RESUMO
Biliary atresia (BA) is classically described at the neonatal age. However, rare cases of BA in older infants have also been reported. We report four cases of late-onset BA in infants older than 4 weeks (3 males, 1 female), and describe the diagnostic and management difficulties. One of the cases had a late-onset (29 weeks) presentation with a successful surgical procedure. We highlight the importance of this unusual differential diagnosis in infants with cholestatic syndrome, who may benefit from Kasai surgery, regardless of age.
Assuntos
Humanos , Masculino , Feminino , Lactente , Atresia Biliar/diagnóstico , Transtornos de Início Tardio/diagnóstico , Fígado/patologia , Atresia Biliar/patologia , Atresia Biliar/cirurgia , Biópsia , Diagnóstico Diferencial , Artéria Hepática/patologia , Transtornos de Início Tardio/patologia , Transtornos de Início Tardio/cirurgiaRESUMO
La atresia biliar es una grave enfermedad que se manifiesta en los recién nacidos, y se desconoce su causa. La inflamación y destrucción progresiva de los conductos biliares conducen a la aparición de ictericia, coluria y acolia entre la segunda y sexta semana de vida. Como existen múltiples causas de colestasis neonatal en esta etapa de la vida, es necesario realizar un diagnóstico y derivación precoz para ofrecer un tratamiento quirúrgico, con el fin de restablecer el flujo biliar. Alrededor del 80% de los pacientes normalizan la bilirrubina luego de la portoenterostomía (operación de Kasai), realizada antes de los 45 días de vida. Si la operación fracasa, el trasplante hepático surge como única alternativa. La atresia biliar debe diagnosticarse durante el primer mes de vida y ser considerada una urgencia quirúrgica.
Biliary atresia is a serious disease of unknown cause, affecting newborns. An inflammation and progressive destruction of the bile ducts lead to jaundice, dark urines, and acholia, between the second and sixth weeks of life. Neonatal cholestasis could be due to several different diseases, thus a diagnosis of biliary atresia and early derivation for surgical treatment are necessary to allow a restoration of the bile flow. Eighty percent of the children normalize serum bilirubin after the portoenterostomy (Kasai operation), if they are operated before their 45 days of life. When Kasai operation fails, a liver transplantation is the only possibility. Biliary atresia must be diagnosed before the first month of life and must be considered as a surgical emergency.