Hemorragia intracraneal como forma de presentación de atresia biliar: presentación de dos casos Translated title: Intracranial hemorrhage as initial presentation of biliary atresia: two cases report

Abstract

La atresia biliar en lactantes se presenta habitualmente con la tríada ictericia, acolia y coluria, y ocasionalmente con sangrado intracraneal, nasal o gastrointestinal. Presentamos dos niñas, de cuatro y dos meses, que fueron asistidas por presentar convulsiones, cefalohematoma y sopor. En la tomografía computada cerebral se halló hemorragia subdural en una paciente e intraventricular y parenquimatosa en la otra. Al ingreso, presentaban antecedentes, signos clínicos y de laboratorio de colestasis, sin diagnóstico etiológico. La niña con hematoma subdural requirió drenaje quirúrgico. La paciente con sangrado intraventricular y parenquimatoso no requirió cirugía y se le administró vitamina K. Se diagnosticó atresia de vías biliares mediante centellograma con HIDA y colangiografía intraoperatoria previa al procedimiento de Kasai (portoenteroanastomosis). Ambas niñas presentaron buena evolución neurológica a los seis meses. Requirieron trasplante de hígado alrededor del año de vida. La atresia biliar se debe considerar en el diagnóstico de lactantes pequeños que presentan sangrado agudo y colestasis.

Translated abstract

Biliary atresia in infants occasionally presents as intracranial, nasal or gastrointestinal bleeding, instead of the classical triad of jaundice, acholia and choluria. We present two female infants aged four and two months, who were hospitalized with convulsive episode, cephalohematoma and drowsiness. Computed tomography fndings were subdural hemorrhage in one patient and intraventricular and parenchymal bleeding in the other one. At admission they have history, clinical and laboratory signs of cholestasis of unknown etiology. The patient with subdural hemorrhage required surgical drainage. The other girl with intraventricular and parenchymal bleeding received vitamin K and no surgery. Biliary atresia was diagnosed and treated in both girls. At six months both had an adequate neurological outcome and required liver transplantation at one year old. Biliary atresia should be considered in all infants with sudden acute bleeding and cholestasis.