Management of Laryngotracheoesophageal Cleft

Abstract

Laryngotracheoesophageal cleft (LTE) is a rare disorder that is still associated with a high mortality. Often the mortality is associated with other coexisting severe congenital anomalies, but even in those infants in whom the LTE cleft is the only anomaly, the mortality rate is still unacceptably high. This paper explores two avenues aimed at reducing the mortality. There are two main factors responsible for the high mortality. First, the diagnosis may be difficult and hence delayed; and second, once the diagnosis is made, management reported to date does not adequately prevent aspiration of stomach contents, which is the main mechanism of the infant's death. With respect to early diagnosis, the key symptom is respiratory distress exacerbated by attempts at feeding. The key diagnostic radiological procedure is a cineesophagram which shows a high spillover of contrast into the trachea on swallowing. The definitive diagnosis is made by endoscopy. Endoscopic evaluation can be confusing but if the anomaly is considered by the surgeon, an important maneuver at endoscopy, under general anesthesia, is to pass an endotracheal tube into the larynx. The tube stents open the edges of the cleft, which can clearly be seen while performing esophagoscopy, thus confirming the diagnosis. With respect to early management, the commonest cleft is a partial LTE cleft. Initially the airway should be maintained either by placement of an endotracheal tube or a tracheotomy. The more entensive the cleft, the more easily does the indwelling tube slip over the edge of the defect into the esophagus. In all but the mildest of clefts, gastric decompression and alimentation are required. This still allows for considerable reflux of gastric contents into the lungs, and we advocate a high total gastric section with a double gastrostomy to minimize the possibility of aspiration of stomach contents. After the infant's general condition is stabilized, definitive early surgical repair is undertaken. This paper includes a detailed case report of one patient, illustrating the above points of early diagnosis, total gastric division and early repair.