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Review
. 2022 Jul 5;13(4):33-46.
doi: 10.4292/wjgpt.v13.i4.33.

Biliary atresia and congenital disorders of the extrahepatic bile ducts

Ali Islek  1 , Gokhan Tumgor  2
Affiliations
Review

Biliary atresia and congenital disorders of the extrahepatic bile ducts

Ali Islek et al. World J Gastrointest Pharmacol Ther. .

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.

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Conflict of interest statement

Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.

Figures

Figure 1
Figure 1
Liver biopsy of a patient with biliary atresia. A: Cholestasis (arrow) and ballooning degeneration (asterisks) (hematoxylin-eosin, magnification × 100); B: Portal fibrosis (hematoxylin-eosin, magnification × 100); C: Bile duct proliferation (Cytokeratin 19, magnification × 100).
Figure 2
Figure 2
Classificiation of choledochal cysts[4].
Figure 3
Figure 3
An endoscopic retrograde cholangiopancreatography image of type IVa choledochal cyst.
See this image and copyright information in PMC

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