DOI: https://doi.org/10.36347/sjmcr.2026.v14i06.055

Pages: 1556-1562

Portal cavernoma cholangiopathy represents a rare biliary complication of chronic extrahepatic portal vein obstruction. Although radiological biliary abnormalities are frequently detected in patients with portal cavernoma, symptomatic disease remains uncommon and usually occurs after years of evolution. We report the case of a 19-year-old female with chronic portal cavernoma secondary to neonatal portal vein thrombosis related to umbilical catheterization, complicated by portal hypertension and recurrent upper gastrointestinal bleeding. She presented with progressive cholestatic jaundice without abdominal pain or infectious syndrome. Imaging demonstrated severe biliary dilatation associated with choledocholithiasis, intrahepatic lithiasis, peri-cholédocien venous collaterals, and massive splenomegaly in the setting of chronic portal cavernoma. Endoscopic retrograde cholangiopancreatography (ERCP) allowed temporary biliary drainage; however, recurrent cholangitis occurred despite stenting, requiring repeat ERCP. Due to persistent biliary compression and advanced portal hypertension, the patient ultimately underwent staged surgical management with cholecystectomy, roux-en-y hepaticojejunostomy, and mesenterico-caval shunt. This case highlights the progressive nature of portal cavernoma cholangiopathy, the role of multimodal imaging in diagnosis, and the importance of multidisciplinary management in advanced symptomatic forms.