Repair of thoracoabdominal ectopia cordis with myocutaneous flaps and intraoperative tissue expansion.

Abstract

Thoracoabdominal ectopia cordis is a congenital malposition of the heart and abdominal viscera, which are externally situated, carrying an extremely poor prognosis. We report a case of a complete thoracoabdominal ectopia cordis that had undergone successful coverage over the exposed heart and abdominal contents. A few hours after birth, the defect was repaired with an expanded polytetrafluoroethylene soft-tissue patch that was gradually reduced by suture plication. Coverage was completed 48 hours later with bilateral pectoralis major and rectus abdominis myocutaneous flaps. Intraoperative tissue expansion minimized the cardiac compression produced by surgical reconstruction of the defect. Our patient, who is now 36 months old, represents the seventeenth case to survive reconstructive attempts.