Caroli’s disease associated with biliary papillomatosis and cholangiolithiasis
03 July 2018 (eFirst)
A 52-year-old man presented with constant right upper quadrant pain and repeated episodes of fever for 6 months, accompanied with cutaneous and scleral icterus. Laboratory tests suggested a rise in bilirubin. A computed tomography (CT) scan showed obvious dilatation of the intra- and extrahepatic bile ducts, and patchy high density shadows in the intrahepatic bile ducts that were thought to be stones ([Fig. 1]).
Duodenoscopy showed that the orifice of the duodenal papilla was shaped like the mouth of a fish and a large amount of mucus was expelled ([Fig. 2]). T-tube cholangiography confirmed the dilated hepatic bile ducts ([Fig. 3]). The patient then underwent biliary exploration. During surgery, a large amount of mucus could be seen in the bile duct, and the T-tube was indwelling in the common bile duct. After the surgery, the patient still had abdominal pain, the T-tube drainage was problematic with mucus discharge observed, and there was no significant regression of the jaundice.
The patient underwent cholangioscopy 6 weeks after surgery, which showed diffuse and labyrinth-like dilatation of the intrahepatic bile ducts ([Fig. 4 a]), which was thought to be Caroli’s disease with cholangiolithiasis. Diffuse papillary protrusions could be seen on the intrahepatic bile duct wall, with a large amount of mucus ([Fig. 4 b]). A biopsy was taken, which subsequently confirmed the diagnosis of biliary papillomatosis ([Fig. 5]). Most of the stones were removed under cholangioscopy ([Video 1]). After stone extraction, the patient’s abdominal pain was relieved and the degree of jaundice gradually decreased.
Video 1 Cholangioscopy showing diffuse dilatation of the intrahepatic bile ducts, with a large amount of mucus and cholangiolithiasis. A biopsy is taken and stones are extracted.
Caroli’s disease is a rare autosomal recessive disorder, which leads to intrahepatic stones and recurrent cholangitis when progressive. It is characterized by a biliary abnormality consisting of segmental saccular dilatations of the large intrahepatic bile ducts  . Biliary papillomatosis is also a rare disease entity with a strong malignant potential, which is characterized by multiple papillary adenomas involving both the intrahepatic and extrahepatic biliary tree . This patient had a wide range of lesions due to papillomas, which could not be completely resected. The only complete and effective treatment for this condition is liver transplantation .
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* Chi Yuan and Jianrong Liu are considered co-first authors of this work.
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