The hepatic portoenterostomy (HPE) is the accepted initial operation for biliary reconstruction for biliary atresia, but in a select group of patients with patent distal extrahepatic bile ducts (PDEBD), a hepatic portocholecystostomy (HPC) may also be considered. A10 year old boy presented with sudden onset of jaundice following a successful HPC at 2 months of age. Radiographic evaluation revealed excretion into a distended gallbladder with distal biliary obstruction. He underwent a successful Roux-en-Y cholecystojejunostomy and remains jaundice-free two years later. Cholecystojejunostomy is an effective salvage operation for patients who develop late distal biliary obstruction after an HPC.
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