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. 2018 Mar;25(2):68-73.
doi: 10.1159/000480708. Epub 2017 Oct 5.

Biliary Atresia - Clinical Series

Bárbara Neto  1 , Mariana Borges-Dias  1 , Eunice Trindade  2 , José Estevão-Costa  1 , José Miguel Campos  1
Affiliations

Biliary Atresia - Clinical Series

Bárbara Neto et al. GE Port J Gastroenterol. 2018 Mar.

Abstract
in English, Portuguese

Introduction: Biliary atresia is the main cause of death by hepatic failure and the main indication for liver transplant in children. This study aims to analyze the population with this diagnosis, treated between 2000 and 2015 at Hospital de São João.

Material and methods: Descriptive, observational, and retrospective study, including the patients with biliary atresia, diagnosed and treated between January 1, 2000 and December 31, 2015. We analyzed epidemiologic, clinical, biochemical, and image data, as well as registered complications and present status.

Results: Eighteen patients were evaluated. The median age at time of Kasai portoenterostomy was 63 days of life, with better prognosis for those patients who had surgery before 72 days. The procedure was successful in 2/3 of cases. There was a significant association between recurrent cholangitis and survival. Five cases of transplant and 2 deaths, one of them after transplant, were registered. Survival with native liver was 77.8%, 72.2%, and 64.2% at 1, 5, and 10 years of follow-up, respectively.

Discussion: The presentation and evolution of patients was similar to other studies. However, there was a higher surgical success and survival rates at 5 and 10 years of follow-up than most series. Age at surgery and recurrence of cholangitis were the only factors significantly related to prognosis.

Conclusion: In spite of the low number of patients (1,125/year), our results were similar to those of other reference centers.

Introdução: A atresia das vias biliares representa a principal causa de morte por insuficiência hepática e a principal indicação para transplante hepático em idade pediátrica. O objetivo deste estudo é analisar a população com este diagnóstico, tratada entre 2000 e 2015 no Hospital de São João.

Material e métodos: Estudo descritivo, observacional e retrospetivo, incluindo-se doentes com atresia das vias biliares diagnosticados e tratados entre 1 de Janeiro de 2000 e 31 de Dezembro de 2015. Foram analisados dados epidemiológicos, clínicos, bioquímicos, imagiológicos, idade à data da cirurgia, complicações ocorridas e o estado atual.

Resultados: Foram avaliados 18 doentes. A idade mediana à data da portoenterostomia de Kasai foi de 63 dias de vida, verificando-se um melhor prognóstico nos pacientes operados antes dos 72 dias. Foi constatada drenagem biliar adequada em 2/3 dos casos aos 6 meses. Verificou-se uma associação significativa entre a ocorrência de episódios recorrentes de colangite e a sobrevida. Registaram-se 5 transplantes e 2 óbitos, um dos quais pós-transplante. A sobrevida com fígado nativo foi de 77.8%, 72.2% e 64.2% ao 1º, 5º e 10º anos de seguimento, respetivamente.

Discussão: A apresentação e evolução dos pacientes coincidiu, de um modo geral, com o descrito na literatura. No entanto, constatou-se uma taxa de sucesso operatório e de sobrevida com fígado nativo aos 5 e 10 anos superiores à maioria das séries. A idade à data de cirurgia e a recorrência dos episódios de colangite foram os únicos fatores que se mostraram significativamente relacionados com o prognóstico.

Conclusão: Apesar do número reduzido de doentes (1,125/ano), foram registados resultados comparáveis a outros centros de referência.

Keywords: Biliary atresia; Kasai portoenterostomy; Liver transplantion.

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Figures

Fig. 1
Fig. 1
Perioperative cholangiography that confirms the diagnosis of biliary atresia.
Fig. 2
Fig. 2
Evolution of case numbers with follow-up time. w, weeks; m, months; y, years.
Fig. 3
Fig. 3
Evolution of studied cases.
Fig. 4
Fig. 4
Kaplan-Meier curves, representing native liver survival during postoperative time.
See this image and copyright information in PMC

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