Hepatopulmonary fusion associated with congenital diaphragmatic hernia: A case series

Hepatopulmonary Fusion (HPF) is an exceedingly rare anomaly associated with right sided congenital diaphragmatic hernia (CDH) and has a high mortality rate. Three neonates were cared for at our pediatric medical center for management of their CDH and coinciding HPF. Case 1 was a former term female who had a prenatal diagnosis of partial anomalous pulmonary venous return (PAPVR). She developed respiratory distress shortly after birth, which prompted further imaging. She underwent a computed tomography angiography (CTA) that showed a right CDH and abnormal perfusion of the superior aspect of the right hepatic lobe, suggestive of anomalous pulmonary venous return to the right portal system. HPF was suspected based on that finding. At 2 months old she underwent a successful separation of the fusion and a mesh repair of the CDH. The patient survived to the hospital discharge. Case 2 was a late preterm female with a prenatal diagnosis of a right CDH and images on prenatal echocardiogram suggestive of atypical venous return to the liver. HPF was diagnosed incidentally during the repair of the CDH. The fusion was separated with electrocautery and a stapler. The CDH defect was repaired with a mesh. The patient survived to the hospital discharge. Case 3 was a former term female who underwent repair of her right CDH on day of life 5. Her HPF was identified at the time of the CDH repair and was separated using electrocautery and a stapler. Her course was notable for refractory pulmonary hypertension with infectious complications, which eventually resulted in her demise during the index hospitalization. While HPF is typically encountered incidentally in the operating room during the repair of a right CDH, it should be suspected in patient who have vascular anomalies of the right hepatopulmonary region.