Strategies for managing Type IV laryngotracheoesophageal clefts at Great Ormond Street Hospital for Children.

OBJECTIVE: To review our strategy for the treatment of patients with Type IV laryngotracheoesophageal cleft-a very rare congenital malformation.

METHODS: Retrospective review of nine cases of Type IV laryngotracheoesophageal cleft managed between October 1994 and January 2004 at Great Ormond Street Hospital for Children, London.

RESULTS: Three children were not operated upon (Group A) because of serious co-morbidity and parental decision not to opt for repair; they died at the ages of 2, 7 and 14 days, respectively. Six cases were repaired (Group B) at ages ranging from 2 to 53 days, all using an anterior cervico-thoracic approach. Two cases were operated upon using conventional ventilation, three using cardiopulmonary bypass and one using extracorporeal membrane oxygenation. There was no intra-operative mortality. The number of operative and diagnostic procedures varied from 4 to 37. Two operated cases expired at the ages of 11 days and 25 months; both were operated upon using cardiopulmonary bypass and had significant cardiac co-morbidity. Post-operative microlaryngoscopy and bronchoscopy showed dehiscence in the cleft repair ranging from 1mm to 2cm in five cases. This was observed at the distal end in three patients and just below the vocal cords in two cases. Three cases underwent further repair which was successful. The most recent case repaired using extracorporeal membrane oxygenation required less heparin when compared with those done on cardiopulmonary bypass and had the best post-operative result. Two patients were finally decannulated. The total duration of diagnostic and operative procedures ranged from 9 to 26h and 30min. The hospital stay in the operated cases varied from 9 days to 2 years 2 months 3 days, and their ICU stay ranged from 9 days to 10 months 7 days. All four of our patients with clefts which ended above the carina are alive after multiple repairs whereas all five cases with clefts extending all the way to the carina died (repair was attempted in two).

CONCLUSION: Early diagnosis and repair are essential for successful treatment of Type IV laryngotracheoesophageal clefts. We believe it is best to repair this defect via an anterior cervico-thoracic approach, with or without a median sternotomy, and extracorporeal membrane oxygenation is now our preferred method of gas exchange during such repair. The longest Type IV clefts extending all the way to the carina have the worst prognosis. The decision to operate or not should be based upon the associated co-morbidity and fully informed parental choice, since treatment entails significant morbidity and mortality.