Tuberous sclerosis - neurosurgical indications in intraventricular tumors

Thirteen cases of intraventricular tumors associated with tuberous sclerosis are described. The most common picture showed the "forme fruste", i.e. there were no signs of the typical clinical triad - metal retardation, seizures, and adenoma sebaceum - and there was usually no family history of tuberous sclerosis. There was a high incidence of increased intracranial pressure (76,9%). CT has made possible the preoperative diagnosis of an intraventricular tumor which is suggestive of tuberous sclerosis. The best surgical results were obtained by total excision of the tumor. The shunt procedures are discussed as a temporary surgery for relief of increased intracranial pressure and as a preparation of the patient for the final excision of the tumor. All the tumors were classified as subependymal giant cell astrocytomas according to the new classification of the World Health Organization.