WOFAPS 2025 8th World Congress of Pediatric Surgery

Zeinab Badri Faris, Musab Mohammed, Esam Taha, Abdelrahman Elnour

Sudan Medical Specialization Board

Background: Hepatopulmonary fusion (HPF) is a rare congenital anomaly most often associated with right-sided congenital diaphragmatic hernia (CDH). It involves herniation of the liver into the thoracic cavity with partial or complete fusion to hypoplastic lung tissue. This fusion disrupts normal pulmonary development, impairs gas exchange, and contributes to pulmonary hypertension. Surgical management is particularly challenging due to the variable nature of the fusion, which may range from fibrovascular connections to true parenchymal fusion.

Objective: To describe the surgical management and outcomes of three HPF cases associated with right-sided CDH, emphasizing the fusion variants and the tailored operative strategies employed.

Results: All three cases were managed by a multidisciplinary team comprising pediatric and hepatobiliary surgeons. In Case 1, 60% of the liver was herniated and fused to a rudimentary hypoplastic right lung, with additional herniation of the transverse colon and jejunum. Case 2 involved 40% liver herniation fused to the lung, along with the right kidney and small bowel. In Case 3, complete fusion of the liver to the lung was noted. Postoperatively, Cases 1 and 2 required mechanical ventilation for 4 and 5 days, respectively, and nasogastric feeding was initiated on postoperative day 2. Both were discharged in stable condition and remain on follow-up. Case 3, however, succumbed to respiratory failure two hours after surgery.

Conclusion: HPF presents significant surgical challenges, and outcomes are closely tied to the degree of lung hypoplasia and fusion extent. Successful management requires individualized surgical approaches and close multidisciplinary collaboration, especially in resource-limited settings.