8.4 Laryngeal Clefts
Key Features
Laryngeal clefts are a rare congenital anomaly.
They are frequently associated with other anomalies.
Clinical presentation varies with the extent of the cleft. The patient may have problems with the airway, feeding, and voice.
Most clefts are short, but complete laryngotracheoesophageal clefts have a mortality rate greater than 90%.
Laryngeal clefts represent a rare cause of stridor. Failed fusion of the posterior cricoid lamina and incomplete development of the tracheoesophageal septum results in a laryngeal cleft, an abnormal communication of the larynx and esophagus. Laryngeal clefts are usually sporadic nonsyndromic congenital abnormalities. Most are associated with other nonsyndromic congenital abnormalities, including tracheoesophageal fistula (TEF), esophageal atresia, congenital heart disease, cleft lip and palate, micrognathia, glossoptosis, laryngomalacia, gastrointestinal and genitourinary anomalies. Rarely, they are attributable to a specific syndrome [Opitz-Frias′s and Pallister-Hall′s syndrome, VATER (v ertebral defects, imperforate a nus, t racheo e sophageal fistula, and r enal dysplasia) association].
Epidemiology
Laryngeal clefts are rare. Congenital anomalies of the larynx are found in only 0.5% of the population, and clinically symptomatic laryngeal clefts constitute only 0.3 to 0.5% of all congenital laryngeal anomalies. Males are more likely to be affected than females (3:1). Thirty percent of laryngeal clefts are associated with maternal polyhydramnios. TEF is present in ~ 25% of patients with a laryngeal cleft and is associated with a higher failure rate of surgical repair.
Clinical
Signs and Symptoms
There are no pathognomonic findings. Clinical symptoms depend on the extent of the cleft. Small clefts, whose anatomic involvement is limited to the interarytenoid musculature, present with stridor and feeding problems. There may be coughing, choking, stridor, aspiration pneumonias, or cyanotic episodes. Occasionally, patients with small laryngeal clefts may be asymptomatic. However, the most severe clefts are accompanied by aphonia, severe upper airway obstruction, and respiratory distress. Stridor occurs due to anterior collapse of posterior supraglottic structures. Cyanosis and stridor are exacerbated with feeding. In utero, polyhydramnios due to impaired swallowing of amniotic fluid by the fetus is associated with laryngeal clefts.
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