Caroli Disease: When Your Ducts Aren't in a Row

Roberto R. González, MD; William H. Brown, MD; David L. Carr-Locke, MD


May 02, 2012

Case Presentation: Epigastric Pain and Fever

A 53-year-old white woman was seen in the inpatient setting for epigastric pain and fever.


She had a history of congenital adrenal hyperplasia, for which she took prednisone 5 mg orally every day. The pain and fever began 2 days before admission.

She denied nausea, vomiting, diarrhea, jaundice (icterus), dark urine, or clay-colored stools. Upon further questioning, she mentioned that she had had chronic, intermittent right upper quadrant and epigastric pain for more than 20 years, with worsening severity. Ten years before this presentation, she had undergone cholecystectomy for chronic cholecystitis, but her pain did not resolve postoperatively. She denied use of alcohol, tobacco products, illegal drugs, supplements, over-the-counter medications, or herbal remedies.

Clinical Evaluation

On examination, the patient was in acute pain. Her sclerae were anicteric. Active bowel sounds were auscultated, and her abdomen was soft. She had severe tenderness on palpation of the right upper quadrant and epigastrium. Rebound tenderness and guarding were absent. No organomegaly was identified.

Laboratory evaluation revealed that her liver function tests, basic metabolic panel, international normalized ratio, complete blood count, and amylase and lipase values were all within normal limits. Blood cultures done on admission were positive for gram-negative bacteria.

Abdominal ultrasonography was performed, revealing a common bile duct 1.2 cm in diameter, no evidence of obstructing calculi, normal-appearing hepatic parenchyma, and a 3-cm cystic structure in the left lobe of the liver. Endoscopic ultrasonography (EUS) confirmed a dilated common bile duct, with no obstructing calculi and no evidence of tumor or mass. The pancreatic duct was normal. See Figures 1a-1c.

Figures 1a-1c. Results of endoscopic ultrasonography. 1a. Normal pancreatic duct. 1b. Dilated proximal common bile duct. 1c. Dilated distal common bile duct.

Endoscopic retrograde cholangiopancreatography (ERCP) revealed a stenotic ampulla, and sphincterotomy was performed. The intrahepatic biliary tree appeared mildly dilated proximally on fluoroscopy but did not show additional significant intrahepatic abnormality (Figure 2).

Figure 2. ERCP revealing mildly dilated proximal intrahepatic bile ducts and small cystic structures in the liver parenchyma filled with contrast (arrow).

Magnetic resonance cholangiopancreatography (MRCP) was performed and revealed numerous cystic structures throughout the hepatic parenchyma in communication with the biliary tree, including the 3-cm cyst seen on previous ultrasonography (Figure 3). This confirmed the diagnosis of Caroli disease.

Figure 3. MRCP showing numerous cystic structures in communication with the biliary tree, including the 3-cm cyst seen on ultrasonography (arrow).

The patient was given intravenous antibiotics for bacteremia and was discharged 1 week later on ursodeoxycholic acid and oral pain medication. She is currently being considered for liver transplantation.


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