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From question on page 1272

The magnetic resonance cholangiopancreatography scan showed hepatic fibrosis, multiple renal cysts (fig 1A), and multiple ectatic, dilated, irregular intrahepatic biliary ducts (fig 1B). The unifying diagnosis was that of Caroli’s disease with autosomal recessive polycystic kidney disease (ARPKD) and congenital hepatic fibrosis. The patient has not attended for upper gastrointestinal endoscopy to establish the presence of oesophageal varices.

Also known as congenital cystic dilatation of the intrahepatic biliary tree, Caroli’s disease is rare. In most cases inheritance is autosomal recessive. Two types have been described: type I, usually affecting a single lobe, and type II, associated with hepatic fibrosis. Other associations include ARPKD, medullary sponge kidney, and medullary cystic disease. Clinical findings are recurrent right upper quadrant pain, pyrexia, and sepsis. Type II disease presents with signs and symptoms of portal hypertension. Complications include recurrent cholangitis, biliary calculi, and an increased risk of cholangiocarcinoma. Treatment options include antibiotics, endoscopy for evidence of varices, lobectomy, and liver transplantation.

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