[About the problem of congenital laryngo-tracheo-esophageal cleft]

Abstract

The congenital laryngo-tracheo-oesophageal cleft is an extremely rare anomaly representing an incomplete or absent separation of larynx and trachea from the oesophagus. Depending on the extent of the anomaly, the phenomenon is classified into laryngeal cleft (type I), cleft affecting the superior part of the trachea (type II) and defect extending to the bifurcation (type III). We report on 3 cases. A boy with a type II cleft died at the age of two days due to respiratory insufficiency conditioned by aspiration, before surgery would have been possible. In another boy with a type II cleft closure was successfully performed in several sessions. A girl with a type III cleft died at the age of five months after the defect had been almost completely closed in two sessions. The right main bronchus and the right lung were hypoplastic and also the left main bronchus was partly represented by skin formation only. This resulted in progressive respiratory insufficiency. Both boys had an associated malformation of the G syndrome type. Despite the unhappy outcome of the last of three cases mentioned above, it seems that stepwise closure is less associated with surgical risk than surgery in a single session. A review of the 24 cases of cleft described in the literature shows that these children present very serious treatment problems and that there is a high incidence--amounting to 60%--of associated malformations, especially of the lungs and bronchi.