Romboencefalosinapsis como causa de ventriculomegalia severa. Reporte de dos casos / Rhomboencephalosynapsis as a cause of severe vetriculomegaly. Report of two cases

ABSTRACT

We report two cases of a rare pathology of the fetal cerebellum, rhomboencephalosynapsis, a defect characterized by the partial or complete absence of the cerebellar vermis, which is currently associated with severe ventriculomegaly due to defects in the formation of the cerebral aqueduct. These are the first cases reported in Peru

RESUMEN

Reportamos dos casos de una patología rara del cerebelo fetal, la romboencefalosinapsis, defecto caracterizado por la ausencia parcial o completa del vermis cerebeloso, frecuentemente asociada a ventriculomegalia severa por defectos en la formación del acueducto de Silvio. Estos constituyen los primeros casos reportados en Perú.