Biliary atresia: evaluation on two distinct periods at a reference pediatric service

Arq Gastroenterol. 2014 Jan-Mar;51(1):53-8. doi: 10.1590/s0004-28032014000100011.

Abstract

Context: Biliary atresia is a progressive, idiopathic, fibro-obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction exclusively in the neonatal period.

Objectives: To assess the differences regarding age at referral, age at surgery, duration of propaedeutics and waiting time for surgery between two groups of infants in different periods.

Methods: Retrospective study of infants diagnosed with biliary atresia on two periods: 1983-1993 and 1998-2011.

Results: Biliary atresia was diagnosed in 129 infants, being 48 in casuistic I and 81 in casuistic II. The median age at admission was 94 and 60 days, respectively (P = 0.0001). On evaluating patients who had undergone portoenterostomy before 120 days of age, no difference was observed regarding the duration of propaedeutics or waiting time for surgery (P = 0.15), but difference was found when comparing the age at surgery (P = 0.002). Among those infants with no biliary flow and without liver transplantation or death after 18 post-operative months, the estimated probability of survival was 44.6% and 38.7% in casuistics I and II, respectively. In casuistic I, all infants who showed biliary flow were alive during the observation period and, in casuistic II, 80.3% were alive after 7 years of follow-up.

Conclusions: Even though patients were admitted and treated earlier, it is clear that surgery could be done sooner. Delay in referral and timely propaedeutics were the main contributors.

Publication types

  • Comparative Study

MeSH terms

  • Biliary Atresia / mortality
  • Biliary Atresia / surgery*
  • Female
  • Humans
  • Infant
  • Liver Transplantation
  • Male
  • Portoenterostomy, Hepatic
  • Retrospective Studies
  • Survival Analysis
  • Time Factors
  • Treatment Outcome