"La Hipertension Portal Idiopatica (HPI) es un sindrome clinico-patologico caracterizado por hipertension portal no cirrotica. Su prevalencia es baja en occidente y no hemos encontrado casos comunicados en nuestro pais. La patente histologica caracteristica de HPI es fibrosis y obliteracion de las venas porta intrajepaticas (flebosclerosis), fibrosis portal y periportal, y formacion de vasos aberrantes. Presentamos un caso que nos fue derivado por hepatoesplenomegalia y pancitopenia. Al tercer dia de internacion presento dolor abdominal y mediante una TAC abdominal se le diagnostico un infarto esplenico . Posteriormente se le realizo una esplenectomia y biopsia hepatica. Los parametros hematologicos mejoraron despues de la operacion. Los estudios de imagenes y endoscopicos confirmaron la presencia de hipertension portal: circulacion colateral, vena porta permeable y dilatada y varices esofagicas. Los cambios histologicos del higado concordaron con el diagnostico de HPI. Los signos y sintomas caracteristicos de HPI son hemorragias digestivas relacionadas con la hipertension portal y pancitopenia secundaria al hiperesplenismo y su principal causa de muerte es la hemorragia por varices esofagicas. Como complicacion infrecuente de la HPI se ha descripto la trombosis de la vena Porta. En cambio el infarto esplenico, es una rara complicacion de la hipertension portal por cirrosis y del transplante hepatico, pero no se han publicado casos en pacientes con HPI. (AU)."@es
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