UCLA

The perinatal management of newborns with giant omphaloceles requires careful suspicion concerning unexpected associated malformations. We describe a newborn with a combination of incomplete Pentalogy of Cantrell consisting of giant omphalocele, anterior congenital diaphragmatic hernia (CDH) and pericardial absence complicated by a hepatic rapidly involuting congenital hemangioma (RICH). A giant omphalocele was detected prenatally. Postnatally, staged closure of the omphalocele was planned. A mass of the liver was noted and (mis-)taken for a hematoma. In the further course, the baby developed cardiorespiratory insufficiency due to a central CDH which was excised. Subsequently, staged closure of the omphalocele became impossible. Specific workup revealed a mass in the left liver lobe. The mass was resected and the abdominal wall defect repaired. Histology confirmed the diagnosis of an RICH. The surgical treatment of newborns with giant omphaloceles requires a multidisciplinary neonatal support and an elaborate pediatric surgical armamentarium to cope with additional malformations.