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21-03-2024 | Primary Sclerosing Cholangitis | Gastrointestinal

Spontaneous perforation of the common bile duct: an uncommon complication of primary sclerosing cholangitis

Authors: Lucas Dargent, Sara Lemoinne, Karine Louvion, Pauline Iorio, Christophe Corpechot, Sanaâ El Mouhadi, Quentin Vanderbecq, Olivier Chazouillères, Lionel Arrivé

Published in: European Radiology

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Abstract

Objectives

To describe the MR features and prognosis of patients with an uncommon complication of primary sclerosing cholangitis (PSC) characterized by a spontaneous perforation of the common bile duct (CBD) resulting in a peri-biliary collection and a pseudo-cystic appearance of the CBD.

Methods

A single-center cohort of 263 patients with PSC who had at least two MRIs between 2003 and 2022 and a minimum follow-up of 1 year was retrospectively analyzed. MRI data (characteristics of CBD perforation and MR features of PSC) and clinical data were assessed. Analysis of survival without liver transplantation according to type of PSC (classical or CBD spontaneous perforation) was performed according to the Kaplan–Meier method and the curves were compared using the Log-Rank test.

Results

A total of nine (3.4%) PSC patients (5 males) had perforation of the CBD with a median age at diagnosis of 18 years compared to 33 years for the control group (p = 0.019). The peri-biliary collections were variable in appearance (fusiform or pedunculated), with a diameter ranging from 5 to 54 mm. All nine patients showed intra- and extra-hepatic bile duct involvement, dysmorphia, and high ANALI scores. The clinical course was characterized by numerous complications in most patients, and five patients (56%) underwent liver transplantation at a median time of 5 years from diagnosis, compared to 40 patients (16%) in the control group (p = 0.02).

Conclusion

The spontaneous perforation of the common bile duct is an uncommon complication of primary sclerosing cholangitis that affects young patients and is associated with a poor prognosis.

Clinical relevance statement

This uncommon complication of primary sclerosing cholangitis with perforation of the common bile duct resulting in a peri-biliary collection and a pseudo-cystic appearance of the common bile duct is characterized by a poor prognosis in younger patients.

Key Points

• Among 263 patients with primary sclerosing cholangitis (PSC), nine patients (3.6%) had an uncommon complication characterized on MRI by perforation of the common bile duct (CBD).
• This perforation of the CBD was responsible in all nine cases for the formation of a peri-biliary collection, giving a pseudo-cystic appearance to the CBD.
• The spontaneous perforation of the common bile duct is an uncommon complication of primary sclerosing cholangitis that affects young patients with a poor prognosis.
Literature
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go back to reference Novellas S, Caramella T, Fournol M, Gugenheim J, Chevallier P (2008) MR cholangiopancreatography features of the biliary tree after liver transplantation. AJR Am J Roentgenol 191:221–227CrossRefPubMed Novellas S, Caramella T, Fournol M, Gugenheim J, Chevallier P (2008) MR cholangiopancreatography features of the biliary tree after liver transplantation. AJR Am J Roentgenol 191:221–227CrossRefPubMed
Metadata
Title
Spontaneous perforation of the common bile duct: an uncommon complication of primary sclerosing cholangitis
Authors
Lucas Dargent
Sara Lemoinne
Karine Louvion
Pauline Iorio
Christophe Corpechot
Sanaâ El Mouhadi
Quentin Vanderbecq
Olivier Chazouillères
Lionel Arrivé
Publication date
21-03-2024
Publisher
Springer Berlin Heidelberg
Published in
European Radiology
Print ISSN: 0938-7994
Electronic ISSN: 1432-1084
DOI
https://doi.org/10.1007/s00330-024-10704-9