Staged reconstruction of large congenital diaphragmatic defects with synthetic patch followed by reverse latissimus dorsi muscle

Background/Purpose: Synthetic repair of large congenital diaphragmatic defects (>90%) invariably will lead to recurrence, progressive chest wall deformity, and restrictive pulmonary disease. Staged reconstruction with living, growing tissue can help avoid these complications. Methods: Between November 1995 and December 1999, 5 patients (median age, 25 months) with diaphragmatic agenesis underwent staged replacement with a reverse latissimus dorsi (RLD) flap. All required extracorporeal membrane oxygenation (ECMO) support at birth followed by synthetic patch (polytetrafluoroethylene or PTFE) diaphragm closure. Clinical evidence of patch disproportion, including recurrence (n = 3), chest wall deformity (n = 3), radiographic findings (n = 2), and restrictive respiratory patterns (n = 1), provided indication for replacement. The procedure involves removal of the original patch via thoracotomy followed by transposition of a RLD flap (based on the paraspinous and intercostal perforating vessels) into the defect through the bed of the 10th rib. Two patients underwent concomitant fundoplication. Results: RLD flap was completed successfully in all 5 patients. Median length of stay after the procedure was 8 days. With a mean follow-up of 28 months (15 to 64 months), there have been no recurrences and no complications related to the procedure. Respiratory status and chest wall deformity have improved. There was neither evidence of paradoxical chest wall movement nor obvious limitation of the ipsilateral upper extremity on physical examination. The RLD flaps have grown proportionately to the children and remained at a stable level on chest x-ray. Conclusions: Staged reconstruction with patch closure followed by definitive reverse latissimus dorsi flap repair is a safe and highly effective treatment option in patients with diaphragmatic agenesis. A planned replacement should be considered in all patients with severe diaphragmatic hernias and patch closure before the development of thoracic complications.