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 palsy. Computed tomography (CT) and magnetic resonance imaging (MRI) demonstrated an enhancing lesion in the region of the right third nerve with bony erosion of the posterior clinoid process. At exploration, the lesion proved to be a cavernous angioma arising from the substance of the third nerve. Three other cases of third nerve cavernous angioma have been reported. One of these lesions also occurred in a patient with this unusual genetic syndrome. The surgical management and possible role of the genetic defect in the pathogenesis of this lesion are discussed.