Staged repair of ectopia cordis

Ectopia cordis is a challenging congenital anomaly that generally has defied repair because of the magnitude of the deformity and the associated defects, particularly the intracardiac anomalies. We were fortunate to be presented with a boy who had successfully undergone an operation on the day of his birth to provide skin coverage over his bare ectopic heart. Subsequent studies disclosed no intracardiac anomalies. This paper describes the second stage of his repair designed to place the heart within the thorax and construct a bony shield anterior to it.