Laryngomalacia, shown in the image below, is a congenital abnormality of the laryngeal cartilage. It is a dynamic lesion resulting in collapse of the supraglottic structures during inspiration, leading to airway obstruction. It is thought to represent a delay of maturation of the supporting structures of the larynx. Laryngomalacia is the most common cause of congenital stridor and is the most common congenital lesion of the larynx.
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The epiglottis is small and curled on itself (omega-shaped). Approximation of the posterior edges of the epiglottis contributes to the inspiratory obs....
Laryngomalacia may affect the epiglottis, the arytenoid cartilages, or both. When the epiglottis is involved, it is often elongated, and the walls fold in on themselves. The epiglottis in cross section resembles an omega, and the lesion has been referred to as an omega-shaped epiglottis. If the arytenoid cartilages are involved, they appear enlarged. In either case, the cartilage is floppy and is noted to prolapse over the larynx during inspiration. This inspiratory obstruction causes an inspiratory noise, which may be high-pitched sounds frequently heard in other causes of stridor, coarse sounds resembling nasal congestion, and low-pitched stertorous noises. More severe compromise may be associated with a lower ratio of the aryepiglottic fold length to the glottic length.
A classification system has been proposed. In type 1 laryngomalacia, the aryepiglottic folds are tightened or foreshortened. Type 2 is marked by redundant soft tissue in any area of the supraglottic region. Type 3 is associated with other disorders, such as neuromuscular disease and gastroesophageal reflux.
Laryngomalacia is the most common cause of chronic inspiratory noise in infants, no matter which type of noise is heard. Infants with laryngomalacia have a higher incidence of gastroesophageal reflux, presumably a result of the more negative intrathoracic pressures necessary to overcome the inspiratory obstruction. Conversely, children with significant reflux may have pathologic changes similar to laryngomalacia, especially enlargement and swelling of the arytenoid cartilages. Some of the swelling of the arytenoid cartilages and of the epiglottis may be secondary to reflux.
Occasional inflammatory changes are observed in the larynx, which is referred to as reflux laryngitis. When the epiglottis is involved, gravity makes the noise more prominent when the baby is supine.
The exaggerated inspiratory effort increases blood return to the pulmonary vascular bed. This could account for the increased likelihood of pulmonary artery hypertension in infants with hypoxemia.
Frequency is unknown. Often, the diagnosis is presumed.
Mortality/Morbidity
Rarely, the lesion may cause enough hypoxemia or hypoventilation to interfere with normal growth and development. In severe cases, when laryngomalacia may be associated with gastroesophageal reflux, feeding problems such as choking or gagging may occur.
Race
No known race predilection has been reported.
Sex
Although previous reports in predominately white populations have reported a male predominance (58-76% of cases), a more recent study of a more ethnically diverse population demonstrated no significant difference between males and females.[1]
Age
Although this is a congenital lesion, airway sounds typically begin at age 4-6 weeks. Until that age, inspiratory flow rates may not be high enough to generate the sounds. Symptoms typically peak at age 6-8 months and remit by age 2 years.
Late-onset laryngomalacia may be a distinct entity, which can present after age 2 years.
The usual history in patients with laryngomalacia is of inspiratory noises that begin during the first 2 months of life. Sounds typically start at age 4-6 weeks, but they may begin in the nursery or as late as age 2-3 months.
Noises are inspiratory and may sound like nasal congestion, with which they are initially confused. However, the noises persist and no nasal secretions are present. The noise may be more high pitched, crowing stridor.
Noise is often increased when the baby is supine, during sleep, during agitation, during upper respiratory infection episodes, and, in some cases, during and after feeding.
The baby's cry is usually normal, unless concomitant reflux laryngitis is present.
Usually, no feeding intolerance is noted, although occasional choking or coughing with feedings may be noted if the baby has reflux and/or aspiration. Noises may increase during and after feeding.
Upon examination, the baby is usually happy and appropriately interactive.
Mild tachypnea may be present.
Other vital signs are normal, and oxygen saturation is usually normal.
One can usually detect nasal airflow.
The noise may be increased if the baby is placed supine.
The cry is normal. Hearing the baby's cry during the examination is important. An abnormal cry suggests pathology at or near the vocal cords.
The noise is purely inspiratory. The sounds may best be heard just above the sternal notch.
The rest of the examination findings are unremarkable, although another airway lesion may also be present in infants with laryngomalacia.[2]
Laryngomalacia may present primarily with snoring and/or sleep-disordered breathing.[3] Also, swallowing dysfunction may be present in a significant proportion of children.[4] Therefore sleep disordered breathing and swallow dysfunction should be considered in children, older than 3 months, presenting with these upper airway complaints.[5]
Laryngomalacia is a congenital abnormality of the larynx. The pathology is unknown. In cases in which redundant or tight tissue has been removed, it is histologically indistinct from normal tissue.
These studies are the best studies used to confirm the diagnosis. However, in an infant with typical inspiratory noises (worse when supine) who have a normal cry and normal growth and development, clinical diagnosis is not unreasonable.
A pediatric pulmonologist or pediatric otorhinolaryngologist may perform flexible laryngoscopy or bronchoscopy. Bronchoscopy under anesthesia has been shown to be more sensitive and specific than bronchoscopy in infants who are awake.
Direct visualization of the airway reveals an omega-shaped epiglottis that prolapses over the larynx during inspiration.
Enlarged arytenoid cartilages that prolapse over the larynx during inspiration may also be present.
In more than 90% of cases, the only treatment necessary for laryngomalacia is time. The lesion gradually improves, and noises disappear by age 2 years in virtually all infants. The noise steadily increases over the first 6 months, as inspiratory airflow increases with age. Following this increase, a plateau often occurs with a subsequent gradual disappearance of the noise. In some cases, the signs and symptoms dissipate, but the pathology may persist into childhood and adulthood. In those cases, symptoms or signs may recur with exercise or sometimes with viral infections.
Children with severe retractions, cyanotic spells, and apneas during sleep may have obstructive sleep apnea associated with laryngomalacia. These children should be evaluated with a sleep study. Supraglottoplasty may be of benefit in children with severe symptoms of laryngomalacia (see below).[6] Thus, a detailed sleep history should be taken in all infants with symptoms of laryngomalacia.
Infants with laryngomalacia have a higher incidence of gastroesophageal reflux and swallowing dysfunction.[4] Thus, acid suppression and swallowing therapy have been used in children with symptoms of moderate laryngomalacia.[7]
If the baby has clinically significant hypoxemia (defined as a resting oxygen saturation < 90%), supplemental oxygen should be administered. Recent data suggest infants with laryngomalacia and hypoxemia may more readily develop pulmonary hypertension.[8] Therefore, children with hypoxemia should periodically undergo evaluation for pulmonary hypertension.
If the baby has normal cry, normal weight gain, normal development, and purely inspiratory noise that developed within the first 2 months of life, then no further workup may be necessary. Parents may be told that laryngomalacia is the most likely diagnosis, and they can be assured of its natural history.
If the picture is not obvious or if the parents are not completely reassured, diagnostic procedures include fluoroscopy and flexible laryngoscopy or bronchoscopy. Flexible bronchoscopy with the child anesthetized is more specific and sensitive than flexible bronchoscopy in a child who is awake.
Special concerns
There is a distinct group of older children (aged >2 years) with late-onset laryngomalacia, or occult laryngomalacia, who do not present with the typical congenital symptoms of noisy breathing. Children manifest symptoms during feeding, exercise, or sleep.[9] Many are identified with snoring or sleep-disordered breathing as initial symptoms and are diagnosed with laryngomalacia upon direct visualization of the airway. In late-onset laryngomalacia, supraglottoplasty may be beneficial for cases of moderate-to-severe obstructive sleep apnea associated with significant apnea-hypopnea index on sleep study.[10, 11] However, other causes for obstruction, such as adenotonsillar hypertrophy, should also be evaluated.
In severe cases in which the laryngomalacia interferes with ventilation enough to impair normal eating, growth, and development, a surgical approach is possible.[12]
Approximately 10% of patients with severe congenital laryngomalacia require surgical intervention because of failure to thrive, significantly elevated carbon dioxide or hypoxemia, severe obstructive sleep apnea, pulmonary hypertension, or cor pulmonale. Operations include simple tracheotomy or supraglottoplasty in which support structures are tightened and excess tissue on the epiglottis is removed.[29] Laser epiglottopexy has been successful.[13, 14]
A meta-analysis by Farhood et al found that the Apnea-Hypopnea Index (AHI) improved by a mean of 12.5 points after supraglottoplasty for laryngomalacia with obstructive sleep apnea, however, 88% (29 of 33 children) had residual disease.[15]
Recent studies have demonstrated in severe cases of laryngomalacia that require supraglottoplasty, there is significant improvement post-operatively in overall quality of life of caregivers and infants, with a high degree of parental satisfaction, especially in parental perception of swallowing among these children.[30, 31, 32]
If the parents require another opinion or if the lesion is clinically severe, consultation with a pediatric pulmonologist or pediatric otorhinolaryngologist may help.
Prognosis is excellent. Most babies outgrow the condition by their second birthday, many by the first. In some cases, even though the signs and symptoms dissipate, the pathology persists. Such patients may have stridor with exercise later in life.
Laryngomalacia may be more common in children with Down syndrome, in whom it may persist beyond the second birthday.
What is laryngomalacia?What is the pathophysiology of laryngomalacia?How is laryngomalacia classified?How is laryngomalacia characterized in infants and children?What is the incidence of laryngomalacia in the US?What is the mortality and morbidity associated with laryngomalacia?How does the prevalence of laryngomalacia vary among racial groups?How does the prevalence of laryngomalacia vary by sex?How does the prevalence of laryngomalacia vary by age?What are the signs and symptoms of laryngomalacia?Which physical findings are characteristic of laryngomalacia?What is the primary presentation of laryngomalacia?What causes laryngomalacia?What are the differential diagnoses for Laryngomalacia?What monitoring is needed in patients with laryngomalacia?What is the role of imaging studies in the evaluation of laryngomalacia?What is the role of laryngoscopy and bronchoscopy in the evaluation of laryngomalacia?What are the treatment options for laryngomalacia?When is a sleep study indicated in the management of laryngomalacia?When is acid suppression and swallowing therapy indicated in the treatment of laryngomalacia?When is supplemental oxygen indicated in the treatment of laryngomalacia?What is late-onset laryngomalacia and how is it treated?When is surgery indicated in the treatment of laryngomalacia?Which specialist consultations may be needed in the treatment of laryngomalacia?What dietary restrictions are recommended for laryngomalacia?What activity restrictions are recommended for laryngomalacia?Which medications are used in the treatment of laryngomalacia?What is the duration of laryngomalacia?What is the indication for acid suppression for the treatment of laryngomalacia?What is the outpatient treatment of laryngomalacia?When is inpatient care indicated for laryngomalacia?What medications are indicated for laryngomalacia?How is laryngomalacia prevented?What are possible complications of laryngomalacia?What is the prognosis of laryngomalacia?
Stephanie Lovinsky-Desir, MD, MS, Assistant Professor of Pediatric Pulmonology, Morgan Stanley Children’s Hospital of New York-Presbyterian, Columbia University College of Physicians and Surgeons
Disclosure: Nothing to disclose.
Coauthor(s)
Laura Anne Conrad, DO, Fellow, Division of Pediatric Pulmonology, New York-Presbyterian-Columbia University Medical Center
Disclosure: Nothing to disclose.
Specialty Editors
Mary L Windle, PharmD, Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Nothing to disclose.
Charles Callahan, DO, Professor, Chief, Department of Pediatrics and Pediatric Pulmonology, Tripler Army Medical Center
Disclosure: Nothing to disclose.
Chief Editor
Denise Serebrisky, MD, Associate Professor, Department of Pediatrics, Albert Einstein College of Medicine; Director, Division of Pulmonary Medicine, Lewis M Fraad Department of Pediatrics, Jacobi Medical Center/North Central Bronx Hospital; Director, Jacobi Asthma and Allergy Center for Children, Jacobi Medical Center
Disclosure: Nothing to disclose.
Additional Contributors
Michael R Bye, MD, Professor of Clinical Pediatrics, State University of New York at Buffalo School of Medicine; Attending Physician, Pediatric Pulmonary Division, Women's and Children's Hospital of Buffalo
Boggs W. Laryngomalacia Commonly Presents With Snoring or Swallowing Dysfunction. Reuters Health Information. Available at http://www.medscape.com/viewarticle/825146. May 15, 2014; Accessed: June 16, 2015.
The epiglottis is small and curled on itself (omega-shaped). Approximation of the posterior edges of the epiglottis contributes to the inspiratory obstruction. Used with permission from Oxford University Press [Benjamin B. Atlas of Paediatric Endoscopy: Upper Respiratory Tract and Oesphagus. New York, NY: Oxford University Press; 1981.]
The epiglottis is small and curled on itself (omega-shaped). Approximation of the posterior edges of the epiglottis contributes to the inspiratory obstruction. Used with permission from Oxford University Press [Benjamin B. Atlas of Paediatric Endoscopy: Upper Respiratory Tract and Oesphagus. New York, NY: Oxford University Press; 1981.]
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