LARYNGOMALACIA

Laryngomalacia refers to collapse of the supraglottic structures during inspiration (movie 1

and picture 3) [5,6]. It is the most common congenital anomaly of the larynx [7,8]. It is
distinct from tracheomalacia (an abnormally compliant trachea), which is far less common.
Laryngomalacia causes inspiratory stridor, whereas tracheomalacia causes expiratory or
biphasic stridor. In addition, tracheomalacia is often associated with other congenital
malformations such as trachea-esophageal fistula or vascular rings. Tracheomalacia is
discussed in greater detail separately. (See "Congenital anomalies of the intrathoracic airways
and tracheoesophageal fistula", section on 'Tracheomalacia'.)

Etiology — The etiology of laryngomalacia is not clearly defined, and different mechanisms

may apply in different infants. Proposed mechanisms include [8-10]:

●Delayed maturation or "hypotonia" of the supporting cartilaginous structures of the larynx

●Redundant soft tissue in the supraglottis

●A foreshortened or tight aryepiglottic fold

●Underlying neuromuscular disorders

●Supraglottic inflammation or edema

Gastroesophageal reflux (GER) has been reported to occur more commonly in children with
laryngomalacia compared with other children [11]. Whether GER causes laryngomalacia is
uncertain. It is quite possible that the two occur together because of a common underlying
mechanism (immature airway and sphincter tone), Nevertheless, in clinical practice it is
widely noted that antireflux medications seem to improve laryngomalacia symptoms. The
reason for this effect is unclear since infants typically do not produce much gastric acid.

In histologic studies, surgically excised supraglottic tissues from infants with laryngomalacia
are not distinct from supraglottic tissues from infants without laryngomalacia, weakening the
"malacia" or soft-cartilage theory. The frequent concurrence of gastroesophageal reflux, the
variation in severity with state of consciousness, and the new onset of laryngomalacia after
neurologic injuries support the hypothesis that it is a neuromuscular disorder [9].

Clinical features — Laryngomalacia manifests with intermittent low-pitched "wet"

inspiratory stridor, usually in the neonatal period [7,8,12,13]. In most children with
laryngomalacia, stridor is loudest at four to eight months of age and resolves by 12 to 18
months. Hoarseness is not a characteristic finding.

The degree of airway obstruction is dynamic and thus the intensity of stridor fluctuates.
Stridor is more intense during upper respiratory tract infections and is often worse in the
supine position, improving when prone [7]. In infants with mild to moderate laryngomalacia,
the stridor is usually loudest when feeding or sleeping and may disappear completely when
crying (although this may be difficult to detect due to the noise of the cry). Patients with
severe laryngomalacia, conversely, may have the loudest stridor during crying [7]. In some
infants, the stridor may be present only during sleep or relaxation (state-dependent
laryngomalacia) [12,13].
Other presenting features include [7,9,14-16]:

●Snoring and/or sleep-disordered breathing – 25 percent

●Swallowing dysfunction and/or feeding difficulties – 10 to 50 percent

●Gastroesophageal reflux and laryngopharyngeal reflux (reflux beyond the esophagus to the
larynx, oropharynx, or nasopharynx) – 60 to 70 percent

Children with mild forms of laryngomalacia breathe and feed well despite their noisy
respiration. More severe forms result in suprasternal or substernal retractions, poor feeding,
sleep-disordered breathing, and failure to thrive [15,16].

Up to 20 percent of infants with laryngomalacia have additional airway anomalies, but life-
threatening anomalies are uncommon [5,17]. Laryngomalacia may be isolated or may occur
in association with congenital syndromes (eg, Down syndrome, DiGeorge (22q11 deletion)
syndrome) or other non-airway anomalies [5]. (See "Down syndrome: Clinical features and
diagnosis" and "DiGeorge (22q11.2 deletion) syndrome: Clinical features and diagnosis".)

Diagnosis — The diagnosis of laryngomalacia is usually suspected based upon the history

and physical examination [7]. It is confirmed with flexible fiberoptic laryngoscopy (movie 1)
[10]. An experienced pediatric care provider need not refer every child with mild inspiratory
stridor to a pediatric otolaryngologist. Children with significant or progressive stridor, apnea,
cyanotic episodes, or poor growth require endoscopic confirmation.

Once the diagnosis of laryngomalacia is confirmed with flexible fiberoptic laryngoscopy, the
clinician should assess severity and consider additional evaluation for associated airway
abnormalities.

●Severity – The severity of laryngomalacia is determined by the degree and sequelae of

airway obstruction and the response to conservative interventions (eg, position change,
feeding modifications, treatment for gastroesophageal reflux). For example, intermittent
noisy breathing in an infant who is otherwise feeding and growing well would be considered
mild, whereas an infant with considerable stridor associated with feeding difficulties, poor
growth, apnea, or cyanotic episodes is considered to have severe laryngomalacia.

●Associated anomalies – Ancillary airway evaluation (eg, direct laryngoscopy and

bronchoscopy or flexible fiberoptic bronchoscopy) for concurrent laryngotracheal
malformations (eg, esophageal atresia, tracheoesophageal atresia) is warranted in infants with
cyanosis, apnea, hoarseness, feeding difficulty, poor growth, or atypical stridor [5,10,17,18].
Ancillary airway evaluation is lower yield in infants with less severe symptoms, and
performing it as a routine in this setting is controversial [5,10,17,18].

For children who have normal or equivocal findings on awake laryngoscopy yet have a
clinical presentation highly suggestive of laryngomalacia, drug-induced sleep endoscopy
(DISE) may be useful to evaluate for state-dependent laryngomalacia [19]. Some children
show signs of supraglottic collapse only during sleep. DISE can identify this variant and help
define the areas of supraglottic prolapse [20]. DISE is not without risk and thus should only
be pursued in patients with considerable symptoms. We do not advise DISE in young infants.
DISE is most commonly used to evaluate older children with residual obstructive sleep apnea
following adenotonsillectomy. The procedure is discussed in greater detail separately. (See
"Adenotonsillectomy for obstructive sleep apnea in children", section on 'Drug-induced sleep
endoscopy'.)

Numerous other potentially dangerous airway abnormalities may have similar clinical
presentations. Hoarseness is not characteristic of laryngomalacia. Other causes of stridor
include subglottic stenosis, vocal cord paralysis, vascular ring, laryngeal mass (eg, cyst or
hemangioma), subglottic hemangioma, and tracheomalacia (table 1A-B). The assessment of
stridor in children is discussed separately. (See "Assessment of stridor in children".)

Management — The management of laryngomalacia depends upon the severity. In the

majority of otherwise normal children, laryngomalacia is not dangerous and resolves
spontaneously.

●Mild laryngomalacia – Infants with mild laryngomalacia (intermittent mild stridor with no
other symptoms) may be followed clinically with frequent monitoring to make sure that
weight gain is adequate [7,10]. Stridor usually resolves by 12 to 18 months of age.

●Moderate to severe laryngomalacia – Infants with moderate or severe laryngomalacia

(stridor with feeding difficulty, dyspnea, tachypnea, cyanosis, apnea) should be referred to an
otolaryngologist for full endoscopic evaluation and possible intervention [7,10]. Medical
management may be adequate therapy for some infants with moderate laryngomalacia [21];
however, infants with severe laryngomalacia often require surgical intervention.

Medical management consists of acid suppression, speech and swallow therapy, and/or high-
calorie formula. Infants known to have associated GER should be treated for reflux [10].
Therapy for GER is often empiric in patients with laryngomalacia, though a diagnostic
evaluation may be appropriate for some children. Data supporting one approach versus the
other are extremely limited [22-25]. Feeding evaluation and speech therapy services can help
guide interventions to improve feeding (eg, texture modification, bottle pacing, augmenting
feeding schedule). Management of GER and swallowing dysfunction are discussed in greater
detail separately. (See "Gastroesophageal reflux in infants", section on 'Treatment options'
and "Aspiration due to swallowing dysfunction in infants and children", section on
'Management'.)

Children with severe laryngomalacia often benefit from surgery to remove redundant
supraglottic tissue (picture 4). In experienced hands, this surgery can produce dramatic
improvements in breathing, feeding, and growth with little morbidity [5,7,26-28]. Parents
report improved quality of life for children undergoing supraglottoplasty [29]. Two meta-
analyses found that for children with obstructive sleep apnea due to laryngomalacia,
supraglottoplasty is associated with improvement in the apnea-hypopnea index and oxygen
saturations; however, most children have residual symptoms after the procedure [30,31]. (See
"Adenotonsillectomy for obstructive sleep apnea in children", section on 'Supraglottoplasty'.)

Possible complications of supraglottoplasty include supraglottic or glottic scarring and

subsequent chronic aspiration or dysphonia, so surgery should not be undertaken for minimal
indications (ie, to ease parental anxiety about noisy breathing). The risks and benefits of
supraglottoplasty should be carefully considered in children with underlying neuromuscular
disease. Neuromuscular disease is not a contraindication to supraglottoplasty, but the benefit
of improving airway obstruction must be weighed with the risk of worsening aspiration in
this setting [10].