Background: Congenital diaphragmatic hernia is defined as patent pleuroperitoneal canal through the diaphragm. CDH can be divided into two types, anterimedial retrosternal hernia (Morgagni hernia), and the posterolateral hernia (Bochdalek hernia). Right-sided CDH that is associated with hepatopulmonary fusion is considered as rare congenital anomaly. In the literature review, there is only 32 reported cases of hepatopulmonary fusion. The prevalence of hepatopulmonary fusion in right sided congenital diaphragmatic hernia is 3 in 1000 infants.

Case summary: A 5 days old baby boy, was born full term to a 41-year-old mother. He was delivered by spontaneous vaginal delivery with birth weight of 3.4 kg and the (APGAR) scores was 7 and 9. Immediately after delivery, he developed respiratory distress and admitted to neonatal intensive care unit. The infant was treated with nasal oxygenation, intravenous fluids, and parenteral antibiotics. Then, chest X-ray was done, showing right-sided CDH. Computed tomography was done to differentiate between CDH and eventration. Chest fluoroscopy was performed and suspicion raised between right-sided CDH and right eventration. After patient stabilization, right thoracoscopy was performed during which the right lung found to be adherent to the liver. Thoracoscopy was carried out and there were many adhesions. So, it was decided to proceed to thoracotomy to separate the liver from lung, which was technically challenging. There was also shared vasculature between the liver and the lung in addition to the fusion. The atelectatic part of the lung was dissected with the rim of the liver in order to be able to repair CDH. Gore-Tex mesh was used in order to close the huge diaphragmatic defect.

Conclusion: The detection of the right hepatopulmonary fusion that is combined with right-sided CDH was considered as novel intraoperative finding in the presence of Bochdalek hernia