Outcome of Early Hepatic Portoenterostomy for Biliary Atresia

Abstract

Although these data suggest that outcomes are worse for patients who undergo the procedure before 30 days of age, they may reflect a difference in the pathogenesis of biliary atresia that brings it to clinical attention earlier and may provide further evidence that biliary atresia is a phenotype for a number of distinct underlying disease processes.

A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000

“…Early presentation and surgery has been demonstrated by some to be a risk factor for poor outcome. 16 In this study, this was observed with respect to patients with BASM who tended to have early diagnosis and worse prognosis; however, it was not confirmed among the other subjects.…”

Impact of age at Kasai operation on short- and long–term outcomes of type III biliary atresia at a single institution

“…Although many researchers advocated the advantage of early operation [2], some noted an adverse effect of early operation, especially during the first month of life [3]. Regarding the impact of the timing of Kasai operation on long-term outcome, a few reports have been published [4,5]; however, it is still very uncertain.…”

Pathogenesis of biliary atresia: defining biology to understand clinical phenotypes

“…41 This association implies that a younger patient will have an earlier phase of disease and perhaps a lesser degree of liver injury. However, age alone is not a uniform predictor of outcome as demonstrated by the poor survival with the native liver of a cohort of young infants with biliary atresia after hepatoportoenterostomy, 42 suggesting that other factors influence the clinical course of disease, such as the coexistence of severe cardiovascular disease, cytomegalovirus infection, and the type and extent of liver pathology at diagnosis. Prominent histological findings of inflammation in the liver at diagnosis has been reported to correlate with a poor response to hepatoportoenterostomy.…”