MEDICAL IMAGES

Caroli Syndrome
Jackson J. Liang, DO, and Patrick S. Kamath, MD

52-year-old man presented with fevers and right upper quadrant abdominal pain of 3 weeks duration.
He had experienced multiple prior episodes
of cholangitis and was taking rotating antibiotics to prevent attacks. Magnetic resonance
cholangiopancreatography (Figure 1) revealed
diffuse fusiform and saccular intrahepatic and
extrahepatic bile duct dilation, consistent with
Caroli disease. No abscesses or stones were visualized. Blood cultures grew Escherichia coli and
Enterococcus gallinarum. Symptoms improved after 2 weeks of intravenous antibiotic therapy.

Caroli disease is an autosomal recessive disorder characterized by intrahepatic bile duct

dilation and renal cysts (Figure 2). Our patient
had Caroli syndrome or complex, which
comprises Caroli disease plus congenital hepatic brosis, portal hypertension, and autosomal
recessive polycystic kidney disease. Recurrent
cholangitis, sepsis, and cholangiocarcinoma
may also develop. Variceal bleeding may occur
in patients with Caroli complex. Recurrent
cholangitis is preferably treated with antibiotics; orthotopic liver transplant may be required when antibiotics are ineffective.

FIGURE 1. Magnetic resonance cholangiopancreatogram (3-dimensional fast spin echo, without

gadolinium) demonstrating cystic dilation of the
intrahepatic and extrahepatic bile ducts.

FIGURE 2. Magnetic resonance image of the

abdomen (coronal view, fast spin echo, without
gadolinium) revealing cystic biliary dilation and
the presence of multiple bilateral renal cysts
(arrows).

Mayo Clin Proc. n June 2013;88(6):e59 n http://dx.doi.org/10.1016/j.mayocp.2013.01.027

www.mayoclinicproceedings.org n 2013 Mayo Foundation for Medical Education and Research

Downloaded from ClinicalKey.com at Universidad Francisco Marroquin August 03, 2016.

For personal use only. No other uses without permission. Copyright 2016. Elsevier Inc. All rights reserved.

From the Department of

Internal Medicine (J.J.L.)
and Division of Gastroenterology and Hepatology
(P.S.K.), Mayo Clinic,
Rochester, MN.

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