EN
TÜRKÇE
Poster - 68
RIGHT CONGENITAL DIAPHRAGMATIC HERNIA (CDH) ASSOCIATED WITH HEPATO-PULMONARY FUSION (HPF): CASE PRESENTATION AND REVIEW OF LITERATURE
Ahmed Azzam 1, Eniola Folaranmi 2, Ceri Jones 2
1 Cairo University Faculty of Medicine, Department of Pediatric Surgery, Cairo University Specialized Pediatric Hospital, Egypt
2 Cardiff and Vale UHB
Introduction:
Hepatopulmonary fusion (HPF) is an exceptionally rare congenital anomaly associated with right-sided congenital diaphragmatic hernia (CDH). It is characterized by fusion between the herniated liver and the hypoplastic right lung, presenting significant diagnostic and surgical challenges. Because HPF is rarely identified preoperatively, surgeons should maintain a high index of suspicion in patients with right-sided CDH.
Case Presentation:
A 3-day-old full-term female neonate presented with respiratory distress. Chest radiography demonstrated right-sided CDH with herniation of abdominal viscera into the thoracic cavity, while echocardiography showed normal cardiac anatomy and pulmonary pressures. Thoracoscopic exploration revealed a hypoplastic right lung and complete intrathoracic herniation of the liver, necessitating conversion to an open approach. Following reduction of the bowel, dense adhesions between the liver, hernia sac, and lung parenchyma were encountered, confirming HPF. Limited dissection resulted in a minor lung parenchymal injury, which was repaired primarily. Because complete separation of the fused liver and lung was considered unsafe due to significant vascular communications, the fused tissue was preserved. The diaphragmatic defect was reconstructed using a Gore-Tex mesh, and a chest drain was inserted. The postoperative recovery was uneventful. Computed tomography angiography performed three weeks later confirmed abnormal vascular connections between the liver and lung, and histopathology of the excised hernia sac demonstrated fibro-adipose and pulmonary tissue.
Conclusions:
HPF should be considered in all cases of right-sided CDH, particularly when complete liver herniation is present. Preoperative imaging may increase diagnostic suspicion; however, the diagnosis is frequently established intraoperatively. Careful operative planning and individualized surgical management are essential to minimize complications and optimize patient outcomes in this rare congenital condition.