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Surgery of biliary atresia.

Authors
Type
Published Article
Journal
Scandinavian journal of surgery : SJS : official organ for the Finnish Surgical Society and the Scandinavian Surgical Society
Publication Date
Volume
100
Issue
1
Pages
49–53
Identifiers
PMID: 21482505
Source
Medline
License
Unknown

Abstract

Biliary atresia (BA) is a progressive fibro-obliterative cholangiopathy of unclear etiology affecting varying degrees of both extra- and intrahepatic biliary tree resulting in obstructive bile flow and cholestasis in neonates. The diagnostic work-up is designed to diagnose or rule out BA without any unnecessary delay. Kasai portoenterostomy is a palliative operation performed to establish bile drainage from microscopic bile ductules that remain in the porta hepatis. It is advantageous to perform portoenterostomy as early after birth as possible for better chances of success. Actuarial native liver 5-year survival rates range from 30% to 60% after portoenterostomy, and about 20% of all patients undergoing Kasai portoenterostomy during infancy survive into adulthood with their native liver. Portoenterostomy remains as the first line operative treatment in BA while liver transplantation serves as a salvage treatment when portoenterostomy fails or liver function gradually deteriorates after initially successful establishment of bile flow.

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