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Related Experiment Video

Updated: Nov 8, 2025

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Diffuse Versus Localized Caroli Disease: A Comparative MRCP Study.

Maïté Lewin1,2, Christophe Desterke2,3, Catherine Guettier2,4

  • 1Service de Radiologie, AP-HP Hôpital Paul Brousse, 12-14 Ave Paul Vaillant Couturier, 94800 Villejuif, France.

AJR. American Journal of Roentgenology
|April 21, 2021
PubMed
Summary
This summary is machine-generated.

Magnetic resonance cholangiopancreatography (MRCP) can diagnose Caroli disease (CD) by identifying diffuse intrahepatic bile duct dilatation with saccular and fusiform features. Localized findings may indicate primary intrahepatic lithiasis.

Keywords:
Caroli diseaseMRIbile ductscongenital lithiasis

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Area of Science:

  • Hepatobiliary imaging
  • Gastroenterology
  • Radiology

Background:

  • Caroli disease (CD) is a rare congenital disorder characterized by saccular dilatation of intrahepatic bile ducts.
  • Distinguishing between diffuse and localized forms of CD is crucial for prognosis and management.

Purpose of the Study:

  • To evaluate the Magnetic Resonance Cholangiopancreatography (MRCP) features differentiating diffuse and localized Caroli disease.
  • To establish diagnostic criteria for Caroli disease using MRCP findings.

Main Methods:

  • A multicenter retrospective study identified 66 patients diagnosed with diffuse or localized Caroli disease between 2000 and 2019.
  • Patients underwent MRCP, and imaging findings were correlated with histopathology where available.
  • Statistical analyses, including chi-square tests, t-tests, and Kaplan-Meier survival analysis, were used for comparison.

Main Results:

  • Diffuse Caroli disease (n=45) showed characteristic IHBD dilatation (saccular/fusiform), a "central dot" sign, and peripheral "funnel-shaped" signs, often associated with congenital hepatic fibrosis.
  • Localized disease (n=21) presented with intrahepatic biliary calculi in all cases and left liver atrophy in 86% of patients.
  • Overall survival was significantly lower in patients with diffuse Caroli disease compared to localized disease (p=0.03).

Conclusions:

  • Diffuse intrahepatic bile duct dilatation with saccular and fusiform features, along with the peripheral "funnel-shaped" sign on MRCP, are key diagnostic indicators for Caroli disease.
  • Localized intrahepatic bile duct dilatation on MRCP may primarily suggest primary intrahepatic lithiasis rather than Caroli disease.