Chest wall reconstruction in a patient with Cantrell syndrome

Abstract

Cantrell syndrome is a very rare congenital anomaly with up to five features: a midline, upper abdominal wall abnormality, lower sternal defect, anterior diaphragmatic defect, diaphragmatic pericardial defect, and congenital abnormalities of the heart. This report describes our experience of performing a reconstruction of a chest wall defect in a Cantrell syndrome case with herniation of the heart. The patient was a 1-month-old female infant who received surgical patch repair of a ventricular septal defect (VSD) and atrial septal defect (ASD) at the Department of Cardiac Surgery. Subsequently, the patient underwent reconstruction at the second-stage surgery. A rhomboid skin flap with an inferior pedicle was used to close the defect. In this process the flap, including portions of the rectus abdominis muscles, was elevated and transferred into the defect. The sectioned ends of the divided pectoralis major muscles were sutured together to simultaneously reconstruct the muscles. It has been 2 years since the surgery, and the defect is covered with normal skin, and the protrusion of the heart from the chest wall and the externally visible pulsation have been resolved. The progress has been very good functionally and cosmetically.