OCULOMOTOR NERVE CAVERNOUS ANGIOMA IN A PATIENT WITH ROBERTS-SYNDROME

A 25-year-old man with Roberts Syndrome (SC-phocomyelia) is described who presented with an acute onset of a complete right third nerve pals y. Computed tomography (CT) and magnetic resonance imaging (MRI) demon strated an enhancing lesion in the region of the right third nerve wit h bony erosion of the posterior clinoid process. At exploration, the l esion proved to be a cavernous angioma arising from the substance of t he third nerve. Three other cases of third nerve cavernous angioma hav e been reported. One of these lesions also occurred in a patient with this unusual genetic syndrome. The surgical management and possible ro le of the genetic defect in the pathogenesis of this lesion are discus sed.