouth, pharynx, larynx and oesoph- oesophageal stenosis, laryngotracheal cicatrisation
agus can result. Public health measures such as and scarring of the tissues of the mouth and phar-
safer containers and increased awareness of the ynx, sometimes requiring gastrostomy feeding and
dangers have greatly reduced the incidence, but long-term tracheostomy.
BUTTON BATTERIES
‘Button batteries’ are ubiquitous now in household delayed and occur long after the battery has been
electronic devices, hearing aids and some toys removed.
(Figure 4.4). They are extremely powerful and
can be quickly destructive if they come in con- If you suspect a child has ingested a button battery,
tact with tissues. In the ear canal, they may cause make immediate arrangements to get them to an
intense inf lammation with bone erosion and they operating theatre for pharyngo-oesophagoscopy and
can cause septal perforation and long-term steno- removal. Tere is some evidence that oral administra-
sis in the nose. A battery is especially destructive tion of honey may be a helpful frst-aid measure. Te
if swallowed when it may impact at the cricopha- degree of urgency is such that the child should have
ryngeus or mid-oesophagus and erode through a general anaesthetic even with a full stomach, as the
the lumen. Fatal mediastinal bleeds have been balance of risk of tissue destruction from the battery
reported, and prolonged oesophageal stenosis is versus the hazards of possible aspiration of stomach
not uncommon. The destructive effects can be contents is such as to warrant immediate endoscopy.
ENT Foreign Bodies 17

(a) (b)
Figure 4.4 (a) Button batteries (coin shown to illustrate size) and (b) some common household items that require
button batteries.
FURTHER READING
KEY POINTS Houston R, Powell S, Jafray B, Ball S. Clinical
guideline for retained button batteries. Arch
● Don’t persist in the emergency department Dis Child. 2021;106(2):192–4. doi: 10.1136/
(ED) trying to remove a nasal or ear for- archdischild-2019-318354.
eign body if the child is fractious or dis- Shafer AD, Jacobs IN, Derkay CS et al. Management
tressed. Refer to the ENT clinic or arrange and outcomes of button batteries in the
a general anaesthetic. aerodigestive tract: a multi-institutional
● Modern ‘button batteries’ are very pow- study. Laryngoscope. 2021;131(1):E298–E306.
erful and can cause severe destruction of doi: 10.1002/lary.28568.
tissues. If you suspect a child has ingested
or inhaled a button battery, get the child
to an operating theatre for removal of the
battery without delay – even if the child
has a full stomach.

5 SEPSIS IN THE HEAD
AND NECK
INTRODUCTION
Te ears, nose and throat are the entry portals to the and fulminant mediastinitis may be secondary to an
upper respiratory tract and are subject to a range infection that started in the upper respiratory tract,
of infections which can spread to adjacent areas – and haematogenous spread of pyogenic organisms
notably the fascial spaces of the neck, the periorbital can cause devastating systemic sepsis.
tissues and the mastoid air cells. Intracranial sepsis
DEEP NECK SPACE INFECTIONS

Te fascial layers of the neck – the superfcial and
deep cervical fascia – are distributed so as to cre-
ate a number of potential spaces where abscesses can
collect and expand (Figures 5.1 and 5.2) and deep
neck space (DNS) infections can develop. Infection
in the oropharynx, especially the tonsil, can easily
spread to cause suppuration in the parapharyn- Pharyngeal wall
Parotid gland
geal space, the peritonsillar space or, in very young
children, the retropharyngeal lymph nodes. Dental Parapharyngeal
space
infections can spread to the submental and subman-
Digastric and
dibular space. Te fora are mainly the pyogenic Tonsil
stylohyoid muscles
(pus-producing) organisms – Streptococcus pneu-
moniae, Staphylococcus aureus and Haemophilus Sternocleidomastoid
Greater cornu muscle
infuenzae – but anaerobes and unusual organisms of hyoid bone
may be implicated.
An abscess in the parapharyngeal space produces a Internal carotid

tense, painful swelling in the side of the neck in a artery, jugular vein
febrile child, with erythema and cellulitis of the skin.
Admit the child, commence IV hydration, antibiotics, Figure 5.1 Neck spaces – coronal view.
DOI: 10.1201/9780429019128-5 Sepsis in the Head and Neck 19

Retropharyngeal
space
Lateral
parapharyngeal
space
Sublingual Submental (a)

space space
Figure 5.2 Neck spaces – lateral view.
analgesia as required and measure baseline observa-

tions. If the child is especially toxic or there is no
early response to antimicrobial therapy, arrange for
incision and drainage under general anaesthetic. Te
abscess is fuctuant in the early stages and incision
and drainage are likely to allow pus to escape, but
the abscess may have become frm and indurated and (b)
organised such that there is little or no pus (a phleg-
mon). Ultrasound scanning can help by showing the Figure 5.3 (a,b) Parapharyngeal abscess. The
fuid. A computed tomography (CT) scan may be abscess has become organised (phlegmon).
helpful especially if it can be undertaken without a
general anaesthetic (Figure 5.3).
Abscess Larynx
A retropharyngeal abscess occurs typically in Sternomastoid Pharynx
younger children (up to the age of about 3 years)
who tend to have prominent lymph nodes in the
retropharyngeal space. Te child will fex their neck
and extend the head; dysphagia or a painful swallow
causes drooling. Stertorous breathing suggests some
degree of airway obstruction, and the child is at risk
of asphyxia.
Treatment is initially with systemic antibiotics, pro-

gressing to incision and drainage if there is no rapid Cervical spine
resolution. Anaesthesia in these children can be dif-
cult as the pharyngeal swelling makes ET intubation Figure 5.4 Retropharyngeal abscess.
especially challenging. Tey ofen need to be trans-
ferred to a specialist paediatric unit for transoral antimicrobial therapy may sufce, but a large abscess
incision and drainage (Figure 5.4). will require incision and drainage. Some surgeons
advocate ‘quinsy tonsillectomy’ – a ‘hot’ tonsillec-
Peritonsillar abscess (quinsy) is mainly a condition tomy – particularly if the child would need a general
of older children and young adults. If treated early, anaesthetic for incision and drainage.

Suppuration in the f loor of the mouth can DNS infections usually resolve with a good outcome,
complicate a dental infection. Incision and drain- but infection may spread to the mediastinum, along
age may be very unrewarding as there is extensive the carotid sheath, and to the intracranial structures.
cellulitis with induration of the tissues (Ludwig’s Haematogenous spread can give rise to septicaemia
angina). with devastating consequences.
MASTOID ABSCESS
Acute otitis media (AOM) invariably involves some Intense IV antimicrobial therapy may sufce in
degree of infammatory changes in the mucosa of the the early stages, but a fuctuant abscess may need
mastoid air cells. Redness of the skin and a fuctuant drainage, to include a cortical mastoidectomy and
swelling of the tissues over the mastoid process (Figure an extensive myringotomy to allow pus to escape
5.5) suggest that the infection has escaped the bony con- from the middle ear. Te diagnosis is clinical but a
fnes of the temporal bone and formed a sub-periosteal CT scan may delineate the extent of the abscess and
abscess which, if unchecked, will rupture through the help with surgical planning.
periosteum, involve the venous sinuses (venous sinus
thrombosis) and extend beyond the dura potentially to Gradenigo’s triad/syndrome – VI nerve palsy, pain
cause serious neurological complications. in the distribution of the trigeminal nerve and otitis
Figure 5.5 Acute mastoiditis with sub-periosteal abscess.
Sepsis in the Head and Neck 21

media – occurs when an abscess forms in the septic thrombosis of the internal jugular vein, usu-
petrous apex. Bezold’s abscess is a collection of pus ally secondary to mastoiditis due to the bacterium
in the neck due to tracking of infection behind Fusobacterium necrophorum.
the sternomastoid. Lemierre’s syndrome is a
ORBITAL CELLULITIS
Paranasal sinus infections can be complicated by the orbit (orbital cellulitis) ensue and may progress
spread beyond the bony confnes of the sinuses. to abscess formation with proptosis requiring urgent
Frontal sinus abscess (Pott’s pufy tumour) is now decompression (incision and drainage usually via an
very rare. Sinogenic intracranial abscess is uncom- external approach at the medial canthus) or even, in
mon but orbital sepsis is still a frequent mode of pre- extreme cases, to cavernous sinus thrombosis with
sentation of children with sinus infection. Infection ophthalmoplegia and a serious risk to vision. Admit
easily breaks through the thin party wall between the child, commence IV hydration, antibiotics, and
the ethmoid sinuses and the orbit (the lamina papy- consider surgery if there is an abscess.
racea). Swelling and erythema of the sof tissues of
INTRACRANIAL COMPLICATIONS
Otitis media, mastoiditis and sinusitis can be com- or focal neurological signs should raise suspicion.
plicated by spread of infection beyond the dura Imaging is especially helpful here. Te child with
(Figures 5.6–5.8). Severe headache, pain out of pro- suspected intracranial sepsis needs urgent admis-
portion to the otoscopic fndings, extreme systemic sion, antimicrobial therapy and neurosurgical
toxicity and, most of all, alteration in consciousness review.
5
3 4
6
7 Figure 5.6 Some potential complications of otitis media:
1
1. Mastoiditis; 2. Petrous apex abscess; 3. Extradural
abscess; 4. Intracranial abscess – cerebral or cerebellar;
5. Subdural abscess; 6. Labyrinthitis; 7. Venous sinus
8 thrombosis; 8. Facial palsy.

Figure 5.7 Frontal lobe sinogenic abscess. Figure 5.8 CT scan showing otogenic intracranial
abscess.
SYSTEMIC SEPSIS
Tere is increasing recognition that children can ideally in consultation with an experienced paedi-
develop a life-threatening, rapidly progressive atric team.
exaggerated infammatory response to infection.
Tis has come to be known as ‘sepsis’ and is a
time-critical emergency that warrants immediate
intervention. Children can deteriorate alarmingly KEY POINTS
quickly, and an apparently healthy baby with what
initially seems a mild respiratory infection may ● Te ears, nose and throat are the entry
become moribund in a short period. If you have any portals to the upper respiratory tract and
worries regarding a child’s worsening condition, be are subject to a range of infections which
mindful of the local arrangements with regard to can spread to adjacent areas.
paediatric early warning signs (PEWS), monitor the ● Sepsis is a time-critical emergency.
child’s condition carefully and commence antimi- Children can deteriorate very quickly.
crobial and fuid replacement therapy immediately,
Sepsis in the Head and Neck 23

Patel S, Burgess A. Guideline for the child presenting
FURTHER READING to hospital with lymphadenitis or a lymph node
https://qicentral.rcpch.ac.uk/resources/systems-of- abscess. Available at: www.entuk.org/paediatric-
care/recognition-and-response-to-sepsis-in- guidelines (accessed 21 January 2022).
the-paediatric-emergency-care-setting/ Royal Patel S, Burgess A. Guideline for the child presenting
College Paediatrics and Child HealthGuidelines to hospital with tonsillitis or quinsy. Available at:
re sepsis in children, May 2020 (accessed 29 www.entuk.org/paediatric-guidelines (accessed
March 2022). 21 January 2022).
Patel S, Burgess A. Guideline for the child present- Patel S, Burgess A, Marsh C. Guideline for the child
ing to hospital with otitis media or mastoiditis. presenting to hospital with pre-septal or post-
Available at: https://www.entuk.org/paediatric- septal cellulitis. Available at: www.entuk.org/
guidelines (accessed 21 January 2022). paediatric-guidelines (accessed 21 January 2022).

6 THE EXTERNAL EAR
DEVELOPMENT OF THE EAR

Te external ear includes the pinna and the external the frst two branchial (pharyngeal) arches fuse to
ear canal. Te pinna and the outer part of the ear become the external ear, and developmental abnor-
canal are made of cartilage, covered by perichon- malities are fairly common.
drium and skin. A series of ‘hillocks’ derived from
CONGENITAL ANOMALIES
‘Minor’ anomalies
These include skin tags, pre-auricular sinuses,
appendages, cysts and ‘accessory auricles’. Tey can
be upsetting for parents but can usually be treated
surgically if they give rise to aesthetic concerns or
recurrent infection. A ‘pre-auricular sinus’ is a small
blind-ending pit, lined with squamous epithelium
and lying just in front of the pinna. Tis is usually
innocuous but can become infected; it is easily
removed by wide local excision, ideally when the
child is about 2 years old (Figure 6.1). A ‘pre-auricu-
lar sinus’ is not to be confused with a branchial or
‘frst arch’ abnormality, which is usually lower (below
the tragus), and can be a marker for a complex tract
(fstula) running into the ear canal (see Chapter 26).
Some congenital abnormalities, including the not-
uncommon ‘accessory auricle’, are shown in
Figure 6.2.
Figure 6.1 Pre-auricular sinus.
DOI: 10.1201/9780429019128-6 The External Ear 25

(a) (b) (c)
Figure 6.2 Congenital external ear abnormalities: (a) Deformity of the pinna with accessory auricle; (b) acces-
sory auricle with microtia (c) accessory auricle with normal pinna.
(a) (b)
Figure 6.3 (a,b) Prominent ear with poorly developed anti-helical fold.
Prominent ears of about 4 to 5 years as ideal, before the child is set-

tled in school. Tere are multiple surgical techniques
‘Protruding ears’ (ofen unkindly referred to as ‘bat to re-contour the anti-helical fold. Careful preopera-
ears’) are a common reason for referral of a child tive planning, including clinical photography and
to an otolaryngology or plastic surgery clinic. Te detailed counselling of the parents regarding the
child is ofen subject to bullying and name-calling expectations of surgery, is important.
and may become very distressed. Te typical defor-
mity is a poorly developed or absent anti-helical fold Microtia
(Figure 6.3). If the parents of a newborn baby pres-
ent, some degree of moulding of the cartilage can be More serious abnormalities of development range
brought about by the use of specially designed splints from microtia (literally a ‘small ear’) to anotia, which
(EarBuddies™, Figure 6.4) but, in older children, the is absence of all of the structures that make up the
treatment is surgical. Most authorities regard the age external ear (Figure 6.5).

Figure 6.4 Splint to correct prominent ear. Figure 6.5 Baby with high-grade microtia
Microtia can be unilateral (far more common, 4 : 1) or decision making. Surgery is ofen best delayed until
bilateral, and has a prevalence of 2.5 in 10 000 births. the patient is old enough to contribute to the decision-
In addition to the external ear deformity, there may making process, which will afect the child for the
be canal atresia or stenosis. Tere can be associated rest of their life.
ossicular, middle ear and inner ear abnormalities.
Te issues are both aesthetic and functional; associ- Immediate management should focus on optimis-
ated hearing loss is common but not universal. ing hearing. Babies with microtia need a detailed
and skilled hearing assessment within the frst
In the majority of cases, microtia presents as an iso- few weeks of life. Both air and bone conduction
lated deformity in an otherwise healthy child, but it thresholds should be determined, and a plan can
can be part of a spectrum of abnormalities in syn- then be made for early audiological rehabilitation
dromes such as branchio-oto-renal syndrome, hemi- if needed.
facial microsomia, Goldenhar syndrome and Treacher
Collins syndrome. Maternal rubella infection – now In microtia with canal atresia, there is usually a
uncommon due to widespread vaccination – has severe conductive hearing loss on the afected side.
been considered a factor in microtia. Some drugs in Inner ear function tends to be good, resulting in
pregnancy such as thalidomide (no longer used) and some ability to hear on the afected side. Te hear-
isotretinoin (sometimes given for acne) have also been ing in the contralateral ear is usually normal and,
implicated, but the aetiology is usually unknown. if so, parents can be reassured that speech and lan-
guage development should progress well. If there is
When a surgeon frst meets a baby with microtia, bilateral conductive loss, the baby can be ftted with
they will need to consider a long and hopefully bone-conducting hearing aids straight away. Te
rewarding relationship with the patient and fam- Sofband® (see Chapter 12, Figure 12.3) has proven
ily. Surgical expertise is best ofered in a team that popular and successful for use with younger chil-
should be able to provide audiological assessment dren and babies. Te child can then be considered
and rehabilitation as well as psychological support for bone-anchored hearing aids (BAHAs) when they
throughout the patient’s journey. Although there is are a little older.
sometimes a strong parental desire to expedite sur-
gery, the decision should be patient-led with as much Te baby should have a full medical paediatric assess-
information and support provided to help with the ment to include whatever investigations are deemed
The External Ear 27

appropriate to look for associated anomalies, (e.g. participates fully in the decision as to which route to
an abdominal ultrasound to screen for brachio-oto- take, with guidance from both clinician and parent.
renal syndrome).
Although there are psychological issues for the
Imaging (CT scan with or without magnetic reso- patient with microtia, most children do not seem to
nance imaging, MRI) can be lef to a later date but be unduly afected until the age of 6 or 7. Te other
can give important information regarding the struc- important reason to wait until the child is older
ture of the middle ear and the inner ear. before surgery is to allow the child to grow physically.
Te Sofband is also used in children with unilateral Pinna reconstruction using the patient’s own costal
microtia as the benefts of intervention in single- cartilage can produce excellent aesthetic results in
sided hearing loss are increasingly acknowledged. skilled hands. Tis usually happens at around 9 or 10
years of age. It is a procedure performed in at least two
Te child with microtia is at increased risk of middle stages scheduled around 6 months apart. Te alterna-
ear disease including cholesteatoma, which can be tive approach is to use a prosthesis, using percutaneous
difcult to pick up by otoscopy if there is canal steno- abutments to anchor the prosthesis to the skull.
sis. Imaging then becomes increasingly important,
and the child should have regular audiological and Modern prostheses, fashioned by a skilled techni-
otological surveillance. cian and customised to the child and the defect, will
give a realistic-looking pinna that exactly matches
A child with microtia presents to the ENT surgeon the opposite side. Te prosthesis has a fxed lifespan
within the frst few weeks or months of life, but a deci- and needs to be changed every 2 years. In addition, as
sion with regards to defnitive surgical correction is the skin–implant interface is prone to irritation, the
best lef for several years. Parents will be anxious and patient and family are committed to daily lifelong
will need careful counselling and support, including care of the implant site.
a full early discussion with a team experienced in the
management of this condition and what the possi- Correction of canal atresia and middle ear surgery for
bilities are. Te default option – and by far the best microtia are both highly specialised and technically
approach in the frst few years – is simply to observe, demanding. Te aim is to achieve good hearing, but
and plan intervention as needed when the child is the serious potential complications of such surgery
much older. include facial paralysis, sensorineural hearing loss,
and a re-stenosis requiring further surgery. With the
Te aesthetic deformity can be addressed by recon- excellent hearing results achieved by a BAHA – and
structive surgery, ofen using autologous graf mate- the increasingly better BAHAs becoming available –
rial, or a prosthetic implant. It is best if the child corrective surgery is now rarely indicated.
ACQUIRED PATHOLOGY
Infammation Syringing or gentle suction is usually efective in a
cooperative child, and hard wax can be sofened by
Skin disease such as eczema and psoriasis may mani- the use of warm olive oil drops for a week or so. True
fest as otitis externa. otitis externa is far less common in children than
in adults. It can occur following contamination with
Impacted cerumen can be problematic in children, water – particularly chlorinated or infected water
ofen made worse by enthusiastic parents who poke (swimmer’s ear) – requiring regular suction and the
objects in the ear in an attempt to remove the wax. use of local antimicrobial and steroid preparations to

Figure 6.6 Haematoma of the pinna.
keep it under control. An acute staphylococcal infec-

tion may produce a tense infammatory swelling Figure 6.7 Bruising caused by NAI.
(furuncle) as in adults. A streptococcal infection can
spread to the pinna to cause a painful erythematous studs, infection, and tears to the lobule if earrings are
swelling (erysipelas). Infection along the tissue planes forcibly removed.
of the cartilage (perichondritis) can, if unchecked,
give rise to fbrosis and deformity.
KEY POINTS
Trauma
Blunt trauma ● Prominence of the pinna (‘bat ears’) can
cause a great deal of distress to parents and
Blunt trauma to the pinna may cause subcutane- children.
ous bleeding leading to a haematoma (Figure 6.6). ● Improved prostheses have transformed the
Untreated, this can become infected with scarring management of microtia.
and deformity. Consider early evacuation and appli- ● Reconstructive surgery for microtia is best
cation of a pressure dressing. A haematoma may be deferred until the child is old enough to
the result of an injury sustained during sport or the make the decision.
‘rough and tumble’ of the children’s playground but
be alert to the possibility of non-accidental injury
(NAI). ‘Pinchmarks’ on the rim of the pinna are said
to be pathognomonic of NAI and are caused by rough
FURTHER READING
pinching of the pinna between the fnger and thumb
UK care standards for the management of patients with
when an adult grasps the child by the ear (Figure 6.7).
microtia and atresia. Available at: http://www.
bapras.org.uk/docs/default-source/commission
Penetrating trauma ing-and-policy/microtia-and-atresia--care-
standards.pdf?sfvrsn=2 (accessed 24 January
Penetrating trauma is uncommon. Complications of
2022).
ear-piercing are sometimes seen such as embedded
The External Ear 29

7 HEARING LOSS IN
CHILDHOOD
INTRODUCTION
Childhood deafness may be sensorineural, conduc- due to acquired childhood pathologies, mainly
tive, or mixed and includes auditory neuropathy and OME and chronic suppurative otitis media (CSOM)
auditory processing disorders. (see Chapters 9 and 11).
Permanent childhood hearing impairment hearing Despite improved screening and awareness of the
(PCHI) can be congenital or acquired. It can remain importance of early detection and rehabilitation,
stable, deteriorate progressively, fuctuate or mani- which have brought about greatly improved out-
fest later in life. Conductive loss due to middle ear comes, deafness in childhood remains a huge global
pathology tends to present later, and it is usually challenge.
INCIDENCE
Between one and two newborn children per thousand difculties. Tese fgures are even higher in the devel-
are born ‘deaf’, i.e. with a bilateral permanent child- oping world, where diagnosis is ofen late and rehabili-
hood hearing impairment (BPCHI). Tis is usually tation opportunities limited. Screening will detect the
defned as a hearing loss (HL) of at least 40 dB HL in great majority of babies with congenital deafness, but
the better hearing ear. About one-quarter of these chil- delayed-onset hearing loss, ofen caused by conditions
dren will have ‘profound’ hearing loss (see Chapter 8) present at or before birth (e.g. intrauterine cytomega-
and up to one-third will have signifcant associated lovirus), is well recognised, hence the need for ongoing
medical issues such as developmental delay or learning surveillance for children considered ‘at risk’.
AETIOLOGY
Te question ‘why is my baby deaf’ is of great con- diagnosis, and it is important – but not always pos-
cern to parents. Deafness is a manifestation of one sible – to determine an exact aetiology. Tis helps
or more pathological processes rather than a fnal to outline prognosis and may facilitate specifc
DOI: 10.1201/9780429019128-7 Hearing Loss in Childhood 31

Congenital hearing loss
Environmental
• ‘TORCHES’ infections Genetic
• Ototoxicity
• Perinatal hypoxaemia
• Hyperbilirubinaemia
Syndromic Non-syndromic
• Usher’s syndrome • Single-gene defects
• Waardenburg’s (e.g. connexin 26)
syndrome • X-linked and
• Pendred’s syndrome mitochondrial defects
Details can be found at

www.rarediseases.org
Figure 7.1 Causes of congenital hearing loss.
treatment strategies e.g. antiviral medication in some new gene mutations causing non-syndromic deaf-
limited circumstances, and perhaps precise genetic ness. Mutations in the gene that codes for connexin
interventions in the future. A defnitive diagnosis 26 – a gap junction protein – are a particularly
may also enable referral to appropriate specialists common fnding in permanent congenital hearing
e.g. endocrinologists for Pendred’s syndrome, and impairment (PCHI).
genetic counselling services.
Te maternal infections that may be associated with
Congenital (present at birth) hearing loss is tradition- PCHI are ofen referred to by the acronym TORCHES
ally divided into genetic (or inherited) causes – more (Toxoplasmosis, Other, Rubella, Cytomegalovirus,
ofen than not nowadays with a known chromosomal and Herpes Simplex). Toxoplasmosis and rubella are
or gene abnormality – and environmental causes such now rare in the developed world, but cytomegalo-
as prenatal maternal infection or birth complications virus (CMV) in pregnancy is not at all uncommon
such as prolonged hypoxaemia (Figure 7.1). Overall, and about one in a hundred newborns will have some
about 50% of cases of congenital deafness are geneti- evidence of CMV infection. Only a small number of
cally inherited. About another 25% are due to a non- these (about 5%) will have PCHI but, as the hearing
genetic, environmental or acquired pathology. loss may not manifest for the frst few weeks, these
cases may be missed by newborn screening proto-
In about one-third of the inherited or genetic cases, cols and babies with CMV should be referred for
the hearing loss is one manifestation of a ‘syndrome’ full audiological evaluation and surveillance. CMV
(see Chapter 3), known as syndromic hearing loss is now the commonest intrauterine infection in
(see Chapter 8). Most of the remainder are due to humans, and up to 80% of women of reproductive
recessive genes, a small number are due to dominant age are seropositive. Some 25% of cases of congenital
genes, and a very small number are due to X-linked hearing loss are now associated with CMV infection.
or mitochondrial defects. Non-syndromic genetic Polymerase chain reaction (PCR) testing of the baby’s
causes of deafness are highly variable, with diferent saliva or urine in the frst 3 weeks of life will confrm
and ofen unpredictable inheritance patterns. the diagnosis of congenital infection. Tere is some
evidence to support the use of antiviral agents (val-
Te specifc genetic defect is not always estab- gonociclovir) for congenital CMV but only in chil-
lished, but improved detection techniques and gene dren with disseminated infection. Trials of antiviral
sequencing are leading to the regular reporting of therapy for CMV-associated hearing loss are ongoing.

SCREENING AND SURVEILLANCE
Screening is the testing of individuals among an
apparently disease-free population to identify those
who warrant further investigation to diagnose a spe-
cifc condition, in this case PCHI. Surveillance for
hearing loss is focused on ongoing regular testing and
support to include children who may have ‘passed’
the newborn screen but are still at risk of later-onset
PCHI. Some of the conditions that warrant surveil-
lance include babies ‘at risk’ due to conditions such
as extreme prematurity, very low birth weight, severe
hyperbilirubinaemia, maternal TORCH infection,
ototoxic medication and meningitis.
Most western healthcare systems have introduced

‘universal’ newborn screening (i.e. of every newborn
baby regardless of risk factors), typically by mea-
suring otoacoustic emissions (OAEs, Figure 7.2). Figure 7.2 Baby undergoing ‘screening’ by detection
Tese are measurable acoustic responses generated of OAEs.
in the outer hair cells. Te test is easy to perform
and relies on inserting an ear tip with a micro-
phone and a speaker and recording the response microtia, neonatal meningitis or CMV where the risk
to an acoustic signal. OAEs are less reliable if there of PCHI is high but it may not be picked up by OAEs.
is middle ear fuid. Auditory brainstem response Many healthcare systems have an additional ‘screen-
(ABR) tests rely on picking up electrical signals on ing’ at the time of school entry, typically around fve
the skin in response to an auditory stimulus. Tis years of age, using Pure Tone Audiometry or an age-
usually requires general anaesthesia in the newborn, appropriate hearing test. Many children with OME
but it may be needed in some circumstances such as (Chapter 9) are identifed in this way.
EARLY INVESTIGATIONS
Investigative protocols differ in different health- planning treatment. Genetic counselling may also
care systems. Once a baby has ‘failed’ the screen- be appropriate.
ing test, early audiological evaluation is essential
to confirm or refute (false positive) the diagnosis. Full audiological and otological assessment and
Once the diagnosis is confirmed, parents will medical evaluation by an experienced paediatric
need intensive and ongoing support for what is a team is essential. Genetic testing, CMV testing,
devastating and life-changing diagnosis for their and imaging (CT and/or MRI scanning) are usu-
newborn baby. Early involvement of an experi- ally arranged. More detailed investigations may
enced multidisciplinary team makes for the best be needed depending on the fndings, and in con-
outcomes. sultation with the parents, so that a management
plan can be put into efect as soon as possible. Te
Initial investigation should focus on determining purpose of a screening programme is to ensure
aetiology, identifying associated conditions, and early intervention, and the most efective early
Hearing Loss in Childhood 33

Figure 7.3 Audiogram in a case of functional hearing loss.

intervention is amplifcation, initially with use of Auditory processing disorders
one or more usually two hearing aids. Enormous
technological advances in the design and manu- Variously referred to as auditory neuropathy or
facture of hearing aids have taken place in recent auditory dysynchrony (AD), the preferred term is
decades, and children beneft from hearing aids auditory neuropathy spectrum disorders (ANSDs).
from as early as the frst few weeks of life, i.e. as Tis is an ‘umbrella’ term for a group of conditions
soon as they can be ftted. characterised by normal OAEs with normal or near
normal ABRs. Older children show normal or near-
normal PTA but have difculty processing normal
Unilateral hearing loss speech. Te pathology is uncertain, it is thought
to relate to auditory nerve dysfunction but with a
Traditional teaching that one ear was entirely
normal cochlea. Te range of conditions encoun-
sufcient for optimum development of language
tered is probably far more complex, in most cases,
and communication skills is misplaced. Single-
the aetiology is unknown, and many cases are now
sided deafness (SSD) warrants early identifcation
acknowledged to have a genetic basis. Tere is a high
and active intervention to ensure these children
incidence of comorbidity, including attention defcit
maximise their potential. If recognised, SSD may
disorder (ADD), ASDs, and apraxia and developmen-
warrant amplifcation (usually with conventional
tal delay. Amplifcation may help following rigorous
hearing aids) and ongoing surveillance, includ-
audiological evaluation, but these children require
ing awareness and support in the home and school
intensive support with early language intervention
environment.
by skilled teachers to achieve their full potential.
‘Functional’ hearing loss

This is probably a better term than ‘non-organic
hearing loss’ as the latter is sometimes used to KEY POINTS
refer to subjects who feign hearing loss for finan-
cial gain (e.g. compensation for alleged noise- ● Te outcomes for babies with congenital
induced hearing loss). These children are not hearing loss have greatly improved, but
‘feigning’ deafness and it is counterproductive hearing impairment remains a signifcant
in the extreme to suggest they are. Adolescents, worldwide cause of disability.
more often girls, sometimes present with reported ● Early recognition and intervention are
hearing loss, often ‘confirmed’ at pure-tone essential to ensure optimum outcomes for
audiometry (PTA), but with what seems normal deaf children.
responses to conversation and normal speech ● Children ‘at risk’ of congenital hearing
discrimination in everyday life and without any loss who pass the newborn screening test
evidence of ear or other auditory pathology. ABRs should be considered for ‘surveillance’ as
are normal (Figure 7.3). There may be a back- they may present with later-onset hearing
ground history of psychological distress, anxiety, loss.
high academic expectation, school problems or ● SSD should be recognised so children can
bullying. Treatment is expectant and improve- be referred early for investigation and
ment is usual but the child and family may need rehabilitation.
psychological referral and support.
Hearing Loss in Childhood 35

nhs.uk/conditions/baby/newborn-screening/
FURTHER READING hearing-test/ (accessed 24 January 2022).
Liming BJ, Carter J, Cheng A et al. International Nicolas S, Gallois Y, Calmels MN. Quality of life
Pediatric Otolaryngology Group (IPOG) con- of children treated for unilateral hearing loss:
sensus recommendations: Hearing loss in the a systematic review and meta-analysis. Arch
pediatric patient. Int J Pediatr Otorhinolar- Dis Child. 2021;106(11):1102–10. doi: 10.1136/
yngol. 2016;90:251–8. doi: 10.1016/j.ijporl.2016. archdischild-2020-320389.
09.016. Wilson BS, Tucci DL, Merson MH, O'Donoghue GM.
NHS. Newborn hearing screening. (Details of the Global hearing health care: new fndings and
newborn screening programme (UK) and perspectives. Te Lancet 2017;390(10111):2503–
resources for parents.) Available at: https://www. 15. doi: 10.1016/S0140-6736(17)31073-5.

8 HEARING TESTS
INTRODUCTION
Age-appropriate assessment is essential to the early Table 8.1 Degrees of hearing loss defned.
detection, categorisation and management of hearing
impairment in childhood. Degrees of hearing loss Degree Hearing
are defned in Table 8.1. Objective tests do not require of hearing loss loss range (dB HL)
the cooperation of the child but rely on picking up
electrophysiological signals in response to sound. Mild 26–40
OAE tests – widely used in newborn screening (see Moderate 41–55
Chapter 7) and ABR tests are the main objective tests. Moderately severe 56–70
ABR involves placing sensors on the child’s head to
record electrophysiological changes in response to a Severe 71–90
sound signal fed in via an earphone. Te child needs
to be asleep and may require sedation or anaesthe-
sia. Subjective tests measure a response that the child
volunteers when they hear a presented test sound.
BEHAVIOURAL OBSERVATION AUDIOMETRY

Behavioural observation audiometry (BOA) involves toy is activated. Tese tests require a trained and
recording changes in activity – ofen head turning – skilled audiometrician and a sound-proofed test
in response to a sound stimulus (distraction test). room. Incorporating a test into a game that the child
It was widely used in babies up to the age of about learns and enjoys – putting men in a wooden boat in
6 months, but objective tests are now generally pre- response to a sound stimulus (play audiometry) – may
ferred in this age group. be appropriate for toddlers and preschool children
who are not old enough for pure-tone audiometry.
In a visual reinforcement audiometry (VRA) test,
sounds of diferent frequencies and loudness are Pure-tone audiometry (PTA) is the standard test
presented. When the child hears the sound, they method for adults and older children. Most children
turn their head and a ‘reward’ such as a fashing can be taught to undertake PTA from the age of about
DOI: 10.1201/9780429019128-8 Hearing Tests 37

4 years onwards, but the test requires concentration indicates when they hear the signal (Figure 8.1). A
and active participation. A series of signals (‘pure graph is then plotted of loudness against frequency
tones’) of diferent frequencies and at diferent loud- to give an easy-to-read assessment of hearing via air
ness levels is presented via headphones, and the child conduction (AC) across a range of frequencies.
Figure 8.1 Hearing test (PTA) in progress.
Figure 8.2 Audiogram.

Bone-conduction testing is now performed using a More specialised tests such as speech discrimination
vibrating device (bone conductor) placed on the skin tests which test the child’s capacity to hear words at
over the mastoid. A signifcant diference between diferent loudness levels may be needed in particular
AC and bone conduction (BC) (air–bone gap) sug- situations.
gests a conductive deafness, as occurs in OME
(Figure 8.2).
IMPEDANCE AUDIOMETRY (TYMPANOMETRY)

Tis is not a test of hearing but gives important infor-
mation about the ‘compliance’ and by implication the
degree of ‘stifness’ of the eardrum and middle ear. A
small probe in the ear canal emits a tone via a micro-
phone, and a speaker measures the sound refected
from the drum at diferent levels of air pressure
(Figure 8.3). In practice, compliance correlates well
with the mobility of the eardrum and middle ear. A
‘fat’ (Type B) tympanogram with reduced middle-ear
compliance across a range of air pressures is character-
istic of a middle ear efusion. A ‘Type C’ trace with a
negative middle ear pressure peak is highly suggestive
of Eustachian tube dysfunction (see Chapter 9). Figure 8.3 Normal tympanogram.
FURTHER READING
KEY POINTS NHS guidance for hearing tests for children. Available
at: https://www.nhs.uk/conditions/hearing-
● Make sure the chosen test is suitable for tests-children/ (accessed 29 March 2022)
the child’s developmental age. Tis is not
always the same as the chronological age.
● Tympanometry – with otoscopy and pure
tone audiometry – is a helpful adjunct to
the diagnosis of OME.
Hearing Tests 39
9 OTITIS MEDIA WITH
EFFUSION
INTRODUCTION
Fluid in the middle ear is an inevitable part of child- least 3 months. Most cases resolve with no treat-
hood. It occurs during an episode of acute otitis media ment. The majority of affected children probably
(AOM) when it causes localised pain and systemic do not come to the attention of ORL or audiology
upset, but a painless efusion – usually self-limiting – professionals.
is a common event in children and, apart from a short
period of mild hearing loss, causes no adverse efects. Persistent OME will need active management and, if
untreated, may result in suboptimal educational and
Otitis media with effusion (OME) is defined as cognitive outcomes for the child.
the persistent presence of middle ear f luid for at
INCIDENCE AND AETIOLOGY

Most children develop OME during the preschool in the winter months. Exposure to tobacco smoke
years, with a lower incidence beyond the age of 6 or (passive smoking), respiratory allergy and close con-
7 years. OME may follow an unresolved episode of tact with other children such as occurs in daycare cen-
AOM, but there is not always a history of an earlier tres and crèches have been implicated. Breastfeeding
acute episode. Te efusion is mucinous (‘glue ear’) or seems to ofer some protection. Children at particular
serous and may be sterile. It is thought to be produced risk include those with clef palate, Down syndrome
by goblet cells in the middle ear and Eustachian tubal (trisomy 21) and craniofacial disorders. Cystic fbro-
mucosa. Eustachian tubal dysfunction has long been sis (CF), ciliary dyskinesia and immune dysfunction
implicated as part of the aetiology of OME but, in also predispose to OME. Tere is increasing interest
truth, the cause is not completely understood. Tere in the role of ‘bioflms’ – including in the adenoidal
is a seasonal variation, the condition being commoner tissue (see Chapter 15) in the pathogenesis of OME.
DOI: 10.1201/9780429019128-9 Otitis Media with Effusion 41

CLINICAL PRESENTATION AND INVESTIGATIONS
Many children have no symptoms. Some present
with hearing loss, noticed at home or at school. Some
have earache, a sensation of pressure in the ear or
a vague sensation of balance disturbance. Tinnitus
is uncommon. Otoscopic appearances can be very
variable, but the typical fndings are of a bulging
translucent drum (Figure 9.1), sometimes with
bubbles or an air–fuid level. Sometimes – especially
in long-standing cases – there may be retraction of
the drum onto the ossicles or even the medial wall
of the middle ear. Te diagnosis is clinical but con-
frmed by PTA in children who can do a PTA, i.e.
usually children over 4 years old, assuming the child
has reached the expected developmental milestones
and is co-operative. PTA will show a conductive loss
(Figure 9.2), with an ‘air-bone gap’ of up to 35 db. A
tympanogram will typically show a ‘fat’ trace (Type Figure 9.2 PTA showing typical bilateral conductive
B, Figure 9.3). loss in OME.
1.0
Compliance value (mL)
Type C Type A
0.5
Type B
0
–300 –200 –100 0 100 200
Middle ear pressure (daPa)
Figure 9.3 Tympanometry is a useful adjunct in the

diagnosis of OME. A ‘Type b’(fat) trace is typical, but
Figure 9.1 Otoscopic view of OME. a ‘Type c’ trace is also commonly found.
MANAGEMENT
Most children with OME will have complete reso- ‘Watchful’ waiting refers to expectant management
lution of their efusions without any treatment. All but with serial observation so that treatment can be
children and their teachers and families will ben- ofered if the hearing loss is prolonged or becomes
eft from advice regarding good hearing strategies especially problematic. Multiple treatment modali-
(Box 9.1). ties have been subjected to rigorous analysis, but the

Clinical, audiometry, tympanometry
Box 9.1 Hearing strategies
• Avoid competing auditory stimuli (e.g.
Otherwise healthy child Comorbidity, e.g.
music, TV) when addressing the child. trisomy 21, cleft palate
• When speaking to the child, get their
attention, look directly at them, and
speak slowly and clearly. Active observation Multidisciplinary
• Alert the child’s teacher and consider or watchful wait assessment and follow-up
placing the child near the front of the
classroom.
Resolution OME persists tympanostomy tubes
if needed
evidence base for treatments other than tympanos-
tomy tubes and amplifcation (hearing aids) is sparse.
Consider tympanostomy ENT and audiology
Medical treatments such as antibiotics, mucolytics follow-up
and antihistamines are largely inefective. Systemic

or intranasal steroids may be of some beneft but Figure 9.4 Algorithm showing management strategy
have signifcant side efects. Auto-infation devices for OME.
(balloons through which the child can infate the
Eustachian tube using a nozzle introduced to the
nose) and the more invasive approach of Eustachian
tubal balloon infation all enjoy some popularity
but, in practice, the two most ofen used modalities
of active treatment are ventilation tubes and ampli-
fcation (hearing aids). Adjuvant adenoidectomy is
recommended in some circumstances, for example,
in recurrent cases that need a second insertion of
tympanostomy tubes, or if there is an independent
reason to recommend adenoidectomy (e.g. recalci-
trant allergic rhinitis or persistent rhinosinusitis).
Many healthcare systems have well-developed guide- (a)
lines to help clinicians and parents decide on the
best approach, usually suggesting an initial trial of
expectant treatment and intervention only if symp-
toms are persistent (Figure 9.4).
Te tympanostomy tubes (ventilation tubes or ‘vents’)

most widely used are ‘grommets’ with ‘T-tubes’ (Figure
9.5) reserved for situations where long-term ventilation
of the middle ear is required. Tympanostomy tubes are
associated with a risk of permanent perforation of the
eardrum, about 2% with grommets but as high as 40%
with T-tubes. Troublesome infection and discharge may (b)
also complicate the use of ventilation tubes, but these
are usually manageable by a short course of ciprofoxa- Figure 9.5 Tympanostomy tubes: (a) grommet,
cin drops. Tympanosclerosis (scarring with calcifed (b) T-tube.
Otitis Media with Effusion 43

deposits in the drum and sometimes within the middle tympanostomy tubes, but many children are reluctant
ear) can also occur as a reaction to ventilation tubes. to use hearing aids. Tey are usually recommended
where OME is expected to run a prolonged course
Hearing aids seem equally efective and are preferred such as in children with Down syndrome and in OME
by some parents due to the long-term adverse efects of associated with clef-palate.
FURTHER READING
KEY POINTS NICE. Otitis media with efusion: What information
and advice can I give to parents of children with
● Middle ear efusion is almost universal at otitis media with efusion (OME)? Available at:
some time in children. https://cks.nice.org.uk/topics/otitis-media-with-
● Most cases improve with no intervention. efusion/management/management/#advice-to-
parents (accessed 20 January 2022).

10 ACUTE OTITIS MEDIA
INTRODUCTION
Acute infammatory changes in the middle ear are an media (AOM) by their second birthday. Infection is
almost universal feature of childhood and most chil- typically short-lived and self-limiting, but AOM can
dren will have had one or more episodes of acute otitis have serious sequelae and complications.
PATHOGENESIS
Infection is usually initially viral. Te middle ear is part
of the upper respiratory tract and is in communication
with the pharynx via the Eustachian tube, hence the
common viral pathogens – parainfuenza virus, respi- Acute otitis media
ratory syncytial virus (RSV), coronaviruses, etc. – may
all be implicated. Te mucosa of the middle ear and
Eustachian tube become sufused and oedematous, Resolves Fluid persists in
Drum perforates
with hyperaemia of the tympanic membrane, and an (usual outcome) middle ear
increase in secretions into the middle ear, which may
become tense and engorged causing the child pain
and distress. Ofen, this resolves quickly but bacterial
superinfection can supervene due to the proliferation Complications (rare),
e.g. mastoiditis,
of pyogenic organisms such as Streptococcus pyogenes,
intracranial sepsis
Staphylococcus aureus and Moraxella catharhalis. Tis
causes increased pain, tense swelling, with an infam-
matory exudate into the middle ear and the mastoid Perforation persists
Otitis media may Otitis media with
air cells. Te tympanic membrane may perforate, usu- Chronic suppurative
be recurrent
ally accompanied by a purulent external discharge and otitis media (CSOM)
some relief of pain. Te possible outcomes of AOM are
shown in Figure 10.1. Figure 10.1 Algorithm showing outcomes of AOM.
DOI: 10.1201/9780429019128-10 Acute Otitis Media 45

PREDISPOSING FACTORS
Most children who develop AOM are otherwise due to earlier exposure to environmental patho-
healthy, and ear infections are an expected part of gens in the community. Passive exposure to
childhood. Children at greater risk include those tobacco smoke is a risk factor, as are a number of
with immune dysfunction such as children receiv- medical conditions (e.g. clef palate). Te role of
ing chemotherapy, children with specifc disor- the adenoids is disputed, but it seems likely that
ders of immunity and children with CF. AOM is adenoids may harbour and shed bacteria that colo-
to a degree seasonal and is more prevalent in the nise and proliferate in the Eustachian tube, hence
autumn and winter months. Breastfeeding ofers the place of adenoidectomy in the management of
some protection, and children looked afer in day- recalcitrant cases of recurrent acute otitis media
care centres seem to be more at risk, presumably (RAOM).
DIAGNOSIS AND INVESTIGATIONS

AOM implies the presence of pathogens in the
middle ear but, in practice, the diagnosis is almost
always made clinically with no attempt to identify
or culture the ofending organisms. Otalgia, pyrexia
and a distressed child with otoscopic features of
infammation (reddened bulging drum, Figure
10.2) are the usual fndings. Other conditions such
as tonsillitis, ‘teething’ or uncomplicated respira-
tory infection can have similar features, and this
diagnostic uncertainty has made evaluation of the
evidence for treatment, particularly antimicrobial
therapy, very difcult.
Figure 10.2 Otoscopic view in AOM.
MANAGEMENT
AOM is painful, and the frst requirement is to make clinicians, and parents, are understandably uneasy
sure the child has adequate analgesia. Paracetamol, about withholding antibiotics, especially if there is evi-
stepping up to ibuprofen if needed, with good hydra- dence of bacterial infection (e.g. severe pain or puru-
tion and general support will usually be adequate. Te lent discharge). Impending or actual complications
role of antimicrobial therapy is still not fully established are, of course, an absolute indication for antibiotics,
or agreed upon despite numerous studies and the pub- and the frst choice based on known pathogens is usu-
lication of multiple guidelines. Te evidence suggests ally amoxycillin, with a cephalosporin as second line.
that antimicrobials have little efect on the duration
or severity of symptoms and an uncertain efect on Surgery has a limited role, but myringotomy to
the frequency of complications or long-term sequelae. release a tense bulging eardrum may be considered
Despite increasing concern regarding the overenthu- in recalcitrant cases where there has been little or no
siastic use of antibiotics and the development of resis- response to medical therapy, or where complications
tance to standard antibiotics in the community, many have developed.

RECURRENT INFECTION
Tis is not well defned, but most authorities would
accept that more than three documented episodes of FURTHER READING
AOM in a 6-month period warrant the term ‘RAOM’. Suzuki HG, Dewez JE, Nijman RG, Yeung S.
Expectant treatment is best, if at all possible, but Clinical practice guidelines for acute otitis
this condition can cause a great deal of distress to media in children: a systematic review and
parents and children, and there is some evidence to appraisal of European national guidelines.
support the use of long-term prophylactic antibiot- BMJ Open. 2020;10(5):e035343. doi: 10.1136/
ics. Tympanostomy tubes (grommets) are sometimes bmjopen-2019-035343.
considered, but the evidence is unclear and there is
no doubt that grommets are associated with specifc
risks including recurrent mucopurulent discharge
and long-term tympanic membrane perforation.
Adenoidectomy may help remove the aggregated
communities of bacteria or ‘bioflms’ thought to be
responsible for recurrent infections.
KEY POINTS
● Tere is very little evidence to support the

routine use of antibiotics in uncomplicated
otitis media in children. Antibiotics make
little diference to symptoms or complica-
tions. A ‘delayed’ or ‘back-up’ prescription
may be appropriate.
● Although rare, complications of otitis
media can be devastating. Advise parents
to seek medical advice if symptoms persist
or deteriorate, or if the child becomes sys-
temically unwell.
Acute Otitis Media 47

11 CHRONIC OTITIS MEDIA
INTRODUCTION
Chronic otitis media (COM) refers to a spectrum the eardrum, and the more destructive condition
of conditions including chronic suppurative otitis cholesteatoma. COM may follow one or more epi-
media (CSOM) in which the eardrum is perfo- sodes of acute otitis media (AOM) but may also
rated with (active) or without (quiescent or inac- arise without a defnite antecedent history of acute
tive) ear discharge, localised retraction pockets of infection.
PREVALENCE
COM, particularly perforation of the eardrum, is poverty, deprivation and poor primary healthcare
a common fnding in children, but the majority of can contribute, and prevalence is much higher in
perforations occur in the course of an acute infec- the developing world where CSOM is an important
tion and heal well. Ethnicity is an important fac- public health problem and a signifcant cause of
tor, with COM occurring much more frequently childhood deafness. Perforations are found in up to
in indigenous Australians, native Americans and 2% of children who have had tympanostomy tubes
children of Inuit heritage. Socioeconomic factors, (grommets).
CLINICAL FEATURES
Discharge, usually mucopurulent, and deafness anaerobic organisms such as are more often found
are the main presenting symptoms. COM may be in cholesteatoma (Pseudomonas aeruginosa) may
‘silent’ and only detected on otoscopic examina- cause a scanty and sometimes fetid discharge.
tion. Pyogenic organisms (Streptococcus pyogenese, Occasionally, COM can present with compli-
Staphylococcus aureus and Moraxella catharalis) cations such as facial palsy and intracranial
can cause a profuse mucopurulent discharge, but sepsis.
DOI: 10.1201/9780429019128-11 Chronic Otitis Media 49

THE PERFORATED EARDRUM
Perforation of the eardrum may follow a single epi-
sode of AOM, or there may be a history of recur-
rent AOM. Some cases are iatrogenic, usually as a
complication of tympanostomy tubes, and about 2%
of cases of tympanostomy tubes are complicated by
long-term perforation.
Te site of the perforation is important, as a central

perforation (Figure 11.1) is less likely to be associ-
ated with cholesteatoma than a perforation in the
attic region, with associated bone destruction, but
the older classifcation of perforations as ‘attico-
antral’ or ‘tubo-tympanic’ is too simplistic and no
longer considered useful. A dry inactive or quiescent
perforation is ofen asymptomatic and may only be Figure 11.2 Old healed perforation. A thin mem-
suspected or discovered at otoscopic examination. brane has closed the perforation but there is cal-
Many perforations heal, usually with no adverse cifcation of the surrounding parts of the eardrum
(tympanosclerosis).
efects but sometimes leaving residual scarring or
retraction of the tympanic membrane, ofen with
calcifcation – ‘tympanosclerosis’ – of the drum or material, sometimes more or less constantly but
the middle ear structures (Figure 11.2). What is more ofen with long spells where there is no dis-
really important in planning management is whether charge. Hearing loss is variable and, if there is no
there is a cholesteatoma, hence the division of CSOM ossicular disease, it may be very minimal.
into CSOM without cholesteatoma (Figure 11.1) and
CSOM with cholesteatoma (Figure 11.3). A ‘wet’ perforation with intermittent or constant
discharge should be managed initially with intensive
A perforation may be active or inactive. A ‘wet’ medical treatment. Regular aural toilet in the form
perforation will discharge, usually mucopurulent of cleaning of debris, gentle suction and the use of
Figure 11.1 Dry central perforation. Inactive or qui- Figure 11.3 Attic perforation with underlying
escent CSOM. cholesteatoma.

antibiotic drops (ciprofoxacin) with strict adherence keen on watersports also warrant consideration of
to keeping the ear dry and free of external contami- surgery. Several techniques are described, the most
nants is ofen enough to dry up the infection. popular relying on temporalis fascia harvested from
behind the ear and used as an ‘underlay’ to form a
A dry inactive perforation may need no intervention. scafold over which new epithelium grows to close
If the child is symptomatic (i.e. with a signifcant the defect. Te optimum age for tympanoplasty is
hearing loss or recurrent troublesome discharge), sur- debated, but most otologists wait until the child is
gery in the form of tympanoplasty (myringoplasty) about 7–8 years old, by which time Eustachian tubal
to close the perforation, usually with native fascia, function will have improved making for better long-
may be considered. Children who are especially term outcomes.
RETRACTION POCKETS
Retraction of all or part of the eardrum is a com- their approach to management of retractions, with
mon sequel of Eustachian tubal dysfunction and some preferring a ‘wait and see’ approach except for
may complicate both AOM and OME. Small retrac- the most extreme cases, and some preferring early
tion pockets cause little in the way of adverse efects, intervention. Surgical excision, with or without
particularly if the middle ear is well ventilated, but cartilaginous reinforcement of the resulting defect
more severe retraction can obliterate the middle ear (tympanoplasty), ventilation of the middle ear (tym-
space and cause signifcant conductive deafness, panostomy tubes) and attention to Eustachian tubal
with the potential for ingress of squamous epithe- dysfunction (adenoidectomy, balloon dilatation) are
lium into the middle ear clef with erosion of bone all advocated. Te evidence base for any treatment
and formation of cholesteatoma. Otologists difer in approach is uncertain.
CHOLESTEATOMA
Keratinising squamous epithelium is not normally be due to ectopic cell rests and can involve the petrous
present in the middle ear and, if a mass of such tis- apex with or without middle ear involvement.
sue is found in the middle ear and/or the mastoid
air cells, it is termed a cholesteatoma. It is liable Cholesteatoma is similar histologically in adults
to recurrent infection and is potentially erosive, and children, but there are important diferences
involving the ossicles and surrounding structures in pathogenesis and behaviour. Most otologists will
including the tegmen, facial nerve and petrous describe paediatric cholesteatoma as being more
apex. Te precise aetiology is unknown but the aggressive and more destructive, such that early
more common acquired form is thought to occur intervention and prolonged follow-up are essential.
as a consequence of persistent ingrowth of epithe- Te child’s Eustachian tubal function may be worse,
lium from the external ear beyond the annulus and making treatment more difcult, and the greater
into the middle ear clef. It may begin as a retrac- pneumatisation of the mastoid air cells in children
tion pocket and is associated with Eustachian tubal makes for more extensive spread and more challeng-
dysfunction. ing surgery.
Most cholesteatomas are acquired, but congenital Diagnosis is clinical, but imaging (CT scanning) is
cholesteatoma is well recognised in children and highly recommended to assess the extent, look for
presents as a mass behind an intact eardrum. It may bony destruction and plan surgery (Figure 11.4).
Chronic Otitis Media 51

KEY POINTS
● COM, particularly perforation of the ear-

drum, is a common fnding in children,
but the majority of perforations occur in
the course of an acute infection and heal
Figure 11.4 CT scan showing extensive cholestea-
well.
toma with bone erosion (arrows).
● Cholesteatoma in children can be very
aggressive, with rapid and extensive bone
Treatment of cholesteatoma is surgical, and the
destruction.
special challenges in children are such that experi-
enced surgeons with access to good imaging, oto-
endoscopic techniques as needed and intraoperative
facial nerve monitoring should undertake this sur-
FURTHER READING
gery. Various techniques are described, with some
Schilder AGM, Marom T, Bhutta MF et al. Panel
otologists preferring to keep the canal wall intact
7: Otitis media: treatment and complications.
(canal wall up, CWU), and some preferring the better
Otolaryngol Head Neck Surg. 2017;156(4_
intra-operative access of the canal wall down (CWD)
suppl):S88–S105. doi: 10.1177/0194599816633697.
approach. Tese techniques are described and evalu-
PMID: 28372534
ated in more detail in standard otology textbooks.

12 SURGERY FOR
CHILDHOOD
HEARING LOSS
INTRODUCTION
Te role of the surgeon in managing children with hearing device (BCHD) – usually a bone-anchored
permanent childhood hearing impairment is now hearing aid (BAHA) – is considered where there is
largely focused on implantation otology, i.e. the use microtia, chronic otitis externa or in SSD where
of implants to the skull or the ear to channel acous- the rationale is to divert sound to the contralateral
tic signals to the auditory nerve and auditory cor- ear via the skull bones. In general, BCHDs depend
tex. Tere have been enormous strides in this area in on good cochlear function, and many patients
recent years with continuing improvements in tech- who have used both traditional hearing aids and
nology and outcomes. Only the essential principles BCHDs will express a preference for BCHDs. For
of the main varieties of implants are presented here. children where a BAHA is planned, the skull bone
needs to have developed so that it is thick enough
Where children need amplification (e.g. in bilat- to accommodate the implant, usually at about 2
eral conductive, sensorineural and mixed loss and years of age. A Softband® can be a useful way of
in many cases of unilateral loss), the first option ensuring good amplification until the child is a
will be to use a hearing aid. A bone-conducting little older.
BONE-CONDUCTING HEARING DEVICES

BCHDs may be implantable − i.e. requiring sur- skull bones to the cochlea using a device worn
gery to position all or part of the device − or on the head. They are usually well tolerated in
non-implantable. babies and younger children but some children
can be very sensitive to objects over or around
Non-implantable BCHDs the head. Non-implantable BCHDs are useful in
microtia, chronic otitis externa and sometimes
All these work on the principle of sound conduc- in single-sided sensorineural deafness. A trial
tion transmitted transcutaneously through the of a non-implantable BCHD is nearly always
DOI: 10.1201/9780429019128-12 Surgery for Childhood Hearing Loss 53

recommended before progressing to an implant-
able BCHD.
Examples of non-implantable BCHDs include:
● Sofband® (Figure 12.1).

● MED-EL ADHEAR® Adhesive – an adhesive
adapter attached to the skin behind the ear
and a sound processor. Te sound processor
‘clicks’ on to the adapter. Children who don’t
tolerate a headband may fnd this easier to wear
(Figure 12.2).
● BCHD attached to spectacle frames.
Implantable BCHDs
Tese are of two main types, percutaneous and
transcutaneous.
Percutaneous devices involve an osseo-integrated

implant which projects through the skin so as
to anchor an ‘abutment’ to which a processor – Figure 12.1 Child using a Softband®
which amplifes and conducts sound – is attached.
Acoustic energy is transmitted to the skull bones
and thence to the middle ear and cochlea. A BAHA
(Figure 12.3) is one such device. It is ‘passive’ in
that the processor simply amplifes and transmits
the sound energy.
In the alternative transcutaneous devices, the

processor is not directly attached to bone but
attaches to a magnet under the skin. Te MED-EL®
Bonebridge system is one such device. Te parts
embedded under the skin bring about vibration of
the skull bone via the implanted ‘transducer’. Te
external ‘processor’ – attached to the skin via a
magnet – picks up the sound, converts it to elec-
trical signals and transmits them to the implanted
parts (‘transducer’) and thence to the skull bones
and the cochlea where they are received as sound.
Tis is an ‘active’ rather than a passive device and
an ‘under-the-skin’ system with no open scars or Figure 12.2 MED-EL ADHEAR®, a transcutaneous
wounds (Figure 12.4). BCHD.

(a) (b)
Figure 12.3 (a,b) A BAHA and how it effects transmission.
Figure 12.4 MED-EL® Bonebridge. The external processor connects with the internal components transcutane-
ously via a magnet. The internal processor and transducer are completely covered (i.e. with no parts projecting
through the skin).
Surgery for Childhood Hearing Loss 55

(a) (b)
Figure 12.5 (a,b) Components of a cochlear implant.
COCHLEAR IMPLANTATION
Cochlear implantation has revolutionised the treat- development of good speech discrimination. This
ment of severe and profound hearing loss in chil- is because of the phenomenon of ‘neural plastic-
dren. Te cochlear implant (CI) is an implantable ity’ within the central nervous system, with a far
device that electrically stimulates the cochlear spi- greater capacity for adaptability in the immature
ral ganglion cells when the hair cells are not func- auditory neural networks including the brainstem.
tioning efectively, such that neural impulses are There is increasing evidence now to support the
transmitted along the auditory nerve to the cortex practice of bilateral implantation and to consider
where they are processed to produce awareness of implantation for SSD as devices become more
sound including speech intelligibility. Te compo- sophisticated.
nents of a CI are shown in Figure 12.5. Tere is an
external device (processor) consisting of a micro- Surgical techniques have improved in recent
phone, speech processor and transmitter coil. Te years with implantation taking place largely in
internal component is made up of a receiver/stimu- designated centres where there is an experienced
lator which picks up the signal from the processor multidisciplinary team. Intensive support in the
and generates electrical impulses which are then postoperative period under the supervision of a
delivered via an electrode array to the auditory skilled audiology team is essential to a success-
nerve. ful outcome. Complications are uncommon and
include very rare instances of facial nerve trauma,
The indications for cochlear implantation have but more commonly issues with skin faps which
widened in recent years and are under constant may mean the device has to be removed or re-
review, but any child with confirmed severe or implanted. Long-term device failure is uncommon
profound hearing impairment may be eligible. but regular follow-up and surveillance of children
Conventional hearing aids are tried in the first is needed post implantation. Intensive supportive
instance, and the child is referred to a multi- rehabilitation under the supervision of a dedicated
disciplinary team for full audiometric, medical team including audiologists and teachers is vital to
and radiological assessment. Early implantation long-term success, especially in the early days fol-
(as early as the first year of life) is crucial to the lowing surgery.

AUDITORY BRAINSTEM IMPLANT
An auditory brainstem implant (ABI) is a device is unsuitable, such as in cases of neurofbromatosis
which is implanted in the cochlear nucleus in the type 2 (NF2), where the cochlear nerves have been
brainstem rather than the inner ear. It may be useful irreparably damaged. Experience with ABIs in chil-
in very a small number of circumstances when a CI dren is very limited.
MIDDLE EAR IMPLANTS

Te idea of a device within the middle ear to aug- FURTHER READING
ment hearing is an attractive alternative to conven- Bagatto M, Gordey D, Brewster L et al. Clinical con-
tional hearing aids, and a few such devices are now sensus document for ftting non-surgical trans-
available. ‘Active’ surgically implanted middle ear cutaneous bone conduction hearing devices to
implants (MEIs) may stimulate the ossicles or the children. Int J Audiol. 2021;1–8. doi: 10.1080/
inner ear. Experience in children is very limited, 14992027.2021.1939449.
but MEIs may become more useful as technology Gordey D, Bagatto M. Fitting bone conduction hear-
improves. ing devices to children: audiological practices
and challenges. Int J Audiol. 2021;60(5):385–92.
doi: 10.1080/14992027.2020.1814970.
KEY POINTS NICE. Guidance: Cochlear implants for children
and adults with severe to profound deafness.
● Surgery for PCHI is now largely focused on Available at: https://www.nice.org.uk/guidance/
implantation. ta566 (accessed 24 January 2022).
● Technological developments in this feld Simon F, Roman S, Truy E et al. Guidelines (short
are rapidly advancing, and new techniques version) of the French Society of Otorhino-
and devices regularly become available. laryngology (SFORL) on pediatric cochlear
● Cochlear implantation has transformed implant indications. Eur Ann Otorhinolaryngol
outcomes for children with congenital Head Neck Dis. 2019;136(5):385–91. doi: 10.
hearing loss. 1016/j.anorl.2019.05.018.
Surgery for Childhood Hearing Loss 57

13 BALANCE DISORDERS
INTRODUCTION
Children and adolescents frequently complain of Many cases are due to normal maturation of the phys-
‘dizziness’, or cause concern to their parents because iological systems that contribute to maintaining bal-
they are clumsy, fall easily or seem to struggle ance; the vestibular apparatus does not fully mature
with their balance. Te great majority of causes until well into the teenage years. Migrainous disorders
are benign, but clinicians need to be aware of ‘red are far more common in children than was previously
fag’ symptoms and signs that warrant urgent fur- acknowledged and ofen present atypically, not always
ther investigation. Reasons for ‘dysequilibrium’ are with a headache. True vestibular disorders in children
legion and, while clinicians need to be vigilant, seri- are uncommon and, when they occur, they tend to be
ous progressive pathology in children does not ofen self-limiting and characterised by early and efective
present in this way. Some ‘red fag’ presentations are compensation. Most presentations of ‘dizziness’ are
shown in Box 13.1. due to non-vestibular pathology. Some causes of bal-
ance dysfunction in children are shown in Box 13.2.
Box 13.1 ’Red fags’ in children with Box 13.2 Some common causes of
‘dizziness’ imbalance
• Persistent or recurrent headache With hearing loss
• Persistent or recurrent vomiting
• OME
• Suspected seizures
• CSOM
• Focal neurological symptoms/signs e.g.
• Surgery to brain or ear
‘tingling’, numbness
• Ototoxic drugs
o Abnormal eye movements
o Blurred vision Without hearing loss
o Change in behaviour
• Migraine (includes benign paroxysmal
• Cardiac symptoms e.g. syncope,
vertigo of childhood, BPVC)
palpitations
• Vestibular neuronitis
• Functional disorders
DOI: 10.1201/9780429019128-13 Balance Disorders 59

CLINICAL PRESENTATION
Take a detailed and thorough history. Te duration of associated with balance dysfunction. ‘Functional’ (or
symptoms can be of great diagnostic help. ‘Dizziness’ ‘psychogenic’ vertigo as it used to be known) is com-
lasting for a few seconds suggests benign paroxysmal mon, and the rapid hormonal changes of adolescence
positional vertigo (BPPV) but this is rare in children. can ofen be accompanied by symptoms of ‘dizzi-
Episodes lasting a few hours may be due to migraine, ness’ caused by postural (orthostatic) hypotension.
or benign paroxysmal vertigo of childhood (BPVC) Adolescence is also a time when young people may
which is now thought to be a variant of migraine. be subject to exam pressures, bullying, intense social
Vestibular neuronitis and acute labyrinthitis symp- media activity and sometimes family disharmony, all
toms last for a few days, but compensation in chil- of which can manifest as somatic symptoms includ-
dren is much more rapid than in adults. Associated ing balance disorders.
symptoms can help narrow the diagnosis; nausea and
vomiting suggest vestibular pathology but may occur Hearing loss is a feature of otitis media, and hearing
in migraine. Neurological features such as seizures loss with tinnitus occurs in Menière’s disease, but
or cranial nerve palsies are alarming and warrant this is rare in children. OME is frequently associated
urgent investigation to rule out intracranial pathol- with clumsiness, falls, and ‘dizziness’.
ogy. Anxiety, depression and eating disorders may be
EXAMINATION AND INVESTIGATION

Clinical examination starts as the child enters the referral, including imaging (CT/MRI scanning) of
consulting room. Observe their gait, posture and the head. Age-appropriate audiometry and tym-
motor function. A thorough ENT examination is panometry are an integral part of a routine ENT
important, particularly otoscopy, and a basic neu- examination. More sophisticated investigations are
rological examination to include evaluation of the not always needed unless dictated by the fndings of
function of the cranial nerves is helpful. Look for initial clinical assessment. Specialist clinics ofer ves-
nystagmus. Any fndings that suggest a focal neu- tibular function tests.
rological lesion warrant urgent investigation and
MANAGEMENT
Once sinister or progressive causes have been medication to subdue the acute attacks, especially if
excluded, parents and children are usually happy to there is accompanying headache.
be reassured that there is no ominous pathology. Most
conditions are managed expectantly and will resolve. Specialist vestibular clinics will ofer specifc strat-
Advice and counselling with regard to migraine, egies such as the Epley maneouvre for BPPV and
with an emphasis on reassurance and avoiding ‘trig- vestibular rehabilitation exercises for prolonged
gers’, may be all that is needed. Avoid medication if vestibular failure. If imbalance is secondary to otitis
you can, but drugs such as pizotifen and propranolol media, the treatment is that of the primary condi-
have a place in controlling severe symptoms. Very tion (e.g. grommets for OME and tympanomastoid
troublesome migraine may require prophylaxis and surgery for cholesteatoma).

KEY POINTS
● Parents and clinicians worry that symptoms of ‘dysequilibrium’ can be due to serious intracranial
pathology, but this is extremely uncommon. Brain tumours very rarely present with imbalance as
the sole symptom.
● Apart from audiometry, few if any investigations are needed to evaluate the ‘dizzy’ child.
● Imaging is rarely helpful unless there is a defnite and specifc indication.
● Vestibular migraine (or a migraine variant) is the commonest cause of episodes of imbalance in
children.
● Avoid medication if at all possible. Most cases are self-limiting.
● Te main role of the clinician is to take a careful history, exclude sinister pathology and provide
reassurance and support.
● OME is frequently accompanied by balance dysfunction. Te mechanism is unknown.
Ratnayake S, Crunkhorn R, Wong J, Dasgupta

FURTHER READING S. Vestibular migraine in children. Audacity
van de Berg R, Widdershoven J, Bisdorf A et al. Magazine. 2021;March Edition:30-33. Available
Vestibular migraine of childhood and recurrent at: https://cloud.3dissue.net/30176/30074/
vertigo of childhood: diagnostic criteria consensus 30346/49789/# (accessed 6 June 2022)
document of the Committee for the Classifcation w w w.jv r-web.org /i ma ge s/ IC V D -Rec u r rent-
of Vestibular Disorders of the Bárány Society and Vertigo-of-Childhood-Diagnostic-Criteria-
the International Headache Society. J Vestib Res. V20.pdf
2021;31(1):1–9. doi: 10.3233/VES-200003.
Balance Disorders 61
14 FACIAL PALSY
INTRODUCTION
Te facial nerve is far more superfcial in children and is especially at risk from a low post-aural inci-
than in adults. Tis makes it more vulnerable to sion (Figure 14.1). Children with Down syndrome
trauma including operative injuries. As the mas- or craniofacial deformity may have particularly
toid process is rudimentary in the newborn and complex or unusual facial nerve anatomy, making
in early childhood, the intratemporal portion of tympanomastoid surgery in this group especially
the nerve is much more exposed than in adults challenging.
Figure 14.1 Anatomy of the facial nerve in an infant. (a) Intra-temporal course. The mastoid process is higher
and less well developed than in the adult. (b) Extra-temporal course. The posterior auricular nerve and the
nerve to the digastric branch off, and within the substance of the parotid gland, the nerve divides into the motor
branches to the muscles of facial expression. The nerve here is more superfcial than in the adult, and vulner-
able to injury.
DOI: 10.1201/9780429019128-14 Facial Palsy 63

AETIOLOGY If the nerve is traumatised during surgery, it may
simply be ‘bruised’ (neuropraxia), in which case it
will recover. Remove any source of pressure on the
Facial palsy is much less common in children than in nerve including packing/dressings in the ear and
adults. Both congenital and acquired aetiologies are protect the eye while awaiting recovery. If the nerve
described (Box 14.1). is severed, the outcome is poor even with repair.
A facial palsy evident in the newborn may be due

Box 14.1 Common aetiologies to birth trauma. This can complicate a difficult
forceps delivery, or in some instances a traumatic
• Idiopathic (Bell’s palsy) non-instrumental vaginal delivery where there is
• Trauma (includes iatrogenic, birth excessive pressure from the mother’s sacral prom-
trauma) ontory on the baby’s skull. Unravelling the precise
• Otitis media cause can be difficult and may lead to medicole-
• Congenital gal disputes; a congenital facial palsy of unknown
• Intracranial mass aetiology presents in a similar way but rarely
recovers.
Otitis media can be complicated by facial palsy.

Acquired palsy AOM with an intact drum can cause a neuropraxia,
As in adults, the commonest cause is Bell’s palsy, or especially if the bony covering of the nerve is incom-
idiopathic facial paralysis. Tis usually comes on plete (dehiscent). Although alarming, and a cause of
over a few days but may only be noticed as a ‘sud- great distress to parents, facial palsy complicating
den’ presentation, progressing to a complete palsy an AOM almost always recovers. A facial palsy in
which then recovers, typically over a period of days CSOM is more ominous and suggests erosion of the
or weeks. It is almost always unilateral. A viral aeti- bony canal covering the nerve. Tis is an indication
ology with infammatory swelling of the perineural for early surgery.
tissues in the region of the stylomastoid foramen
leading to neuropraxia has been postulated, but this Rarely, malignant disease – an intracranial tumour, a
is unproven. Tere is some evidence that early treat- rhabdomyosarcoma of the head and neck, or leukae-
ment with systemic corticosteroids hastens resolu- mic infltration of the temporal bone – can present
tion. Antiviral medication such as acyclovir has been as a facial palsy. Modern imaging has greatly helped
advocated, but the evidence is uncertain. If there are early diagnosis in these situations and should be con-
no unusual clinical features and the palsy recovers sidered if there are any atypical clinical features such
completely, investigations are not generally needed. as associated neurological signs, headache or failure
to improve.
Trauma may be caused by temporal bone fractures,
usually as part of a serious head injury, or may be Congenital facial palsy
iatrogenic, for instance inadvertent damage to the
nerve during tympanomastoid surgery, parotidec- ‘Obstetric’ palsy has been alluded to above.
tomy or surgery to the neck including excision of
lymph nodes or a pharyngeal arch anomaly. Facial Isolated or multiple cranial nerve palsies including
palsy is a devastating complication with profound facial palsy occur for no apparent reason, and facial
and life-long implications for the child and family palsy is an important component of some childhood
and it is not an acceptable outcome for any but the syndromes including Moebius syndrome, Poland
most serious indications for surgery (e.g. malignant syndrome and some cases of CHARGE syndrome
disease). (see Chapter 3).

Hemifacial microsomia (as in Goldenhar’s syndrome) Neonatal asymmetric crying facies presents as asym-
describes a spectrum of anomalies afecting one side metry of the lower lip when the baby cries. It may be a
of the face causing both functional and aesthetic manifestation of facial nerve dysfunction and occurs
asymmetry. in 0.6% of live births.
MANAGEMENT OF FACIAL PALSY

Take a careful history, including a birth history, and Long-term management of established facial palsy
examine the child with an emphasis on looking for is complex. Muscle and fascial slings and a variety
neurological fndings, particularly in the other cra- of nerve and muscle reanimation techniques includ-
nial nerves. Otoscopy and, where possible, audiologi- ing nerve transfers/anastomoses, nerve repairs and
cal evaluation are important. Document the severity free grafs are used, usually in specialised multidis-
of the paresis and record the pattern of movement of ciplinary team clinics.
each of the regions of the face (i.e. the upper, middle
and lower parts).
KEY POINTS
Look carefully at the eye in case there is evidence of
early corneal damage or conjunctivitis. If there are ● Facial palsy due to AOM almost always
unusual clinical features or any suggestion of a mass, recovers completely.
imaging can be immensely helpful. MRI with gado- ● Make sure to protect the cornea, especially
linium enhancement is ideal, if available, and will at night.
show the architecture of the nerve from the brain-
stem to the intraparotid branches.
Early treatment should focus on protecting the eye. FURTHER READING

Children may not tolerate an eyepatch, but a little tape Psillas G, Antoniades E, Ieridou F, Constantinidis
over the eyelids at night can help to reduce the risk of J. Facial nerve palsy in children: a retrospec-
exposure of the cornea. If you make the diagnosis of tive study of 124 cases. J Paediatr Child Health.
Bell’s palsy early, then oral steroids for a few days may 2019;55(3):299–304. doi: 10.1111/jpc.14190.
be worthwhile, as may the addition of acyclovir.
Facial Palsy 65
15 THE PHARYNX AND
ORAL CAVITY
INTRODUCTION
Te lymphoid tissue of the pharynx surrounds the T cells and their function is to help develop both cell-
entrance to the aerodigestive tract. Te palatine ton- mediated and humoral immunity. Tey are prone to
sils, the adenoids and the lingual tonsils collectively recurrent infection in childhood; tonsillectomy and
constitute Waldeyer’s ring. Te lymphoid follicles adenoidectomy remain among the commonest surgi-
that make up these structures are composed of B and cal procedures performed in children.
ACUTE PHARYNGITIS AND TONSILLITIS

Viral infection of the pharynx with acute enlargement Diagnosis of acute tonsillitis is largely clinical, and
of the tonsils is an almost universal part of childhood. most children are managed in primary care settings
Pain, difculty swallowing, fever and enlarged tender rather than by ORL specialists. Acute tonsillitis can
neck nodes with erythema and swelling of the ton- usually be managed with symptomatic treatment,
sils are the presenting features. Acute viral infections
are usually self-limiting. Any of the common upper
respiratory viruses (rhinoviruses, adenoviruses,
coronaviruses) can be responsible, with the Epstein
Barr virus (EBV) causing a particularly severe form
of acute pharyngitis especially in adolescents (infec-
tious mononucleosis). Bacterial infection is generally
with one of the common pyogenic organisms, includ-
ing group A beta-haemolytic Streptococcus pyogenes
(GABHS), Haemophilus infuenzae, Streptococcus
pneumomiae. Symptoms are more severe, with
marked pain, malaise and sometimes an exudate on
the tonsils (Figure 15.1). Anaerobic organisms can be
implicated in severe infections, particularly if quinsy
(peritonsillar abscess) develops. Figure 15.1 Acute tonsillitis.
DOI: 10.1201/9780429019128-15 The Pharynx and Oral Cavity 67

i.e. analgesics (paracetamol), fuids and observation Some cases of tonsillitis progress to complications.
for signs of deterioration. Resolution is the norm Tese include spread of infection to the tissue planes
with or without antibiotic treatment but, if antibi- beyond the pharynx (quinsy, parapharyngeal abscess,
otics are deemed appropriate, penicillin is efective retropharyngeal abscess), severe airway obstruction
against most of the common causative organisms. and, rarely, generalised septicaemia (see Chapter 5).
NON-INFLAMMATORY CONDITIONS OF THE TONSILS

As the tonsils are composed of lymphoid tissue, they tonsillectomy, even if there is no history of recur-
may be the site of presentation of a lymphoma, or rent sore throats.
a non-lymphoid malignancy in the head and neck.
Asymmetry in the size of the tonsils is common in Gross enlargement of the tonsils can compromise
younger children, especially following one or more swallowing and contribute to obstructive sleep
episodes of tonsillitis, and can cause concern and apnoea (OSA) (see Chapter 16).
alarm to both doctors and parents. In the great
majority of cases this is harmless but, if there is sus- Occasionally, the tonsil can be the source of recur-
picion of a lymphoma, then tonsillectomy for histol- rent spontaneous bleeds due to prominent fragile
ogy is warranted. vessels on the mucosal surface. Tis can be so severe
as to warrant tonsillectomy.
Te tonsils are ofen invaginated by multiple deep
crypts, in which cheesy debris and sometimes Immune complex disorders in childhood (e.g. post-
food particles or even calcifed material (tonsillo- streptococcal glomerulonephritis and rheumatic
liths) can collect. Tonsillar debris may contribute fever) due to tonsillitis were important causes of renal
to halitosis. Teenagers in particular can fnd this failure and mitral and aortic valvular heart disease in
so distressing that it warrants consideration of a the past but are far less ofen seen in modern practice.
TONSILLECTOMY
Te indications for tonsillectomy have become more
sharply defned in recent years. Many healthcare
systems and national ORL societies have guidelines Box 15.1 SIGN guidelines for
to help parents and clinicians make good evidence- tonsillectomy (recurrent tonsillitis)
based decisions. Recurrent tonsillitis remains the
commonest reason for tonsillectomy in childhood. • Episodes due to tonsillitis
Most clinicians would ofer tonsillectomy to chil- • Episodes disabling and prevent normal
dren who fulfl the criteria in accordance with the functioning
Scottish Intercollegiate Guidelines Network (SIGN) • Seven episodes in preceding year or
guidelines (Box 15.1). One of the difculties is that • Five episodes in each of preceding
ORL doctors are presented with a child who has a 2 years or
history of episodes of sore throat that have been • Three episodes in each of preceding
managed by the primary care team, and it is not 3 years
always certain that the child has had true tonsillitis.

Mild viral pharyngitis, even if recurrent, is not a are not breached, leading to much less postoperative
good indication for surgery, hence the importance pain and discomfort.
of a careful history to enquire about neck nodes,
fever, a painful swallow and a period of several Surgery is ofen carried out on a day-case basis,
days’ misery, usually with absence from school, all with minimal risk of complications. Some degree
of which are typical of true bacterial tonsillitis. OSA of pain and discomfort is probably inevitable, easily
(see Chapter 16) is the other common indication for managed with simple analgesics, but bleeding and
tonsillectomy. airway obstruction are now much less common.
Bleeding of such severity as to warrant return to
Surgical techniques vary. Te traditional approach theatre in the immediate postoperative period (pri-
is ‘cold steel dissection’ with ties or diathermy hae- mary haemorrhage) can occur in as many as 2%
mostasis, but a variety of ‘hot’ techniques includ- of cases and is a surgical emergency. A later bleed
ing coblationTM (controlled ablation) have become (secondary haemorrhage) is ofen thought to be
popular in recent years. Tese have the advantage of due to infection. Quoted rates vary up to over 10%
reduced perioperative bleeding and permit ‘intracap- depending on the criteria use to defne a ‘second-
sular’ tonsillectomy so that the pharyngeal muscles ary bleed’.
ADENOIDS
Te adenoids are rudimentary at birth but enlarge tissue. Te main complication of adenoidectomy is
to occupy a substantial part of the nasopharynx bleeding. Velopharyngeal insufciency, character-
between the ages of about 2 and 7 years. Adenoids ised by escape of air from the nasal cavity during
can obstruct the nasopharyngeal airway and the phonation (rhinolalia aperta), is ofen noted in the
orifces of the Eustachian tube, contributing respec- weeks and months following surgery but is usually
tively to OSA and OME. Tere has been increasing temporary. Persistent cases can be troublesome and,
focus in recent years on the role of the adenoids very occasionally, warrant corrective pharyngeal or
as a reservoir of chronic infection. ‘Bioflms’ are palatal surgery.
aggregates of bacteria in a complex mucopolysac-
charide matrix which is resistant to conventional ‘Grisel’s syndrome’ is postoperative hypermobil-
antimicrobial therapy and may contribute to recur- ity of the neck, causing pain and impaired rota-
rent infections in the nose, sinuses and middle ear. tion with the potential for injury to the cervical
Adenoidectomy – ofen in association with an inter- spinal cord in extreme cases. It was considered a
vention such as tonsillectomy (typically for OSA) or complication of adenotonsillectomy, particularly in
insertion of grommets – is a common ORL proce- children with Down syndrome due to pre-existing
dure in children. Indications include OSA, OME laxity of the ligaments that support the atlantoaxial
and persistent rhinitis that has been resistant to joint. It is now accepted that this can occur follow-
medical therapy. Blind curettage with a sharp blade ing any surgery that involves manipulation of the
has been the traditional technique, but ORL special- neck and, while children with Down syndrome are
ists are increasingly moving towards surgery under at increased risk, it is important that surgeons and
direct vision (coblation and suction diathermy) theatre personnel exercise great care when manip-
using an endoscope and a screen/monitor, permit- ulating any child’s neck during anaesthesia and
ting much more accurate and thorough removal of surgery.
The Pharynx and Oral Cavity 69

TONGUE TIE
Te lingual frenulum connects the undersurface of
the tongue with the foor of the mouth. If it is unusu-
ally short or tight (anklyoglossia), tongue movement
may be restricted (Figure 15.2). Parents will notice
a notch at the tip of the tongue when the child pro-
trudes the tongue, and the newborn baby may have
difculty with breastfeeding due to being unable to
‘latch’. If the frenulum is divided in the frst few days
of life, feeding can be greatly improved. In older chil-
dren, the adverse efects of tongue tie are less cer-
tain. Whether a severe tongue tie can interfere with
the development of speech is uncertain, but many
children and parents will present for treatment due
to perceived speech difculties or difculty with, Figure 15.2 Tongue tie.
for example, playing wind instruments, or with aes-
thetic concerns. especially thickened and fxed, a frenuloplasty with a
Z-plasty type repair. Newborns will tolerate a frenu-
Treatment in most cases involves simple division of lotomy without the need for anaesthesia, but older
the frenulum, with, in rare cases where the tissue is children will need a general anaesthetic.
MACROGLOSSIA
Enlargement of the tongue such that it protrudes
beyond the incisors at rest is termed ‘macroglossia’
(Figure 15.3). True enlargement is less common than
‘pseudomacroglossia’, i.e. the tongue size is normal
but protrusion is due to poor muscle control or a rela-
tively small oral cavity. Tis occurs in some cases of
cerebral palsy and in Down syndrome. True mac-
roglossia may be due to hypertrophy of the tongue
musculature (‘primary’ macroglossia, e.g. Beckwith–
Wiedemann syndrome, congenital hypothyroidism),
or infltration of the tongue (secondary macroglos-
sia, e.g. lymphangioma, mucopolysaccharidosis).
Symptoms include drooling, ulceration of exposed
mucosa, speech difculties, airway obstruction and Figure 15.3 Macroglossia. A pyogenic granuloma
aesthetic concerns. has developed on the exposed mucosa.
Most children with macroglossia will be under the

care of a paediatrician, who will supervise the neces- cases, a tracheostomy. Surgical reduction of the
sary investigations, but the ORL team may be asked tongue (partial glossectomy) is usually undertaken
to help if treatment is needed. Te child may need in a specialist centre by an experienced team with
airway support with a Guedel airway or, in severe appropriate back-up facilities including a PICU.

CLEFT LIP AND PALATE
Clef of the lip and/or palate occurs in about 1 in 700
births. Most cases are of unknown aetiology, but
factors that increase the risk include maternal use of
some anticonvulsant agents (phenobarbital, phenyt-
oin), maternal use of alcohol and tobacco, and ste-
roids. Dedicated multidisciplinary teams including
a clef surgeon, orthodontist, speech and language
therapist, and otolaryngologist are now well estab-
lished and ofer optimum care for these children and
their families. ORL issues include otitis media, nasal
deformity with functional and aesthetic issues, and
airway obstruction.
Otitis media is so prevalent in children with clef pal-

ate as to be almost universal. Te ORL and audiology
specialist will need to monitor hearing and treat OME
and retraction pockets as needed. Most ORL special-
ists who look afer children with clef palate will advise
caution regarding the use of tympanostomy tubes, as
the OME issues may be prolonged and amplifcation
Figure 15.4 Bifd uvula.
using hearing aids may be more appropriate.
Some conditions (e.g. Pierre Robin sequence, see

Chapter 3) are associated with signifcant airway
obstruction. A nasopharyngeal airway (NPA) may
tide things over until the child is able to man-
age without airway support. Some children with KEY POINTS
clef palate will have other deformities of the head
and neck which necessitate a tracheostomy (see ● Tonsillitis is usually viral. Antibiotics are
Chapter 25). not always needed.
● Asymmetry of the size of the tonsils is
A variety of palatal clefing of particular impor- common and causes much parental anxi-
tance to ORL is the ‘submucous clef palate’. Tere ety, but it is rarely due to serious pathology.
is no ‘gap’ in the mucosa of the palate, but a dias- Te size of the tonsils fuctuates. Carefully
tasis of the underlying muscles sometimes with a assess each case clinically.
bifd uvula (Figure 15.4), a notch in the hard palate ● Tongue tie may interfere with breastfeed-
and a visible furrow along the midline of the sof ing in the newborn. Make sure the baby
palate (Calnan’s triad). Treatment is rarely needed, is referred and treated early if there are
but extreme caution needs to be taken if adenoidec- any concerns. Tongue tie in older children
tomy is contemplated, as the adenoids may provide is rarely problematic and won’t usually
useful bulk behind the sof palate to aid closure need surgery. Te efect on speech is very
during phonation, and overenthusiastic adenoidec- uncertain.
tomy can cause velopharyngeal insufciency.
The Pharynx and Oral Cavity 71

Otolaryngol Head Neck Surg. 2019;160(2):187–205.
FURTHER READING doi: 10.1177/0194599818807917.
ENT UK Guidelines. Available at: https://www. Patel S, Burgess A. Paediatric Pathways: Tonsillitis
entuk.org/resources/155/ent_uk_tonsillectomy_ and peritonsillar abscess (quinsy) pathway for
commissioning_guide (accessed 25 January 2022). children presenting to hospital. Available at:
Mitchell RB, Archer SM, MD, Ishman SL, Rosenfeld https://bsac.org.uk/paediatricpathways/tonsilli-
RM et al. Clinical Practice Guideline: Tonsille- tis-quinsy.php (accessed 25 January 2022).
ctomy in children (update)–Executive summary.

16 OBSTRUCTIVE
SLEEP APNOEA
INTRODUCTION
Te spectrum of sleep-disordered breathing (SDB) children, primarily up to the age of about 5 years.
ranges from simple snoring to severe obstructive OSA is especially common in children with Down
sleep apnoea (OSA). Te characteristic feature of OSA sundrome (trisomy 21), in obese children (although
is cessation of breathing (apnoea) due to partial or the relationship is not nearly as direct as it is in adults,
complete upper airway obstruction during sleep for and most children with OSA are of normal weight), in
periods long enough and frequent enough to cause children with neurological conditions such as cerebral
hypoxaemia. Prevalence is between 2% and 5% of palsy and in children with craniofacial anomalies.
PATHOPHYSIOLOGY
Good quality sleep in children is essential for nor- hypertension, and cor-pulmonale are all recognised
mal growth and development. Rapid eye movement long-term consequences of untreated OSA. More
(REM) sleep is especially disturbed in OSA with immediate efects include daytime sleepiness, irri-
the potential for signifcant long-term adverse con- tability, hyperactivity, cognitive dysfunction and
sequences for the child’s health. Failure to thrive, behavioural issues.
ventricular hypertrophy, systemic and pulmonary
Te typical presentation is with snoring, ofen very turning’ in the bed followed by an arousal response
noisy and prolonged, leading to disturbed sleep for when the child ‘wakes themselves up’ and recom-
the child and family. Parents will describe apnoeic mences normal breathing. Tese apnoeic spells are
episodes during which the child stops breathing, alarming for parents who worry that the child may
usually while struggling to get air into the respira- go on to prolonged apnoea, but the hypoxia brought
tory system as evidenced by recession of the chest about by an apnoeic episode precipitates a pro-
in younger children, unusual postures, ‘tossing and nounced response in the respiratory centre (‘arousal’)
DOI: 10.1201/9780429019128-16 Obstructive Sleep Apnoea 73

so that oxygen saturation is restored. Daytime day (e.g. mouth-breathing). Te adverse efects
symptoms such as tiredness, irritability and poor on the quality of life of both child and family are
attention are common, and the child will ofen considerable and usually greatly improve following
show features of airway obstruction during the adenotonsillectomy.
INVESTIGATIONS
Various parental questionnaires have been used to
screen for OSA, and it is likely that the disorder is
even more common than the current referral pattern
would suggest, i.e. many children have undiagnosed
OSA. Parents will ofen come to the ORL clinic with
a mobile-phone video clip showing the child dur-
ing sleep, and this can be really helpful to demon-
strate the apnoeic spells and the attempts to breathe
against resistance. Defnitive diagnosis of OSA
requires measurement of the number of ‘apnoeas’
(complete cessation of airfow) and ‘hypopnoeas’
(reduction in airfow) during a defned period –
typically an hour – i.e. the apnoea/hypopnoea index
(AHI). ‘Mild’ OSA is associated with an AHI of up Figure 16.1 Large obstructing tonsils.
to 5, with an AHI of greater than 5 classifed as mod-
erate to severe. Te ‘gold standard’ diagnostic tool
is polysomnography (PSG), a full ‘sleep study’ to have ‘central’ apnoea, where the apnoeic spells are
include electroencephalography, electromyography due not to obstruction but to an abnormal pattern of
to measure chest wall movement, nasal fow mea- arousal in the respiratory centre. SDB can, of course,
surement and a record of respiratory efort in addi- be a mixture of OSA and central apnoea. More sophis-
tion to oxygen saturation. Very few units have the ticated investigations, including PSG or a variant of
capacity or resources for PSG, hence the reliance on PSG depending on local investigative facilities, may be
overnight pulse oximetry, which can be recorded at needed in these children.
home on a portable device.
Tere is increasing interest in drug-induced sleep
Pulse oximetry is widely used as a screening tool endoscopy (DISE). Te child is given a titrated dose of
and diagnostic modality and to ‘triage’ patients who an anaesthetic agent (usually propofol) and the sur-
may need more intensive perioperative care, includ- geon passes a fexible endoscope to observe the move-
ing overnight stay or even a short stay in a high- ments of the tongue, palate and pharyngeal muscles.
dependency unit (HDU). Oxygen desaturation is Te technique is especially helpful in children who
considered a ‘proxy’ measurement to record apnoea have had adenotonsillectomy but present with resid-
or hypopnoea but there are limitations and, in prac- ual symptoms. Adenoidal remnants, tongue base or
tice, the diagnosis of OSA is usually made clinically, pharyngeal wall prolapse and, in some cases, laryn-
based on a typical history ofen (but by no means gomalacia may be picked up in this way.
always) in a child with obvious adeno-tonsillar
enlargement (Figure 16.1). A few centres use dynamic MRI to study the move-
ment of various parts of the pharynx and upper
Some children – especially children with a background airway during respiration. Tis requires general
neurological condition such as cerebral palsy – will anaesthesia and is still undergoing evaluation.

MANAGEMENT
Te great majority of children with OSA will improve with mixed (central and obstructive) apnoeas and
following adenotonsillectomy. New techniques of who have severe OSA that has not responded to
intracapsular tonsillectomy (‘tonsillotomy’), includ- surgery may need treatment with continuous posi-
ing the use of the Coblator™, have led to less intraoper- tive airway pressure (CPAP). Very occasionally, a
ative bleeding and a reduced need for opiate analgesia nasopharyngeal airway, surgery to the craniofacial
and facilitated same-day surgery in many cases. Local skeleton or even a tracheostomy will have to be
arrangements vary according to facilities, the experi- considered.
ence of the clinical team, parental expectation and
resources. In the UK, children over the age of 2 years
or with a weight of 12 kg are suitable for surgery in
their local hospital without the need for HDU facili- KEY POINTS
ties, and children of 1 year and over (corresponding
weight 10 kg) can have their surgery locally if there is ● OSA has a signifcant efect on the quality
access to an HDU. Children with signifcant comor- of life of both the child and family.
bidity are best referred to a specialist centre where ● Most children improve greatly following
PICU and support services are available (Box 16.1). adenotonsillectomy.
Box 16.1 Risk factors in the

management of OSA FURTHER READING
Benedek P, Balakrishnan K, Cunningham MJ,
• Severe cerebral palsy
Friedman NR et al. International Pediatric
• Achondroplasia
Otolaryngology Group (IPOG) consensus
• Neuromuscular disorders (moderately
on the diagnosis and management of pedi-
or severely affected)
atric obstructive sleep apnea (OSA). Int J
• Signifcant craniofacial anomalies
Pediatr Otorhinolaryngol. 2020;138:110276.
• Mucopolysaccharidosis
doi: 10.1016/j.ijporl.2020.110276.
• Signifcant comorbidity (e.g. complex
Marcus CL, Moore RH, Rosen CL, Giordani B
or uncorrected congenital heart
et al. A randomized trial of adenotonsil-
disease, home oxygen, severe cystic
lectomy for childhood sleep apnea. N Engl
fbrosis)
J Med. 2013;368(25):2366–76. doi: 10.1056/
• When onsite support from tertiary
NEJMoa1215881.
medical specialties is needed (e.g.
Narayanasamy S, Kidambi SS, Mahmoud M,
metabolic, haematology)
Subramanyam R. Pediatric sleep disordered
breathing: a narrative review. Pediatric Medicine
vol. 2 (October 2019). Available at: https://
Adenotonsillectomy in OSA is associated with sub-
pm.amegroups.com/article/view/5050/html
stantial improvements in quality-of-life measure-
(accessed 25 January 2022).
ments. Some children with signifcant comorbidities,
Obstructive Sleep Apnoea 75

17 CONGENITAL NASAL
DISORDERS
INTRODUCTION
Nasal obstruction in the newborn is an emergency nasal airfow at birth will cause severe hypoxaemia,
requiring urgent referral and treatment. Babies are only relieved when the baby breathes through the
obligate nasal breathers, and any obstruction to mouth.
EMBRYOLOGY OF THE NOSE

Te nose and sinuses develop from the primitive requiring immediate airway support (a Guedel air-
foregut. Two epithelial elevations (nasal placodes) way followed in many cases by a tracheostomy).
appear at about the fourth intrauterine week. Tey
fuse to form the lateral nasal walls and the midline Te developing nose is closely related to the primi-
septum extends dorsally to separate the nose into the tive forebrain, from which it becomes separated by
two nasal cavities, each closed behind by the bucco- the bony structures of the anterior skull base, includ-
nasal membrane. A persistent bucconasal membrane ing the cribriform plate. Te developing brain may
presents as choanal atresia. herniate into the nasal cavity, giving rise to a menin-
gocoele or encephalocoele, which can then present as
Partial or complete agenesis of the nose (arrhinia) a nasal mass.
has been described and is a neonatal emergency
CHOANAL ATRESIA
Failure of canalisation of the posterior nasal aper- neonatologist will try to gently pass a small suction
tures (choanae) may be bony, membranous or mixed. catheter from the anterior nares into the nasophar-
It is usually bilateral and presents at birth with ‘cycli- ynx. If it fails to pass bilaterally, consider the diag-
cal cyanosis’. Te baby has complete nasal obstruc- nosis of choanal atresia. A good confrmatory test is
tion and becomes hypoxaemic except during mouth to place a cold stainless-steel spatula or mirror just
breathing, hence it becomes almost impossible to feed under the baby’s anterior nares during a breath cycle
the child. If the diagnosis is suspected, the midwife or to test for misting and condensation (mirror test).
DOI: 10.1201/9780429019128-17 Congenital Nasal Disorders 77

Figure 17.1 CT scan showing choanal stenosis Figure 17.2 The nasal choanae (endoscopic view,
(arrowed). 120° telescope).
Neonatal rhinitis and obstruction of the nose due to detailed view of the posterior nares on a monitor
secretions is commoner than choanal atresia and in (Figure 17.2) to facilitate trans-nasal surgery under
many suspected cases no atresia is found. vision – is more popular.
Te frst step in management is to secure a safe airway. When the posterior nasal apertures have been estab-
A Guedel tube in the oral cavity may sufce to enable lished – usually with serial bougie dilatation – bone and
safe transfer to a paediatric centre, but ET intubation sof tissue including in the region of the thickened vomer
may be needed, especially as many of these children may be removed with bone forceps and a microdebrider
have associated medical conditions. to enlarge the orifces and fashion good-sized choanae.
Some surgeons use postoperative stents, but many pre-
Imaging (CT scanning) helps to confrm the diag- fer not to. Te baby can soon feed but will need careful
nosis and plan defnitive treatment (Figure 17.1). follow-up as recurrent stenosis is not uncommon.
A little nasal suction and a few drops of a decon-
gestant (e.g. 0.5% ephedrine) help to clear the nares In some children, choanal atresia is an isolated
and make for a more helpful image. Defnitive anomaly, but it may be associated with a series of
treatment is surgical and should be undertaken as linked congenital defects – CHARGE association.
quickly as the baby is stable. Delay may compro- Features may include some or all of: coloboma, heart
mise breastfeeding in particular and, if immediate anomalies, atresia of the choanae, renal anomalies,
treatment is not possible, a nasogastric tube is best. genital hypoplasia, ear anomalies, and it is impor-
tant that babies are screened by a paediatrician.
Tere are a variety of surgical techniques for repair.
Older methods relied on an open transpalatal Unilateral choanal atresia is much less common and
approach, but improved modern endoscopes – espe- does not usually present until later, usually with
cially the 120-degree telescope which permits a highly unexplained nasal discharge.
PYRIFORM APERTURE STENOSIS

Te bony orifces in the midfacial skeleton on either nares make up the ‘pyriform aperture’. Stenosis here
side of the nasal septum that mark the anterior is much less common and usually due to excessive

prominence of the nasal process of the maxilla. It Defnitive repair, if needed, is more challenging
ofen mimics choanal atresia but the diagnosis and may require an open rhinoplasty or midfacial
becomes clear on CT scanning. degloving approach. Tere is an association with
sometimes very signifcant intracranial anomalies
Te early management is the same as for choanal such as holoprosencephaly (a defect in the develop-
atresia: establish a safe airway, and serial dilata- ment of the midline intracranial structures) and a
tion with decongestant drops and saline irrigation single central incisor tooth. Again, paediatric liaison
may tide things over and avoid the need for surgery. for screening is important.
NASAL ENCEPHALOCOELE
A portion of the developing brain may herniate
through the bony skull base and become ‘trapped’
below the cribriform plate within the nasal cav-
ity. Tis will present as a nasal mass and persist
in the nose, ofen with acute nasal obstruction in
the newborn. An encephalocoele contains both
meninges and neurological tissue in direct con-
tinuity with the intracranial structures (Figure (a)
17.3). A meningocoele is similar but contains no
neurological tissue, just the meninges and cerebro-
spinal fuid. A glioma contains nerve tissue (glial
cells, usually with fbrous and vascular tissue) but
is discrete from the intracranial contents, i.e. it has
become ‘pinched of’. Glial heterotopia refers to the
presence of a mass of such tissue in an aberrant
site, i.e. the nasal cavity or the nasopharynx where (b)
it may have migrated some distance from its intra-
cranial origin.
Detailed and skilled imaging is essential in the man-

agement of these lesions. Teir possible connection
to intracranial structures leaves the child at risk of
catastrophic intracranial sepsis, especially in the
(c)
case of injudicious surgery or biopsy. MRI is most
suitable if available and will demonstrate intracra-
Figure 17.3 Congenital nasal masses.
nial connection if present. Treatment is surgical.
(a) Encephalocoele. Neural tissue projects into the
Gliomas may be removed trans-nasally, but enceph- nasal cavity. (b) Meningocoele. Meninges with con-
alocoeles and meningocoeles, especially if large, will tained CSF projects into the nasal cavity. {c) Glioma.
ofen need an open combined ORL and neurosurgi- Neural tissue in the nasal cavity but no remaining con-
cal approach. nection to the cranial cavity.
Congenital Nasal Disorders 79

CONGENITAL NASAL MASSES
A variety of cysts – derived from embryonic tissue – Nasoalveolar and nasolabial
can present in and around the nasal structures in cysts
the newborn.
Nasoalveolar cysts in the foor of the nose and naso-
labial cysts along the lateral nasal wall may cause aes-
Dermoid cyst thetic concerns. Tey can usually be removed safely
Te commonest congenital nasal mass is a dermoid by enucleation, taking care not to leave any epithelial
cyst . Tis is typically a smooth midline swelling on remnants behind.
the dorsum of the nose under the skin. Tere may
be an external ‘punctum’. It is thought to result from
Odontogenic or dentigerous
inclusion of epithelial cells along lines of fusion,
hence the tendency for it to occur in the midline. cysts
Te cyst contains thick, ofen viscous fuid, with Odontogenic (or dentigerous) cysts occur in associa-
ectodermal and mesodermal components, some- tion with the development and eruption of the teeth
times including skin appendages and hair follicles. and may occur in the maxillary antrum and the foor
It may become infected, but more ofen the par- of the nose.
ents will present with aesthetic concerns. Careful
evaluation including detailed imaging is essential,
not least to exclude an intracranial connection as Thornwaldt’s cyst
these lesions can invaginate deeply into the midline
nasal cartilages and beyond, making excision very Tornwaldt’s cyst is a midline usually asymptomatic
challenging. nasopharyngeal cyst, increasingly found as endo-
scopic inspection of the nasopharynx (e.g. during
Treatment is surgical. Very small discrete lesions can adenoidectomy) becomes more commonplace.
be removed with a single incision on the nasal dor-
sum, or endoscopically, but an external rhinoplasty
approach can make for a more satisfactory aesthetic Teratoma
result. Larger and more extensive lesions will require
Teratoma is a neoplastic cystic lesion containing all
liaison with neurosurgical colleagues and may need
three germinal cell layers. It may present as a frm
a frontonasal approach via a forehead incision.
obstructing nasal mass. Very large teratomas may
be diagnosed in utero and may be associated with
Nasolacrimal duct cysts maternal polyhydramnios. Investigation is via CT
and MRI scanning.
Nasolacrimal duct cysts may preset to the ORL clinic
with nasal obstruction, or to the eye clinic with Te treatment is surgical, and regular follow-
epiphora. If they require surgery, a trans-nasal endo- up may be assisted by serial alphafetoprotein
scopic approach is usually possible. measurements.

Figure 17.4 Nasal haemangioma.
VASCULAR MALFORMATIONS
Haemangiomas of the head and neck are relatively efects. Medical treatment is with propranolol, but in
common, usually cutaneous, and do not always need highly aggressive lesions – especially if they encroach
treatment (Figure 17.4). Large proliferating haeman- on the orbit and threaten vision – mitomycin C can
giomas in and around the nose may cause severe be considered.
airway compromise as well as worrying aesthetic
THE NASAL SEPTUM

Some degree of deviation of the nasal septum is so a poor aesthetic result (‘saddle nose’). Prominent
common as to be normal. Tis may be due to com- blood vessels on the nasal septum (Little’s area or
pression of the facial skeleton at delivery. Unless Kiesselbach’s plexus) can cause nosebleeds (epi-
there is gross cosmetic deformity or severe airway staxis) but in children a dilated vein just under the
obstruction, nasal septal surgery is usually discour- septal mucosa can be to blame. Tis usually responds
aged before the mid to late teens due to the risk of well to silver nitrate cautery.
Congenital Nasal Disorders 81

FURTHER READING
KEY POINTS Moreddu E, Rizzi M, Adil E, Balakrishnan K
et al. International Pediatric Otolaryngology
● Neonatal rhinitis is much more common Group (IPOG) consensus recommendations:
than choanal atresia. Diagnosis, pre-operative, operative and post-
● Early correction of choanal atresia is operative pediatric choanal atresia care. Int
important to enable the baby to feed. J Pediatr Otorhinolaryngol. 2019;123:151–5.
● Avoid biopsy or instrumentation of con- doi: 10.1016/j.ijporl.2019.05.010.
genital nasal masses until you have good
quality imaging. Many congenital nasal
masses have an intracranial connection.

18 ACUTE RHINOSINUSITIS
INTRODUCTION
‘Colds’, ‘snifes’, viral upper respiratory tract infec- generally looked afer by their parents or carers at
tions and nasal discharge are a universal feature home and do not usually need medical attention
of childhood. Children with these conditions are (Figure 18.1). As the mucosa of the nose and the
Figure 18.1 Acute rhinosinusitis (ARS) is an almost universal experience in childhood.
DOI: 10.1201/9780429019128-18 Acute Rhinosinusitis 83

paranasal sinuses is one continuous epithelial lin-
ing, it is inevitable that rhinitis is accompanied by Box 18.1 Acute rhinosinusitis in
sinusitis, hence the term ‘rhinosinusitis’ is preferred children
to describe infammation in this region. Given the
number of healthcare professionals (paediatricians, ARS in children is defned as: sudden onset
family doctors, nurses, etc.) who attend to children of two or more of the symptoms:
with rhinosinusitis and the ubiquity and variety of the • nasal blockage/obstruction/congestion
presentations treated, the defnitions, terminology • discoloured nasal discharge
and therapeutic recommendations can be confusing. • cough (daytime and night-time)
Te European Position Paper on Rhinosinusitis and
Nasal Polyps (EPOS) clarifes some of these issues for <12 weeks; with symptom-free intervals
and contains considered evidence-based guidelines if the problem is recurrent; with validation
for the diagnosis and management of rhinosinusitis, by telephone or interview.
including acute rhinosinusitis (ARS), in both adults
and children (Box 18.1). Source: European Position Paper on Rhinosinusitis
and Nasal Polyps 2020 (EPOS2020), p. 2.
ANATOMICAL CONSIDERATIONS
Te confguration and pneumatisation of the sinuses the orbit (orbital cellulitis, see Chapter 5). Te max-
in children markedly difer from those of the adult. illary sinuses are rudimentary at birth, not reach-
At birth and in the early years of life only the eth- ing their adult volume and position until the teens.
moid sinuses are air-flled. Tey are intimately Te frontal sinus is the last to develop and may not
related to the bony orbit, separated just by a thin be present before the age of 10 years. Te sphenoid
plate of bone, the lamina papyracea, hence the ten- sinus is rarely pneumatised before the age of about
dency for sinus infections in children to extend to 5 years.
PATHOPHYSIOLOGY
It is almost inevitable that children develop acute common causative organisms, but coronaviruses,
infections of the nose and sinuses, ofen as many as infuenza and parainfuenza viruses and the respira-
ten or more episodes in a year. Te diagnosis is nearly tory syncytial virus (RSV) may be found. All children
always made clinically without recourse to endos- are susceptible but predisposing factors include win-
copy or imaging. Tere is a brisk mucosal infam- ter months, environmental exposure such as is the
matory reaction with swelling and oedema of the norm in schools and nurseries, exposure to tobacco
nasal and sinus mucosa, nasal obstruction and nasal smoke (passive smoking) and immunodefciency. A
discharge. Features include rhinorrhea (runny nose) very small number of children have ciliary motility
and pyrexia, sometimes with cough and facial pain. disorders, and anatomical factors such as nasal sep-
Te child may have irritability and malaise but many tal deviation and/or (dental) sepsis may predispose
experience minimal symptoms and little disruption particularly to bacterial infection. Te role of allergic
to normal life. Adenoviruses and rhinoviruses are rhinitis (AR) is unclear.

Care pathways for acute rhinusitis (ARS)
Two ARS symptoms Check for likely ABRS Self-care

One of which should be nasal ≥ 3 of the following: • Self-education/e-Health
obstruction and/or discoloured • Fever above 38°C • Decongestants <10 days
Self-care pharmacy
discharges • Double sickening • NSAIDs / paracetamol

• Unilateral disease • Herbal medicine
± facial pain/pressure • Severe pain • Zinc
± reduction or loss of smell • Raised ESR/CRP • Vitamin C
<10 days • Consider saline spray/rinses
• Avoid antibiotics
Refer to/treatment by primary care
Check for likely ABRS Symptoms >10 days or increased after 5 days Appropriate therapy
≥ 3 of the following: • INCS
• Fever above 38°C • Decongestants <10 days
Primary care
• Double sickening • Herbal medicine

• Unilateral disease ≥3 episodes of ABRS last year? • Saline spray/rinses
• Severe pain • Avoid antibiotics
• Raised ESR/CRP
Consider antibiotics Improvement after

No other investigations 10 days of antibiotics?
Refer secondary/tertiary care
PRESENCE OF ALARM SYMPTOMS

Secondary/tertiary care
diagnosis and treat accordingly • Periorbital oedema/erythema • Severe headache

(e.g. odontogenic, fungal ball, • Displaced globe • Frontal swelling
• Double vision • Signs of sepsis
or • Ophthalmoplegia • Signs of meningitis
non-sinus diagnoses • Reduced visual acuity • Neurological signs
(e.g. migraine)
IMMEDIATE REFERRAL
Figure 18.2 EPOS management algorithm for ARS.
OUTCOMES
The infection is generally self-limiting and resolves children are prone to repeated episodes – more than
with or without treatment. If symptoms worsen four per annum – recurrent acute rhinosinusitis
after 5 days, or persist for 10 days or more, the EPOS (RARS). This may be confused with AR and with
guidelines suggest the term ‘post-viral ARS’. Some bronchial asthma. Whether R ARS is a causative
Acute Rhinosinusitis 85
factor in the development of bronchial asthma is rhinosinusitis (ABRS). Clinically, this presents as a
unclear. change in the nature of the rhinorrhea, which may
become thickened, purulent and greatly discoloured,
In a few children, the viral infection is complicated with increased pyrexia and systemic upset, raised
by bacterial superinfection. Tis is typically with infammatory markers such as C-reactive protein
pyogenic organisms such as Streptococcus pneu- (CRP) and ‘double sickening’. Tis is when the child’s
moniae, Haemophilus infuenzae, Strep. Milleri and symptoms suddenly worsen following a period of
Moraxella catarrhalis and is termed acute bacterial apparent improvement.
MANAGEMENT
Treatment is largely supportive and symptomatic. applicable if there is a true bacterial infection. Te
Multiple therapeutic interventions have been used most appropriate antibiotics in most situations are
and evaluated for ARS. Tese include systemic amoxycillin or a cephalosporin. Tis must be bal-
and local decongestants, intranasal corticosteroids anced against the alarming rise in antimicrobial
(INCSs) and systemic steroids, antibiotics, probi- resistance in all communities and the morbidity
otics, antipyretics, herbal remedies, vitamin C and associated with antibiotics for the individual child.
homeopathic remedies. Te evidential base for more Despite these concerns, antibiotics are widely used
or less all of these is at best uncertain, and EPOS in practice and are ofen demanded by parents. Te
has declined to recommend any specifc treatment EPOS guidelines/algorithm (Figure 18.2) presents a
for routine use. Te role of antibiotics is especially considered and well evidence-based template for use
contentious. Te efect, if any, is small, and only in primary and secondary care.
COMPLICATIONS
Although usually no more than a nuisance, albeit and life-threatening complications. Tese include
with a considerable efect on the child and par- orbital cellulitis and intracranial sepsis (see
ents’ quality of life, ARS can give rise to serious Chapter 5).
KEY POINTS FURTHER READING

Dennison SH, Hertting O, Bennet R, Eriksson M
● Upper respiratory tract infections and et al. A Swedish population-based study of com-
nasal discharge are universal features of plications due to acute rhinosinusitis in children
childhood. 5–18 years old. Int J Pediatr Otorhinolaryngol.
● Te European Position Paper on Rhino- 2021;150:110866. doi: 10.1016/j.ijporl.2021.110866.
sinusitis and Nasal Polyps (EPOS) contains Fokkens WJ, Lund VJ, Hopkins C, Hellings PW et al.
considered evidence-based guidelines for European Position Paper on Rhinosinusitis
the diagnosis and management of ARS. and Nasal Polyps 2020 (EPOS2020). Rhinology
● Antibiotics are only required if there is a 2020;58(Suppl S29):1–464. doi:10.4193/Rhin20.600.
true bacterial infection. Available at: https://epos2020.com/Documents/
supplement_29.pdf (accessed 26 January 2022).

19 CHRONIC
RHINOSINUSITIS
INTRODUCTION
Acute rhinosinusitis usually resolves but a small (CRS). Tis is characterised by rhinorrhea and nasal
proportion of children will go on to have persis- obstruction (Box 19.1) and is an important cause of
tent symptoms. Rhinosinusitis persisting beyond long-term morbidity in children.
12 weeks is referred to as chronic rhinosinusitis
PATHOPHYSIOLOGY
CRS is primarily an infective condition, but there
is some overlap with AR and the two may coexist. Box 19.1 Defnition of chronic rhino-
Pyogenic organisms – Streptococcus pneumoniae,
sinusitis in children
Haemophilus inf luenzae, Moraxella catarrha-
lis – elicit a chronic infammatory reaction caus- Chronic rhinosinusitis (with or without nasal
ing oedema and exudation of the nasal mucosa. polyps) in children is defned as: the pres-
Clinically, the mucosa is sufused and swollen, ence of two or more symptoms one of
especially in the region of the inferior and middle which should be either nasal blockage /
turbinates, causing obstruction of the sinus ostia. obstruction / congestion or nasal discharge
Te sinus epithelium becomes engorged, sometimes (anterior/posterior nasal drip):
with a purulent discharge, which causes congestion
• ± facial pain/pressure
and facial pain. ‘Bioflms’ are now thought to play
• ± cough
an important role. Tese are aggregates of bacteria,
secretions and host infammatory cells in a com- for ≥12 weeks; with validation by tele-
plex mucopolysaccharide matrix which adheres to phone or interview.
mucosal surfaces and is relatively resistant to both
host defences and antimicrobial therapy. Te bio- Source: European Position Paper on Rhinosinusitis
and Nasal Polyps 2020 (EPOS 2020), p. 3.
flm forms a reservoir of continuing infection and,
periodically, sheds bacteria which can migrate and
DOI: 10.1201/9780429019128-19 Chronic Rhinosinusitis 87

establish further bioflms at adjacent sites. Bioflms contribute to CRS, making elimination of the infect-
on both the nasal mucosa and adenoids may ing organisms very difcult.
CLINICAL FEATURES
Prolonged nasal congestion and discharge are mucosa – particularly marked in the region of the
the dominant symptoms. Examination will show middle turbinates – and sometimes crusting and
nasal obstruction, often a mucopurulent exu- exudation.
date (Figure 19.1), congestion and swelling of the
Figure 19.1 Nasal discharge, often of a mucopurulent exudate, is a dominant symptom of CRS.
INVESTIGATIONS
CRS is a clinical diagnosis, and few children need ciliary dyskinesia (PCD) or cystic fbrosis (CF) can
complex investigations. Torough nasendoscopy present to the ENT clinic. Arrange appropriate
can be undertaken in the ORL clinic. Most children investigations if you are suspicious; for example,
have no underlying conditions, but some will have consider a ‘sweat test’ or genetic testing to exclude
anatomical abnormalities of the nose (e.g. severe CF particularly if the child has nasal polyps. If there
septal deformity) or, in very rare cases, an undiag- is diagnostic uncertainty or coexisting AR, skin tests
nosed unilateral choanal atresia. A small number of may be helpful. Imaging (CT scanning) will show
children have immunodefciency, and ORL special- extensive mucosal disease and may demonstrate an
ists need to be alert to the possibility that primary antrochoanal polyp or an unexpected nasal mass.

TREATMENT
Treatment of CRS is primarily medical. Saline nasal guidelines recommend that surgery should only be
irrigation is safe and efective and may be well toler- considered following ‘appropriate medical treat-
ated by some children. Te evidence base for most ment’ (AMT), and despite the poor evidence base to
pharmacological interventions in children is weak, support the use of antibiotics, most ORL specialists
but INCSs are usually recommended, certainly will recommend a course of antibiotics before con-
before contemplating any surgical treatment. EPOS sidering surgery.
SURGERY
Tere is good evidence to support adenoidectomy FURTHER READING
for recalcitrant CRS, perhaps because the adenoids Fokkens WJ, Lund VJ, Hopkins C, Hellings PW
act as a reservoir of continuous infection. Maxillary et al. European Position Paper on Rhinosinusitis
antral lavage has been widely used in the past and and Nasal Polyps 2020 (EPOS2020). Rhinology.
is ofen combined with adenoidectomy. Te exact 2020;58(Suppl S29):1–464. doi: 10.4193/
role of functional endoscopic sinus surgery (FESS) Rhin20.600. Available at: https://epos2020.
is not fully established, but it is frequently used com/Documents/supplement_29.pdf (accessed
with some evidence of symptomatic improvement. 26 January 2022).
Middle meatal antrostomy and limited removal of
diseased mucosa rather than extensive exenteration
of the paranasal sinuses is probably all that is needed.
Turbinate reduction surgery is ofen ofered as a last
resort to improve severe nasal obstruction, but the
efect, if any, tends to be short-lived.
KEY POINTS
● CRS is an important cause of long-term
morbidity in children.
● ‘Bioflms’ on the nasal mucosa and ade-
noids may contribute to CRS, making
elimination of the infecting organisms
very difcult.
● Treatment is primarily medical with sur-
gery reserved for cases that have failed to
respond to intensive pharmaco-therapy.
Chronic Rhinosinusitis 89
20 ALLERGIC
RHINOSINUSITIS
INTRODUCTION
Allergic rhinosinusitis (AR) is becoming increas- and it is now apparent that efective recognition and
ingly common with a prevalence varying between management not only improve symptoms but help to
10% and 25% in children. It causes severe adverse control concomitant bronchial asthma.
efects on the quality of life of both child and family,
PATHOGENESIS
AR is an infammatory condition caused by the cel- which cause swelling, oedema and hypersensitivity
lular response to an allergen to which the child has of the mucosa. Te typical allergens at play in chil-
earlier been exposed. It is mediated by immuno- dren are the house dust mite, grass and tree pollens,
globulin E (IgE) which binds to receptor cells – ‘mast moulds and spores, and animal (pet) dander. Tere is
cells’ – in the host. Te nasal mucosa is especially a strong genetic component to the aetiology of AR. It
sensitive but the pharynx, the oral cavity and par- is more common in western populations and, while
ticularly the conjunctival mucosa are frequently the exact reasons for this are unknown, smaller fam-
involved, such that the term AR is now best thought ily size, earlier exposure to environmental pollutants
of as allergic rhinoconjunctivitis. Degranulation of and reduced exposure to community infections may
these cells releases a variety of infammatory media- be some of the factors at play.
tors such as histamine, leukotriene C4 and others
CLINICAL FEATURES
The dominant symptoms in most children are conjunctivitis are more common than realised
nasal discharge (usually a thin watery rhinor- and should be enquired about. Some children
rhoea), nasal obstruction, sneezing and itch- will experience itching and discomfort in the pal-
ing. Swelling and itching of the eyelids due to ate and the pharynx, with symptoms worsening
DOI: 10.1201/9780429019128-20 Allergic Rhinosinusitis 91

Figure 20.1 Hay fever.
during periods of exposure to the allergen, such as life may be very considerable, with reduced school
to pollens and grasses in the spring and summer attendance and interference with sport, leisure
(hay fever, Figure 20.1). The effects on quality of and exercise.
DIAGNOSIS AND INVESTIGATIONS

AR is typically managed by family doctors and the and measurement of the local reaction (a ‘wheal’).
diagnosis is clinical, with little recourse to endos- Blood tests to measure serum IgE – both total IgE
copy or investigations. Te history is key and, on and allergen-specifc IgE – are sometimes done but
examination, the nasal mucosa may be moist, swol- they are less accurate than skin tests. Tests in more
len, erythematous or sometimes pale with swelling specialised clinics include nasal smears to identify
of the inferior and middle turbinates. If the child is eosinophils, ‘nasal challenge’ to provoke response
seen in an ORL clinic, nasendoscopy will show fur- to a specifc allergen, and measurement of nitrous
ther evidence of boggy oedematous nasal mucosa, oxide (NO). Imaging is not routinely helpful, but CT
and skin tests to identify specifc allergens are ofen scanning may be needed if there is nasal polyposis
considered. Tese take the form of intradermal or suspicion regarding an alternative diagnosis (e.g.
injection of a series of allergens with observation antrochoanal polyp).
TREATMENT
Early and appropriate management of AR is essential good rhinitis control reduces the risk of the develop-
not only to improve the quality of life of the child and ment of bronchial asthma and helps to improve the
family but because there is increasing evidence that progress of asthma in children already afected.

Allergen avoidance is the frst step in AR manage- Sodium chromoglycate, a ‘mast cell stabiliser’, can
ment. If the child is allergic to house dust mite, be used as a nasal spray, and oral montelukast, a leu-
then mattress protectors and acaricides for carpets kotriene receptor antagonist, may have a place.
and sof furnishings can help. Most parents will
take measures to reduce exposure to grasses, pol- ‘Immunotherapy’ has enjoyed increased popularity
lens and pet dander, for example, but eliminating in recent years. Te principle is that controlled expo-
common allergens, including pollens and grasses, sure to the allergen may bring about desensitisation.
is not easy. Two methods are commonly employed: sublingual
immunotherapy (SLIT) and subcutaneous immu-
Pharmacotherapy usually starts with a systemic or notherapy (SCIT). Tese may be available in a small
intranasal antihistamine, preferably a non-sedating number of centres and in limited circumstances, but
one such as cetirazine or desloratadine. INCSs give the evidence base for their use in children is cur-
very good symptom control. A spray is usually bet- rently poor, and there is a risk of anaphylaxis.
ter tolerated than ‘drops’ and, while inevitably there
is some systemic absorption, this is least with the Surgery has little to ofer in the management of AR,
low bioavailability preparations (e.g. mometasone but turbinate reduction techniques have been popu-
and futicasone). Leukotriene receptor antagonists lar in the past where there is gross turbinate hyper-
tend to be reserved for more severe disease, and a trophy and failure to improve nasal obstruction
short course of oral glucocorticoids may be con- despite intensive medical therapy.
sidered for extreme exacerbation of symptoms,
for example, if the child needs symptom control
during school examinations. Despite the low bio-
availability of modern INCSs, there is a concern KEY POINTS
regarding systemic absorption, and it is important
to bear in mind that some INCSs can now be bought ● AR has a very considerable impact on the
without prescription, so parents and children may child’s quality of life.
‘self-medicate’. ● Good control of AR is an important aspect
of the management of bronchial asthma.
Nasal decongestants – local and systemic – are ● AR is a medical disorder. Surgery has little
discouraged in children, but are sometimes used or no place in management.
for short periods to control severe exacerbations.
Fokkens WJ, Lund VJ, Hopkins C, Hellings PW et al.

FURTHER READING European Position Paper on Rhinosinusitis
Cingi C, Muluk NB, Scadding GK. Will every and Nasal Polyps 2020 (EPOS2020). Rhinology.
child have allergic rhinitis soon? Int J Pediatr 2020;58(Suppl S29):1–464. doi:10.4193/Rhin20.600.
Otorhinolaryngol. 2019;118:53–8. doi: 10.1016/j. Available at: https://epos2020.com/Documents/
ijporl.2018.12.019. supplement_29.pdf (accessed 26 January 2022).
Allergic Rhinosinusitis 93
21 NON-INFLAMMATORY
ACQUIRED SINONASAL
DISORDERS
INTRODUCTION
Tumours of the nose and paranasal sinuses infrequent ly missed until t hey are ver y
in children are uncommon and hence not advanced.
ANGIOFIBROMA
Tis is a benign but locally invasive fbrovascular embolisation and help to control what can be tor-
tumour almost exclusive to adolescent boys. Tere rential perioperative bleeding if resection is planned.
may be a hormonal mechanism to explain this but
the exact aetiology is unknown. Te tumour arises Te treatment is surgical removal, even if the mass is
from the sphenopalatine foramen and extends into small, as progression is inevitable. Early disease may
the nasal cavity and the nasopharynx, compressing be amenable to endoscopic removal; larger tumours
the bony facial skeleton and the skull base. Typical will require an open approach. Tis type of surgery
presentations are nasal obstruction and epistaxis. tends to be concentrated in a few specialist centres
Modern imaging techniques – MRI scanning and where clinicians have gained experience in a num-
CT – will demonstrate the mass and delineate the ber of techniques such as midfacial degloving, lat-
margins. Te MRI appearance is highly charac- eral rhinotomy and maxillary osteotomy, sometimes
teristic with a pronounced ‘blush’ (Figure 21.1). combined with endoscopic clearance.
Angiography is not only diagnostic but may facilitate
FIBROUS DYSPLASIA
Tis is a benign lesion of bone which may afect functional nasal problems. It can be ‘monostotic’, i.e.
the craniofacial skeleton causing aesthetic and in a single bony locus, or ‘polyostotic’ where it can be
DOI: 10.1201/9780429019128-21 Non-infammatory Acquired Sinonasal Disorders 95

(a) (b)
Figure 21.1 MRI appearance of angiofbroma: (a) axial (b) coronal.
present in more than one site, ofen including one of

the long bones (e.g. the femur or tibia). It can occur
in older children or young adults, and while not a
true neoplasm, the involved bone expands rapidly
causing facial deformity, dental malocclusion and
encroachment onto the orbit. Diagnosis is by imag-
ing, with a characteristic appearance (‘ground glass’)
on plain X-ray and on CT scanning (Figure 21.2).
Treatment is conservative as far as possible, but sur-
gery to debulk large lesions may be appropriate for
cosmetic reasons. A small number of these children
have associated endocrine abnormalities (McCune–
Albright syndrome) and should be screened by a
paediatrician. Figure 21.2 CT scan showing fbrous dysplasia of
the maxilla.
JUVENILE OSSIFYING FIBROMA

Juvenile ossifying fbroma (JOF) is a true neo- is characteristic, showing a radiolucent lesion with
plasm which can occur in the craniofacial skeleton. variable calcifcation. Treatment is surgical and com-
Proptosis, nasal obstruction, epistaxis and facial plete excision may be very difcult.
deformity can all ensue. Te radiological appearance

MALIGNANCY
Malignant disease in the mucosal or skeletal ele-
ments of the nose in childhood is rare, but as a large
number of the malignancies that present in children
occur in the head and neck, it is important for the
ORL specialist to be vigilant. An expanding facial
swelling warrants immediate investigations as it
is highly suggestive of malignancy (Figure 21.3).
Arrange imaging and, if necessary, biopsy if suspi-
cious. Lymphoma, Ewing’s sarcoma, rhabdomyosar-
coma, haemangiopericytoma and nasopharyngeal
squamous carcinoma have all been described in the
nasal cavity and nasopharynx in children.
Figure 21.3 An expanding facial swelling, highly

suggestive of malignant disease.
NASAL TRAUMA
Despite being highly active and prone to various
injuries, young children present relatively infre-
quently with nasal trauma compared to their young
adult counterparts. Check for septal haematomas,
associated injuries such as facial fractures and for
bony deformity (Figure 21.4). Te nasal skeleton is
relatively sof and pliable in children and deformity
requiring surgical correction is not common. If there
is a deformity, manipulation under anaesthesia will
usually bring about a satisfactory aesthetic result.
KEY POINTS
● Consider fexible nasendoscopy in adoles- Figure 21.4 Nasal fracture.

cent boys who present with a nosebleed,
especially if bleeds are recurrent or unex- FURTHER READING
pectedly profuse. Wilson MN, Nuss DW, Zacharia Bek, Snyderman
● Avoid biopsy of a nasal mass until you have CH. Surgical management of juvenile nasopha-
good quality imaging. Bleeding from an ryngeal angiofbroma. Oper Tech Otolaryngol -
angiofbroma may be torrential. Head Neck Surg. 2019;30:22-9.
Non-infammatory Acquired Sinonasal Disorders 97

22 THE OBSTRUCTED
AIRWAY
INTRODUCTION
A baby’s airway is especially vulnerable to obstruc- Te infant may struggle hard to breathe, causing
tion, given its narrow calibre and the high oxygen immense alarm and distress to parents and health-
needs of the infant. Te larynx and trachea in the care staf alike. Specifc causes of airway obstruction
newborn are less rigid than in an adult or an older in children and their management are covered in
child, and hence more susceptible to compression. A the next three chapters. Tis chapter focuses on the
small reduction in the diameter of the airway lumen – general principles of looking afer a child with acute
such as occurs with mucosal swelling in an acute airway obstruction.
respiratory infection – can have a profound efect.
Tis will depend on the cause, but some features tachypnoea, recession of the sof thoracic cage on
are constant (Box 22.1). A baby with an obstructed inspiration, and visible indrawing of the trachea
airway will be distressed, often demonstrating (tracheal tug). Te child struggles to feed. Stridor
refers to a high-pitched noise, typically on inspira-
tion, although expiratory stridor can present as well,
especially if the obstruction is lower in the airway
Box 22.1 Features of airway (e.g. in tracheomalacia). Stridor on inspiration and
obstruction in infants expiration (biphasic) is ominous and suggests severe
airway compromise. Te low-pitched ‘snoring’-type
• Stridor noise associated with pharyngeal airway obstruction
• Tachypnoea is more ofen referred to as stertor, but the terms ‘stri-
• ‘Tracheal tug’ dor’ and ‘stertor’ are ofen used imprecisely. Te noisy
• Chest recession breathing associated with lower airway obstruction
• Feeding diffculties (e.g. in bronchial asthma) is usually referred to as
‘wheezing’.
DOI: 10.1201/9780429019128-22 The Obstructed Airway 99

With increased work needed for breathing, a child
can maintain good oxygen saturations for a consider- Box 22.2 ’Red fags’ in a child with
able period of time, but progressive airway obstruc- airway obstruction
tion will tire the child and eventually cause reduced
breathing, cyanosis and cardiorespiratory arrest. • Biphasic stridor
• Reduced stridor after a prolonged period
Be alert to the ‘red fags’ to look for in a child with of struggle (‘Beware the quiet child’)
airway obstruction (Box 22.2). • Cyanosis – a very late sign of
impending acidosis and cardiac arrest
• Bradycardia
EARLY MANAGEMENT
Te child may need immediate resuscitation in the the cause and should be given early, usually by the
acute situation. Healthcare personnel looking afer subcutaneous route.
children with potential airway obstruction need
to be conversant with the principles of Advanced Continue to monitor – pulse oximetry, vital signs –
Paediatric Life Support™ (APLS). Te frst priority and support the airway throughout including, if
is to ensure the oropharyngeal airway is patent, i.e. appropriate, the use of a face mask with hand ventila-
check for a foreign body or a prolapsed tongue and tion. If there is no improvement, the child may need
apply gentle suction to remove secretions. High-fow an alternative airway. Tis is typically via ET intuba-
oxygen using a re-breathing bag (Ambu-bag™) can tion or the use of a laryngeal mask if personnel skilled
buy a little time. Nebulised adrenaline – 2 ml of 1 in at intubation or the use of the mask are available. If
1000 solution in normal saline – may help if there is ET intubation is impossible, or if there is a proximal
extensive mucosal swelling or bronchoconstriction, obstruction, a cricothyroidotomy may be considered
and it is essential to establish good IV access early on. (Figure 22.1). Tis is very much a procedure used only
Corticosteroid therapy – dexamethasone 0.4 mg per in extremis and should be an absolute last resort, par-
kg – has been shown to greatly improve the manage- ticularly in the very young for whom ET intubation is
ment of children with airway obstruction whatever much preferred.
Thyroid lamina Thyrohyoid membrane
(a) (b)
Trocar and cannula
Cricoid cartilage
Figure 22.1 (a,b) Cricothyroid puncture should be used only as a last resort.

ASSESSMENT
Rapid and life-threatening airway obstruction in It is usually easily accomplished in babies by ask-
babies is uncommon, and more ofen stridor is ing the parent to hold the infant on their lap, and in
gradual, intermittent and of varying severity. Once an older child you may be able to secure the child’s
the emergency situation is under control, focus your cooperation.
attention on diagnosis and treatment. Clinical fea-
tures can help to narrow the diagnosis, and a careful More thorough endoscopy including rigid laryngo-
history and examination will dictate the next steps. tracheobronchoscopy requires general anaesthesia
A rapid onset suggests an acute infection – especially with the use of Hopkins rod telescopes and a record-
if the child is febrile – or an inhaled foreign body. ing monitor. Tis will usually be undertaken in a
Hoarseness or an abnormal cry suggests laryngeal centre that has specialist experience in the anaes-
pathology such as recurrent respiratory papilloma- thetic and surgical management of children with
tosis (RRP) in an older child or a cord palsy in a paediatric airway pathology.
baby. Feeding difculties and aspiration can occur
in tracheo-oesophageal fstula (TOF) or laryngeal Imaging can supplement endoscopy: for example,
clef, and the birth and neonatal history can alert the if tracheal compression is noted at endoscopy, then
clinician to a web, mucosal cysts or laryngotracheal a CT or MRI scan may demonstrate an abnor-
stenosis. mal mediastinal vessel. MRI scanning is useful
to demonstrate the extent of a haemangioma or a
Te primary diagnostic technique is airway endos- lymphangioma, and contrast studies such as tube
copy. Laryngoscopy is a relatively straightforward oesophagography and bronchography can be useful
outpatient procedure for many children (fexible to demonstrate a TOF or to delineate the extent and
awake airway endoscopy) and should be the frst severity of tracheobronchomalacia.
investigative technique if the child will tolerate it.
DEFINITIVE TREATMENT
Clearly, treatment depends on the cause. Te child KEY POINTS
will ofen need to be transferred to a specialist cen-
tre. Protocols and resources for the transfer of the
● A small reduction in the diameter of the
acutely ill child have greatly improved in recent years
airway of a young child can have a pro-
and close liaison between the referral centre and the
found efect.
unit where the child presents is important. Te child
● Be aware of the common features of airway
may need ET intubation to facilitate safe transfer (see
obstruction in an infant, particularly the
Chapter 1). In some circumstances, a ‘retrieval team’
‘red fags’.
may need to travel to help support the local team and
● Be conversant with the principles of APLS.
facilitate transport.
● Treatment in a specialist centre may be
required.
FURTHER READING
Advanced Life Support Group (ALSG). Advanced
Paediatric Life Support: A practical approach
to emergencies, 6th edn. Wiley-Blackwell; 2016.
The Obstructed Airway 101

23 CONGENITAL
LARYNGEAL DISEASE
INTRODUCTION
Te laryngotracheal structures develop from the pathology will typically, but not always, present as
primitive foregut. Major anomalies of the air pas- stridor in the newborn. Conditions such as haeman-
sages ofen occur in association with oesophageal giomas and some cases of laryngotracheal stenosis
anomalies, some of which (e.g. tracheal agenesis) do not manifest themselves until later.
are incompatible with survival. Congenital airway
LARYNGOMALACIA
Tis is the commonest cause of stridor in infancy. It swollen mucosa particularly over the arytenoids, and
may present immediately at birth, but it more ofen virtually the whole of the supraglottis can prolapse
becomes problematic in the frst few weeks of life as into the glottis on inspiration.
the baby’s oxygen needs increase and the child begins
to feed. It is characterised by harsh inspiratory stri-
dor, made worse when the child feeds or becomes very
active. It usually improves when the child is positioned
prone and can become less pronounced when the baby
is asleep. Most cases are mild and resolve spontane-
ously, but severe cases can cause great distress, feed-
ing difculties, failure to thrive and repeated hospital
admissions due to acute exacerbations. Te aetiology
is not fully understood but is thought to relate to the
comparative sofness of the baby’s laryngeal cartilages
such that they are ‘drawn in’ during respiration. Tis
is easily seen at endoscopy when the epiglottis takes
on an ‘omega’ shape with short aryepiglottic folds and
the arytenoid cartilages are rapidly drawn together
on inspiration, partially or completely occluding the Figure 23.1 Laryngomalacia – endoscopic view of
airway (Figure 23.1). Tere may be redundant and the supraglottis.
DOI: 10.1201/9780429019128-23 Congenital Laryngeal Disease 103

Mild cases need no intervention. Tere is an associa- mucosa. Excessive tissue removal risks postoperative
tion with gastro-oesophageal refux, but the precise aspiration, and a conservative approach is preferred.
relationship is unclear and the role of anti-refux treat-
ment is uncertain. More severe cases warrant referral
to a paediatric ORL clinic where fexible endoscopy will
demonstrate the dynamic features and help reassure
the parents. Persistent cases, or where there is failure to
thrive, diagnostic uncertainty or concern about asso-
ciated conditions will need admission for rigid airway
endoscopy. Tis is to confrm the diagnosis, exclude
any other pathology and, if appropriate, improve the
symptoms by surgery. Te preferred technique for most
paediatric otolaryngologists is an ‘aryepiglottoplasty’
(Figure 23.2). Tis involves division of the aryepiglot- Figure 23.2 Aryepiglottoplasty. The mucosal fold
tic folds to open the laryngeal introitus, sometimes between the epiglottis and the arytenoid cartilage
combined with removal of excessive and redundant (aryepiglottic fold) is divided.
VOCAL CORD PALSY

Vocal cord palsy can be unilateral or bilateral. It child has a safe airway. Investigations should include
can be a difcult diagnosis, hence is ofen missed. imaging; skilled ultrasonography can demonstrate
Te typical presentation is with respiratory distress the paretic cord. MRI can demonstrate intracranial
immediately afer birth, ofen with a weak or absent pathology in bilateral palsy, or a mediastinal lesion
cry, feeding difculties, aspiration and marked stri- compressing the recurrent laryngeal nerve in uni-
dor. Te degree of airway compromise depends on lateral palsy. Some cases occur following cardiac
the position of the paralysed cord(s), and some chil- surgery but more ofen the aetiology is unknown.
dren – especially with bilateral palsy – will require Defnitive treatment in the form of cord lateralisa-
tracheostomy. Most cases resolve in time, so it is wise tion or, in some cases, laryngotracheal reconstruc-
to persist with conservative treatment as long as the tion may be needed.
LARYNGEAL WEB
Webbing or atresia can occur at various sites in endoscopy, and it can be tempting to divide it
the larynx, but most commonly at the level of in the expectation that this will give immediate
the vocal cords (Figure 23.3). Mild cases may and sustained relief of airway obstruction, but
not require intervention, but severe cases where almost always the visible ‘web’ is the upper limit
there is near-complete airway obstruction will of a longer atretic segment, and more exten-
need definitive surgical repair, often involving sive surgery is required. Balloon dilatation is
laryngotracheal reconstruction. A preliminary an increasingly popular strategy to expand the
tracheostomy can be life-saving. A laryngeal web airway.
may seem fairly innocuous when discovered at

Figure 23.3 Laryngeal web.
CONGENITAL LARYNGOTRACHEAL STENOSIS

Te congenital form is a type of atresia and, in severe expand as the child gets older. More severe cases
cases, can amount to near-total agenesis of the airway, may need laryngotracheal reconstruction or cri-
which is fatal. Milder forms, particularly isolated sub- coid resection (see Chapter 24). ‘Complete tracheal
glottic stenosis (SGS) can present with repeated episodes rings’ is a particularly difcult condition where the
of stridor, especially when the child develops a respira- entire tracheal circumference is encircled by rigid
tory infection, and can elude diagnosis for many years. cartilaginous rings rather than the normal horse-
Te diagnosis is made at rigid endoscopy. Children shoe shape which does not extend to the posterior
with Down syndrome are particularly susceptible. tracheal wall. Tis is especially challenging surgi-
cally (slide tracheoplasty) and requires very highly
Most cases can be managed conservatively in specialised care.
the expectation that the calibre of the airway will
LARYNGOTRACHEAL CLEFT
Tis is essentially a defect in the party wall between in type 1 where there may be few or no symptoms.
the larynx in front and the oesophagus behind. Mild Feeding difculties, aspiration, repeated episodes of
cases (type 1) extend as far as the vocal folds, whereas airway obstruction and recurrent respiratory infec-
the most severe (type 4) extend into the trachea as tions may alert the clinician, and the diagnosis is
far as the carina. Tere is a strong association with confrmed at endoscopy. Imaging (videofuoroscopy)
other anomalies, particularly tracheo-oesophageal can demonstrate the overspill of contrast from the
fstula (TOF). Presentation may be delayed, especially oesophagus into the airway.
Congenital Laryngeal Disease 105

Type 1 cases may need no treatment or may respond the posterior wall of the larynx. More severe cases
to simple measures such as thickening the child’s may require tracheostomy, feeding gastrostomy, and
feeds. If surgery is needed, it can be performed endo- eventual repair via laryngofssure or even open tho-
scopically, freshening and apposing the mucosa in racotomy, with uncertain and ofen poor outcomes.
TRACHEO-OESOPHAGEAL FISTULA
TOF is a persistent communication between the oesoph- Fistula Fistula
agus and the trachea, ofen in association with atresia
of the oesophagus. Tere are several anatomical vari- Atresic upper
ants, the commonest being an atretic oesophagus with oesophageal segment
the blind stump extending into the mediastinum and
a distal oesophageal remnant communicating with the
trachea lower down (Figure 23.4). A much rarer variety,
but of particular importance to the ORL specialist, is
the ‘H’ fstula where there is no oesophageal atresia and
an intact trachea but there is a fstulous communication
between the two, typically in the mediastinum. Te
fstula opening in the trachea can be found at tracheo-
bronchoscopy in a child with recurrent unexplained
respiratory infections, but it can be very elusive.
(a) (b)
TOF is ofen associated with other congenital abnor-

Figure 23.4 (a) Blind-ending oesophageal pouch and
malities including vertebral, anal, cardiac, tracheal, a fstula between the lower oesophageal lumen and the
renal and limb (VACTERL). Tracheomalacia is com- trachea. (b) ‘H’-type. The fstula connects the oesopha-
mon, and some children will require tracheostomy geal and tracheal lumens directly. A ‘pit’ may be evident
in addition to surgical repair of the TOF, ofen for a on the posterior tracheal wall at tracheoscopy.
prolonged period. ‘Aortopexy’ – anchoring the aortic
arch which is closely attached to the anterior tracheal to hoist the trachea forward and increase the patency
wall – to the ventral surface of the sternum may help of the lumen.
HAEMANGIOMA
Haemangiomas (‘birth marks’) are common, mostly get to see an otolaryngologist. Te diagnosis is made at
occurring on the skin. Not all are obvious at birth, endoscopy when a smooth, sof (pear-shaped) subglot-
and they proliferate during early infancy only to tic swelling is seen. Imaging can help to delineate it.
become apparent during the frst few months. Airway
haemangiomas are typically in the subglottic region, Even if the airway obstruction is incomplete, active
sometimes but by no means always in association with treatment is needed as the lesion will grow rapidly
cutaneous lesions. Te child presents with worsening before it involutes and may cause complete airway
stridor from about 2 months, eventually progressing to obstruction if untreated. Treatment in the past was
severe airway obstruction. Late presentation is uncom- surgical, ofen involving a tracheostomy or pro-
mon but the child may be in extremis by the time they longed steroid therapy, but the method of choice

now is the use of propranolol over a period of about ideally, these children will be managed in a multidis-
4–6 weeks under the supervision of a paediatrician ciplinary team which includes an otolaryngologist.
or a cardiologist with expertise in this area. Surgery Most infantile hemangiomas disappear in childhood
may be appropriate in some circumstances and, if lef untreated.
VASCULAR COMPRESSION
While not abnormalities of the tracheobronchial tree, cross and compress the trachea just above the carina
some vascular anomalies can compress the trachea (vascular sling), and there are some less common
and present as airway obstruction. Te commonest variants. Te otolaryngologist may notice an inden-
of these is a double aortic arch (vascular ring). Te tation in the anterior tracheal wall at endoscopy, and
aorta splits into two segments, one passing behind the diagnosis is confrmed by imaging. Early referral
the oesophagus and one in front of the trachea, com- to the paediatric cardiac service is advised, and the
pressing both. An aberrant innominate artery may prognosis is usually excellent.
TRACHEOMALACIA
Partial or complete collapse of the trachea or bronchi close of. Diagnosis is confrmed by endoscopy, ofen
during respiration is caused by a lack of rigidity in the supplemented by contrast bronchography, especially
developing cartilage. To some degree, this is physi- if there is a prominent element of bronchomalacia.
ological, but it can be severe and even life-threaten-
ing. It is ‘primary’ if it occurs due to segmental or Treatment is difcult and may involve pressure venti-
complete maldevelopment of the tracheal rings, and lation – continuous positive airway pressure (CPAP)
‘secondary’ if it occurs as a result of compression, or bilevel positive airway pressure (BiPAP) – until
(e.g. by a vascular ring or in association with a TOF) there is spontaneous improvement. A tracheostomy
where it can complicate surgical repair. In severe may be needed to facilitate ventilation or to bypass a
cases, the child will have what parents and physicians malacic segment. Some cases respond to ‘aortopexy’,
ofen refer to as ‘dying spells’ when the airway com- where the aorta is hitched forward and anchored to
promise gives rise to apnoea and cyanosis, especially the sternum, thus pulling the mediastinal structures,
brought about by expiration as the smaller airways including the trachea, with it and opening the lumen.
RARE LARYNGOTRACHEOBRONCHIAL CONDITIONS

Laryngeal cysts and laryngocoeles may be congeni- anterior laryngeal clef and the rare cri du chat syn-
tal. A laryngocoele is an outpouching of the laryngeal drome (a ‘mewing’ cry due to persistent opening of
mucosa which projects beyond the confnes of the the posterior glottis during phonation in association
larynx; a saccular cyst is a discrete, fuid-flled ‘sac’ with microcephaly, now known to be due to a partial
which does not communicate with the airway but deletion of chromosome 5) are among the fndings
develops in the mucosa of the airway, ofen in the that can be noted at laryngotracheoscopy. Congenital
vallecula or the glottis. If they obstruct the airway, bronchial cysts and rare mediastinal tumours may
they can be removed surgically. A bifd epiglottis, sometimes present to the otolaryngologist.
Congenital Laryngeal Disease 107

FURTHER READING
KEY POINTS Laryngomalacia. BMJ best practice guideline.
Available at: https://bestpractice.bmj.com/
● Laryngomalacia is common. Mild symp- topics/en-gb/754 (accessed 6 June 2022)
toms need no intervention. Consider
endoscopy if symptoms are severe, the
baby is failing to thrive or there is diag-
nostic uncertainty.
● Propranolol is the frst-choice manage-
ment strategy for airway haemangiomas.
Surgery is now rarely needed.

24 ACQUIRED
LARYNGOTRACHEAL
DISEASE
INTRODUCTION
Acute infections – including diphtheria and epi- reliance on assisted ventilation via indwelling ET
glottitis – were an important cause of childhood tubes – developed. Laryngotracheal infections
morbidity and mortality until well into the twen- remain an important global health issue but vac-
tieth century and major indications for tracheos- cination programmes, improved knowledge and
tomy in children. Cicatrisation of the subglottis understanding of neonatal care and better ET
due to ET intubation assumed importance in the tubes have all greatly reduced the importance of
latter part of the century as neonatal care improved both infections and iatrogenic airway problems in
and paediatric intensive care facilities – with their the developed world.
INFECTION
Acute epiglottitis obstruction may not be immediately apparent but
can very quickly progress. Settle and calm the child
Typically due to Haemophilus infuenza B (Hib), this as much as possible and admit them to hospital for
condition was rampant for much of the twentieth urgent observation and treatment. Avoid anything
century. Widespread implementation of Hib vacci- that might precipitate laryngospasm such as over-
nation in children has greatly reduced its incidence, enthusiastic examination, the tongue depressor or
but cases still occur occasionally due to vaccine endoscope until you are satisfed that the airway is
failure, and rarely due to organisms other than Hib, secured, usually by ET intubation. Commence IV
particularly in vulnerable children such as those antibiotics and carefully monitor the airway until
with compromised immune systems. Children aged the swollen oedematous epiglottis and the sur-
between about 2 and 7 years are especially suscep- rounding structures have returned to normal before
tible. Tey present with a rapidly progressing febrile considering extubation. Tis is preferably accom-
illness, sore throat and painful swallow. Airway plished in the controlled setting of a PICU.
DOI: 10.1201/9780429019128-24 Acquired Laryngotracheal Disease 109

Acute laryngotracheobronchitis Recurrent respiratory
papillomatosis
Acute laryngotracheobronchitis (ALTB) is com-
monly referred to as ‘croup’, which describes the RRP is a now uncommon condition occurring
typically harsh cough which accompanies a com- both in adults and children (juvenile-onset recur-
mon viral infection in children. It is more preva- rent respiratory papillomatosis, JORRP) character-
lent in the winter months and can be due to one of ised by warty excrescences in the respiratory tract
a variety of viruses including the respiratory syn- (Figure 24.1). It is caused by the human papillo-
cytial virus (RSV) or a parainfluenza virus. The mavirus (HPV), principally types 6 and 11, hence
usual age of onset is about 18 months. The mucosa the expectation that it may become a very rare
of the larynx, trachea and bronchi becomes suf- condition as widespread HPV vaccination pro-
fused and oedematous with a marked reduction grammes are established. Transmission is thought
in the calibre of the airway and the child becomes to be via the birth canal, although cases have
progressively more distressed. In severe cases stri- been recorded in babies born by Caesarian sec-
dor is marked, leading on to respiratory failure. tion. Colonisation of the maternal genital tract –
Mild cases can be managed at home. The response usually asymptomatic but sometimes in asso-
to systemic steroids (oral dexamethasone 0.4 mg ciation with genital warts – is common (25% or
per kg) is usually very rapid, but more severe cases more), but JORRP is very rare so it is likely that
will need hospital admission for observation with there are some host susceptibility factors at play.
serial monitoring (pulse oximetry), nebulised JORRP presents with hoarseness in children typi-
adrenaline, oxygen therapy and sometimes, in cally from the age of 4 years and upwards although
exceptional circumstances, airway support includ- it may manifest in younger children, usually an
ing ET intubation. indication of more aggressive disease. Untreated,
the exophytic lesions will proliferate and obstruct
Some children are prone to recurrent episodes of ‘croup’ the airway. Diagnosis is confirmed at endoscopy
and may be referred to the ORL service for investiga- by observing the characteristic warty lesions, and
tion to rule out an underlying cause. Very occasionally, treatment is surgical.
cases of undiagnosed laryngotracheal stenosis present
in this way and endoscopy can be reassuring, but more Te preferred technique nowadays is repeated removal
ofen there is no structural abnormality and the symp- of the lesions at endoscopy by a microdebrider. Of the
toms are due to mucosal hypersensitivity. Ofen, these various excision techniques – which include the use of
children go on to develop bronchial asthma and con- the CO2 laser and Coblator™ – this seems to produce
tinued monitoring and surveillance under the supervi-
sion of a paediatrician may be needed.
Bacterial (membranous)
tracheitis
Tis is a much more serious condition and can
complicate an apparently innocuous bout of croup.
Te tracheal mucosa becomes infected with slough-
ing and exudation due to pyogenic bacteria such as
Staphylococcus aureus, Streptococcus pyogenes and
Strep. pneumonia. Te child will require admission
to a PICU and is at risk of overwhelming sepsis and
of potentially fatal mediastinitis. Te otolaryngolo- Figure 24.1 The larynx in RRP. The warty excres-
gist may be required to undertake a therapeutic air- cences cause dysphonia progressing to airway
way endoscopy, removing exudate and slough. obstruction.

the least damage to surrounding tissues. Repeated sur- Interferon, acyclovir, ribavirin and various vac-
geries are the norm, some children requiring endos- cines have been tried. Tere is some evidence to
copy only every 6 months and some needing disease support the therapeutic use of the HPV vaccine.
debulking every few weeks to maintain a safe airway. Cidofovir (an antiviral agent originally developed
Resolution is expected by puberty, but some children to treat CMV) has enjoyed a vogue as an intra-
have recalcitrant disease persisting into adulthood. lesional agent injected at the time of surgery and,
Te lesions may progress at an alarming rate and while there have been good reports of efcacy,
cause concern regarding impending airway compro- there is uncertainty with regard to the potential
mise, but traditional advice is to avoid tracheostomy long-term toxicity.
except in the most extreme circumstances. Seeding of
disease within the trachea and bronchi can be fatal, Vaccination of adolescent girls and, in many coun-
causing lung cavitation and respiratory failure, and tries, boys as well has begun to reduce the preva-
there is a small risk of malignant transformation. lence of genital HPV, with an expected reduction in
the incidence of both JORRP and carcinoma of the
Medical treatments are available as an adjunct to uterine cervix in women already apparent in many
surgery, but the evidence for their use is uncertain. countries.
INTUBATION TRAUMA AND EXTUBATION FAILURE

Premature babies, ofen with multiple signifcant under endoscopic control has become more widely
and life-threatening medical issues, are now rou- available and may be helpful in mild cases. Te ‘cricoid
tinely managed on highly specialised neonatal and split’, using a vertical incision through the midline of
paediatric intensive care units (PICUs) with excellent the cricoid cartilage, ofen possible by an endoscopic
survival prospects. Many require assisted ventilation, approach, may permit the introduction of a slightly
typically with the help of an indwelling ET tube, larger ET tube for a few days to enable another attempt
sometimes for long periods. As this practice became at extubation, but repeated failure suggests that SGS
commonplace from the 1960s onwards, it became has occurred and more radical intervention is needed,
apparent that a number of children developed scar- sometimes necessitating a temporary tracheostomy.
ring and stenosis of the airway, presumably as a direct
consequence of prolonged or traumatic intubation.
Trauma at the level of the cords caused glottic webs;
trauma further down caused SGS. Tracheostomy was
the inevitable consequence until surgical techniques
to reconstruct the airway became widely available.
Severe SGS is less common nowadays, but iatro-

genic injury to the larynx and trachea still occurs.
Granulation tissue on the free edge of the cords is a not
uncommon fnding at endoscopy. Mucosal cysts in the
larynx and upper trachea are now frequently identi-
fed in intubated babies and may compromise extuba-
tion. Large fuid-flled cysts may require uncapping at
endoscopy (Figure 24.2). Failure to extubate a baby
due to swelling of the subglottic mucosa may respond
to systemic steroids for a few days, followed by another
attempt at extubation under endoscopic vision to check
for cysts, swelling and oedema. Balloon dilatation Figure 24.2 Subglottic cysts viewed at endoscopy.
Acquired Laryngotracheal Disease 111

SUBGLOTTIC STENOSIS
SGS implies irreversible scarring of the subglottic (LTR), using a graf, ofen autologous rib cartilage, to
airway (Figure 24.3). It is traditionally graded by expand the scarred airway (Figure 24.4). Some chil-
the Cotton–Meyer classifcation depending on the dren may be suitable for cricotracheal resection (CTR)
extent to which the calibre (cross-sectional area) of with removal of the lower rim of the cricoid cartilage
the airway is reduced (Box 24.1). and part of the upper trachea and anastomosis of the
free ends (Figure 24.5). Te aim is to enable the child
Grade 1 and some grade 2 cases may not require to manage without a tracheostomy.
corrective surgery; steroid therapy sometimes with
repeated balloon dilatation may sufce. More severe
disease will need defnitive correction.
Surgery for SGS is now best carried out in a limited

number of centres where the child can be looked afer
by an experienced team. Te most widely performed
traditional surgery is laryngotracheal reconstruction
Figure 24.4 Laryngotracheal reconstruction. A linear

incision is made through the midline of the larynx (laryn-
gofssure) and extended into the upper trachea. A rib
graft is fashioned and inserted in the resultant defect to
expand the stenosed airway.
Figure 24.3 Subglottic stenosis – endoscopic

appearance.
Box 24.1 Cotton–Meyer classifca-

tion of SGS
• Grade 1: <50% reduction
• Grade 2: 50–70% reduction
• Grade 3: >70–90% reduction
• Grade 4: 100% occlusion Figure 24.5 Cricotracheal resection. Cartilage is resected
and the resultant free edges anastomosed as shown.

LARYNGOTRACHEAL TRAUMA
Not all cases of laryngeal trauma are iatrogenic. trauma, although uncommon, is occasionally
Swallowed caustic substances can cause serious reported as is thermal injury due to inhalation of hot
and life-changing injuries to the airway, and blunt air during exposure to fres.
VOICE DISORDERS
Vocal cord nodules in children may cause hoarseness
FURTHER READING
and are best managed conservatively, ideally with
Meites E, Stone L, Amiling R et al. Signifcant
the help of a speech and language therapist (SALT).
declines in juvenile-onset recurrent respiratory
Cysts, polyps and very rarely neoplasms may occur
papillomatosis following human papillomavirus
in the larynx and present with dysphonia. Vocal cord
(HPV) vaccine introduction in the United States.
palsy may be iatrogenic following thoracotomy with
Clin Infect Dis. 2021;73(5):885–90. doi: 10.1093/
injury to the recurrent laryngeal nerve. It is usually
cid/ciab171.
temporary, but long-standing cases may respond to
injection techniques.
KEY POINTS
● Acute epiglottitis is an acute airway emer-

gency. Admit the child, avoid any instru-
mentation of the airway (including the
tongue depressor) and start antibiotics
immediately.
● The management of ‘croup’ has been
greatly improved by widespread use of
steroids (dexamethasone).
● RRP is a viral disease. HPV vaccination
should make this already rare condition
even less common.
● Tink of RRP in any child with unex-
plained hoarseness. Make sure you get a
view of the larynx.
● Avoid surgery for children with vocal cord
nodules if at all possible. Refer to a SALT.
Acquired Laryngotracheal Disease 113

25 TRACHEOSTOMY
INTRODUCTION
Tracheotomy refers to a surgical opening in the tra- a mature track from the tracheal lumen to the skin
chea. It is used to bypass the upper (pharyngeal and of the neck (i.e. it includes a ‘stoma’), this is a tra-
laryngeal) airway and to permit air entry into the cheostomy, but the terms are used interchangeably.
trachea and bronchi. When the opening has formed Tracheotomy in children may be lifesaving.
INDICATIONS
Multiple medical and surgical conditions in chil-
dren lead the clinician to consider tracheostomy
Box 25.1 Avoiding tracheostomy:
but remember that this is just one of a number of
options that may be appropriate for dealing with an
some techniques for airway support
obstructed airway. Techniques for providing airway • High-fow oxygen via a facemask
support and maintenance in children have improved • Nebulised adrenaline 2 mL (1 in 1000)
(Box 25.1). Te three main categories of indications • Steroids – dexamethasone 0.4 mg per
for tracheostomy are to bypass obstruction, to facili- kg can be given orally
tate ventilation and to permit tracheal aspiration. • A Guedel airway
• Nasopharyngeal airway (NPA)
• Endotracheal (ET) intubation
• Non-invasive ventilation, e.g. via nasal
prongs or a face mask
TECHNIQUE
Fortunately, emergency tracheotomy is very rarely indi- obstructed and the child is unable to breathe – then you
cated. If you cannot gain access to a child’s airway in may need to make an emergency opening into the lar-
any other way – for example, if the larynx is completely ynx via the cricothyroid membrane. A wide-bore needle
DOI: 10.1201/9780429019128-25 Tracheostomy 115

is best; defnitive measures can be considered once the gland may be in the way and can be divided, but it
immediate crisis is resolved. Tis is only really feasible is ofen easy simply to displace it. When the fascia
in the older child, as the cricothyroid membrane is not is cleared from the tracheal cartilage, you should
well developed before the age of about 3 years. be able to see the rings clearly. Be certain you have
identifed the rings; check with your assistant and
As with any surgical intervention, detailed pre- feel the ET tube by rolling it gently between fnger
operative counselling of the parent(s) and fully and thumb before placing two ‘stay sutures’ just lat-
informed consent are vital. Formal tracheotomy is eral to the midline, one on either side (Box 25.2).
usually undertaken in controlled conditions in a You can then use these to help open the tracheotomy
fully anaesthetised child with an ET tube in place and to facilitate insertion of a tracheostomy tube
(Figure 25. 1). (Figure 25.3).
Te author’s preference is for a transverse inci- Liaise closely with the anaesthetist throughout, and
sion, securing haemostasis with bipolar diathermy. make sure the tube is the right length. If it is too
Remove a pad of subcutaneous fat (Figure 25.2) and short, it will pop out of the neck; if it is too long, it
continue the dissection staying in the midline, down will go into the bronchus on one side and the other
to the pretracheal fascia. Te isthmus of the thyroid lung will not be ventilated. Take care that the tube
is the right calibre and length. Te size of the ET
tube is a good guide. Some important diferences
between adult and paediatric tracheostomy are
shown in Box 25.3.
Box 25.2 Tracheostomy

• Keep your dissection to the midline.
• Find the tracheal rings and do not make
an incision in the tracheal wall until you
are certain you have identifed them.
• Liaise closely with the anaesthetist to
make sure the airway is safe before
you insert a tracheotomy tube.
Figure 25.1 Child positioned for tracheostomy.
Figure 25.3 Stay sutures facilitate safe positioning

Figure 25.2 Removal of a fat pad. of the tube.

Box 25.3 Important differences between adult and child tracheostomy
• Childrens’ tissues are much softer and more easily compressed
• A child‘s airway has a narrower lumen with not enough room for an inner tube.
• Never excise cartilage in a child – the lumen is too narrow and the risk of stenosis is high.
• A cuff is usually not helpful.
• It is easy to enter the thorax – watch for pneumothorax.
• Mediastinal vessels may be high and encroach the surgical feld.
• A child is much more likely to suffer from excessive granulations at and above the stoma
and at the distal end of the tube.
COMPLICATIONS
Tese may be immediate (perioperative), delayed (i.e. in the days post-surgery) or late. Some important
complications are shown in Box 25.4.
Box 25.4 Complications of tracheostomy

Immediate
• Bleeding
• Pneumothorax
• Airway obstruction
Intermediate
• Tube dislodged
• Tube obstructs
Late
• Accidental decannulation
• Blocked tube
• Granulations
POSTOPERATIVE CARE
Postoperative care starts in the operating room. the child’s neck and the tape. Leave the stay sutures
Check the tube well before you open the trachea and in and tape them down (Figure 25.4). Carers looking
have a range of sizes ready. Secure the tube well so it afer the child need to know what to do in the event
does not pop out. Make sure the tapes holding it in of an emergency. Have a spare tube with an intro-
place are neither too loose nor too tight. You should ducer at the bedside at all times and ensure adequate
be able to comfortably get your index fnger between suction and humidifcation facilities are available.
Tracheostomy 117
Te child needs constant nursing care and obser-
vation for the frst 24 hours, with easy access to a
skilled resuscitation team. Te tube can be changed
afer about 5 days, by which time the tract will be
mature and stable.
Figure 25.4 Tracheostomy tube in position.
TRACHEOSTOMY TUBES IN CHILDREN

Tubes have improved greatly in recent years. Silastic to the narrow calibre of the airway, and fenestrated
tubes are fexible yet strong, cause less tissue reaction tubes are very rarely used due to the risk of excessive
than older rubber tubes, and are easily inserted by granulation tissue. Cufed tubes may be needed to
parents and carers provided they are well trained. help ventilation but, in general, are best avoided for
Children’s tubes typically have no inner tube due long-term use.
HOME CARE
Many tracheotomised children are managed at distressing. Inspired air needs to be moistened and
home. Tis can be a daunting prospect for parents, ideally warmed, as the nose and upper airway are
carers and teachers, all of whom need support and bypassed. A period of several weeks’ inpatient care
training. Parents need to understand that a tra- is usually required to train parents about suction,
cheotomy, although ofen lifesaving, has a series of tube change technique, humidifcation, stoma care
adverse efects and comes with some risk. A child and resuscitation. Looking afer a child with a tra-
with a tracheostomy will need regular suctioning of cheostomy will have a profound efect on the fam-
the chest to remove secretions. In the early stages, ily dynamics, work patterns, etc., and can be highly
the child will be unable to speak as they will not be demanding. Good community support with access
able to project air into the larynx. Tis will afect the to skilled nurses and regular follow-up makes for a
child’s ability to cry, which many parents fnd very far smoother path for parent and child.
DECANNULATION
Sometimes, a child is best managed with a trache- has resolved. Tere are diferent protocols and plan-
otomy indefnitely but, in most cases, decannulation ning regimes but, generally, a few days’ inpatient care
(removal of the tracheostomy tube) can be planned to assess the airway and ensure the child can manage
once the condition that required the tracheostomy without the tracheostomy tube is needed.

TRACHEOCUTANEOUS FISTULA
A persistent leakage of mucus and air through the
stoma is not uncommon even long afer successful
decannulation, especially if the tube has been in place KEY POINT
for a prolonged period (Figure 25.5). Ofen the ‘fstula’
will heal spontaneously but, if it persists for more than ● A tracheostomy is a life-changing event
about 6 months, it may need surgical closure. for parents and child. Te family will need
intensive counselling prior to surgery, and
extensive and skilled training to facilitate
care of the child at home.
FURTHER READING
Manchester University NHS Foundation Trust.
National tracheostomy safety project. Available at:
https://www.tracheostomy.org.uk/healthcare-
staf/emergency-care-child (accessed 14 January
2022).
Figure 25.5 Tracheocutaneous fstula.
Tracheostomy 119
26 CONGENITAL DISORDERS
OF THE NECK
INTRODUCTION
Te development of the head and neck in the investigation and management. Congenital disor-
embryo is complex, and it is no surprise that con- ders include developmental cysts, vascular anoma-
genital anomalies in this region are fairly common- lies and abnormalities of the embryonic pharyngeal
place. Disorders in infants and children that present arches. Not all of these are immediately apparent
with a neck mass are very diferent from those that at birth; they may present when the child is a little
occur in adults and require a diferent approach to older.
DERMOID CYST
Te lines of fusion during embryonic development scan will show a discrete cyst, with – in contrast with
may close with the inclusion of epithelial cells. Tese a thyroglossal cyst – no ‘track’ extending upwards
cells may be of ectodermal and mesodermal origin, (Figure 26.1).
with sebaceous glands and sometimes hair follicles.
Te cells produce a cheesy, sebaceous material and Treatment is surgical, for aesthetic reasons but not
gradually enlarge to present as a cyst. Te typical least to confrm the diagnosis. A simple cystectomy
position is in the midline of the neck where it may is adequate but, as the distinction between a dermoid
be confused with the more common thyroglossal and a thyroglossal cyst is not always made preopera-
duct cyst. Clinically, a dermoid cyst forms into a tively, many children will have the more extensive
frm mobile mass, which, because it is not attached ‘Sistrunk’s’ procedure. Dermoid cysts can present
to the tongue base, does not protrude on swallowing. in other sites, notably the dorsum of the nose (see
Imaging is the frst-line investigation. An ultrasound Chapter 17).
DOI: 10.1201/9780429019128-26 Congenital Disorders of the Neck 121

(a) (b)
Figure 26.1 Dermoid cyst: (a) longitudinal view, (b) transverse view.
THYROGLOSSAL DUCT CYST

Te thyroid gland develops high in the neck at the Treatment is surgical. Thyroglossal cysts may
tongue base and descends behind the hyoid during become infected, making subsequent surgery
intrauterine life to take up its position encircling the more difcult due to scarring. Simple cystectomy
trachea and the alae of the thyroid cartilage. Descent is inadequate and liable to lead to recurrence. Te
is via a lumened structure – the ‘thyroglossal duct’ – ‘Sistrunk’s’ procedure is the classical operation for
which eventually atrophies. If the duct persists, it a thyroglossal cyst and includes removal not only
may form a cyst, typically in the midline but some- of the cyst but of the tissue above, extending to the
times a little to one side. Clinically, this is evident as tongue base and including the mid-portion of the
a frm swelling, said to move on protrusion of the hyoid bone (Figure 26.2). Te important thing is
tongue due to its attachment to the tongue base. Tis not to leave any residual epithelial cells which could
sign is inconstant and unreliable. Te swelling is not cause the cyst to reform. Wide excision to include
always in contact with the tongue, and the diagnosis adjacent sof tissue which could harbour cell rests or
should be confrmed by imaging. Ultrasound scan- a remnant of the tract is the key to successful frst-
ning will show a cyst, and the radiologist may be able time surgery.
to demonstrate a tract going to the tongue base. It is
prudent to ask the radiologist to note the presence of
a normal thyroid, as there have been rare reports of
thyroglossal cysts that contain the only remaining
functioning thyroid epithelium. CT and MRI scan-
ning are not usually needed.
Figure 26.2 Excision of a thyroglossal duct cyst.

LINGUAL THYROID
Tis is a rare anomaly in which some or all of the thy- If treatment is required, it is usually medical, i.e.
roid gland fails to descend and is found at the level of suppression of activity and growth by thyroid stim-
the foramen caecum of the tongue. It may be the only ulating hormone (TSH) under the supervision of an
functioning thyroid tissue. It is usually asymptom- endocrinologist. Surgery is very rarely needed.
atic but may cause dysphagia, airway obstruction or
bleeding. MRI scanning demonstrates the mass well.
THE BRANCHIAL ARCHES

Te structures of the head and neck in the devel-
oping embryo are derived from a series of bars of
mesenchymal tissue – the ‘branchial’ or ‘pharyngeal’
arches. Six arches are described, and they are sepa-
rated by external clefs and internal pouches. Te
frst ‘arch’ gives rise to the maxilla and mandible,
and the second gives rise to some of the structures
that form the ear. Major anomalies (agenesis or par-
tial agenesis) of these arches form the basis of some
craniofacial anomalies such as hemifacial micro-
somia and mandibulofacial dysostosis. More com-
monly, developmental anomalies may give rise to
cysts (fuid-flled sacs), sinuses (blind-ending tracts)
or fstulas (abnormal communications between two
structures, e.g. the skin and the pharynx), which can Figure 26.3 Excision of a frst branchial cleft tract.
become infected and will ofen require surgery. The tract has been dissected out and separated from its
opening in the foor of the ear canal. The yellow sloop
First-arch anomalies give rise to cysts, sinuses or fs- is over the facial nerve trunk.
tulas in the pre-auricular area. Tey are highly chal-
lenging surgically because of the potentially close
A third or fourth branchial clef abnormality can
relationship to the facial nerve (Figure 26.3).
give rise to recurrent neck abscess, ofen involving
the thyroid gland, as these arches are closely related
Second-arch fstulas are the commonest manifesta-
to the thyroid and parathyroid glands. Te typical
tion of arch anomalies to require surgery. Tey usually
anomaly is a sinus extending from the pyriform fossa
present in older children or in young adults, some-
to deep in the neck. Surgical excision including a
times following one or more infections. Te external
part of the thyroid lobe was the traditional approach,
opening is in the skin of the upper neck and the inter-
but some surgeons now advocate endoscopy with
nal orifce may be deep in the pharynx, ofen within
identifcation of the pharyngeal orifce which can be
the tonsil. Surgical excision involves following the
cauterised.
tract upwards to its internal opening, taking care to
navigate the carotid sheath and the hypoglossal nerve.
Congenital Disorders of the Neck 123

VASCULAR AND LYMPHATIC MASSES
Tese are now classifed as either ‘vascular tumours’ mass, sometimes unilocular (macrocystic) but more
or ‘vascular malformations’. ‘Haemangiomas’ or ofen multilocular (microcystic), which results from
‘birth marks’ are the commonest variety of vascular abnormal development of the lymphatic system.
tumour, and are usually innocuous, self-limiting and Tey can be extremely large and may be made up
require no treatment. Tey may cause alarming swell- of complex interdigitating processes that extend
ing, for example in the parotid gland where they can throughout the head and neck and are almost
be mistaken for a malignant tumour (see Chapter 28). impossible to safely remove surgically. Tey may
Biopsy is not ofen needed as the radiological features cause functional problems (e.g. airway obstruction
(particularly MRI scanning) are so distinctive. If they and dysphagia) and are a source of major concern
encroach on important structures – notably the orbit because of their aesthetic appearance. Te diagnosis
or the airway – they may need to be more actively is clinical, confrmed by imaging; MRI scanning is
treated. In recent years, propranolol has been found especially helpful (Figure 26.4).
to limit growth, and steroids, both systemic and
intralesional, are sometimes used. Laser therapy or, Treatment may be expectant in small lesions, but
in very rare cases, surgical excision may be required. sclerotherapy using a variety of sclerosant materials
(e.g. OK-432, an inactive strain of Strep. pyogenes)
Te commonest ‘vascular malformation’ in paedi- under the supervision of an interventional radiolo-
atric practice is a lymphangioma. Tis is a cystic gist has become popular in recent years.
(a) (b) (c)
Figure 26.4 (a–c) Large lymphangioma.
FIBROMATOSIS COLLI
Previously known as ‘sternomastoid tumour’, this con- tissue. It presents in the newborn. Early recognition and
dition is characterised by torticollis and a lump in the intensive treatment with physiotherapy are essential to
sternomastoid muscle. Te aetiology is unknown but it reduce the risk of permanent deformity of the neck.
is most likely an idiopathic intense fbrosis of the muscle

‘EXIT’ PROCEDURE
Advances in prenatal imaging are now such that some
congenital masses are diagnosed in utero (Figure 26.5). KEY POINTS
Tis means that the obstetrician/neonatologist can
plan for immediate intervention in the event of a life-
● A simple ‘cystectomy’ is inadequate for
threatening lesion of the head and neck, such as a lesion
a thyroglossal duct cyst and risks recur-
that might cause immediate airway compromise (con-
rence. Remove a good cuf of tissue around
genital high airway obstruction syndrome, CHAOS).
the cyst to include a portion of the hyoid
An immediate tracheotomy or ET intubation while
bone and follow the track upwards to the
the newborn baby is still receiving placental support is
tongue base.
termed ex utero intrapartum treatment (EXIT).
● Imaging is the frst-line investigation for
almost all neck masses. An ultrasound
scan under the supervision of an experi-
enced paediatric radiologist is ofen all that
is needed.
● Surgery for cysts, fstulas and sinuses to
the branchial arches is demanding, with a
risk to the cranial nerves. Refer to an expe-
rienced surgeon and consider facial nerve
monitoring.
FURTHER READING
Trimble KG, McCadden L. Cysts and sinuses of
the head and neck. In Watkinson JC, Clarke
RW, Aldren CP et al (eds). Scott-Brown’s
Otolaryngology, Head and Neck Surgery, 8th
Figure 26.5 Scan of expectant mother showing edn. CRC Press; 2018.
baby with large extracranial mass.
Congenital Disorders of the Neck 125

27 ACQUIRED DISORDERS
OF THE NECK
INTRODUCTION
Masses can arise from any of the structures in the causing parental alarm and needing investigation
neck including lymph nodes, the thyroid gland and and management. Lymph node enlargement – usu-
the salivary glands. Enlargement may be in response ally benign and of no pathological signifcance – is by
to physiological stimuli but can be due to pathology, far the commonest cause of a neck mass in children.
CERVICAL LYMPHADENOPATHY
Te lymph nodes in the neck enlarge in response to rapidly. Associated skin changes (e.g. discoloration
infections, mainly viral, in the upper respiratory tract. or discharge) warrant more careful investigation.
Fluctuation in size of these nodes is therefore physi- Red fags for cervical lymphadenopathy are listed in
ological, and part of the process whereby the child Box 27.1.
develops immunity to common upper respiratory
pathogens. Lymphadenopathy is probably a misnomer,
given the ubiquitous nature of cervical lymph node Box 27.1 Red fags for cervical
enlargement in children. A fnding of one or more
lymphadenopathy
enlarged neck nodes in a child is a frequent cause of
referral to the ORL service and may cause a great deal • Solitary node
of parental distress. Te parent and the referring clini- • Large node (more than 2 cm)
cian will be anxious to exclude malignancy, including • Nodes in unexpected site, e.g.
one of the lymphoproliferative disorders. supraclavicular
• Marked asymmetry
Management starts with a careful history and exami- • ‘Rubbery’ or hard on palpation
nation. Multiple discrete nodes and nodes that fuc- • Puckering or tethering of skin
tuate in size rather than continue to expand are • Broken skin
clearly less of a worry. Small mobile nodes (up to • Unusual ultrasound fndings
2 cm in diameter), long-standing nodes and bilateral • Systemic symptoms, e.g. unexplained
symmetrical nodes are of less concern than single, fever or malaise
large or matted nodes or nodes which have enlarged
DOI: 10.1201/9780429019128-27 Acquired Disorders of the Neck 127

Te investigation of frst choice is ultrasound scan- cytomegalovirus (CMV) or Epstein Barr Virus (EBV)
ning. An experienced radiologist is invaluable here may be useful. Infectious mononucleosis (EBV infec-
and they will look for features of normal architec- tion) is a common cause of ofen massive cervical
ture in the nodes. If the hilar architecture is normal, adenopathy in adolescence. Diagnosis is confrmed
particularly if there are adjacent nodes with a simi- by analysis of the blood flm, which will show the
lar appearance, then the node is almost certainly a atypical lymphocytes, with positive serology (mono-
benign ‘reactive’ node and, provided there is noth- spot and Paul Bunnel tests).
ing in the history or examination to warrant further
investigation, the parents can be reassured that no If a lymphoma is suspected, the child will need a full
intervention is needed (Figure 27.1). Cystic inclu- work-up to include a chest X-ray looking at the medi-
sions, loss of defnition of the hilar ‘strands’ and astinum, blood tests and bone marrow biopsy.
calcifcation all suggest an abnormal node and will
warrant further investigation, in some cases biopsy Some other specifc infectious agents that can cause
(Figure 27.2). Blood tests for serology to demonstrate true lymphadenopathy are human immunodefciency
(a) (b)
Figure 27.1 (a,b) Reactive lymph node. Ultrasound scan shows normal hilar architecture.
(a) (b)
Figure 27.2 (a,b) Ultrasound scan of an abnormal node. Loss of defnition of hilum.

virus (HIV), Mycobacterium tuberculosis, Bartonella Cervical tuberculosis is now far less common than
henselae (cat-scratch disease), and non-tuberculous NTM in western communities, but it still occurs and
mycobacteria (NTM). clinicians need to be alert to make the diagnosis. It is
most ofen found in children and adolescents who have
HIV infection in children is ofen associated with a history of recent foreign travel and may present as a
cervical lymphadenopathy, which may precede the solitary ‘rubbery’ neck node with or without systemic
development of full-blown acquired immunodef- symptoms and with or without discharge through the
ciency syndrome (AIDS). Multiple intrasalivary gland skin. Imaging may show a mass of nodes that have rup-
cysts with enlargement of the parotids is another fea- tured through the cervical fascia to form a ‘dumb-bell’
ture of cervical HIV in children. Treatment is under shaped structure, one part superfcial to the fascia and
the supervision of a paediatric infectious diseases one part deep (‘collar-stud abscess’). Imaging may show
expert but the ORL doctor may be asked to under- calcifcation which is highly suggestive of the diagnosis.
take a biopsy, not least to exclude a malignancy as Treatment is by antituberculous chemotherapy under
these children are at greatly increased risk of lym- the supervision of an infectious diseases clinician.
phomatous change in the neck nodes.
NON-TUBERCULOUS MYCOBACTERIA
NTM is a chronic granulomatous infection caused
by mycobacteria other than the tuberculosis bacillus,
hence the earlier term atypical mycobacteria (ATM).
It is becoming increasingly common and typically
afects the cervical lymph nodes with a particular
afnity for the intrasalivary lymphoid tissue, hence
the frequency of infection in and around the parotid
and submandibular regions. Te age of onset is about
2–4 years, and infection is thought to occur by oral
ingestion. Te pathogens are soil saprophytes such
as Mycobacterium bovis, M. avium intracellulare and
M. scrofulaeum. Tese probably fnd their way into
domestic water supplies and may accumulate in the
limescale deposits on bathroom and shower fttings.
Older children and adults seem to have developed
immunity and are only at risk if they have immune
dysfunction.
Te lesions are slow-growing, sometimes with a

characteristic violet appearance of the overlying
skin (Figure 27.3) and sometimes with a discharg-
ing sinus that leaks a sticky efuent onto the skin.
Tey are self-limiting and resolve over a period of
months or sometimes years, but the aesthetic efects
can be very distressing in the meantime. Diagnosis
is largely clinical but supported by imaging, which
will ofen show a ‘collar-stud’ type abscess (Figure Figure 27.3 Non-tuberculous mycobacteria (NTM).
27.4) or a multicystic appearance with fuid levels Note the skin changes.
Acquired Disorders of the Neck 129

Treatment is controversial. Te disease is benign and
self-limiting, and the child will recover completely
even if just managed by serial observation. Tis may
leave an unsightly scar, but healing can be remark-
ably good and scarring is ofen not nearly as bad as
parents fear. Tere is some evidence to support the
use of antituberculous chemotherapy to hasten reso-
lution, but these drugs are not without side efects
and the therapeutic beneft seems to be small.
Surgery is curative if the whole of the diseased mass is

removed but, as the nodes are ofen intimately related
to the parotid and submandibular glands, there is
a risk to the facial nerve trunk and to the marginal
Figure 27.4 Collar-stud abscess. mandibular branch of the facial nerve. For this reason,
many surgeons will adopt a ‘wait and see’ approach
within a mass of nodes. Identifcation of the organ- and only operate on easily accessible nodes or where
ism by either by staining or culture is notoriously dif- there is repeated infection, skin changes and serious
fcult. Histology, if needed, will show granulomatous concern regarding the aesthetic outcome. Te decision
infammation but without the caseation (necrotic is clinical and made in consultation with the parents.
foci) typical of tuberculosis.
LYMPHOMA
Malignant tumours of the lymphoid system (the com- Although a diagnosis of a lymphoma is a devastat-
monest malignancy in the neck in children) present ing event in the life of the child and the parents, the
with enlarged lymph nodes. Previous exposure to long-term prognosis – depending on stage, histology
EBV is thought to predispose, but EBV exposure is and treatment – may be excellent with greater than
very common and lymphomas are very uncommon. 90% survival for patients with many forms of early
Lymphadenopathy, especially if large, prolonged or disease.
in association with fever, weight loss and systemic
symptoms, may warrant urgent excision biopsy. Lymphomas were classifed as Hodgkin’s lymphoma
Aspiration biopsy cytology (ABC) or fne-needle or non-Hodgkin’s lymphoma based on the histology;
aspiration (FNA) is far less helpful in the diagnosis however, as chemotherapy regimens have improved,
of neck masses in children than it is in adults and this is now too simplistic to enable optimum thera-
precise diagnosis and staging of lymphomas requires peutic strategies. Classifcation and staging systems
a good-sized specimen so the pathologist can study have become both more focused and more complex
the detailed architecture of the lymph node. in recent years. Most paediatric oncologists use
the World Health Organization/Revised European
Reed–Sternberg cells, large multinucleated and American Lymphoma (WHO/REAL) system to clas-
highly distinctive cells found within the afected sify the histological type and the most recent modif-
nodes, are pathognomonic of Hodgkin’s lymphoma, cation of the Ann Arbor system to denote the stage.
the most common of the lymphomas that present
in children. It is more common in adolescence, and Te role of the ORL specialist is largely to be alert to
presentation before the age of 5 years is very rare. the possibility of a lymphoma in a child with a neck

mass and to undertake urgent excision biopsy when Chemotherapy strategies are becoming increasingly
needed. Liaise with the oncology and pathology team more refned and tailored to the individual child,
who may require a mixture of fresh tissue and tissue hence the importance of precise histological classif-
sent in formalin. Te child needs to be referred to a cation and accurate staging.
dedicated MDT and the work-up will include imag-
ing, blood tests and possible bone marrow biopsy or
harvesting.
NON-INFECTIVE INFLAMMATORY CONDITIONS

A number of conditions that mimic lymphoma condition is now known to be caused by COVID-19
can give rise to enlarged neck nodes in children. (see Chapter 29).
Rosai–Dorfman disease (sinus histiocytosis with
massive lymphadenopathy) presents with greatly Langerhans cell histiocytosis (LCH) occurs mainly in
enlarged cervical lymph nodes, fever and malaise. It young children (under the age of 5 years). It is a mul-
is of unknown aetiology and generally self-limiting. tisystem disease, again of unknown aetiology, asso-
Diagnosis is histological. ciated with unexplained fever, malaise and weight
loss. LCH, known in the past as Letterer–Siwe dis-
Castleman’s disease is a B-cell lymphoproliferative ease or eosinphilic granulomatosis, is characterised
disorder, sometimes associated with HIV infection. by the presence of lesions – including in the cervical
Te diagnosis is histological. Kikuchi–Fujimoto dis- nodes – which have a distinctive histological appear-
ease is another histological diagnosis that can mimic ance and may initially be mistaken for lymphoma. It
lymphoma, but it is benign and self-limiting. Tese can run a very aggressive course with a signifcant
conditions may have an as yet unconfrmed viral mortality (10%). Children need careful work-up and
aetiology. should be referred to a paediatric oncologist as some
will be considered for chemotherapy.
Kawasaki syndrome – an acute multisystem vasculi-
tis with associated cervical lymphadenopathy – has Tese conditions are sometimes referred to as pseu-
been of particular interest of late as a very similar dolymphomas but they are not true malignancies.
NON-LYMPHOMATOUS MALIGNANCY
In western healthcare settings, cancer is second only Neural tumours include neuroblastoma and benign
to trauma as a cause of death in children. Around 12% neurofbromas as well as some very aggressive neuro-
of childhood cancers occur in the head and neck, the ectodermal tumours.
commonest being lymphoma. Neural tumours, thy-
roid malignancy and sarcomas (rhabdomyosarcoma) Te commonest cause of thyroid dysfunction in chil-
are all described and may present as a neck mass. Te dren worldwide is still iodine defciency, but thyroid
role of the ORL doctor is to be aware and to under- swellings (goitres) are ofen found especially in teen-
take biopsy when needed. Management is under the agers, more ofen in girls. Tey may be completely
supervision of an oncology MDT, and survival rates benign and associated with the hormonal changes
for most childhood cancers have increased greatly in of adolescence, needing no intervention, or they
recent years. may be active nodules (e.g. follicular adenomas with
Acquired Disorders of the Neck 131

associated hyperthyroidism), but the child will need Blunt trauma may result from falls, sports injuries, traf-
investigation and work-up under the supervision of fc accidents and strangulation. Te main concern here
an endocrinologist. Diferentiated papillary cancers, is airway obstruction, which can be delayed, hence the
mixed papillary/follicular and rarely pure follicular need for vigilance with admission and careful moni-
cancers are not common but any thyroid mass needs toring. If there is any concern that the larynx or tra-
careful investigation. Antecedent radiation of the chea have been injured early involvement of the ORL
head and neck is a risk factor for thyroid cancer. team, to include laryngotracheoscopy – ideally under
controlled conditions in the OR avoiding ‘blind’ ET
Investigation starts with an ultrasound scan and, in intubation – is essential.
many centres, an FNA. Tis is one of the few situa-
tions in children where FNA is helpful. Treatment is
controversial and, as these tumours tend to be slow- KEY POINTS
growing and with very high survival rates, limited
surgery is ofen appropriate. ● NTM lesions improve without any treat-
ment. Be cautious about recommending
Te support of an MDT including an oncologist and intervention, especially any surgery that
endocrinologist is invaluable. could risk trauma to the facial nerve or its
branches.
● Precise histopathological diagnosis of
Trauma lymphoma is increasingly important as
treatment regimens become more refned.
Injuries to the neck in children are uncommon, but we Discuss with the oncologist and the pathol-
live in violent times and stab and gunshot injuries in ogist before taking a biopsy and send fresh
teenagers are now frequently seen in trauma centres. tissue straight to the laboratory. Cytology
Self-harm is increasingly reported in children. (FNA/ABC) is rarely helpful.
● A thyroid swelling warrants investigation
Penetrating injuries that breach the platysma have but is usually benign.
the potential to involve vessels, the airway, the
pharynx and oesophagus and the cervical nerves.
Bleeding can be catastrophic. Initial management
is in line with the general principles of resuscita-
FURTHER READING
tion – Airway, Breathing, Circulation. Urgent surgi-
Patel S, Burgess A. Guideline for the child presenting
cal exploration may be needed especially if there are
to hospital with lymphadenitis or a lymph node
features suggestive of active bleeding or a breach of
abscess. Available at: www.entuk.org/paediatric-
the airway, e.g. an expanding haematoma, ‘bubbling’
guidelines (accessed 31 January 2022).
in the wound, surgical emphysema.

28 THE SALIVARY GLANDS
INTRODUCTION
Parotitis due to mumps – an extremely common tumours are rare, and disorders such as NTM (see
childhood infection in days gone by – is now rarely Chapter 27), acute infections, juvenile recurrent
seen due to widespread vaccination. Salivary masses parotitis and lymphoma are more common causes
in childhood may be caused by disease of the epithe- of sialomegaly. Diagnosis is ofen delayed. Surgery,
lial tissue (the parenchyma of the salivary glands) or particularly of the parotid, is especially challenging
diseases of the lymphoid tissue. Epithelial salivary in children.
RANULA
Tis is a mucous retention cyst in the foor of the cystectomy ideally with removal of adjacent salivary
mouth (Figure 28.1). It is occasionally found in the epithelium (i.e. the sublingual gland) is better.
newborn but more ofen presents in toddlers when it
forms a smooth translucent swelling (‘frog’s belly’). It
is thought to occur as a result of outfow obstruction
in the sublingual or one of the minor salivary glands.
Mucinous saliva continues to be produced, becomes
encysted, and the swelling expands along the tissue
planes. Te cyst is found between the mucosa of the
foor of the mouth and the mylohyoid muscle, usu-
ally to one side of the midline. A very large ranula
will extend well into the neck (plunging ranula). Te
appearance causes parental alarm and, if very large,
it can interfere with swallowing.
Diagnosis is clinical but imaging can help treatment

planning. Treatment is surgical. Simple ‘marsupiali-
sation’ or laying the sac open is not enough; a total Figure 28.1 A ranula.
DOI: 10.1201/9780429019128-28 The Salivary Glands 133

SIALADENITIS
Acute infections – particularly of the parotid – may
occur due to a variety of viruses including classically
the mumps virus, which causes bilateral parotitis.
MMR (mumps, measles, rubella) vaccination had
all but eliminated this highly contagious condition
but there have been outbreaks among adolescents
in particular in recent years due to poor vaccine
uptake in some communities. Complications
include meningitis, orchitis in boys and unilateral
sensorineural hearing loss. Diagnosis is confrmed
by serology.
Acute bacterial infection of the parotid (suppu-

rative parotitis, Figure 28.2) can cause extensive
cellulitis of the head and neck with abscess forma-
tion. Premature newborn infants and children with
immunosuppression, poor nutrition or dental sep-
sis are especially at risk. Intensive treatment with
IV antibiotics is needed, and occasionally abscess
drainage, taking great care not to traumatise the very
superfcial facial nerve.
HIV infection in the parotid can give rise to multiple

microcysts, and the diagnosis should be considered
in unexplained parotidomegaly. Figure 28.2 Acute suppurative parotitis.
RECURRENT PAROTITIS
Tis is characterised by repeated episodes of pain- easily managed with analgesics and antibiotics as
ful swelling of one or both parotids. It is common- appropriate.
est in older children and teenagers, and ofen settles
afer puberty. Te aetiology is unknown, and both Contrast studies – cannulating the parotid duct and
infective agents (e.g. EBV) and autoimmune factors demonstrating the ductal architecture – may show
have been implicated. Superinfection with pyogenic strictures and ectatic ducts and may even be therapeutic
organisms can occur with widespread destruction of in fushing debris from the gland. As with management
the acinar and ductal structures of the gland (sialec- of calculi, there is growing interest in techniques to
tasia, Figure 28.3) and even fstula formation to introduce increasingly sophisticated endoscopes to the
the skin but, more usually, symptoms are mild and parotid duct and undertake sialendoscopic treatment.

Figure 28.3 MRI scan showing extensive bilateral parotid duct ectasia.
CALCULI
Salivary stones are much more common in adults antibiotics may be needed if the gland is infected, and
than in children. Tey present with painful enlarge- it may be possible to remove the stone by massag-
ment usually of the submandibular gland. Te stone ing it toward the orifce of the duct or by opening or
is sometimes palpable in the foor of the mouth, but probing the duct orifce at surgery. Tere is increas-
plain X-ray usually demonstrates it well. A course of ing interest in and enthusiasm for sialendoscopy.
SALIVARY TUMOURS
Haemangioma of the parotid is a congenital haemangioma pattern of a proliferative phase fol-
tumour but it will not usually be apparent until lowed by gradual involution.
the child is at least a few months old. It may grow
rapidly, causing discoloration and necrosis of Treatment, if needed, has been greatly improved by
the skin, giving rise to extreme alarm as parents the use of propranolol (see Chapter 28) and some-
and doctors suspect an aggressive malignancy. times laser therapy. Surgery should be avoided, but
Early referral and investigation by MRI scanning very large or aggressive lesions may respond to cyto-
allow a defnitive diagnosis, without the need for toxic agents under the supervision of a paediatric
biopsy (Figure 28.4). Te prognosis is excellent, oncologist.
with the natural history following the expected
The Salivary Glands 135

Lymphomas are considered in Chapter 27.
Salivary epithelial neoplasms in children are rare, but

a higher proportion of childhood parotid tumours
are malignant than is the case in adults, hence the
need for early investigation and continued vigilance.
Pleomorphic salivary adenoma (PSA) is the com-
monest childhood epithelial neoplasm and occurs
most ofen in the parotid.
Malignant tumours include mucoepidermoid car-

cinoma and acinic cell tumours. Diagnosis is ofen
delayed. Management may involve surgery and che-
motherapy and should be undertaken by an experi-
enced team to include a paediatric oncologist.
Figure 28.4 MRI scan of a parotid haemangioma.
PAROTIDECTOMY IN CHILDREN
Conditions such as branchial fstula and lymph- by appropriately trained and experienced surgeons.
angiomas of the head and neck may involve the Facial nerve monitoring is strongly advised. Te risk
parotid, and parotidectomy or a parotidectomy-type to the nerve is very real, given its more superfcial
approach is ofen part of the surgery needed for these position in children and the frequent presence of scar
disorders. Parotidectomy may be part of the strategy tissue due to recurrent infections (e.g. in branchial
for the treatment of malignant disease. Tis is highly fstulas). A facial paresis is a devastating complica-
specialised surgery and should only be undertaken tion in a child.
SIALORRHEA
‘Drooling’ or ‘dribbling’ are the colloquial terms to Treatment at first is focused on measures to
describe salivary overspill from the oral cavity onto improve oromandibular posture, ofen with the
the lips and the skin of the lower face. Tis is not true help of a SALT and orthodontist. Pharmacological
sialorrhea as salivary volume is usually normal; the interventions include the use of antimuscarinic
issue is poor retention of saliva usually due to neu- agents (e.g. hyoscine), frequently administered by
rological disease which interferes with the normal dermal patches. ORL clinicians are ofen involved
swallow, i.e. neuromuscular incoordination. Some as part of the MDT looking afer these children and
degree of ‘drooling’ is normal and physiological up are called upon when conservative measures fail.
to the age of about 4 years, but beyond this it can Many ORL clinicians have developed expertise in
be very troublesome in children with neurodisabil- the injection of Botulinus toxin (Botox A) directly
ity due to a variety of aetiologies, typically cerebral into the parotid and submandibular glands. Botox
palsy. acts by binding to the presynaptic receptors to block

acetylcholine release from parasympathetic secre- postoperative course and the technique is not ofen
tomotor nerves, thus reducing salivary fow. Tere recommended.
is a risk of extravasation and of trauma to the facial
nerve, but accurate placement of the injections
with the help of ultrasound guidance has made the KEY POINTS
procedure far safer. Te efect is temporary, and
the procedure may need to be repeated every few ● Complete removal of a ranula will
months. give a better long-term outcome than
‘marsupialisation’.
Transposition of the submandibular ducts so that ● A parotid tumour in a child is ofen
they open into the pharynx rather than via their malignant.
normal distal orifces in the foor of the mouth is a ● Parotid surgery in children is challeng-
now well-established technique. Many surgeons will ing. Refer to an experienced surgeon and
excise the sublingual glands at the same time. monitor the facial nerve throughout.
Excision of the submandibular glands is an extreme

measure and only considered if all earlier approaches
fail, but it is an efective way to reduce salivary fow, albeit
with serious adverse efects including dental caries. FURTHER READING
Gleeson M. Disorders of the salivary glands in chil-
Duct ligation has been advocated in the past, but dren. In: Clarke R (ed.). Pediatric Otolaryngology:
pain, swelling and recanalisation make for a difcult Practical Clinical Management. Tieme; 2017.
The Salivary Glands 137

29 COVID-19 IN
CHILDREN’S ORL
INTRODUCTION
Severe acute respiratory syndrome coronavirus 2
(SARS-CoV-2) – a virus isolated in Wuhan, China, in
December 2019 – is the causative organism of COVID-
19 (Figure 29.1). Tis new disease spread rapidly
worldwide such that the WHO declared a global pan-
demic in March 2020. Te disease mainly afects the
respiratory system and manifestations may vary from
no symptoms at all to signifcant morbidity requiring
intensive ventilatory support up to organ failure and
death. Higher mortality rates were associated with gen-
der – males faring worse – obesity, and the presence of
pre-existing conditions such as diabetes. Spread is pri-
marily by droplet transmission through the respiratory Figure 29.1 Severe acute respiratory syndrome
mucosa but may occur by direct contact, via the gas- coronavirus 2 (SARS-CoV-2), the causative organism
trointestinal (GI) tract, and via the conjunctiva. Early of COVID-19.
in the course of the pandemic it became apparent that
healthcare personnel, including ORL specialists, were at meant greatly reduced morbidity and mortality, but
particular risk. Large numbers contracted COVID-19, COVID-19 continues to spread and intensive public
which led to serious illness and, in many cases, death. health measures to reduce transmission are still in
place. Widespread vaccination of adults has reduced
Vaccines were quickly developed and were approved the prevalence and severity in the adult population,
for use at the end of 2020 and in early 2021. A world- but cases in children continue to rise, albeit typically
wide vaccination programme is underway which has with few if any symptoms and little morbidity.
ORL SERVICE DELIVERY

COVID-19 brought about seismic changes in for adults and children. ORL interventions – par-
healthcare including the provision of ORL services ticularly in children – involve very close contact
DOI: 10.1201/9780429019128-29 COVID-19 in Children’s ORL 139

between clinician and child with exposure to upper for COVID-19 became the norm, with inevitable dis-
respiratory secretions which form droplets contain- ruptions and delays. Staf shortages – ofen with little
ing viral particles. Healthcare workers (HCW) are or no notice due to colleagues becoming ill or needing
particularly at risk when performing ‘aerosol-gen- to isolate – made planning difcult and uncertain.
erating procedures’ (AGPs) such as airway endos-
copy, tracheostomy, and bone-removal during Some conditions seen in paediatric ORL declined
tympanomastoid surgery. Examination of a child’s in incidence, including acute tonsillitis, AOM and
nose or pharynx may be an AGP, particularly if the OME. Te reasons are unclear but may be related
child cries or screams, and ORL staf are frequently to reduced exposure during ‘lockdowns’ and to
exposed to a high viral load. the school closures that were implemented in
many jurisdictions. Some, but not all, departments
During the early stages and the peak of the pandemic, reported a ‘spike’ in the incidence of cases of idio-
clinic visits were greatly reduced and patients with pathic facial palsy in children, again for reasons that
non-urgent conditions had their scheduled surgeries are not clear.
and outpatient consultations cancelled or rescheduled.
Many consultations took place by telephone, or online Many ORL interventions (e.g. tracheostomy, suction
via ‘Zoom’, ‘FaceTime’ or ‘Teams’. Hospital visits were of respiratory secretions, bronchoscopy) involve the
curtailed and children came to appointments accom- potential release of aerosols containing particles of
panied by only one parent (Figure 29.2). ORL surgery tissue and secretions from the respiratory mucosa
was confned to urgent cases, and preoperative testing and are known as AGPs.
Figure 29.2 Consultations in the COVID era were very different from before. PPE, social distancing, and
reduced numbers of children and parents in waiting rooms all helped to reduce environmental transmission.

Particular care and a high level of personal protec-
tive equipment (PPE) – including the use of the fl-
tering face-piece (FFP) respirators, which ofer much
greater protection to the wearer than standard surgi-
cal facemasks – are needed for these interventions.
National medical associations, public health bod-

ies and ORL societies recommended varying lev-
els of PPE to reduce the risks of COVID-19 spread
during encounters with healthcare professionals
(Figure 29.3). Despite widespread vaccination and
reduced morbidity and mortality fgures, some PPE
requirements are largely still in place. Hospitals
and clinics introduced various changes to reduce Figure 29.3 Varying levels of PPE were recom-
potential exposure, including restricting the num- mended to reduce the risks of COVID-19 spread.
ber of visitors, ofen permitting only one parent
or carer to accompany a child on a hospital visit, Face coverings, double gloving, respirator masks,
keeping numbers in waiting rooms to a minimum, increased time intervals between consultations and lon-
and mandating face coverings for parents and older ger waiting times for surgery have signifcantly altered
children. the dynamics of patient and clinician interaction.
Testing of children, parents and healthcare per- Residents and trainees inevitably had less exposure
sonnel on a regular basis with hospital visits to surgical procedures, and training programmes in
rescheduled in the event of a positive test made many cases had to be extended as a result.
for frequent disruptions to planned operating lists
and clinics. ‘Telemedicine’ with remote consulta- Requirements are now less stringent and subject to
tions by telephone or by computer links became revision, but there is no doubt that COVID-19 has
commonplace. brought about many changes that are here to stay.
Te ‘new normal’ looks likely to continue for some
time to come.
CLINICAL FEATURES OF COVID-19 IN CHILDREN

It is now clear that children and young people (CYP) COVID-19 developed a serious and life-threatening
are susceptible to COVID-19 but the risk of contract- illness characterised by multisystem infammation
ing the disease is considerably lower than in adults. – multisystem infammatory syndrome in children-
Symptoms tend to be minimal or absent, morbidity (MIS-C). Te clinical and laboratory features were
is much less and mortality extremely rare. Cough, not dissimilar to the fndings in other multisystem
fever, disturbances of taste and smell and rhinor- infammatory disorders in children (e.g. Kawasaki
rhoea have all been reported. Te role of CYP in dis- disease).
ease transmission is as yet unclear.
Later manifestations of COVID-19 (long COVID)
Early in the course of the pandemic, it became appar- are described, but the precise efects will only become
ent that a small number of children infected with known in the years to come.
COVID-19 in Children’s ORL 141

CHILDREN’S ORL ‘POST-COVID’
Education and training of healthcare personnel FURTHER READING
was especially challenging, with a reduced number Allen DZ, Challapalli S, McKee S et al. Impact of
of cases for teaching, a prohibition on educational COVID-19 on nationwide pediatric otolaryngol-
gatherings, and the need for many doctors-in-train- ogy: otitis media and myringotomy tube trends.
ing and their teachers to take time of due to ill- Am J Otolaryngol. Mar-Apr 2022;43(2):103369.
ness or the need to isolate. ‘Lockdowns’ in various doi: 10.1016/j.amjoto.2021.103369.
countries involved prolonged closure of schools. COVID-19 information and guidance. Available at:
Te long-term efects on children’s social develop- https://www.yoifos.com/covid-19-information-
ment have yet to be determined. Parental reluctance and-guidance (accessed 31 January 2022).
to present for appointments, hospital policies with ENT UK. Guidance for ENT during the COVID-
regard to waiting lists for routine visits, and a grow- 19 pandemic. Available at: https://www.entuk.
ing backlog of children who were not seen during org/guidance-ent-during-covid-19-pandemic
the height of the pandemic have all made for dif- (accessed 31 January 2022).
fcult times ahead. Hobbs CV, Khaitan A, Kirmse BM, Borkowsky W.
COVID-19 in children: a review and paral-
It is likely that reduced exposure to the common viral lels to other hyperinfammatory syndromes.
illnesses of childhood during periods of ‘lockdown’ Front Pediatr. 2020;8:593455. doi: 10.3389/
or school closures will result in lowered immunity fped.2020.593455.
to these conditions, and many children’s services are
already seeing a surge in admissions of children with,
for example, RSV.
Te legacy of the pandemic on long-term health and

well-being is yet to be determined.
KEY POINTS
● COVID-19 is prevalent in children.

● It is associated with much lower morbidity
than in adults.
● COVID-related fatalities in children are
extremely rare.
● A very small number of children may
present with a multisystem infammatory
picture.
● ORL organisations have been to the fore
in producing efective guidelines for safe
practice.
● Te worldwide pandemic has brought
about changes in ORL clinical practice,
some of which look likely to persist.

INDEX
abscesses ‘balanced’ 3–4 chronic otitis media 49
deep neck spaces 19–21 children with Down syndrome deep neck space 19–21
intracranial 22–3 12, 69 membranous tracheitis 110
mastoid 21–2 equipment 3 parotid gland 134
accessory auricle 25–6 analgesia pharynx 67
achondroplasia 75 in AOM 46 rhinosinusitis 86, 87
acute otitis media (AOM) 21, 45 surgery 4 see also bioflms
diagnosis/investigations 46 angiofbroma 95 balance disorders 59–60
management 46 ankyloglossia 70 Bartonella henselae 12
outcomes 45 antibiotics Beckwith–Wiedemann
pathogenesis 45 acute otitis media 46 syndrome 70
predisposing factors 46 acute rhinosinusitis 86 behavioural observation
recurrent 47 antihistamines 93 audiometry (BOA) 37
acyclovir 64, 65 antimuscarinic agents 136 Bell’s palsy 64, 65
adeno-tonsillar enlargement 74 antiviral agents 64 benign paroxysmal positional
adenoidectomy 69 AOM, see acute otitis media vertigo (BPPV) 60
in AOM 46, 47 aortic arch, double 107 benign paroxysmal vertigo of
chronic rhinosinusitis 89 aortopexy 107 childhood (BPVC) 59, 60
in OME 43 Apert’s syndrome 13 benzodiazepines 3
adenoids 69 apnoea, ‘central’ 74 Bezold’s abscess 22
adenotonsillectomy 69, 75 apnoea/hypopnoea index (AHI) bilevel positive airway pressure
adenoviruses 84 74 (BiPAP) 107
adolescence, balance disorders 60 arrhinia 77 bioflms 41, 47, 69
adrenaline, nebulised 100, 115 aryepiglottoplasty 104 chronic rhinosinusitis 87–8
aerosol-generating procedures associations 11, 12, 13 birth trauma 64
140–1 attention defcit hyperactivity bleeding, tonsils 68, 69
air–bone gap 38, 39, 42 disorder (ADHD) 13–14, 35 bone-anchored hearing aids
airway obstruction 99–101 atypical mycobacteria, see non- (BAHAs) 27, 28, 54, 55
assessment 101 tuberculous mycobacteria bone-conducting hearing aids
defnitive treatment 101 auditory brainstem implant 57 53–5
early management 100 auditory brainstem response implantable 54–5
nasal 77, 78 (ABR) tests 33 non-implantable 53–4
red fags 100 auditory neuropathy spectrum bone-conduction testing 39
airway support, techniques 115 disorders 35 botulinus toxin (Botox A) 136–7
Alder Hey Children’s Hospital 2 auditory processing disorders 35 bougie 3
allergens 91, 93 aural toilet 50–1 branchial clef anomalies 25, 123
allergic rhinosinusitis 91–3 autistic spectrum disorders branchial (pharyngeal) arches
Alport’s syndrome 13 (ASD) 13, 14, 35 25, 123
Ambu-bag™ 100 branchio-oto-renal syndrome 27
amoxycillin 46, 86 bacterial infections breastfeeding 46
anaesthesia 3–4 acute otitis media 45 difculty in tongue tie 70
Index 143
bronchial cysts, congenital 107 computed tomography (CT) dizziness 59–60
bronchus, foreign bodies 16–17 choanal atresia 78 Down syndrome (trisomy 21)
bucconasal membrane 77 cholesteatoma 51–2 11–12, 14, 41, 44, 63, 105
bullying 10 chronic rhinosinusitis 88 anaesthesia 12, 69
button batteries, ingestion 17–18 fbrous dysplasia 96 macroglossia 70
parapharyngeal abscess 20 ‘drooling’/‘dribbling’ 136–7
calculi, salivary glands 135 connexin 26 mutations 32 drug-induced sleep endoscopy
Calnan’s triad 71 consent 4–5 (DISE) 74
case notes 7 tracheostomy 116 ‘dying spells’ 107
Castleman’s disease 131 consultation 7–10
caustic ingestion 17, 113 COVID-19 pandemic 139–41 ear canal
cephalosporins 46, 86 remote 8 atresia 27
cerebral palsy 70, 74 continuous positive airway foreign bodies 15
cerumen, impacted 28 pressure (CPAP) 75, 107 ear discharge, chronic otitis
cervical lymphadenopathy 127–9 coronaviruses 45, 84 media 49
lymphoma 130 corticosteroids EarBuddies 26–7
non-infective infammatory airway obstruction 100 eardrum
conditions 131 intranasal 43 perforation 43, 49, 50–1
CHARGE association (or intranasal (INCS) 89, 93 repair surgery 51
syndrome) 13, 64, 78 oral 115 retraction pockets 51
chest recession 99 systemic 43, 93, 110 education and training,
child abuse 9–10 Cotton–Meyer classifcation 112 COVID-19 pandemic 142
ear trauma 29 COVID-19 pandemic 8, 10 encephalocoele, nasal 79
Fabricated or Induced clinical features in children endoscopes 8
Illness (FII) 10 141–2 endoscopy, airway 101
possible signs 9 ORL service delivery 139–40, endotracheal (ET) intubation 100
child protection 9–10 142 extubation failure 111
choanal atresia 77–8 cranial nerve palsies 64 patient with Down syndrome 12
cholesteatoma 28, 49, 50 craniofacial anomalies 13, 63, trauma 111
aetiology 51 75, 123 endotracheal (ET) tube 3, 4
diferences between adult and cri du chat syndrome 107 length 116
paediatric 51 ‘cricoid split’ 111 eosinophilic granulomatosis
treatment 52 cricothyroid puncture 100 (Letterer–Siwe disease) 131
chronic otitis media (COM) cricotracheal resection (CTR) 112 epiglottis, bifd 107
clinical features 49 ‘croup’ 110 epiglottitis, acute 109
eardrum perforation 50–1 Crouzon’s syndrome 13 epistaxis 81, 95
prevalence 49 cystic fbrosis (CF) 41, 46, 88 Epstein Barr virus (EBV) 67, 128
chronic rhinosinusitis (CRS) cytomegalovirus (CMV) erysipelas 29
87–9 infection 32 ethnicity 49
cidofovir 111 European Position Paper on
ciliary dyskinesia 41, 88 day case (same-day) surgery 2–3 Rhinosinusitis and Nasal
ciliary motility disorders 84 deafness, see hearing loss Polyps (EPOS2020) 84–5,
clef lip and palate 71 deep neck space infection 19–21 86, 89
clinics, dedicated paediatric 1–2 dental infections 19 Eustachian tube
cochlear implantation 56 dentigerous (odontogenic) cysts 80 dysfunction 41, 51
codeine 4 dermoid cyst 80, 121–2 infation 43
‘cold spatula test’ 8 development, normal 8–9 examination 8
collar-stud abscess 129–30 dexamethasone 100, 110, 115 EXIT procedure 125
144 Index
external ear glial heterotopia 79 hearing strategies 42–3
congenital anomalies 25–8 glioma, nasal 79 hemifacial microsomia 27, 65,
development 25 glossoptosis 13 123
infammation 28–9 ‘glue ear’ 41 history taking 7–8
prominent 26–7 Goldenhar’s syndrome 13, 27, 65 Hodgkin’s lymphoma 130
trauma 29 Gradenigo’s triad/syndrome 21–2 house dust mite 91, 93
extradural abscess 22 Grisel’s syndrome 69 human immunodefciency virus
extubation, failure 111 ‘grommets’ 43, 47 (HIV) 128–9, 134
eye protection, facial palsy 65 growth chart 9 human papillomavirus (HPV)
growth and development 8–9 110
Fabricated or Induced Illness Guedel airway 3, 78 hypothyroidism, congenital 70
(FII) 10
facemasks, ventilating 3 haemangiomas 124 ibuprofen 46
facemasks (COVID-19) 141 airway 106–7 immune complex disorders 68
facial nerve imaging 124, 135, 136 immunodefciency 88
anatomy 63 nasal 81 immunoglobulin E (IgE) 92
risk to in parotidectomy 136 parotid gland 135–6 immunotherapy 93
facial palsy 22, 64–5 haematoma, pinna 29 impedance audiometry
acquired 64 Haemophilus infuenzae 19, 67, (tympanometry) 39, 42
congenital 64–5 86, 87 infectious mononucleosis 67
spike in COVID-19 pandemic Haemophilus infuenzae B (HiB) infuenza viruses 84
140 109 insects, ear canal 15
fascia, neck 19 hay fever 92 intracranial complications 22–3,
fbromatosis colli 124 healthcare workers, COVID-19 85, 86
fbrous dysplasia 95–6 risk 140
frst arch anomalies 25 hearing aids 35 Jervel/Lange Neilson syndrome 13
fexible awake airway endoscopy auditory brainstem implant 57 juvenile ossifying fibroma
101 bone-conducting 53–5 (JOF) 96
forceps 3 cochlear implant 56–7 juvenile-onset recurrent
optical 17 microtia 27, 28 respiratory papillomatosis
foreign bodies middle ear implants 57 (JORRP) 110–11
ear 15 in OME 44
ingested 16, 17–18 hearing assessment 33 Kawasaki syndrome 131
inhaled 16–17 behavioural observation 37–9 Kiesselbach’s plexus 81
nose 15–16 impedance audiometry
Fraser competence 4 (tympanometry) 39 labyrinthitis 22, 60
frontal sinus 84 microtia 27 Langerhans cell histiocytosis
functional disorders 10 hearing loss 131
hearing loss 35 aetiology 31–2 laryngeal cysts 107
imbalance 60 degrees defned 37 laryngeal mask airway (LMA) 3
functional endoscopic sinus eardrum perforation 50 laryngeal web 104–5
surgery (FESS) 89 early investigations 33–4 laryngo-tracheobronchoscopy
furuncle 29 functional 35 101
Fusobacterium necrophorum 22 incidence 31 laryngo-tracheoscopy 4
permanent childhood 31, 32 laryngocoeles 107
General Medical Council screening and surveillance 33 laryngomalacia 103–4
(GMC) 9 syndromic 13, 32 laryngoscopes 3
Gillick competence 4 unilateral 35 laryngospasm 109
Index 145
laryngotracheal clef 105–6 mast cells 91 neck masses
laryngotracheal reconstruction mastoid abscess 21–2 non-lymphomatous
104, 112 mastoid process 63 malignancy 131–2
laryngotracheal stenosis, mastoiditis 22 see also cervical
congenital 105 maternal infections 27, 32 lymphadenopathy
laryngotracheal trauma 113 maxillary antral lavage 89 neck spaces 19–21
laryngotracheobronchitis, acute maxillary sinus 84 neck trauma 132
(ALTB) 110 measles, mumps, rubella (MMR) neonatal asymmetric crying
Lemierre’s syndrome 22 vaccine 134 facies 65
Letterer–Siwe disease 131 MED-EL ADHEAR® 54 neural tumours 131
leukotriene receptor antagonists 93 MED-EL® Bonebridge system neurodevelopmental conditions
lingual frenulum 70 54–5 13–14
lingual thyroid 123 mediastinal tumours 107 neuromuscular disorders 75
Little’s area 81 meningocoele, nasal 79 newborn
local anaesthetics 3 micrognathia 12 airway obstruction 99–101
lockdowns 142 microtia 12, 26–8 facial palsy 64
long COVID 141–2 aetiology 27 nasal obstruction 77, 78
lymph node, benign ‘reactive’ canal atresia 27, 28 non-accidental injury (NAI) 29
128 management 27–8 non-Hodgkin’s lymphoma 130
lymphadenopathy, cervical 127–9 middle ear implants 57 non-steroidal anti-infammatory
lymphangiomas 70, 124 migrainous disorders 59 drugs (NSAIDs) 4
lymphoma 68, 128, 130–1 mirror test 77 non-tuberculous mycobacteria
classifcation 130 mitochondrial defects 32 (NTM) 129–30
diagnosis 130 montelukast 93 nose
role of ORL specialist 130–1 ‘Montgomery’ legal judgment 5 choanal atresia 77–8
Moraxella catarrhalis 45, 49, embryology 77
McCune–Albright syndrome 96 86, 87 encephalocoele 79
macroglossia 70 mucopolysaccharidosis 70, 75 examination 8
‘magic cream’ 3 multisystem infammatory foreign bodies 15–16
Magill forceps 3 syndrome in children 141 malignant disease 97
Magill laryngoscopes 3 mumps 133, 134 masses 80–1
magnetic resonance imaging mycobacteria, non-tuberculous pyriform aperture stenosis
(MRI) 129–30 78–9
airway obstruction 101 Mycobacterium tuberculosis 129 trauma 97
angiofbroma 95–6 myringotomy 46 vascular malformations 81
dynamic 74
facial palsy 65 nasal challenge 92 obstructive sleep apnoea (OSA)
lymphangioma 124 nasal decongestants 93 73–5
nasal encephalocoele 79 nasal discharge odontogenic (dentigerous)
parotid haemangioma 135, 136 allergic rhinosinusitis 91 cysts 80
malignancy chronic rhinosinusitis 88 oesophagus, atresia 106
causing facial palsy 64 nasal irrigation, saline 89 OME, see otitis media with
lymphoma 130–1 nasal septum efusion
non-lymphomatous neck deviation 81 ondansetron 4
masses 131–2 prominent blood vessels 81 opiates 4
nose 97 nasoalveolar cysts 80 orbital cellulitis 22
salivary glands 136 nasolabial cysts 80 organisations and societies 5
mandibulofacial dysostosis 123 nasolacrimal duct cysts 80 oropharyngeal airway 3
146 Index
OSA, see obstructive sleep tracheotomised children 116, pure-tone audiometry (PTA) 33,
apnoea 118 37–8
otitis externa 28–9 parotid gland in OME 42
otitis media ectasia 134, 135 report 34
acute, see acute otitis media tumours 135–6 pyriform aperture stenosis 78–9
(AOM) parotidectomy 136
chronic, see chronic otitis parotitis 133, 134 quinsy (peritonsillar abscess)
media (COM) recurrent 134–5 20–1
complications 22–3, 47 Pendred’s syndrome 13, 32
facial palsy 64 perichondritis 29 ranula 133
otitis media with efusion perioperative care 2–3 rapid eye movement (REM)
(OME) peritonsillar abscess (quinsy) sleep 73
aetiology 41 20–1 recurrent acute rhinosinusitis
in clef lip and palate 71 permanent childhood hearing (RARS) 85–6
clinical presentation 42 impairment (PCHI) recurrent respiratory
incidence 41 31, 32 papillomatosis (RRP)
investigations 42 Personal Child Health Record 8 110–11
management 42–4 personal protective equipment Reed–Sternberg cells 130
otoacoustic emissions (OAEs) 33 (PPE) 141 respiratory syncytial virus (RSV)
otolaryngologists, paediatric 1 petrous apex abscess 22 45, 84, 110
otolaryngology (ORL) services Pfeifer’s syndrome 13 retraction pockets 51
1–2 pharyngitis, acute 67–8 retrieval team 101
delivery during COVID-19 pharynx, examination 8 retropharyngeal abscess 20
pandemic 139–40 phlegmon 20 rhinolalia aperta 69
otoscopy Pierre Robin sequence 13, 71 rhinosinusitis 84
acute otitis media 46 pinna acute 83–6
cholesteatoma 50 reconstruction 28 allergic 91–3
eardrum perforation 50 trauma 29 chronic 87–9
in OME 42 pizotifen 60 European Position Paper
oxygen saturation 74, 100 play audiometry 37 (EPOS) 84, 85
pleomorphic salivary adenoma rhinoviruses 84
paediatric early warning signs (PSA) 136 Rosai–Dorfman disease 131
(PEWS) 23 Poland syndrome 64 Royal College of Paediatrics and
paediatric intensive care units pollens 92, 93 Child Health 5
(PICUs) 1, 111 polysomnography 74 rubella infection, maternal
papillomatosis, recurrent postoperative nausea and 27, 32
respiratory 110–11 vomiting (PONV) 4
paracetamol 46 Pott’s pufy tumour 22 saccular cyst 107
parainfuenza viruses 45, 84, 110 pre-auricular sinus 25 saline nasal irrigation 89
paranasal sinus infections 84 ‘pre-med’ 3 salivary glands 133–7
complications 22–3 pre-oxygenation 4 calculi (stones) 135
parapharyngeal abscess 19–20 prenatal imaging 125 masses 133, 135–6
parents 4, 7 propranolol 60, 107, 135 sialadenitis 134
child with obstructive sleep prostheses, external ear 28 screening, hearing loss 33
apnoea 74 pseudolymphoma 131 sensorineural hearing loss,
children with syndromes 14 Pseudomonas aeruginosa 49 syndromes causing 13
during child examination 8 psychological factors 10 sepsis 23
informed consent 4–5 pulse oximetry 74 sequences 11, 12, 13
Index 147
severe acute respiratory submandibular ducts tracheocutaneous fstula 119
syndrome coronavirus 2 ligation 137 tracheomalacia 106, 107
(SARS-CoV-2) 139 transposition 137 tracheostomy 115
clinical features in children submandibular glands, excision avoidance 115
141–2 137 complications 117
sialadenitis 134 suppurative parotitis 134 decannulation 118
sialectasia 134, 135 surgical (operating) lists 2–3 diferences between adult and
sialorrhoea 136–7 sweat test 88 paediatric 117
SIGN guidelines (tonsillectomy) 68 swimmer’s ear 28–9 home care 118
single gene defects 32 syndromes 11–13 indications 115
sinusitis, complications 22–3 causing hearing loss 13, 32 postoperative care 117–18
Sistrunk’s procedure 121, 122 technique 115–16
skin tests, allergic rhinosinusitis 92 T-tubes 43 tracheocutaneous fstula
sleep-disordered breathing tachypnoea 99 119
73–5 temporal bone fracture 64 tracheostomy tubes 118
slide tracheoplasty 105 teratoma, nasal 80 tracheotomy 115
snoring 73 Tornwaldt’s cyst 80 transfer, acutely ill child 101
societies 5 THRIVE technique 4 trauma
socioeconomic factors 49 thyroglossal duct cyst 122 causing facial palsy 64
sodium cromoglicate 93 thyroid cancers 132 external ear 29
Sofband® 27, 54 thyroid dysfunction 131 laryngotracheal 113
specialist centres 2, 101 thyroid gland nasal 97
speech and language therapist development 122 neck 132
(SALT) 71, 113, 136 lingual 123 Treacher Collins syndrome
sphenoid sinus 84 swellings (goitres) 131–2 12, 27
Staphylococcus aureus 19, 45, tobacco smoke, exposure to turbinate reduction surgery
49, 110 84 89, 93
‘sternomastoid tumour’ 124 tongue tie 70, 71 Turner’s syndrome 12
stertor 99 tonsillectomy 68–9, 75 22Q11 deletion syndrome
Streptococcus milleri 86 in OSA 75 (DiGeorge syndrome) 13
Streptococcus pneumoniae 19, 67, SIGN guidelines 68 tympanometry (impedance
86, 87, 110 tonsillitis 67–8 audiometry) 39, 42
Streptococcus pyogenes 45, 49, tonsilloliths 68 tympanoplasty (myringoplasty) 51
110 tonsillotomy 75 tympanosclerosis 43–4, 50
group A beta-haemolytic 67 tonsils tympanostomy tubes (ventilation
stridor 99, 101 asymmetry 68, 71 tubes/ ‘vents’) 43, 47
laryngomalacia 103 enlarged 74
subdural abscess 22 non-infammatory ultrasound scanning
subglottic cysts 111 conditions 68 cervical lymph nodes 128
subglottic stenosis (SGS) 105, 111, TORCHES association 32 collar-stud abscess 130
112 torticollis 124 dermoid cyst 121, 122
Cotton–Meyer classifcation toxoplasmosis 32 thyroglossal cyst 122
112 tracheal ring, complete 105 Usher’s syndrome 32
endoscopic appearance 112 tracheal tug 99 uvula, bifd 71
surgical treatment 112 tracheitis, bacterial
sublingual/subcutaneous (membranous) 110 vaccination
immunotherapy (SLIT/ tracheo-oesophageal fstula COVID-19 139
SCIT) 93 (TOF) 101, 106 HPV 111
148 Index
vascular compression 107 vestibular clinics 60 vocal cord palsy 104, 113
vascular masses 124 vestibular neuronitis 60 voice disorders 113
nasal 81 viral infections
see also haemangiomas acute otitis media 45 Waardenburg’s syndrome
vascular ring 107 causing facial palsy 64 13, 32
VATER (VACTERL) association pharynx 67–8 waiting rooms 2
13, 106 rhinosinusitis 84 Waldeyer’s ring 67
velopharyngeal insufciency 69 visual reinforcement audiometry wheezing 99
venous sinus thrombosis 22 (VRA) 37
ventilating facemask 3 vocal cord nodules 113 X-linked disorders 32
Index 149

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DISEASES O F T H E EAR,

NOSE & THROAT I N

Raymond W Clarke BA, BSc., DCH, FRCS, FRCS(ORL)

Boca Raton London New York

CRC Press is an imprint of the

and by CRC Press

CRC Press is an imprint of Taylor & Francis Group, LLC

Library of Congress Cataloging-in-Publication Data

Names: Clarke, Ray (Raymond) author.

ISBN: 978-1-138-57942-2 (hbk)

Typeset in Minion Pro

ORL SERVICES FOR CHILDREN

DOI: 10.1201/9780429019128-1 Introduction 1

ideally with a trained and registered children’s nurse

Children are best cared for as close to home as possi-

2 DISEASES OF THE EAR, NOSE & THROAT IN CHILDREN

4 DISEASES OF THE EAR, NOSE & THROAT IN CHILDREN

ORGANISATIONS AND SOCIETIES

6 DISEASES OF THE EAR, NOSE & THROAT IN CHILDREN

DOI: 10.1201/9780429019128-2 The Paediatric Consultation 7

NORMAL GROWTH AND DEVELOPMENT

8 DISEASES OF THE EAR, NOSE & THROAT IN CHILDREN

The Paediatric Consultation 9

FUNCTIONAL ORL DISORDERS

10 DISEASES OF THE EAR, NOSE & THROAT IN CHILDREN

SYNDROMES, SEQUENCES AND ASSOCIATIONS

DOI: 10.1201/9780429019128-3 Children with Special Needs 11

Sequences and associations

and conductive. A small external ear canal, a high

12 DISEASES OF THE EAR, NOSE & THROAT IN CHILDREN

Children with Special Needs 13

14 DISEASES OF THE EAR, NOSE & THROAT IN CHILDREN

DOI: 10.1201/9780429019128-4 ENT Foreign Bodies 15

INGESTED FOREIGN BODIES

INHALED FOREIGN BODIES

A foreign body that passes into the bronchus

16 DISEASES OF THE EAR, NOSE & THROAT IN CHILDREN

ENT Foreign Bodies 17

18 DISEASES OF THE EAR, NOSE & THROAT IN CHILDREN

DEEP NECK SPACE INFECTIONS

An abscess in the parapharyngeal space produces a Internal carotid

DOI: 10.1201/9780429019128-5 Sepsis in the Head and Neck 19

Sublingual Submental (a)

Figure 5.2 Neck spaces – lateral view.

analgesia as required and measure baseline observa-

Treatment is initially with systemic antibiotics, pro-

20 DISEASES OF THE EAR, NOSE & THROAT IN CHILDREN

Figure 5.5 Acute mastoiditis with sub-periosteal abscess.

Sepsis in the Head and Neck 21

22 DISEASES OF THE EAR, NOSE & THROAT IN CHILDREN

Sepsis in the Head and Neck 23

24 DISEASES OF THE EAR, NOSE & THROAT IN CHILDREN

DEVELOPMENT OF THE EAR

Figure 6.1 Pre-auricular sinus.

DOI: 10.1201/9780429019128-6 The External Ear 25

Prominent ears of about 4 to 5 years as ideal, before the child is set-

26 DISEASES OF THE EAR, NOSE & THROAT IN CHILDREN

The External Ear 27

28 DISEASES OF THE EAR, NOSE & THROAT IN CHILDREN