The therapy of biliary atresia combining the Kasai portoenterostomy with liver transplantation: a single center experience.

Abstract:

:Survival has improved dramatically for children with extrahepatic biliary atresia (EHBA), but optimal surgical management remains controversial. We have studied 28 infants born between June 1981, and April 1988, who underwent Kasai's portoenterostomy as primary surgical treatment. Those with evidence of subsequent hepatic decompensation were evaluated for liver transplantation (LT). All were cared for by surgeons who perform both the Kasai portoenterostomy and LT. Following the Kasai operation, 16 of 28 patients (57.1%) have achieved total biliary drainage, while 5 of 28 (17.9%) achieved partial drainage, and 7 of 28 (25%) achieved no drainage. Nine of 28 (32.1%) have undergone LT, 4 of whom were transplanted at greater than 2 years of age. To date, 25 of 28 (89.3%) are alive. Twenty three of 25 (92%) living are jaundice-free, 65% of whom have undergone the Kasai operation only. We project that 5 of 28 (17.8%) will come to transplantation, and 12 of 28 (43%) have no current indication that they are likely to come to transplantation. We conclude that combining Kasai's portoenterostomy with LT as needed is an effective therapy for children with EHBA, that the Kasai operation offers significant benefit by delaying LT in many, and that the long-term results of management remain to be determined.

journal_name

J Pediatr Surg

authors

Vacanti JP,Shamberger RC,Eraklis A,Lillehei CW

doi

10.1016/s0022-3468(05)80182-4

subject

Has Abstract

pub_date

1990-01-01 00:00:00

pages

149-52

issue

1

eissn

0022-3468

issn

1531-5037

pii

S0022346890000136

journal_volume

25

pub_type

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