Clin Endosc.  2017 Jul;50(4):400-403. 10.5946/ce.2016.150.

Diffuse-Type Caroli Disease with Characteristic Central Dot Sign Complicated by Multiple Intrahepatic and Common Bile Duct Stones

• Hwang MJ
• Kim TN

• KMID: 2389247
• DOI: http://doi.org/10.5946/ce.2016.150

Abstract

Caroli disease (CD) is a rare congenital malformation of the liver characterized by non-obstructive, segmental, cystic dilatation of the intrahepatic bile ducts (IHDs). The clinical course is usually asymptomatic for the first 5-20 years, and symptoms may seldom occur throughout the patient's life. Bile stagnation leads to recurrent episodes of cholangitis, stone formation, or liver abscesses, and biliary cirrhosis usually occurs years later. Here we report on a 42-year-old man diagnosed with diffuse-type CD with a characteristic central dot sign, who had multiple intrahepatic and common bile duct (CBD) stones. CBD stones were treated successfully with endoscopic retrograde cholangiopancreatography (ERCP).

Keyword

Caroli disease; Choledocholithiasis; Central dot sign; Endoscopic retrograde cholangiopancreatography

MeSH Terms

Adult
Bile
Bile Ducts, Intrahepatic
Caroli Disease*
Cholangiopancreatography, Endoscopic Retrograde
Cholangitis
Choledocholithiasis
Common Bile Duct*
Dilatation
Humans
Liver
Liver Abscess
Liver Cirrhosis, Biliary

Figure

• 

  Fig. 1. Abdominal computed tomography. Multiple dilated intrahepatic ducts with tiny dots of strong contrast are seen in the liver (arrows). These represent portal radicles and constitute the characteristic central dot sign for Caroli disease.

• 

  Fig. 2. Magnetic resonance cholangiography. (A) Bile duct ectasia and irregular cystic dilation of the large proximal intrahepatic bile duct (IHD) with a normal common bile duct are noted. Multiple intrahepatic calculi were also demonstrated. (B) T1-weighted image again reveals central dot signs (arrows), which are enhancing dots within the dilated IHD, representing portal radicles (B).

• 

  Fig. 3. Endoscopic retrograde cholangiopancreatography. (A) Multiple small cystic formations, in intimate anatomical association with biliary branches were demonstrated. Minimum amount of contrast dye was injected to prevent cholangitis. (B) Black pigment stone was removed by basket method.

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