Monolobar Caroli's disease in an adult. Case report

Abstract

Caroli's disease is the dilatation of the segmental intrahepatic bile ducts which generally presents in a diffuse form, but may occasionally involve only a single lobe, commonly the left one. We report the case of a 64-year-old male who presented with a clinical picture of obstructive jaundice, with Caroli's disease in segments II and III of the liver. Preoperative diagnosis was made using abdominal ultrasound and computed tomography scan, confirmed by endoscopic retrograde cholangiopancreatography. The treatment used was segmentectomy II and III (left lobectomy--Couinaud's classification) of the liver. Macroscopic examination of the resected specimen revealed cystic dilatation of the intrahepatic bile ducts and intrahepatic lithiasis. Histologically there was no evidence of malignancy. Liver resection is the treatment of choice for Caroli's disease confined to a single lobe or segment, eliminating the potential for cholangitis, lithiasis and carcinoma.