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A rare type of congenital diaphragmatic hernia occurs in which there is a large opening in the anterior diaphragm between the pericardial and peritoneal cavities without a sac. This hernia is invariably associated with defects in the anterior abdominal wall and with sternal defects. More commonly, it is associated with cardiac anomalies as in the Pentalogy of Cantrell. The etiology of this hernia is undoubtedly different from the more common hernia of Morgangni, which has a sac and few associated anomalies. The etiology may be failure of fusion of the pars sternis area of the septum transversum. Only five other cases have been described.

Twenty-three patients with gastroschisis or omphalocele undergoing delayed ventral herniorrhaphy were reviewed. The ventral defects resulted from elective skin flap coverage in 15 patients, prosthetic silo failure in five, and nonoperative management using escharotic agents in three. Herniorrhaphy was performed at 2 months to 15 years of age. Fascial closure was achieved in a single procedure in 15 patients (65%) and with multiple operations involving prosthetic material in five patients (22%). Three patients (13%) had prosthetic material left in their defects without sequelae. There was no operative mortality from interval herniorrhaphy, and complications were minor. We conclude that conversion of a gastroschisis or an omphalocele to a ventral hernia is strategically useful in an infant with a giant defect, prosthetic silo failure, or when associated anomalies preclude early definitive surgery.