Caroli Disease: A Presentation of Acute Pancreatitis and Cholangitis

Abstract

Caroli disease is a rare congenital disorder resulting from the dilation of large intrahepatic bile ducts. Patients affected with Caroli disease are at increased risk of complications resulting from bile stasis and stone formation. We report the case of a 37-year-old woman with a past surgical history of cholecystectomy who presented to the emergency room with a chief complaint of abdominal pain and nausea. The pain was characteristic of acute pancreatitis but she was hemodynamically stable. Total bilirubin was 4.1 mg/dL with a direct fraction of 3.1 mg/dL, aspartate aminotransferase (AST) and alanine aminotransferase (ALT) were 850 IU/L and 1025 IU/L, respectively. Serum amylase and lipase were elevated at 581 IU/L and 1328 IU/L, respectively. CT scan of abdomen/pelvis without contrast showed common bile duct (CBD) measuring 1.6 cm with intrahepatic biliary system dilation and mild peripancreatic fat stranding. She was diagnosed with acute pancreatitis. On the second day, she developed a temperature of 99.6°F. Hepatitis immunity panel was negative for acute hepatitis. The patient was started on antibiotics (IV ciprofloxacin and metronidazole) for suspicion of acute cholangitis. Endoscopic retrograde cholangiopancreatography (ERCP) was done which showed mild dilated intrahepatic ducts and CBD dilation of 1.6 cm, and a choledochal cyst at CBD. Sphincterotomy was done and good bile drainage was reported. She was later discharged in a stable condition. Caroli disease affects males and females equally and most are diagnosed before the age of 30 years correlated with the onset of symptoms. By far, the most commonly reported symptom is acute cholangitis but pancreatitis occurs rarely. Recurrent bouts of infection lead to portal hypertension, fibrosis of the liver and ultimately end up with an orthotopic liver transplant (OLT). Regular follow-ups are important for disease surveillance and monitoring.

Keywords: caroli disease; cholangitis; congenital; ct; cystic disease; ercp; mrcp; pancreatitis; transplant; ursodeoxycholic acid.

Figure 1. Contrast-enhanced CT scan

Contrast-enhanced CT showing intrahepatic and extrahepatic bile duct dilations with a black arrow pointing out fibrovascular bundles. Photo from Levy AD, Rohrmann CA, Murakata LA, Lonergan GJ. Caroli's Disease: Radiologic Spectrum with Pathologic Correlation. American Journal of Roentgenology.