11/14/24, 10\:58 AM Laryngomalacia

Laryngomalacia

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Laryngomalacia\: congenital abnormality of larynx cartilage causing intermittent airway obstruction.
Presentation\: inspiratory stridor, usually within the
resolve by 18-24 months.
Aetiology\: not fully understood; theories include laryngeal anatomical abnormalities, neuromuscular incoordination, and
gastro-oesophageal re
Risk factors\: male sex, laryngeal anatomical abnormalities, GORD (50-100%), neurological abnormalities (20%), genetic
syndromic disorders (e.g., Down’s syndrome).
Clinical features\: stridor worsened by crying, exertion, lying supine, and feeding; feeding di
severe cases.
Examination
cry.
Diagnosis\: clinical diagnosis con
mucosa, and omega-shaped epiglottis.
Management\:
Conservative\: observation and monitoring growth and development in mild cases.
Medical\: feed thickener, anti-re
to severe cases.
Surgical\: supraglottoplasty for severe cases; tracheostomy in cases with comorbidities; Nissen fundoplication for severe
GORD.
Complications\: life-threatening airway obstruction, failure to thrive, developmental delay, worsening GORD, post-surgery
complications (granuloma formation, worsened aspiration, supraglottic stenosis).
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A comprehensive topic overview

Introduction

Laryngomalacia is a congenital abnormality of the larynx cartilage which causes intermittent airway obstruction
(l a r y n g o \: relating to the larynx, m a l a c i a \: abnormal softening of a tissue).
Patients usually present with inspiratory stridor. In laryngomalacia, stridor is caused by the collapse of the supraglottic
structures (e.g. epiglottis, arytenoid processes) into the airway.
Laryngomalacia is the most common laryngeal anomaly and congenital cause of stridor in infants.
1
Patients usually present in infancy, with inspiratory stridor commencing within the
be at their worst at six to eight months of age before gradual improvement. Most children are free of symptoms by 18-24
months of age.
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Aetiology

The aetiology of laryngomalacia is not fully understood, but there are several proposed theories.
The anatomical theory suggests that laryngomalacia is due to laryngeal anatomical abnormality, particularly short
aryepiglottic folds, prominent arytenoid mucosa, and a curled ‘omega-shaped’ (Ω) epiglottis. It has also been suggested
that
Alternatively, neuromuscular incoordination may contribute to suboptimal laryngeal tone. Additionally, gastro-
oesophageal re
2
Figure 1. Schematic comparison of supraglottic anatomy in normal versus malacic larynx
Figure 2. Clinical photograph of
aryepiglottic folds which will prolapse into the glottic inlet during inspiration

Poiseullie’s law

Poiseullie’s law is a useful concept for understanding the pathophysiology of paediatric airway obstruction. The law states
that the resistance of a tube is inversely proportional to the fourth power of its radius.
In clinical terms (i.e. air being inspired through the larynx), any change in airway radius signi
resistance.
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The radius of the infantile larynx is already very small, so any further narrowing may precipitate life-threatening airway
compromise due to massively increased airway resistance. Conversely, as the infant grows, the airway radius increases
and resistance signi

Risk factors

Risk factors for laryngomalacia include\:
1
Laryngeal anatomical abnormality
Gastro-oesophageal re
Neurological abnormality\: in up to 20% of patients
Male sex\: 2\:1 male\:female incidence ratio
Genetic syndromic disorder\: more common in Down’s syndrome and patients with congenital cardiac disease

Clinical features

History

Typical symptoms of laryngomalacia include\:
1
Stridor worsened by crying, exertion, lying supine, and feeding
Onset of stridor within 2 weeks of birth
A natural progression of symptom severity, peaking at 6-8 months and with the resolution of symptoms by 2 years of age
(70% resolution by 12 months)
Feeding di
A collateral history from the parent or guardian is essential. Reviewing the personal child health record (known as the
“red book” in the UK) is a useful way to objectively assess weight gain and development.

Clinical examination

Examination of all unwell patients should follow the ABCDE approach. For patients with laryngomalacia, particular attention
should be paid to signs of increased work of breathing and respiratory distress.
Typical clinical
Stridor
Nasal
Tracheal tug
Intercostal and subcostal recession
Abdominal respiration
Patients with laryngomalacia usually have a normal cry

Di

The absence of typical features of laryngomalacia would favour an alternative diagnosis. Di
include\:
1,3
Vocal fold palsy
Subglottic stenosis
Laryngeal web
Laryngeal cleft
Laryngeal cyst
Subglottic haemangioma
Tracheomalacia
Vascular ring
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Foreign body aspiration

Investigations

Flexible nasendoscopy (FNE) is the key diagnostic investigation. It is used to assess for typical laryngeal anatomical
features and other risk factors (e.g. mucosal in
the patient awake and breathing spontaneously. If the history is atypical, the infant may require additional assessment
under a general anaesthetic.

Bedside investigations

Relevant bedside investigations include\:
Basic observations (including paediatric early warning score)\: oxygen saturations and respiratory rate are particularly
relevant

Laboratory investigations

There are no laboratory tests which can diagnose laryngomalacia. However, haematological tests (e.g. blood gas, full blood
count, C-reactive protein) or microbiological sampling of sputum may be taken if there is suspicion of underlying or
exacerbating infection.
A chest X-ray may provide evidence of other airway pathology, concurrent lower respiratory tract infection, or foreign body

Imaging

aspiration.

Other investigations

In some cases, laryngomalacia may be coexistent with other abnormalities of the airway, and further investigations are
required.
Other relevant investigations may include\:
Microlaryngobronchosopy (MLB)\: a diagnostic procedure performed under general anaesthetic, whereby a rigid
endoscope is passed through the mouth to visualise the trachea, main bronchi and the structures of the larynx.
Polysomnography (sleep study)\: used to characterise associated obstructive sleep apnoea (OSA). Oximetry and other
data are recorded during sleep. This is useful for making decisions about complex patients with multiple medical
problems. It can be a di

Diagnosis

Laryngomalacia is a clinical diagnosis, based primarily on
Positive diagnostic
shaped epiglottis. This results in the dynamic collapse of the supraglottic tissues on inspiration, causing intermittent
airway obstruction.

Management

Mild laryngomalacia is often managed by paediatricians, with ENT specialist involvement required only in more severe
cases.

Conservative management

In milder cases, patients thrive despite audible stridor and endoscopic features of laryngomalacia. Parents can be
reassured of the high likelihood that this will spontaneously resolve with no long-term issues. For these children,
observation with close monitoring of growth and development is the best strategy.

Medical management

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Medical management is indicated in moderate to severe disease, characterised by increased work of breathing, and
feeding di
Options for medical management include\:
1
Feed thickener (e.g. Carobel) is often helpful in reducing aspiration and regurgitation of milk
Anti-re
commonly used to reduce in
Steroids (e.g. dexamethasone) are used in patients with acute respiratory distress to reduce laryngeal swelling and
increase airway patency. Steroids will work in many cases of airway distress and not just in laryngomalacia.

Surgical management

Surgical techniques can correct anatomical abnormalities in patients with severe disease (10-15% of patients with
laryngomalacia).
1
These patients present with signi
patterns and feeding di
apnoea, hypoxia, and hypercapnia are other factors which indicate severe disease.
Options for surgical management include\:
Supraglottoplasty modi
therapy. Tissue from the lateral portion of the arytenoids can also be removed if arytenoid prolapse is seen and the
condition is severe.
Tracheostomy is a secondary surgical option reserved for patients with other comorbidities which would limit the
e
In very severe cases of GORD, Nissen fundoplication may be considered. This is a surgical procedure to reinforce the
gastro-oesophageal sphincter and reduce acid re

Complications

Complications of laryngomalacia include\:
Life-threatening airway obstruction (occurs very rarely and mostly in cases with other co-morbidities, like infection or
congenital abnormality)
Failure to thrive
Failure to gain weight, dropping centiles on the growth chart
Developmental delay
Worsening of GORD (laryngomalacia both exacerbates and is exacerbated by acid re
Complications of surgery for laryngomalacia include\:
6
Granuloma formation
Worsened aspiration
Supraglottic stenosis
The tracheostomy-related mortality rate is about 2%

References

BMJ Best Practice. Published in 2022. Available from\: [LINK]
Thompson DM. Abnormal sensorimotor integrative function of the larynx in congenital laryngomalacia\: a new theory of
etiology. The Laryngoscope. 2007;117(S114)\:1-33.
Klinginsmith M, Goldman J / StatPearls. Adapted by Geeky Medics. Laryngomalacia. License\: [CC BY]
Doctormichael. L a r y n g o m a l a c i a . License\: [Public domain]
Bruce IA, Rothera MP. Upper airway obstruction in children. Pediatric Anesthesia. 2009;19\:88-99.
https\://app.geekymedics.com/notebook/2747/ 5/611/14/24, 10\:58 AM Laryngomalacia
Cochrane LA, Bailey CM. Surgical aspects of tracheostomy in children. Paediatr Respir Rev. 2006 Sep;7(3)\:169-74.

Reviewer

Miss Tash Kunanandam
Consultant Paediatric Otolaryngologist
Royal Hospital for Children, Glasgow

Related notes

Attention De
Autism Spectrum Disorder (ASD)
Biliary Atresia
Bronchiolitis
Cerebral Palsy

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Contents

Introduction
Aetiology
Risk factors
Clinical features
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