Management and long-term follow-up of patients with types III and IV laryngotracheoesophageal clefts

Background: Laryngotracheoesophageal cleft (LTEC) is a rare congenital anomaly that occurs when the trachea and esophagus fail to separate during fetal development. The 2 most severe forms of LTEC are type III, with extension of the cleft from the larynx to the carina, and type IV, with extension of the cleft into one or both mainstem bronchi. Methods: Over the past 25 years, we have accumulated an experience caring for 9 patients with severe LTEC, including 4 with type III and 5 with type IV. Results: Morbidity and mortality from severe LTEC often result from aspiration and chronic lung disease. Patients with types III (1/4) and IV (5/5) LTEC have an extremely high incidence of microgastria with a shortened esophagus for which fundoplication is ineffective. Because gastric feeding often does not initially increase stomach volume and may cause severe aspiration, we suggest early gastric division with later reconstruction of intestinal continuity in patients with microgastria. Postoperative tracheoesophageal fistulas have occurred in 6 of 9 patients. Conclusions: Generous interposition of vascularized tissue with a multiple-layer closure has helped to prevent further recurrences. Postoperative tracheomalacia may be managed with continuous positive airway pressure and may require customized endotracheal tubes. Evaluation of respiratory and digestive function, school performance, and quality of life for the surviving patients is described. (C) 2005 Elsevier Inc. All rights reserved.