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

• Published in: Arquivos de gastroenterologia Vol. 51; no. 1; pp. 53 - 58
• Main Authors: Queiroz, Thais Costa Nascentes, Ferreira, Alexandre Rodrigues, Fagundes, Eleonora Druve Tavares, Roquete, Mariza Leitão Valadares, Penna, Francisco José
• Format: Journal Article
• Language: English
• Published: Brazil Instituto Brasileiro de Estudos e Pesquisas de Gastroenterologia e Outras Especialidades - IBEPEGE 01-03-2014; Instituto Brasileiro de Estudos e Pesquisas de Gastroenterologia (IBEPEGE)
• Subjects: Atresia biliar; Biliary Atresia - mortality; Biliary Atresia - surgery; Female; GASTROENTEROLOGY & HEPATOLOGY; Humans; Infant; Liver Transplantation; Male; Portoenterostomia hepática; Portoenterostomy, Hepatic; Prognóstico; Retrospective Studies; Survival Analysis; Time Factors; Transplante de fígado; Treatment Outcome; Portoenterostomia hepática; Biliary atresia; Atresia biliar; Prognosis; Hepatic portoenterostomy; Prognóstico; Transplante de fígado; Liver transplantation
• ISSN: 0004-2803; 1678-4219; 1678-4219; 0004-2803
• DOI: 10.1590/s0004-28032014000100011

Biliary atresia is a progressive, idiopathic, fibro-obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction exclusively in the neonatal period.
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.
Retrospective study of infants diagnosed with biliary atresia on two periods: 1983-1993 and 1998-2011.
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.
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.