Atrésia das vias biliares extrahepáticas: estudo clínico retrospectivo

 

Ermelinda Santos Silva¹, Margarida Medina¹, Paula Rocha², Berta Bonet³, J.A. Ferreira de Sousa³, Carlos Enes³

¹ S. Gastrenterologia Pediátrica, CHPorto-HMPia

² S. Cuidados Intensivos Neonatais e Pediátricos, CHPorto-HMPia

³ S. Cirurgia Pediátrica, CHPorto-HMPia

Correspondência

 

RESUMO

Objectivo: Conhecer o comportamento de uma população de doentes com atrésia das vias biliares nas distintas fases da doença e determinar a sua sobrevida e factores prognósticos.

Doentes e Métodos: Estudo retrospectivo, transversal, descritivo, incluindo os doentes com atrésia das vias biliares diagnosticados e tratados de Janeiro de 1992 a Dezembro de 2007; este período foi subdividido em duas partes: P1 de Janeiro de 1992 a Dezembro de 1999 e P2 de Janeiro de 2000 a Dezembro de 2007.

Foram analisadas as seguintes variáveis: dados clínicos e bioquímicos; achados imagiológicos, histológicos e cirúrgicos; idade de realização do Kasai; realização de transplante hepático e resultado.

Resultados: Foram atendidos 15 doentes (seis em P1 e nove em P2). O tempo mediano entre a admissão e a portoenterostomia (PE) de Kasai foi 10 dias (14 dias em P1; 7 dias em P2). A idade mediana de execução da PE de Kasai foi 58 dias (89 dias em P1; 45 dias em P2). A PE de Kasai funcionou em 46,67% (33,33% em P1; 55,56% em P2). A sobrevida global aos cinco anos foi 83.33% (66,67% em P1; 100% em P2). A sobrevida com fígado nativo aos cinco anos foi 41.67% (16,67% em P1; 66.67% em P2).

Conclusões: Apesar do número reduzido de doentes operados/ano (1,125 doentes) os resultados em P2 são comparáveis aos observados nos centros europeus de referência. Esta melhoria foi obtida à custa dum diagnóstico mais precoce, com consequente diminuição da idade de execução da PE Kasai; acresce também a importância da experiência acumulada pela equipa ao longo dos anos.

Palavras-chave: Atrésia das vias biliares, colestase neo­natal, transplante hepático.

 

Biliary atresia: retrospective clinical study

ABSTRACT

Objective: To obtain further insight into the natural history of patients with biliary atresia and to determine survival and prognostic factors.

Patients and Methods: we performed a retrospective cross-sectional, descriptive, case series study, that included all patients with biliary atresia attended in our hospital from January 1992 to December 2007. This period was divided in two parts: P1 from January 1992 to December 1999 and P2 from January 2000 to December 2007.

The following variables were analysed: clinical biochemical, imagiological, histological and surgical data; age of Kasai proce­dure; need of liver transplant and outcome.

Results: There were 15 patients attended (six in P1 and nine in P2). Median time between admission and Kasai procedure was 10 days (14 days in P1; 7days in P2). Median age at Kasai procedure was 58 days (89 days in P1; 45 days in P2). Ka­sai procedure was successful in 46,67% (33,33% in P1; 55.56% in P2). Overall ve years survival was 83.33% (66,67% in P1; 100% in P2). Five years survival with native liver was 41.67% (16,67% in P1; 66.67 % in P2).

Conclusions: Despite the low rate of admissions (1,125 patients/year) the P2 results are similar to those of Reference European Centers. This improvement was achieved with an early diagnosis (early reference of patients and more rapid investigation, with consequent lowering of Kasai procedure`s median age); it was also important the increased experience of the team within the years.

Keywords: Biliary atresia, neonatal cholestasis, liver transplantation.

 

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CORRESPONDÊNCIA

Ermelinda Santos Silva

Serviço Gastrenterologia Pediátrica

Hospital de Crianças Maria Pia – Centro Hospitalar do Porto

Rua da Boavista, 827, 4050 Porto, Portugal

ermelinda.rss@gmail.com