Hepatolithiasis – how we dealt

Abstract

Presence of bile duct stones within the intrahepatic bile ducts, proximal to confluence of the right and left hepatic ducts is known as hepatolithiasis. Rare disease in western world but seen in East Asian Countries and Pacific. The disease can occur with or without concomitant presence of cholelithiasis or choledocholithiasis. Two subjects were admitted from surgery out-patient department, Dr DY Patil Medical College, Pune. A 15-year-old male with progressive Jaundice since 6 months and pain in abdomen. On investigations - he was found to be a beta thalassemia, with USG and MRCP warranting multiple intrahepatic stones, proximal and distal CBD calculus. Another patient was a 18-year-old male from same family presented with similar complains. ERCP guided CBD stone extraction was tried but failed. Selective CBD cannulation done on 4th attempt. But still CBD clearance could not be achieved. Decision to go for CBD exploration and cholecystectomy. Intra operatively complete clearance of stones in both intrahepatic ducts, confluence, proximal and distal CBD. Ampullary stone not cleared so Roux en Y hepaticojejunostomy was done. Post-surgery uneventful, follow up no jaundice, MRCP after 4 weeks no residual stone. Two years follow up - no evidence of new stones in Intrahepatic or extrahepatic biliary system. Hepatolithiasis a rare disease with chances of recurrence, the newer treatments available are management via percutaneous approaches and newer modalities for obstructive jaundice in young. PTCSL, POCSL for intra hepatic stones and transduodenal sphincteroplasty for multiple recurrent CBD stones. As these treatments are not available we were forced to operate.