Caroli's Disease as a Cause of Chronic Epigastric Abdominal Pain: Two Case Reports and a Brief Review of the Literature

Abstract

Caroli's disease is a very rare congenital malformation, currently included in cystic diseases of the biliary tract, and is characterized by ectasia and dilatation of the intrahepatic bile ducts. Two clinical entities can be distinguished, Caroli's disease in which congenital hepatic impairment is limited to cystic dilatation and Caroli's syndrome in which congenital hepatic fibrosis coexists. We present two cases of atypical presentations of Caroli's disease. Case one was a 76-year-old man who was referred to our hospital for chronic non-remitting epigastric pain prior to diagnosis. Magnetic resonance cholangiopancreatography (MRCP) was performed, which revealed findings consistent with Caroli's disease. Laboratory investigation disclosed a raised α-fetoprotein. Left hepatectomy was performed due to suspected cholangiocarcinoma. Morphological findings were compatible with Caroli's disease and no evidence of malignancy was found. Case two was a 47-year-old man who presented with chronic epigastric pain and generalized abdominal discomfort. MRCP revealed findings compatible with Caroli's disease. The patient was discharged with ursodeoxycholic acid treatment and was later admitted twice due to inaugural episodes of cholangitis that were medically managed. Bisegmentectomies II and III were performed for suspected neoplasia after a gradual rise in α-fetoprotein and CA19-9 values were noted during follow-up. The surgical specimen confirmed Caroli's disease and there was no evidence of malignancy. Postoperative periods for both patients were favorable, and they remain asymptomatic and well to date.

Keywords: abdominal pain; bile ducts; caroli's disease; caroli's syndrome; cholangiocarcinoma; cholangitis; hepatectomy; polycystic kidney disease; todani; α-fetoprotein.

Figure 1. Magnetic resonance cholangiopancreatography showing (A) saccular dilatation of the left biliary tree (arrow) and (B) biliary dilatation in the left hepatic lobe (arrow).

Figure 2. Surgical specimen showing cysts and gallstones.

Figure 3. Magnetic resonance cholangiopancreatography showing intrahepatic biliary ectasia (arrow).

Figure 4. Magnetic resonance cholangiopancreatography showing lithiasis of the main bile duct (arrow).