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.
Kawaguchi
Akemi L
AL
Department of Pediatric Surgery, Mass General Hospital for Children, Massachusetts General Hospital, Boston, MA 02114, USA.
Donahoe
Patricia K
PK
Ryan
Daniel P
DP
D000293:Adolescent; D000328:Adult; D001980:Bronchi / Q000002:abnormalities; D002648:Child; D003072:Cognition Disorders / Q000209:etiology; D004066:Digestive System Diseases / Q000209:etiology; D013505:Digestive System Surgical Procedures / Q000009:adverse effects; D004947:Esophagus / Q000002:abnormalities*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007442:Intubation, Intratracheal; D007830:Larynx / Q000002:abnormalities*; D008171:Lung Diseases / Q000209:etiology; D008297:Male; D011015:Pneumonia, Aspiration / Q000209:etiology; D011788:Quality of Life; D012008:Recurrence; D012086:Reoperation; D013270:Stomach / Q000002:abnormalities / Q000601:surgery; D014132:Trachea / Q000002:abnormalities*; D014138:Tracheoesophageal Fistula / Q000209:etiology* / Q000601:surgery