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Current concept of the treatment of biliary atresia

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Abstract

Hepatic portoenterostomy (Kasai operation) for the patient with biliary atresia (BA) can restore the bile flow in approximately 80% of children operated on before 60 days of life [1]. However, in terms of long-term survival, according to a recent nationwide survey among the major pediatric centers in Japan, only 325 of 2013 patients had more than 10 years' survival, and only 157 patients (7.8%) remained jaundice-free with normal liver function [2]. About 20% of BA cases without jaundice are generally able to survive for long periods; and most of those patients have portal hypertension or abnormal liver function [3–5].

As the results of liver transplantation have improved, controversy has arisen over the optimal care of these children [4, 6, 7]. Some investigators have claimed that transplantation is the favored primary therapy for most patients with BA [8]. We are thus at a turning point concerning the primary therapy of BA, which makes it necessary to determine the exact indications for the Kasai portoenterostomy and the timing of liver transplantation. This paper describes our strategy for the optimal treatment of BA patients based on our 117 patients who have had various form of portoenterostomy.

Résumé

L'opération de Kasai (dérivation bilio-entérique au hile) pour atrésie biliaire restaure l'écoulement de la bile chez environ 80% des enfants opérés avant le deuxième mois post-natal. Une enquête récente au Japon a cependant revélé que seulement 7.8% restaient anictériques, et seulement 16% (325/2013) des enfants opérés étaient en vie à 10 ans. Parmi les survivants, la large majorité ont une hypertension portale souvent associée à une fonction hépatique perturbée. La transplantation hépatique serait un traitement particulièrement adapté à cette maladie pour certains. Basée sur les résultats de leur série personnelle de 117 enfants avec une atrésie biliaire, les auteurs présentent un algorithme concernant les indications respectives de l'opération de Kasai seule ou combinée à une transplantation hépatique dans cette maladie.

Resumen

La aplicación de la porto-enterostomía hepática (operación de Kasai) en el paciente con atresia biliar (AB) logra la restauración del flujo biliar en aproximadamente 80% de los niños que se operan ante de 60 días. Sinembargo, en términos de sobrevida a largo plazo, una reciente investigación nacional realizada en el Japón demostró que sólo 325 de 2.013 pacientes exibiía sobrevida de más de 10 años y 157 pacientes (7.8%) permanecían libres de ictericia y con función hepática normal. Apenas 20% de los pacientes con AB sin ictiricia logran sobrevivir por largos períodos; la vasta mayoría tienen hipertensión portal y/o función hepática anormal.

En la medida que mejoran los resultados del trasplante hepático se suscita controversia sobre el manejo óptimo de estos ninos. Algunos investigadores han planteado el trasplante como la modalidad terapéutica de preferencia para la mayoría de los casos de AB. Estamos pues en una encrucijada en cuanto al tratamiento primario de la AB, lo cual hace necesario definir las indicaciones exactas para la porto-enterostomía de Kasai y el momento oportuno de realizar un trasplante hepático. Este artículo describe nuestra estrategia más reciente para el óptimo tratamiento de los pacientes con AB, con base en nuestra serie de 117 casos sometidos a diversas formas de porto-enterostomía. La porto-enterostomía ofrece el beneficio muy significativo de posponer el trasplante hepático; por consiguiente, la porto-enterostomía y el trasplante son procedimientos complementarios y no competitivos.

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Correspondence to Takeshi Miyano M.D..

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Miyano, T., Fujimoto, T., Ohya, T. et al. Current concept of the treatment of biliary atresia. World J. Surg. 17, 332–336 (1993). https://doi.org/10.1007/BF01658700

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Keywords

  • Liver Transplantation
  • Portal Hypertension
  • Biliary Atresia
  • Cual
  • Primary Therapy