Caroli's disease with biliary sludge and calculus

Case contributed by Dr Muhammad Shoyab

Presentation

Painful abdominal lump for 6 months. Fever (100 °F) and jaundice for same duration. Nausea, dyspepsia, occasional dysuria. Tender hepatosplenomegaly. WBC 13000/mm3, S. bilirubin 2.5 mg/dL, ALT 60 IU/L, Alkaline phosphatase 100 IU/L. Urine RME : Yellowish color.

Patient Data

Age: 10 years
Gender: Male

Enlarged liver shadow

Flanks are full

Multiple, large, cystic structures in both lobes of liver and also near porta hepatis

Hepatosplenomegaly.

Large multiloculated cystic lesions in both lobes of liver, communicating with rest of biliary system, having same appearance as bile — diffuse intrahepatic biliary dilation.

Accompanied by intraductal septations, sludge and signal void calculi.

Central dot sign present.

Gall bladder distended. Common bile duct (CBD) not distended.

Hepatosplenomegaly.

Large multiloculated fluid density lesions in both lobes of liver — diffuse intrahepatic biliary dilation.

Gall bladder distended. Common bile duct (CBD) not distended.

Case Discussion

Caroli's disease is defined as congenital non-obstructive dilation of intrahepatic bile ducts.

It is considered by some experts as part of the spectrum of fibropolycystic liver diseases, due to its association with fibrocystic kidney diseases. Some authors also want to classify it as type-V under Todani's classification of choledochal cysts, due to anatomic and physiologic similarity.

Being a congenital entity with possible genetic relations, the disease usually presents in the 2nd or 3rd decade of life or even earlier, like the present case. The patient may present with the classic triad of abdominal pain, right hypochondriac mass and jaundice, or with a combination of related symptoms. Hepatic markers (bilirubin, ALT, alk phosphatase) may be normal or only slightly altered.

Ultrasonography is often the first imaging that provides direct clue to the diagnosis, since it clearly demonstrates the dilation of intrahepatic biliary channels, often with central dot sign. MRCP provides the additional benefit of detecting extra-luminal findings or to identify obstructed ducts. CT or MRI with contrast may be used to identify developing cholangiocarcinoma (10% risk).

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