Hepatobiliary and pancreatic surgery: Caroli disease: practice question

A General Surgery question in the style of the NEET PG paper. Answer it first, then read why the right option is right and why each of the others is wrong.

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General Surgery · Hepatobiliary and pancreatic surgery: Caroli disease

Sample item

A 20-year-old has recurrent episodes of fever, jaundice and right upper quadrant pain. MRCP shows saccular dilatations of the intrahepatic bile ducts that communicate with the biliary tree, and the kidneys show medullary sponge-like change. What is the diagnosis?

Original practice item, not a recalled exam question. Nothing here is recorded.

The answer and the reasoning

D. Caroli disease

Caroli disease is a congenital communicating cystic dilatation of the intrahepatic bile ducts (Todani type V) associated with ductal plate malformations such as medullary sponge kidney and recessive polycystic kidney disease. Stasis causes cholangitis, stones and a risk of cholangiocarcinoma.

Option by option

  • A. Type I choledochal cyst

    A type I choledochal cyst is a fusiform dilatation of the extrahepatic bile duct, not the intrahepatic ducts.

  • B. Recurrent pyogenic cholangitis

    Recurrent pyogenic cholangitis causes intrahepatic pigment stones and duct dilatation, mainly in East Asian patients.

  • C. Primary sclerosing cholangitis

    Primary sclerosing cholangitis gives multifocal strictures and beading, not saccular dilatations.

  • D. Caroli disease (correct)

    Correct. Communicating saccular intrahepatic duct dilatation with a renal cystic disease is Caroli disease.

Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.

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Questions about this General Surgery question

What is the answer to this General Surgery question?

The best answer is D, Caroli disease. Caroli disease is a congenital communicating cystic dilatation of the intrahepatic bile ducts (Todani type V) associated with ductal plate malformations such as medullary sponge kidney and recessive polycystic kidney disease.

Why is "Type I choledochal cyst" not the answer?

A type I choledochal cyst is a fusiform dilatation of the extrahepatic bile duct, not the intrahepatic ducts.

Why is "Recurrent pyogenic cholangitis" not the answer?

Recurrent pyogenic cholangitis causes intrahepatic pigment stones and duct dilatation, mainly in East Asian patients.

Why is "Primary sclerosing cholangitis" not the answer?

Primary sclerosing cholangitis gives multifocal strictures and beading, not saccular dilatations.

What concept does this question test?

It tests Hepatobiliary and pancreatic surgery: Caroli disease in General Surgery. Stasis causes cholangitis, stones and a risk of cholangiocarcinoma.

How hard is this question?

We tag it as a multi-step question: it asks you to combine more than one idea. The tag is our own editorial judgement, not an official difficulty rating, so read the reason for every option, not only the answer.

How should I study Hepatobiliary and pancreatic surgery: Caroli disease?

Learn classic signs and eponyms alongside what they diagnose. Then use the flashcards and the other General Surgery questions on this site to test what stuck.

Is this a real NEET PG question?

No. It is an original practice item (editorial status: sample, version 1). NextPrep does not publish recalled or leaked questions, and independent faculty review of this item has not been completed yet, so confirm important facts in a standard textbook.