Laryngo-tracheo-oesophageal cleft. Clinical features, diagnosis and therapy.
The laryngo-tracheo-oesophageal cleft is marked by a missing anatomical separation of the oesophagus and the larynx. The cleft can be restricted to the dorsal part of the larynx (type I), extend to the upper area of the trachea (type II) or involve the whole of the trachea (type III). In reviewing our three cases and 82 cases in the literature, clinical features, diagnosis, and therapy of this rare condition are presented. The condition leads almost always to life-threatening disturbances in the form of asphyxia and aspiration pneumonia. The diagnosis is made through direct laryngoscopy. Radiological demonstration of swallowing and tracheo-oesophagoscopy are necessary additional investigations. In many cases further severe malformations of organs are found, i.e., oesophageal atresia and tracheo-oesophageal fistulae. Despite early detection the prognosis remains unfavourable with a mortality of 46%. With few exceptions, an early operative correction of the defect is required. Access to the dorsal laryngeal area is achieved usually by a lateral pharyngotomy. Difficulties arise in securing adequate closure of the dorsal larynx, because of lack of sufficient tissue material. Despite secure closure of the laryngo-tracheo-oesophageal cleft, disturbances of swallowing often persist. Development of speech is not impaired.
D001237:Asphyxia / Q000209:etiology; D003680:Deglutition Disorders / Q000209:etiology; D004947:Esophagus / Q000002:abnormalities*; D006801:Humans; D007231:Infant, Newborn; D007828:Laryngoscopy; D007830:Larynx / Q000002:abnormalities*; D008297:Male; D011015:Pneumonia, Aspiration / Q000209:etiology; D011379:Prognosis; D014132:Trachea / Q000002:abnormalities*