eMedicine Specialties > Pediatrics: General Medicine > Pulmonology
Laryngomalacia
Updated: Jul 24, 2009
Introduction
Background
Laryngomalacia is a congenital abnormality of the laryngeal cartilage. 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.
Laryngomalacia: The epiglottis is small and curled on itself (omega-shaped). Approximation of the posterior edges of the epiglottis contributes to the inspiratory obstruction. (From B Benjamin, Atlas of Paediatric Endoscopy, Oxford University Press, NY, 1981, with permission.)
Pathophysiology
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
United States
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
Laryngomalacia has no sex predilection.
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.
Clinical
History
- 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 crying or 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.
- The infant is usually happy and thriving.
Physical
- 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 as many as 20% of infants with laryngomalacia also have another airway lesion.
Causes
- 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.
- No genetic pattern is known.
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References
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Further Reading
Keywords
omega-shaped epiglottis, laryngeal cartilage, malformation of the laryngeal cartilage, chronic inspiratory noise, gastroesophageal reflux, reflux laryngitis, hypoxemia, hypoventilation, choking, laryngomalacia, congenital stridor, swollen arytenoid cartilages, pulmonary artery hypertension, hypoxemia, hypoventilation, feeding problems, treatment, diagnosis
