Laryngotracheoesophageal cleft

Laryngotracheoesophageal cleft (LTEC), also known as laryngeal cleft or posterior laryngeal cleft, is a rare congenital malformation characterized by an abnormal communication (cleft) between the larynx and/or trachea and the esophagus along the posterior midline. This defect results from failure of the tracheoesophageal septum to develop properly during embryonic life. The condition primarily affects the respiratory and digestive systems, as the abnormal opening allows food, liquids, and saliva to pass from the esophagus into the airway. The severity of laryngotracheoesophageal clefts varies widely and is classified into four types (Benjamin-Inglis classification). Type I (submucous or interarytenoid cleft) is the mildest form, extending no further than the level of the vocal folds. Type II extends below the vocal folds into the cricoid cartilage. Type III extends into the cervical trachea, and Type IV, the most severe, extends into the thoracic trachea and may involve the carina. Key symptoms include chronic aspiration, recurrent pneumonia, stridor, a weak or hoarse cry, feeding difficulties, coughing or choking during feeds, and failure to thrive. In severe forms, life-threatening respiratory distress may occur shortly after birth. Laryngotracheoesophageal clefts may occur in isolation or in association with other congenital anomalies, including tracheoesophageal fistula, esophageal atresia, congenital heart defects, and other midline defects. Some cases are associated with Opitz G/BBB syndrome or Pallister-Hall syndrome. Diagnosis is confirmed by microlaryngoscopy and bronchoscopy. Treatment depends on the severity of the cleft. Mild (Type I) clefts may be managed conservatively with thickened feeds, anti-reflux measures, and swallowing therapy, or may be repaired endoscopically. More severe clefts (Types II–IV) typically require open surgical repair, which may involve a lateral pharyngotomy or thoracotomy approach. Prognosis is generally good for milder forms but is more guarded for Types III and IV, which carry significant morbidity and mortality.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Laryngotracheoesophageal cleft