Peribiliary cyst

Last revised by Yahya Baba on 19 Jan 2023

Peribiliary cysts occur in the setting of chronic liver disease where it is a rare, benign, and often asymptomatic disorder. They consist of cystic formations around the intrahepatic biliary ducts, primarily in a hilar distribution. Unlike choledochal cysts (for example in Caroli disease), peribiliary cysts do not communicate with the biliary tree

Peribiliary cysts are very rare and occur in the setting of severe chronic liver diseaseportal hypertension 1, and in the setting of autosomal dominant polycystic kidney disease (ADPKD).

These asymptomatic cysts are diagnosed incidentally in the severe chronic liver disease cohort who have screening tests for hepatoma formation. Rare cases of mass effect have been described resulting in obstructive jaundice 2,4.

Histologically, peribiliary cysts have benign-appearing columnar or cuboidal epithelial cell lining 3,4. They develop as dilatation of glands within the periductal tissue 3. They vary in size and have been shown to gradually increase in size and number over time 3.

Features include well-demarcated cysts (uniform, low echogenicity with posterior acoustic enhancement) of varying size in a hilar distribution. In particular, a positive diagnosis is made with the presence of the cystic dilatation on both sides of the portal veins, whereas dilatation of intrahepatic bile ducts usually appears on one side. Background features of chronic liver disease are associated. 

Low (fluid) density, well-defined intrahepatic structures around the liver hilum with no enhancement on a background of cirrhosis.

  • T1: hypointense compared to liver parenchyma

  • T2: hyperintense, that of CSF

  • T1 C+: no enhancement

Peribiliary cysts are a benign and incidental finding. Although they have been shown to grow gradually over time, they require no direct management. Patients will be serially imaged for chronic liver disease. Very rare cases may result in intrahepatic biliary dilatation due to mass effect compressing the intrahepatic ducts.

These were first described by Nakanuma et al. in 1984 1.

Imaging differential considerations include:

  • von Meyenburg complexes: although in this setting biliary hamartomas are more diffuse throughout the liver rather than hilar in distribution and would be more space out

  • Caroli disease: a congenital disorder involving multifocal intrahepatic choledochal cysts of the segmental intrahepatic bile ducts

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Cases and figures

  • Case 1
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  • Case 2
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