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Pediatric Surgery International
Published in: Pediatric Surgery International 9/2021

01-09-2021 | Liver Transplantation | Original Article

The morphological and histopathological assessment of Alagille syndrome with extrahepatic bile duct obstruction: the importance of the differential diagnosis with subgroup “o” biliary atresia

Authors: Masahiro Takeda, Seisuke Sakamoto, Hajime Uchida, Seiichi Shimizu, Yusuke Yanagi, Akinari Fukuda, Takako Yoshioka, Mureo Kasahara

Published in: Pediatric Surgery International | Issue 9/2021

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Abstract

Purpose

The differential diagnosis between Alagille syndrome (AGS) with extrahepatic bile duct obstruction (EHBDO) and biliary atresia (BA) is difficult. We report a case series of AGS with EHBDO with detailed validation of the morphological and histopathological features for the differential diagnosis of BA.

Methods

Six liver transplantations (LTs) were performed for AGS with EHBDO. All patients were diagnosed with BA at the referring institution and the diagnosis of AGS was then confirmed based on a genetic analysis before LT. We verified the morphological and histopathological findings of the porta hepatis and liver at the diagnosis of BA and at LT.

Results

All patients had acholic stool in the neonatal period and were diagnosed with BA by cholangiography. The gross liver findings included a smooth and soft surface, without any cirrhosis. The gross findings of the porta hepatis included aplasia of the proximal hepatic duct, or subgroup “o”, in five patients. The histopathological examination of the EHBD also revealed obstruction/absence of the hepatic duct. There were no patients with aplasia of the common bile duct.

Conclusions

Aplasia of the hepatic duct and the macroscopic liver findings may help in to differentiate between AGS with EHBDO and BA.
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Metadata
Title
The morphological and histopathological assessment of Alagille syndrome with extrahepatic bile duct obstruction: the importance of the differential diagnosis with subgroup “o” biliary atresia
Authors
Masahiro Takeda
Seisuke Sakamoto
Hajime Uchida
Seiichi Shimizu
Yusuke Yanagi
Akinari Fukuda
Takako Yoshioka
Mureo Kasahara
Publication date
01-09-2021
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Surgery International / Issue 9/2021
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI
https://doi.org/10.1007/s00383-021-04932-z

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