Congenital diaphragmatic hernia with hepatic heterotopia: A case series

Introduction Congenital diaphragmatic hernia (CDH) occurs in approximately 2.5 per 10,000 births; heterotopic liver tissue in the hernia sac is even more rare. Case 1 A female patient with prenatally diagnosed left-sided CDH (without herniation of the liver) was delivered at 37 weeks and underwent a primary surgical repair at two days of life. Intraoperatively, an anterior diaphragmatic defect was identified. A thin, long tail of hepatic tissue extended to the anterior diaphragmatic rim, where it was fused to the hernia sac. This was confirmed to be hepatic tissue. The patient recovered well and no hernia recurrence has been identified. Case 2 A female patient with prenatally diagnosed right-sided CDH (with herniation of liver) was delivered at 32 weeks 3 days and underwent a primary surgical repair at three days of life. A pleuroperitoneal membrane was superior to the liver and excised. On pathology, the membrane was confirmed as hernia sac with hepatic heterotopia. The patient was last seen at 6 months of age and is doing well. Conclusion Hepatic heterotopia may be discovered intraoperatively but is often only confirmed upon pathologic examination of the resected CDH specimen. Hepatic tissue within the sac of a CDH does not seem to have any clinical consequences in the short term.