Reconstruction of a Sternal Cleft and Ventral Hernia in an Adolescent with Pentalogy of Cantrell Utilizing Titanium Plates.

Abstract

Pentalogy of Cantrell (PC) is a rare congenital disorder that presents with several midline defects including the abdominal wall, sternum, diaphragm, pericardium, and heart. Surgical intervention for omphaloceles and cardiac defects typically occur in infancy, but the optimal surgical repair for thoracoabdominal wall defects varies by patient. We present a 15-year-old female with PC, who underwent previous omphalocele, diaphragm, and cardiac repair as an infant, with recurrent ventral and diaphragmatic defects as well as a large sternal cleft with ectopia cordis. This case demonstrates the feasibility of delayed definitive surgical repair of the abdominal wall, diaphragm, and sternum utilizing titanium plates, sternal wires, and myocutaneous advancement flaps. While several surgical approaches have been described for repair of sternal defects in infants and children with PC, large defects remain challenging to obtain rigid and lasting cardiac coverage without compressing the heart but still allowing for growth of the chest wall.