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 .
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 .