WOFAPS 2025 8th World Congress of Pediatric Surgery

Nedra Zouabi, Sabrine Ben Youssef, Syrine Laribi, Sami Sfar, Amine Ksia, Mongi Mekki, Mohsen Belghith, Lassaad Sahnoun

Pediatric surgery department, Fattouma Bourguiba Hospital, Monastir, Tunisia

Purpose: Portal cavernoma is the consequence of chronic occlusion of the extrahepatic portal venous system, characterized by the formation of a network of dilated veins through which hepatopetal portal blood flows. In children, it represents a major cause of portal hypertension (PHT). Umbilical catheterization during the neonatal period is a common etiology of portal cavernoma. This study aimed to report our clinical experience in the diagnosis and management of portal cavernoma.

Methods: This is a retrospective study conducted from 2002 to 2025, including 21 children diagnosed with portal cavernoma who underwent surgical management in our department.

Results: Twenty-one cases were included. The mean age was 7.5 years (range: 2 to 14 years). The main reason for hospitalization was signs of portal hypertension (PHT), observed in 15 cases, followed by cholestatic jaundice in 2 cases and pancytopenia in 4 cases. A history of umbilical catheterization was noted in 66.7% of the cases. Abdominal Doppler ultrasound confirmed PHT in all patients. Endoscopic examination revealed the presence of esophageal varices in every case. The most common laboratory abnormality was pancytopenia. A thrombophilia workup was ordered for all patients but was completed in only 14; it was normal in 13, while the 14th patient showed decreased protein S and C levels along with a heterozygous Factor V mutation. Portography was performed in all patients who underwent surgical shunting, with a mesenterico-Rex shunt in 13 cases, a mesenterico-caval anastomosis in 6 cases, and a splenorenal anastomosis in 2 cases. Postoperative outcomes were uneventful, and patients are being followed regularly in outpatient clinic.

Conclusion: Portal cavernoma is a debilitating condition in children that requires a multidisciplinary approach. The etiological workup should include screening for prothrombotic disorders as well as investigation of a history of umbilical catheterization during the neonatal period.