Abstract Introduction Right congenital diaphragmatic hernia (CDH) with hepatopulmonary fusion is a rare subtype in which the herniated liver becomes abnormally fused to the right lung parenchyma through a fibrovascular connection. This creates a complex and high-risk clinical scenario that demands a multidisciplinary approach and meticulous preoperative preparation to optimize outcomes. In this case report, we present four cases of children born with right congenital diaphragmatic hernia with hepatopulmonary fusion, one of which did not require surgical correction. Case 1 A term female infant presented with severe respiratory distress immediately after birth, requiring intubation and NICU admission. Imaging revealed a right CDH, and surgical repair on day four showed fusion between the right lung and liver. The diaphragmatic defect was closed around the fused portion since separation was unsafe. Postoperative imaging identified venous anomalies, including an absent infradiaphragmatic IVC and a prominent hemi-azygos vein. She was extubated at 24 days and discharged at six and a half weeks. During infancy, she had transient respiratory distress and reflux that resolved. At age 12, she remains well without chronic symptoms, though imaging shows persistent right lung hypoplasia and herniation of abdominal contents causing right atrial compression. Pulmonary function testing demonstrates mild restrictive and obstructive defects. Case 2 A healthy term male presented at age four for coin ingestion, with incidental discovery of right CDH and hepatopulmonary fusion with pulmonary sequestration. He underwent coil embolization of collateral arteries to correct a left-to-right shunt. Spirometry showed a mild restrictive pattern. He remains healthy, with good exercise tolerance and no recurrent respiratory infections, though chronic right lower lobe atelectasis is suspected. Case 3 A preterm male (34 + 5 weeks) with Scimitar syndrome and multiple cardiac anomalies had right CDH repair at four days old, during which partial separation of hepatopulmonary fusion was achieved. He required prolonged respiratory support and was discharged on nocturnal CPAP. Currently, he is thriving with good activity, no chronic respiratory symptoms, and well-controlled reflux. Case 4 A full-term male developed bronchiolitis at six months, during which imaging revealed possible hepatopulmonary fusion or sequestration. He improved with bronchodilators and steroids. At seven months, he showed mild exertional dyspnea but normal development. Surgical intervention was deferred in favor of close monitoring. Conclusion These cases highlight that right CDH with hepatopulmonary fusion presents variably—from severe neonatal distress to incidental discovery in later childhood—and requires individualized, multidisciplinary management. This abstract is funded by: None
S A Al Baroudi (Fri,) studied this question.