Purpose or Case Report: Hepatopulmonary fusion (HPF) is a very rare type of right-sided congenital diaphragmatic hernia (CDH) with a reported prevalence of 3 in 1000 right-sided CDH cases. By June 2020, less than 50 cases have been reported in the literature. Out of 147 patients with right CDH managed at our institution, we found only one case (0.68%) of HPF, which is presented here.

HPF is typically not identified preoperatively, and the presence of HPF changes the operative management. It has been reported that a large right CDH without leftward cardiomediastinal shift and atelectatic lung adherent to the liver are imaging features of HPF.

This patient was a full-term, newborn girl with a normal prenatal course who developed respiratory distress immediately after birth. Chest radiograph at birth demonstrated a right CDH. On day four of life, the patient underwent surgical repair of right CDH. During the procedure, hepatopulmonary fusion involving the cephalad portion of the liver was discovered. The lung and liver could not be separated safely so the diaphragmatic defect was closed around the portion of the liver that extended into the thorax. Postoperative CT angiography of the chest and abdomen demonstrated no abnormal vascular communication between the lungs and liver; however, anomalies of systemic veins were identified. Follow-up chest radiograph at 9 months of age demonstrated a large right CDH that did not include bowel or right-sided colon and was without leftward mediastinal deviation. Concurrent contrast-enhanced CT images of the thorax at this time confirm the right CDH with herniated liver occupying most of the right hemothorax and expected rightward traction of the liver vasculature. However, no significant leftward mediastinal deviation was noted. A small amount of atelectatic lung overlying the liver was also noted, another imaging sign expected with HPF. Last follow-up at age 4, patient was thriving with normal growth and development.
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