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World J Gastrointest Pharmacol Ther. Jul 5, 2022; 13(4): 33-46
Published online Jul 5, 2022. doi: 10.4292/wjgpt.v13.i4.33
Biliary atresia and congenital disorders of the extrahepatic bile ducts
Ali Islek, Gokhan Tumgor
Ali Islek, Gokhan Tumgor, Department of Pediatric Gastroenterology, Cukurova University School of Medicine, Adana 01320, Turkey
Author contributions: Islek A substantially contributed to the conception and design of the paper and the acquisition, analysis and interpretation of the data; Islek A and Tumgor G drafted the article and made critical revisions related to the intellectual content of the manuscript and approved the final version of the article to be published.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Gokhan Tumgor, MD, Professor, Department of Pediatric Gastroenterology, Cukurova University School of Medicine, Balcalı, Adana 01320, Turkey. gtumgor74@yahoo.com
Received: January 15, 2022
Peer-review started: January 15, 2022
First decision: March 8, 2022
Revised: March 10, 2022
Accepted: June 13, 2022
Article in press: June 13, 2022
Published online: July 5, 2022
Processing time: 166 Days and 16.2 Hours
Abstract

Biliary atresia (BA) and choledochal cysts are diseases of the intrahepatic and extrahepatic biliary tree. While their exact etiopathogeneses are not known, they should be treated promptly due to the potential for irreversible parenchymal liver disease. A diagnosis of BA may be easy or complicated, but should not be delayed. BA is always treated surgically, and performing the surgery before the age of 2 mo greatly increases its effectiveness and extends the time until the need for liver transplantation arises. While the more common types of choledochal cysts require surgical treatment, some can be treated with endoscopic retrograde cholangiopancreatography. Choledochal cysts may cause recurrent cholangitis and the potential for malignancy should not be ignored.

Keywords: Bilier atresia; Choledochal cyst; Cholestasis; Conjugated hyperbilirubinemia

Core Tip: Biliary atresia (BA) and choledochal cysts are diseases that cause obstructive cholestasis. While the diagnosis of BA can be rather complicated, it should be made as early as possible and treated with a Kasai hepatoportoenterostomy before the age of 2 mo for a good prognosis. All patients with persistent acholic stool and elevated gamma-glutamyl transferase should be evaluated for BA, although normal ultrasonography will not rule out BA, and such patients are candidates for intraoperative cholangiography. Choledochal cysts can present symptoms at any age, and as recurrent cholangitis attacks will lead to chronic liver disease with potential malignancy, treatment and long-term follow-up are essential.