Clinical and pathological challenges in the diagnosis of late-onset biliary atresia: four case studies

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.

Colestasis intrahepática: un desafío diagnóstico / Intrahepatic cholestasis: a diagnostic challenge

La colestasis, término acuñado por el anátomo-patólogo Hans Pooper en 1956, constituye un verdadero síndrome clínico-analítico, que incluye un amplio espectro de entidades patológicas de severidad y pronóstico variable. Las manifestaciones clínicas principales de la colestasis derivan de la acumulación en plasma de sustancias normalmente excretadas en la bilis, vinculado a la disminución en la excreción biliar al tubo digestivo, base fisiopatológica de este síndrome. En su conjunto, las colestasis pueden clasificarse en intra o extrahepáticas, ictéricas o anictéricas y agudas o crónicas; asimismo, desde una visión etiológica, pueden catalogarse como congénitas, inflamatorias, mecánicas, tóxicas, metabólicas o neoplásicas. En el presente artículo, y a partir del reporte de un caso clínico, se analizan aspectos diagnósticos y terapéuticos del síndrome colestásico, proponiendo un algoritmo de actuación, de acuerdo a la mejor evidencia disponible a la fecha.

Cholestasis, a term coined by Hans Pooper anatomic pathologist in 1956, is a real analytic clinical syndrome that includes a wide spectrum of pathological severity and outcome variable. Major clinical manifestations of cholestasis arising from the accumulation of plasma substances normally excreted in the bile, linked to decreased biliary excretion into the digestive tract, pathophysiological basis of this syndrome. Overall, cholestasis can be classified into intra or extrahepatic, icteric or anicteric and acute or chronic, also from an etiological view can be categorized as congenital, inflammatory, mechanical, toxic, metabolic, or neoplastic. In this article, and from clinical case report, we analyze the diagnostic and therapeutic cholestatic syndrome, proposing an algorithm of action, according to the best evidence available to date.