Congenital laryngomalacia

INTRODUCTION — 

Congenital laryngomalacia is one of the most common causes of noisy breathing in infants. The stridor is heard during inspiration and is caused by prolapse of the supraglottic tissue into the glottic inlet (movie 1 and picture 1).

The clinical features, diagnosis, and management of congenital laryngomalacia in infants and children will be reviewed here. Laryngomalacia is distinct from tracheomalacia (an abnormally compliant trachea), which is far less common. Tracheomalacia is discussed in greater detail separately. (See "Congenital anomalies of the intrathoracic airways and tracheoesophageal fistula", section on 'Tracheomalacia'.)

Other congenital abnormalities of the larynx (eg, laryngeal clefts and webs) and other causes of stridor in infants and children are discussed separately. (See "Congenital anomalies of the larynx" and "Assessment of stridor in children".)

EPIDEMIOLOGY — 

Congenital laryngomalacia is the most common cause of recurrent or chronic stridor in infancy, accounting for approximately 45 to 75 percent of cases referred to otolaryngology specialty clinics [1,2]. The reported prevalence of clinically significant laryngomalacia is approximately 3 to 4 cases per 10,000 live births [3]. It is more common in males than females with a male to female ratio of approximately 1.5:1 [2].

Laryngomalacia can occur in otherwise healthy children, or it may be associated with an underlying medical condition (eg, genetic syndrome, neuromuscular disorder, prematurity). (See 'Associated conditions' below.)

ANATOMY

●Normal laryngeal anatomy – The larynx is comprised of cartilage, muscles (extrinsic and intrinsic), and a mucosal lining (figure 1). The muscles of the larynx act to open and close the glottis and to regulate voice quality.

The mobile, crescent-shaped hyoid bone forms the anterior upper limit of the larynx. In newborns, the larynx is located at the level of C3-C4; it gradually descends to the level of C6-C7 by adolescence.

The larynx is divided into three sections relative to the level of the vocal cords:

•Supraglottic structures – The area just above the vocal folds is called the supraglottic region. The supraglottic structures include the epiglottis, arytenoids, aryepiglottic folds, and false vocal folds.

•Glottis – This includes the vocal folds and the area just below the vocal folds.

•The subglottic region – This includes the region starting 1 cm below the vocal folds and ending in the upper cervical trachea. It corresponds to the cricoid cartilage, which is the only laryngeal cartilage that completely encircles the airway. It is the narrowest part of the airway in infants and children.

●Anatomic classification of laryngomalacia – In infants and children with laryngomalacia, supraglottic tissue collapses into the glottic inlet during inspiration.

The Olney classification schema categorizes laryngomalacia into three types based on supraglottic morphology (figure 2) [4]:

•Type 1 – Prolapse of redundant supra-arytenoid tissue

•Type 2 – Foreshortened aryepiglottic folds with an omega-shaped epiglottis (picture 1 and movie 1)

•Type 3 – Retro-displaced epiglottis that collapses posteriorly during inspiration (movie 2)

Children with laryngomalacia can have anatomic findings that fit into a single category, or they may have a combination of findings. In a systematic review of studies describing characteristics of >1200 children with laryngomalacia, the relative frequency of the different types was as follows [2]:

•Type 1 – 32 percent

•Type 2 – 47 percent

•Type 3 – 10 percent

•Combination of two or more types – 11 percent

A retro-displaced epiglottis (type 3 laryngomalacia) is most commonly seen in children with underlying neurological disorders. (See 'Associated conditions' below.)

PATHOGENESIS — 

The pathogenesis of laryngomalacia is not fully understood. It likely results from a combination of neurologic, anatomic, and inflammatory abnormalities leading to diminished tone of the supraglottic tissue and supporting musculature [1,5,6].

Respiration, swallowing, and neuromuscular tone of the larynx are vagally mediated through the laryngeal adductor reflex. The diminished airway tone and swallowing dysfunction seen in laryngomalacia are thought to result from abnormal sensorimotor integration between peripheral vagal nerve chemoreceptors and the central nuclei that control respiration and swallowing via the laryngeal adductor reflex [1,7].

Anatomic factors (eg, redundant soft tissue in the supraglottis, foreshortened or tight aryepiglottic fold) may also play a role. Another theory is that laryngomalacia results from immature development of laryngeal cartilage [6].

Gastroesophageal reflux (GER) and laryngopharyngeal reflux (LPR) can be detected in most patients with clinically significant laryngomalacia (see 'Associated conditions' below). GER and LPR are thought to contribute to the pathogenesis of laryngomalacia by causing inflammation of the already redundant supraglottic tissue. However, the role that GER and LPR play in the pathogenesis of laryngomalacia is uncertain. The association between GER and laryngomalacia may be explained by a common underlying mechanism (immature neuromuscular tone affecting both the supraglottic airway and the lower esophageal sphincter) rather than a causal relationship.

CLINICAL FEATURES

Presentation

Congenital laryngomalacia — Infants with congenital laryngomalacia may present primarily with respiratory symptoms (eg, noisy breathing), feeding difficulties, or a combination of both [8].

Most infants with congenital laryngomalacia present with noisy breathing that begins in the newborn period. It may gradually become more noticeable over the first few months of life. The stridor produced by laryngomalacia occurs during inspiration and can be intermittent. (See 'Natural history' below.)

The stridor can occur at rest and tends to worsen with activities such as crying, agitation, excitement, or feeding. Stridor is worse in the supine position and improves when the infant is upright. The noise can have a "wet" quality which is often associated with concurrent reflux. Stridor may be more pronounced during intercurrent respiratory illnesses, and some affected children may be diagnosed with recurrent episodes of croup before the possibility of laryngomalacia is suspected. (See "Croup: Clinical features, evaluation, and diagnosis", section on 'Recurrent croup'.)

Expiratory stridor and hoarseness are not characteristic findings of laryngomalacia; these findings should prompt consideration of an alternative diagnosis or secondary airway abnormality. (See 'Differential diagnosis' below and 'Associated conditions' below.)

Infants and children with mild laryngomalacia feed well and have normal weight gain and growth despite their noisy breathing. However, severe laryngomalacia can cause sleep-disordered breathing, apneic/cyanotic episodes, severe feeding difficulties, and/or growth failure [8,9]. In severely affected patients, suprasternal or substernal retractions may be noted on examination (movie 3). (See 'Severity assessment' below.)

In a single-center study of 324 infants and children laryngomalacia who were referred to a specialty center for management, the relative frequency of symptoms was as follows [9]:

●Stridor – 93 percent

●Feeding difficulties – 50 percent

●Apnea – 19 percent

●Retractions – 18 percent

●Cyanosis – 13 percent

●Growth failure – 10 percent

Late presentation — Laryngomalacia presenting after infancy or early childhood is uncommon in the absence of an underlying neuromuscular disorder or genetic syndrome. However, late-onset variants of laryngomalacia have been described. Late presentations may include [10,11]:

●Feeding variant – These patients typically present in the toddler years predominantly with symptoms of swallowing dysfunction and feeding difficulties.

●Sleep variant – These patients typically present at school age with sleep-disordered breathing (eg, persistent obstructive sleep apnea despite adenotonsillectomy). Often, a detailed history will reveal a history of mild stridor in infancy that was not severe enough to warrant intervention.  

●Exercise variant – These patients typically present in later childhood or adolescence with exercise-induced inspiratory stridor.

Patients with these late-onset variants usually have profound arytenoid redundancy and prolapse noted on laryngoscopy but other typical anatomic features of congenital laryngomalacia (shortened aryepiglottic folds, retroflexed epiglottis) may be absent [11].

Late-onset variants are rare, and the management of these patients is beyond the scope of this topic.

Associated conditions — Comorbid medical conditions are common in infants and children with congenital laryngomalacia.

●Gastroesophageal reflux (GER) – GER and laryngopharyngeal reflux (LPR) can be detected in 50 to 90 percent patients with clinically significant laryngomalacia [2,12-16]. GER and LPR are thought to contribute to the pathogenesis of laryngomalacia by causing inflammation of the already redundant supraglottic tissue (see 'Pathogenesis' above). In addition, the negative intrathoracic pressure generated by recurrent episodes of airway obstruction can further promote reflux of gastric contents, resulting in a vicious cycle that exacerbates symptoms of both GER and airway obstruction.

If the supraglottic tissues are exposed to refluxed gastric contents recurrently over a prolonged time, it may cause neuroinflammatory changes that alter laryngeal sensation and tone [1]. This may lead to swallowing dysfunction, difficulty clearing secretions, and noisy breathing that has a "wet" quality.

Acid suppression therapy is commonly used in the management of laryngomalacia with the rationale that it may reduce the adverse effects of GER and LPR in this setting. However, based on the available evidence, it is unclear if acid suppression therapy improves symptoms or reduces the need for surgical intervention in infants and children with laryngomalacia [17,18]. Nevertheless, it remains the mainstay of medical management, as discussed below. (See 'Initial interventions (medical therapy)' below.)

●Neuromuscular disorders – Laryngomalacia is common in children with neuromuscular disorders such as cerebral palsy, hydrocephalus, Chiari malformation, myelomeningocele, muscular dystrophy, or congenital hypotonia [1,19]. Children with an underlying neuromuscular cause of laryngomalacia often also have significant swallowing dysfunction.

Laryngomalacia tends to be more severe and persistent in children with underlying neuromuscular disorders compared with infants with isolated laryngomalacia who are otherwise healthy. (See 'Natural history' below.)

●Genetic syndromes – Laryngomalacia may occur in association with genetic syndromes such as Down syndrome or 22q11.2 deletion syndrome. (See "Down syndrome: Clinical features and diagnosis" and "DiGeorge (22q11.2 deletion) syndrome: Clinical features and diagnosis".)

Patients with underlying genetic syndromes may have more severe symptoms because of other syndromic features that cause breathing difficulties, cyanosis, and/or feeding problems (eg, other airway abnormalities, neuromuscular disease, congenital heart disease) [1,20,21].

●Other associated airway abnormalities – Approximately 15 to 30 percent of infants with laryngomalacia have additional airway anomalies such as subglottic stenosis, tracheomalacia, vocal cord immobility, laryngeal clefts, or tongue base obstruction [2,9,22,23]. The presence of additional airway abnormalities generally causes more severe airway obstruction [20].

●Prematurity – There is a higher prevalence of laryngomalacia and tracheomalacia among infants born very preterm compared with term infants. In the available case series, preterm infants accounted for approximately 15 percent of infants with isolated laryngomalacia and up to 30 percent of those with combined laryngomalacia and tracheomalacia [2,3]. The risk of airway abnormalities is greatest in preterm infants with significant neonatal respiratory morbidity (eg, bronchopulmonary dysplasia and/or requiring intubation during the neonatal intensive care unit hospitalization). (See "Complications and long-term pulmonary outcomes of bronchopulmonary dysplasia", section on 'Central airway disease'.)

Preterm infants are also more likely to have feeding and swallowing difficulties. (See "Neonatal oral feeding difficulties due to sucking and swallowing disorders", section on 'Prematurity'.)

NATURAL HISTORY — 

In children who are otherwise healthy, laryngomalacia typically resolves over time as the child grows. Symptoms typically peak at four to eight months of age and improve by 12 to 18 months; most patients outgrow stridor by 24 months of age [20]. In a review of three retrospective studies including a total of 411 infants and children with laryngomalacia, 89 percent had resolution of symptoms within the first few years of life (time to resolution ranged from 4 to 42 months) [24].

By contrast, laryngomalacia tends to persist in children who have underlying neuromuscular disorders and genetic syndromes [1,25,26].

DIAGNOSIS — 

The diagnosis of laryngomalacia is suspected based upon the history and physical examination. A presumptive diagnosis can be made based upon the characteristic features of inspiratory "wet" stridor beginning in the newborn period and becoming more noticeable over the first few months of life; worse in the supine position, improving when the infant is upright (see 'Presentation' above). The diagnosis is confirmed with awake flexible laryngoscopy (movie 1 and movie 2).

Referral indications — Referral to a pediatric otolaryngologist is warranted for any infant or child presenting recurrent or chronic stridor, especially if it has been present since birth or if the child's symptoms are atypical for croup (eg, recurrent episodes of croup-like symptoms). (See "Croup: Clinical features, evaluation, and diagnosis", section on 'Recurrent croup'.)

The otolaryngologist confirms the diagnosis with flexible fiberoptic laryngoscopy and guides management. Although a presumptive diagnosis of laryngomalacia can often be made based upon the history and physical examination alone, flexible fiberoptic laryngoscopy is generally warranted to exclude other causes of recurrent/chronic stridor (eg, vocal cord paralysis or dysfunction, laryngeal cleft, subglottic hemangiomas). (See 'Differential diagnosis' below.)

The urgency of referral is guided by symptom severity. Infants with severe symptoms warrant expedited referral; those who have significant respiratory distress, apnea, desaturation, and/or inability to feed generally warrant inpatient admission [20].

For infants with mild intermittent inspiratory stridor who are otherwise feeding and growing well, nonurgent referral is appropriate.

Confirming the diagnosis — The definitive diagnosis of laryngomalacia is made with awake flexible fiberoptic laryngoscopy [20]. Typical findings of laryngomalacia include an omega-shaped epiglottis, collapse of the supra-arytenoid tissue, and short aryepiglottic folds (movie 1 and picture 1). A retro-displaced epiglottis may also be present, especially in children with underlying neurological disorders (movie 2).

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 sleep variant laryngomalacia (also called state-dependent laryngomalacia) [27]. Some children show signs of supraglottic collapse only during sleep. DISE can identify this variant and help define the areas of supraglottic prolapse [28]. DISE is performed in the operating room under anesthesia which permits evaluation of the airway in a sleep-like state. 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'.)

Additional evaluation — Once the diagnosis of laryngomalacia is confirmed, additional evaluation may be warranted to evaluate for secondary airway lesions, swallowing dysfunction/aspiration, sleep-disordered breathing, and/or gastroesophageal reflux disease (GERD).

●Evaluation for secondary airway lesions – If there is clinical concern for a secondary airway abnormality (eg, subglottic stenosis, tracheomalacia) we suggest ancillary airway evaluation with airway radiographs (anteroposterior and lateral radiographs of the neck) and/or direct laryngoscopy and bronchoscopy [20,22].

Secondary airway abnormalities may be suspected if:

•The infant has moderate to severe symptoms that are not commensurate with the findings on fiberoptic laryngoscopy, or

•The infant's stridor is atypical for laryngomalacia (eg, expiratory or biphasic stridor or associated with hoarseness)

Ancillary airway evaluation is lower yield in infants with mild symptoms that are typical of laryngomalacia and consistent with the laryngoscopy findings; bronchoscopy is not routinely necessary in this setting [20].

●Swallow evaluation – A formal evaluation of swallowing function should be performed in infants with feeding difficulties; choking, coughing, or regurgitating with feeds; and/or poor weight gain [9,20,29]. The initial step is usually a clinical feeding evaluation by a speech-language pathologist or occupational therapist, followed by a videofluoroscopic swallow study (VFSS) and/or fiberoptic endoscopic evaluation of swallowing (FEES) if warranted based upon the findings of the clinical evaluation. Additional details are provided separately. (See "Neonatal oral feeding difficulties due to sucking and swallowing disorders", section on 'Diagnostic evaluation' and "Aspiration due to swallowing dysfunction in children", section on 'Evaluation'.)

The swallow evaluation provides an objective assessment of swallowing and airway protection that informs management decisions regarding the need for feeding modifications or surgical intervention. Approximately 15 percent of patients with moderate laryngomalacia and up to 70 percent with severe laryngomalacia demonstrate aspiration on formal swallow evaluation [30].

●Polysomnography – A formal sleep study is not necessary in most infants with congenital laryngomalacia. Technical challenges of performing polysomnography in young infants and uncertainty regarding its diagnostic accuracy in this age group preclude using it as a routine in this setting. Instead, we generally use the clinical criteria described below to characterize the severity of airway obstruction, which informs management decisions. (See 'Severity assessment' below.)

However, polysomnography can be useful in select circumstance (eg, if there is clinical suspicion for central apnea, in older children with sleep-disordered breathing). Additional details regarding polysomnography in infants and children are provided separately. (See "Overview of polysomnography in infants and children" and "Evaluation of suspected obstructive sleep apnea in children", section on 'Polysomnography'.)

●Diagnostic evaluation for GERD – Most infants and children with clinically significant laryngomalacia are treated empirically with acid suppression therapy, and a diagnostic evaluation for GERD is not necessary. (See 'Initial interventions (medical therapy)' below.)

However, a diagnostic evaluation for pathologic GERD may be appropriate in select cases (eg, patients with severe or refractory symptoms). The diagnostic approach for GERD is described elsewhere. (See "Gastroesophageal reflux disease in children and adolescents: Clinical manifestations and diagnosis", section on 'Diagnostic approach'.)

Severity assessment — We use the Thompson classification system to characterize the severity of laryngomalacia. It is based chiefly on the infant's symptoms [1,31]:

●Mild disease – In mild laryngomalacia, symptoms are generally limited to intermittent stridor. Patients with mild laryngomalacia have normal oxygen levels (baseline resting oxygen saturation >98 percent). They generally feed comfortably and have normal growth. They have a coordinated suck-swallow-breath sequence, though they may occasionally cough, choke, and/or regurgitate with feeding.

Approximately 40 percent of infants with laryngomalacia who present to a tertiary care pediatric otolaryngology practice fall into the mild category [1].

●Moderate disease – Infants with moderate disease have persistent stridor at baseline. They may have intermittent cyanosis, though their resting oxygen saturation levels are generally in the normal range (approximately 95 percent). They usually have associated feeding difficulty caused by difficulty coordinating the suck-swallow-breath sequence. These patients tend to tire during feeding and are described by caregivers as difficult to feed. They also have frequent coughing and choking episodes, regurgitation, and post-prandial emesis. Without intervention, these problems can lead to aspiration or weight loss secondary to caloric expenditure from increased work of breathing.

Approximately 40 percent of infants with laryngomalacia who present to a tertiary care pediatric otolaryngology practice fall into the moderate category [1].

●Severe disease – Infants with severe disease have persistent stridor at baseline that is associated with recurrent cyanosis, apneic pauses, feeding difficulties, aspiration, and growth failure. They have pronounced suprasternal and subcostal retractions that can lead to pectus excavatum (movie 3). They generally have low resting oxygen saturation levels (approximately 85 to 90 percent). Without intervention, chronic hypoxemia can lead to pulmonary hypertension and cor pulmonale. As discussed below, patients with severe disease often require surgical intervention. (See 'Severe laryngomalacia' below.)

Approximately 20 percent of infants with laryngomalacia who present to a tertiary care pediatric otolaryngology practice fall into the severe category [1].

Various other severity classification schemas for laryngomalacia have been described. Some are based purely on anatomic features without correlation to symptom burden [32], while others have attempted to account for both symptoms and anatomic findings [33,34].

DIFFERENTIAL DIAGNOSIS — 

The differential diagnosis includes other causes of recurrent stridor that present in infancy, including subglottic stenosis, vocal cord paralysis, vascular rings, laryngeal clefts, subglottic hemangiomas, tracheomalacia, and recurrent croup (figure 3 and table 1A-B). Laryngomalacia is distinguished from these based upon the endoscopic examination. The assessment of stridor in children is discussed separately. (See "Assessment of stridor in children".)

MANAGEMENT — 

The management of laryngomalacia depends upon the severity, as discussed in the following sections (algorithm 1). Our suggested approach is generally consistent with the recommendations of the International Pediatric Otolaryngology Group (IPOG) [20].

The symptoms of laryngomalacia are burdensome and can create fear and anxiety in the parents/caregivers with substantial negative impact on quality of life for both the infant and the parents/caregivers [35-37]. While, in most cases, this is a self-limited condition that resolves over time and can be managed expectantly, it is important to observe and monitor for symptom progression and adjust management accordingly. Surgical intervention is reserved for a small subset of patients with severe symptoms that persist despite medical management.

Mild laryngomalacia — Infants with mild laryngomalacia (intermittent mild stridor with normal growth and no other symptoms), we suggest conservative management with supportive care and observation [20,38]. Stridor usually resolves by 12 to 18 months of age in these infants. (See 'Natural history' above.)

Conservative management includes the following:

●Ongoing monitoring to ensure that weight gain is adequate. (See "Normal growth patterns in infants and prepubertal children".)

●Anticipatory guidance for the parents/caregivers regarding the typical course of laryngomalacia (ie, that it usually resolves as the infant grows). (See 'Natural history' above.)

●For infants with frequent regurgitation or other concerns for gastroesophageal reflux (GER), simple measures that may be helpful include a trial of thickened feeds, upright positioning after feeds, and avoiding overfeeding [18]. If there is no improvement with these measures, a trial of acid suppression therapy is reasonable. (See "Gastroesophageal reflux in infants", section on 'Additional options for infants with GERD or problematic reflux'.)

Patients should have initial follow-up for symptom assessment within a month. If the patient is stable or improving, subsequent follow-up visits can be spaced to every three to six months [20].

Moderate laryngomalacia — Infants and children with moderate laryngomalacia (persistent stridor associated with feeding difficulty and poor weight gain) should be managed in collaboration with a pediatric otolaryngologist and a clinician experienced in infant swallowing and feeding disorders (eg, a speech or occupational therapist) [20]. Involvement of other specialists may be appropriate depending on the clinical circumstances (eg, a dietitian for infants with poor weight gain, a gastroenterologist for patients with severe GER, a neurologist if there is concern for undiagnosed neuromuscular disease).

Medical management may be sufficient for some infants with moderate laryngomalacia [39]; however, infants with persistent or progressive symptoms despite medical management may require surgical intervention.

Initial interventions (medical therapy) — For most patients with moderate laryngomalacia, we suggest initial medical therapy rather than proceeding directly to surgical intervention.

Medical management includes [20,39]:

●Acid suppression therapy – Acid suppression therapy consists of a histamine type 2 receptor antagonist (H2RA) or proton pump inhibitor (PPI). The choice between an H2RA or PPI is based on cost, availability, and preferences of the clinician and patient. There is no evidence to suggest that one agent is more effective than the other in this setting or that there is additional benefit from using both medications. The dosing, administration, and side effects of these medications are summarized in the table (table 2) and discussed in detail separately. (See "Gastroesophageal reflux disease in children and adolescents: Management", section on 'Pharmacotherapy'.)

Data supporting H2RAs and PPIs in patients with laryngomalacia are limited to observational studies with variable findings [17,18,40]. In a retrospective study of 199 infants with isolated laryngomalacia (ie, not associated with genetic or neuromuscular disorders) who were started on acid suppression therapy (largely with H2RAs) by age six months and followed for up to one year, the proportion of children with airway symptoms declined from 97 to 40 percent and the proportion of children with dysphagia symptoms declined from 75 to 40 percent [17]. However, given the lack of control group in this study, it is unclear if the observed symptom improvements were due to acid suppression therapy or if this merely reflects the expected natural history of laryngomalacia. (See 'Natural history' above.)

By contrast, another retrospective study did not detect any improvements in respiratory exacerbations or need for surgery associated with use of acid suppressive therapy. In this study, which included 236 infants and children with laryngomalacia, 55 percent were treated with acid suppression therapy (consisting of H2RAs in 50 percent, PPIs in 20 percent, and H2RA plus PPI in 30 percent) [18]. In this cohort, acid suppression therapy was associated with a nearly two-fold increased rate of hospitalization for respiratory symptoms and more than three-fold higher likelihood of requiring supraglottoplasty. Given the retrospective nature of the data, confounding may explain the findings (ie, more severely affected patients are more likely to be treated with acid suppression therapy, and this may explain the higher rates of hospitalization and surgery in this group).

●Feeding modification – Swallowing problems are common in infants with moderate to severe laryngomalacia [29]. Improving feeding is essential for the quality of life for caregivers and infants.

Feeding interventions should be tailored to the individual patient and may include measures such as texture modification (eg, thickened feeds), paced feedings, or use of various special nipples for bottle feeding to reduce flow and minimize aerophagia. These interventions are discussed in greater detail separately. (See "Neonatal oral feeding difficulties due to sucking and swallowing disorders", section on 'Management approach' and "Gastroesophageal reflux in infants", section on 'Additional options for infants with GERD or problematic reflux'.)

In a study of 236 infants and children with laryngomalacia who were managed at a single tertiary center, thickening feeds was associated with a lower risk of hospitalization for respiratory complications [18].

●High-calorie feeds to promote weight gain – The approach to nutritional support for infants with poor weight gain is discussed in detail separately. (See "Poor weight gain in children younger than two years in resource-abundant settings: Management", section on 'Nutritional therapy'.)

Persistent or progressive symptoms despite medical therapy — In most infants and children with moderate laryngomalacia, symptoms stabilize and improve with medical management. However, approximately 20 to 30 percent progress to severe disease, requiring surgical intervention, nasogastric (NG) or gastronomy (G) tube feeds, or artificial airway support [1,38]. Those with comorbid conditions and synchronous airway lesions are more likely to progress to severe disease.

For most patients who develop progressively worsening symptoms despite medical management, we suggest supraglottoplasty, as discussed below. (See 'Supraglottoplasty' below.)

Severe laryngomalacia — For infants and children with severe laryngomalacia (persistent stridor associated with severe retractions, cyanosis, apneic episodes, severe feeding difficulties, aspiration, and/or growth failure), initial management consists of the same medical therapies that are used for moderate disease (ie, gastric acid suppression therapy and feeding modifications, including NG or G tube feedings if the infant is unable to feed orally). (See 'Initial interventions (medical therapy)' above.).

However, in severely affected patients, symptoms often do not improve with medical therapy alone, and most patients in this category require surgical intervention.

The urgency of surgical intervention depends on the clinical circumstances. In patients with more extreme manifestations (eg, severe recurrent apneic and cyanotic spells), surgical intervention should be expedited simultaneously with implementing medical therapies. In less extreme cases, it is usually feasible to provide an initial trial of medical therapy before proceeding to surgery.

Supraglottoplasty — Supraglottoplasty is the procedure of choice for treatment of severe or persistent laryngomalacia. Supraglottoplasty removes redundant supraglottic tissue thereby reducing the severity of airway obstruction (picture 2 and movie 4).

●Indications for surgery – Surgical intervention is appropriate for patients with severe symptoms despite medical therapy. Indications for surgery include any of the following [20]

•Apnea (sleep or awake)

•Persistent cyanosis

•Respiratory distress

•Pectus excavatum

•Growth failure due to feeding difficulty

•Cor pulmonale and pulmonary hypertension

There is considerable institutional variability in the use of supraglottoplasty for management of laryngomalacia, with some specialized centers performing only a handful of procedures annually, and other centers reporting >200 supraglottoplasties per year [41,42]. As experience with the procedure has grown and given the low complication rate of the procedure, some pediatric otolaryngologists offer supraglottoplasty as a treatment option for patients with moderate disease who have substantially impaired quality of life. Several studies have demonstrated improvements in quality of life following surgical intervention that correlate with parental/caregiver goals of reducing time spent feeding the infant, achieving successful breastfeeding, and securing child care placement thereby reducing time away from work [35-37]. However, high levels of parental satisfaction with surgery should be balanced against the risks and expense of supraglottoplasty for what is a self-limited disease in most children. In our practice, we generally reserve supraglottoplasty for severely affected patients.

●Supraglottoplasty procedure – Supraglottoplasty is performed under general anesthesia by operating through an endoscope in the oral cavity. The aryepiglottic folds are divided, and the redundant supra-arytenoid tissue that collapses in the airway is removed (picture 2 and movie 4). The result is a wider opening in the supraglottic airway.

Supraglottoplasty may be combined with surgery to address multilevel airway obstruction in children with tracheal disease, tongue base disease, or syndromes associated with anatomic airway obstruction such as Pierre Robin sequence [43]. Success rates are lower in these high-risk patients, and if supraglottoplasty is unsuccessful, tracheostomy placement may ultimately be required. (See 'Failure of supraglottoplasty' below.)

●Recovery and follow-up – Most patients are observed in the hospital and can be discharged the day after surgery [44]. Patients with underlying medical comorbidities, syndromes, and synchronous airway lesions may require observation in the pediatric intensive care unit (PICU) setting; some may require short-term intubation [45,46]. In addition, PICU admission is appropriate for patients who experience significant perioperative airway obstruction or desaturation and for young infants (<2 months), especially preterm infants, given the potential for postoperative apnea in these patients. However, low-risk patients who are otherwise healthy generally do not require PICU admission following supraglottoplasty [47,48]. There are few data on the safety of outpatient supraglottoplasty. In a study reporting on 975 infants and children who underwent supraglottoplasty at a single center over a 10-year period, 24 percent were discharged the same day, 63 percent were observed overnight, and 13 percent required ≥2 nights in the hospital [44]. Factors associated with longer length of stay included young age (ie <5 months); history of apnea; growth failure or G-tube dependence; previous admission for airway problems; and underlying diagnosis of cerebral palsy, genetic syndrome, or cardiac anomaly. Among patients who were discharged the same day or observed overnight, 2.5 percent had an unplanned readmission within 30 days after discharge.

Follow-up after surgery typically occurs at two to three weeks.

The optimal duration of acid suppression therapy after surgery is uncertain. Most otolaryngologists continue acid suppression therapy at least until the surgical site has healed. Some clinicians continue acid suppression until there are no further feeding or aspiration problems.

●Efficacy – In experienced hands, supraglottoplasty can produce dramatic improvements in breathing, feeding, and growth with little morbidity [49,50]. Opening the airway improves the suck-swallow-breath sequence. In patients with aspiration and without underlying neurological disease, aspiration often resolves after surgical intervention.

Reported success rates for supraglottoplasty range from 60 to 95 percent [51,52]. The definition of success varies from study to study; it generally refers to improvement in respiratory status and feeding problems without the need for additional surgical intervention. Success rates tend to be lower in children with concomitant neurologic disease or genetic syndromes. These patients more frequently require revision surgery, tracheostomy, or feeding tube insertion [51]. (See 'Failure of supraglottoplasty' below.)

Airway obstruction symptoms (eg, stridor, cyanotic/apneic episodes) usually improve dramatically after surgery. However, patients with preoperative obstructive sleep apnea (OSA) may continue to have persistent OSA despite intervention. In a meta-analysis of four observational studies involving children with sleep-disordered breathing due to laryngomalacia, supraglottoplasty was associated with improvements in the apnea-hypopnea index (AHI) and oxygen saturation [53]. Two other meta-analyses reported similar findings [2,54]. Patients with a preoperative AHI >12 demonstrate greater improvement; those younger than seven months of age at time of surgery also demonstrate greater impact on AHI [53]. In infants with neurologic disease, other congenital anomalies, or genetic syndromes, OSA tends to persist longer and can take up to four to five years to resolve [55]

Swallowing function improves gradually after supraglottoplasty [41,56]; the pace of improvement varies, depending on whether the infant has other comorbidities. In infants without underlying neurological disease, genetic syndromes, prematurity, or coexisting unrepaired airway lesions, swallowing function and aspiration gradually improve over several months to a year following surgery [29,57]. In one study that included 143 nonsyndromic patients who underwent supraglottoplasty, 25 percent had resolution of dysphagia by 4 months after surgery, 50 percent by 10 months, and 75 percent by 14 months [57]. Persistent feeding difficulties after supraglottoplasty are more common in preterm infants [58]. Patients who do not have sufficient improvement in feeding may require NG or G tube placement for nutrition. Some may require revision surgery. (See 'Failure of supraglottoplasty' below.)

Several studies have documented good catch-up growth following supraglottoplasty among infants with poor weight gain preoperatively [49,59].

●Complications – Possible complications of supraglottoplasty include supraglottic or glottic scarring, chronic aspiration, and dysphonia. These are uncommon, occurring in <10 percent of cases [51]. Some patients may require revision surgery if abnormal scarring occurs. (See 'Failure of supraglottoplasty' below.)

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 against the risk of worsening aspiration in this setting [20].

Failure of supraglottoplasty — Revision surgery may be required for those who do not improve after surgery. Revision surgery may also be required if too little tissue is removed at the initial operation, or abnormal scarring occurs. Revision surgery rates are higher in those with severe GER, neuromuscular disease, hypotonia, or underlying genetic syndromes [25,26,60-62].

If revision surgery is required, further investigation may be warranted to identify potential underlying contributing factors (eg, pathologic or undertreated GER, undiagnosed neurologic conditions such as a Chiari malformation).

Tracheostomy to bypass the supraglottic airway is usually reserved for high-risk patients with supraglottoplasty failure (ie, those with multilevel airway obstruction involving the trachea or tongue base, or syndromes associated with anatomic airway obstruction such as Pierre Robin sequence). Tracheostomy is rarely employed as a first-line operation for laryngomalacia.

Alternatives to surgery — For patients with unacceptably high surgical risk or if the parents/caregivers wish to avoid surgery, the main alternatives to surgery are noninvasive ventilatory support modalities such as continuous positive airway pressure (CPAP; which can be used in the home setting), or high-flow nasal cannula (HFNC; which is generally limited to inpatient care settings). These modalities have been used in children with laryngomalacia with variable success and compliance [63]. The positive pressure provided by CPAP or HFNC may reduce airway obstruction and improve respiratory symptoms in children with moderate to severe laryngomalacia. However, it increases the risk of feeding difficulties and aspiration. Thus, for children with laryngomalacia who are managed with these modalities, oral feeding is often deemed unsafe, and NG or G tube feeds are required.

The use of CPAP in the management of pediatric OSA is discussed in greater detail separately. (See "Continuous positive airway pressure (CPAP) for pediatric obstructive sleep apnea".)

OUTCOME — 

The prognosis for infants and children with laryngomalacia depends on the severity and whether there are associated comorbidities.

●Mild to moderate disease without underlying medical conditions – In children who are otherwise healthy, laryngomalacia usually resolves over time as the child grows. (See 'Natural history' above.)

●Severe disease – Most patients with severe laryngomalacia require surgery, as discussed above (see 'Severe laryngomalacia' above). There are few data on long-term outcomes following supraglottoplasty [51,64]. In the authors' experience, most patients treated with supraglottoplasty do well in the long-term. In our experience, approximately 85 percent of surgically treated patients recover well and do not require any further airway interventions. Approximately 5 percent of patients require revision surgery (eg, dividing a surgical site scar, removing additional redundant supra-arytenoid tissue, or suspending the epiglottis), which usually successfully addresses any residual symptoms. However, approximately 10 percent of patients do not achieve adequate improvement in breathing and swallowing function after supraglottoplasty. These patients usually require a combination of other interventions, including noninvasive positive airway pressure, enteral feeding via a nasogastric or gastrostomy tube, and/or other airway surgery (eg, tongue base procedure or tracheostomy).

●Patients with underlying neuromuscular or genetic conditions – Laryngomalacia tends to persist in these patients. Supraglottoplasty may improve symptoms, but many patients continue to have at least moderate symptoms following surgery [1,25,26]. Approximately 30 to 50 percent of patients require revision surgery and 10 to 40 percent ultimately require tracheostomy [25,26].

SUMMARY AND RECOMMENDATIONS

●Epidemiology – Congenital laryngomalacia is the most common cause of chronic or recurrent stridor in infancy. The reported prevalence is approximately three to four cases per 10,000 live births. (See 'Epidemiology' above.)

●Anatomy and pathogenesis – Congenital laryngomalacia is characterized by short aryepiglottic folds, an omega-shaped epiglottis, and collapse of the supra-arytenoid tissue into the glottic inlet during inspiration (picture 1 and movie 1). The pathogenesis of laryngomalacia is not fully understood. It likely results from a combination of neurologic, anatomic, and inflammatory abnormalities leading to diminished tone of the supraglottic tissue and supporting musculature. (See 'Anatomy' above and 'Pathogenesis' above.)

●Clinical features

•Presentation – Infants with congenital laryngomalacia usually present with inspiratory stridor that begins in the newborn period and peaks at four to eight months of age. Stridor worsens with activities such as crying, agitation, excitement, feeding, or supine positioning. The severity can range from mild intermittent symptoms to severe airway obstruction associated with respiratory distress (eg, retractions (movie 3), tachypnea), apnea, and cyanotic episodes. Feeding difficulties are common and can range from mild (occasional coughing and regurgitation during feeding) to severe (recurrent aspiration events or growth failure dure to inability to feed). (See 'Presentation' above and 'Severity assessment' above.)

•Associated conditions – Comorbid medical conditions are common in infants and children with congenital laryngomalacia and may include (see 'Associated conditions' above):

-Gastroesophageal reflux (GER)

-Neuromuscular disorders

-Genetic syndromes (eg, Down syndrome or 22q11.2 deletion syndrome)

-Secondary airway abnormalities (eg, subglottic stenosis, tracheomalacia)

-Prematurity

●Natural history – In children who are otherwise healthy, laryngomalacia typically resolves over time as the child grows. Most patients outgrow stridor by 24 months of age. By contrast, laryngomalacia tends to persist in children who have underlying neuromuscular disorders and genetic syndromes. (See 'Natural history' above and 'Outcome' above.)

●Referral indications – Referral to a pediatric otolaryngologist is warranted for any infant or child presenting recurrent or chronic stridor. The urgency of referral is guided by symptom severity. Infants with severe symptoms warrant expedited referral; those who have significant respiratory distress, apnea, desaturation, and/or inability to feed generally warrant inpatient admission. For infants with mild intermittent inspiratory stridor who are otherwise feeding and growing well, nonurgent referral is appropriate. (See 'Referral indications' above.)

●Diagnosis – The diagnosis of laryngomalacia is made with awake flexible fiberoptic laryngoscopy demonstrating an omega-shaped epiglottis, short aryepiglottic folds, and collapse of the supra-arytenoid tissue into the glottic inlet during inspiration (movie 1 and picture 1). A retro-displaced epiglottis may also be present (movie 2), especially in children with underlying neurological disorders. (See 'Confirming the diagnosis' above.)

Once the diagnosis of laryngomalacia is confirmed, additional evaluation may be warranted in some cases to evaluate for secondary airway lesions, swallowing dysfunction/aspiration, sleep-disordered breathing, and/or gastroesophageal reflux disease (GERD). (See 'Additional evaluation' above.)

●Differential diagnosis – The differential diagnosis includes other causes of recurrent stridor presenting in infancy, including subglottic stenosis, vocal cord paralysis, vascular rings, laryngeal clefts, subglottic hemangiomas, tracheomalacia, and recurrent croup (figure 3 and table 1A-B). Laryngomalacia is distinguished from these based upon the endoscopic examination. (See "Assessment of stridor in children".)

●Management of mild disease – For infants and children with mild laryngomalacia (ie, those with intermittent stridor but who otherwise feed comfortably and have normal growth), we suggest conservative management with supportive care and observation (Grade 2C). These patients should have ongoing monitoring to ensure weight gain is adequate (algorithm 1). (See 'Mild laryngomalacia' above.)

●Management of moderate to severe disease – Infants and children with moderate to severe laryngomalacia (persistent stridor associated with feeding difficulty, poor weight gain, retractions, apnea, and/or cyanosis) should be managed in collaboration with a pediatric otolaryngologist and a clinician experienced in infant swallowing and feeding disorders (eg, a speech or occupational therapist). Our suggested approach to management is as follows (algorithm 1) (see 'Management' above):

•Initial interventions – For most patients with moderate or severe laryngomalacia, we suggest initial medical therapy rather than proceeding directly to surgical intervention (Grade 2C). Medical management includes (see 'Initial interventions (medical therapy)' above):

-Acid suppression therapy – For all patients with moderate or severe laryngomalacia, we suggest empiric therapy with an acid suppression agent (eg, a histamine type 2 receptor antagonist [H2RA] or proton pump inhibitor [PPI]) (Grade 2C). Dosing, administration, and side effects of these medications are summarized in the table (table 2) and discussed in detail separately. (See "Gastroesophageal reflux disease in children and adolescents: Management", section on 'Pharmacotherapy'.)

-Feeding modification – Feeding interventions should be tailored to the individual patient and may include measures such as texture modification (eg, thickened feeds), paced feedings, or use of various special nipples for bottle feeding to reduce flow and minimize aerophagia. Infants with poor weight gain may require high-calorie formula or breast milk supplements to promote weight gain. These interventions are discussed in greater detail separately. (See "Neonatal oral feeding difficulties due to sucking and swallowing disorders", section on 'Management approach' and "Gastroesophageal reflux in infants", section on 'Additional options for infants with GERD or problematic reflux' and "Poor weight gain in children younger than two years in resource-abundant settings: Management", section on 'Nutritional therapy'.)

•Persistent severe symptoms – For patients with severe laryngomalacia that persists despite medical management, we suggest supraglottoplasty (Grade 2C). Supraglottoplasty removes redundant supraglottic tissue thereby reducing the severity of airway obstruction (picture 2). Complications are uncommon (<10 percent) and may include supraglottic or glottic scarring, chronic aspiration, and dysphonia. Revision surgery may be required in some patients, particularly those with underlying neuromuscular disease or genetic syndromes. (See 'Supraglottoplasty' above.)