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A 12-year-old boy with a history of Caroli syndrome diagnosed via liver biopsy was admitted to the hospital for gastrointestinal bleeding. CT and MRI were performed for liver transplantation evaluation, and revealed diffuse intrahepatic bile duct dilatation (Figure, Movie). The patient underwent a successful liver transplantation.
Images in a 12-year-old boy with Caroli syndrome. (A) Axial and (B) coronal contrast-enhanced CT images and (C) axial and (D) coronal T2-weighted MRI scans reveal irregular cystic dilatation of the intrahepatic bile ducts. The dilatated bile ducts encircle the portal vein, creating a “central dot sign” (arrows in A and C), and connect with normal small bile ducts (arrows in B and D). (E) Coronal three-dimensional volume-rendered image from CT shows the degree and scope of intrahepatic bile duct dilatation more intuitively and stereoscopically.
Movie: The continuous display of coronal and axial planes through cinematic rendering offers a more intuitive visualization of liver enlargement and significant intrahepatic bile duct expansion, clearly illustrating the extent and severity of bile duct dilatation.
Caroli syndrome, also known as type 2 Caroli disease, is defined by intrahepatic ductal dilatation and congenital hepatic fibrosis, and is classified as type V in the Todani classification of congenital cystic dilatation of the bile duct (1–3). Caroli syndrome follows an autosomal recessive inheritance pattern. Bile duct dilatation often leads to cholestasis and cholelithiasis (2,3). As liver fibrosis advances, it can progress to end-stage liver disease, characterized by portal hypertension and associated complications, ultimately necessitating liver transplantation.
Disclosures of conflicts of interest: J.W. No relevant relationships. X.L. No relevant relationships.References
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Article History
Received: Sept 23 2024Revision requested: Oct 22 2024
Revision received: Oct 30 2024
Accepted: Nov 4 2024
Published online: Mar 18 2025
