Unilobar Caroli's disease and its management in an 8-year-old girl
An 8-year-old girl was referred with a history of multiple episodes of epigastric pain, high-grade fever, and jaundice since 5 years of age. The episodes were associated with clay colored stools and high colored urine. Liver function tests were normal except for mildly raised serum alkaline phosphatase (Total bilirubin – 0.3 mg%, Serum glutamic oxaloacetic transaminase (SGOT)/Serum glutamic pyruvate transaminase (SGPT) – 47/34 IU/L, and Serum alkaline phosphatase – 996 IU/L). Ultrasonography revealed dilated intrahepatic biliary radicles (IHBR), dilated common bile duct (CBD) with multiple calculi in gall bladder. Magnetic resonance cholangiopancreatography showed atrophy of left lobe of liver, cystic dilatation of left lobar duct and IHBR, prominent right hepatic duct, multiple gall bladder calculi, dilatation of CBD (1 cm) with no evidence of calculus, and smooth narrowing of CBD in its distal part []. The pancreatic duct was normal with short common pancreatobiliary channel (0.8 cm).\nOn surgical exploration, the left lobe of liver was atrophied, fibrotic and multiple calculi could be palpated in the left lobe of liver. There was a clear demarcation between the healthy right lobe and the diseased left lobe of liver. The gall bladder was filled with multiple calculi. Right hepatic duct and CBD were dilated with no calculus. The left hepatic duct was significantly dilated with presence of multiple calculi. A stone of size 1 cm × 1 cm was present in a large diverticulum arising from left hepatic duct. Left hepatic artery was anomalous and originating from the left gastric artery. An accessory right hepatic artery originated from the superior mesenteric artery. Left hepatectomy was performed with a Roux-en-Y right hepaticojejunostomy. Histopathology of the resected specimen revealed huge, cystic dilation of large intrahepatic bile ducts, ulcerated duct lining, inspissated bile within the lumen of bile ducts, and chronic inflammatory cells.\nThe post-operative period was uneventful. She was put on ursodeoxycholic acid to prevent future episodes of primary hepatolithiasis and cholangitis[] and is asymptomatic at 6-month follow-up.