Issn Print: 0277-2116

Publication Date: 2004/06/01


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P0374 OUTCOME OF KASAI PORTOENTEROSTOMY IN BILIARY ATRESIA - UNIVERSITY OF NORTH CAROLINA EXPERIENCE

K. N. Furuya; K. Freeman; D. von Allmen; L. Taylor; T. Weiner; J. Phillips; S. Lichtman; B. Kassmann; J. Watford

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Author Information: Pediatrics,

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Introduction: Biliary atresia (BA) is a progressive, sclerosing, inflammatory process that leads to obliteration of bile ducts resulting in biliary cirrhosis. The Kasai portoenterostomy (KP) has been performed in an effort to re-establish bile flow but controversy exists as to optimal timing of KP and patient outcome. We examined whether age at KP determined outcome in terms of KP function and patient survival.
Methods: Records of 65 BA pts (University of NC) from 1980–2003 were reviewed. Age at KP, KP function, age at liver transplantation (LT) and race were recorded. A functioning KP (FKP) was defined by complete clearance of jaundice postoperatively (direct bilirubin (DB) < 1.0 mg/dl); partially functioning KP (PKP) by a decrease in DB and drainage on HIDA scan; failed KP if the DB did not decrease. Pts were separated into 2 groups: survival without LT and LT/death. Pts were also stratified based on 1)age at KP (<4 wks; 4–8 0wks; 8–10 wks; 10–12 wks; >12 wks) and 2)by race.
Results: Data was obtained on 62/65 BA pts; 55 underwent KP at a mean/median age of 9.4 wk (range: 1.7–24.5 wk) with 31 Caucasian (C), 19 African American (AA), 3 Asian; 2 Hispanic. Overall KP was successful in 45%; partially functioning in 10%; failed in 45%. No differences were observed between %FKP+PFK vs failed KP or survival without LT regardless of age at surgery except for the 4–8 wk group which had a lower survival rate. 16/55 (29%) pts survived without LT. The mean age at LT of pts with PKP/FKP was 5.4 yr (median 3.9 yr; range 0.6–11.6 yr) and with a failed KP 0.9 yr (median 0.8 yr; range 0.4–2.3 yr). There were no differences in the % survival without LT between pts undergoing KP at <4, 8, 10 or 12 wk vs > 4, 8, 10 or 12 wk respectively. However the overall survival rate without LT was higher among Caucasians (35%) than in AA (16%).
Conclusion: Age at the time of KP did not result in significantly different outcomes in terms of either KP function or patient survival. Survival without LT in AA was significantly lower than in Caucasians however differences in the timing of KP were not observed between these 2 groups.
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