Treatment of Portal Biliopathy-Related Bile Duct Stricture Using a Two-Step Approach: Proximal Splenorenal Shunt and...

Portal biliopathy, also referred to as portal cavernoma cholangiopathy, is a biliary complication of extrahepatic portal vein obstruction caused by cavernomatous transformation of the portal vein and resulting biliary compression. Although biliary abnormalities are common in long)standing portal hypertension, only a minority of patients become symptomatic and require intervention. Herein, we present a 13)year)old patient with long)standing extrahepatic portal vein obstruction who developed pruritus, jaundice, and a distal common bile duct stricture with proximal biliary dilatation secondary to portal cavernoma compression. Despite multiple endoscopic interventions, including biliary stenting, the clinical course was complicated by persistent biliary obstruction and procedure)related bleeding. Proximal splenorenal shunt surgery achieved effective portal decompression and resolution of esophageal varices; however, biliary abnormalities persisted during follow)up. Definitive biliary reconstruction with Roux-en-Y hepaticojejunostomy subsequently resulted in complete clinical and biochemical remission without recurrence during long)term follow)up. This case highlights the importance of an individualized staged management strategy in pediatric portal biliopathy, in which portal decompression may facilitate safer biliary reconstruction, although definitive biliary)enteric reconstruction may still be required in selected patients.