ON THE TREATMENT OF SUBEPENDYMAL GIANT-CELL ASTROCYTOMAS AND ASSOCIATED HYDROCEPHALUS IN TUBEROUS SCLEROSIS

Between 1980 and 1992, 10 children affected by tuberous sclerosis and intraventricular subependymal giant cell astrocytomas were surgically treated at the Institute of Neurosurgery, Section of Pediatric Neurosu rgery, Catholic University of Pome. Nine patients presented with signs and/or symptoms of intracranial hypertension; in all of them the neur oradiological investigations demonstrated the presence of a space-occu pying lesion in the region of the foramen of Monro with secondary vent ricular dilation. In the remaining patient, a 5-month-old male infant, an intraventricular mass was discovered by means of an ultrasound exa mination performed after the first epileptic fit. Three patients under went a ventriculoperitoneal CSF shunt as first surgical procedure; in 2 of them it was subsequently necessary to remove the intraventricular tumor due to the frequent occlusion of the CSF shunt device. Seven su bjects underwent the direct surgical excision of the lesion. In all of them the procedure resulted in the control of the associated hydrocep halus. On the basis of such an experience, the authors conclude that t he surgical removal of the intraventricular tumors in patients with tu berous sclerosis and hydrocephalus is the most appropriate treatment. In fact, in the series considered here, the removal of the tumor was n ot accompanied by significant morbidity, and was followed by improveme nt in clinical conditions. In particular, in cases in whom the occurre nce of hydrocephalus was associated with a worsening in the seizure di sorder, the tumor removal and the correction of intracranial hypertens ion was followed by a significant reduction in frequency or even by th e disappearance of the seizures. However, in no case presenting with m ental impairment was a significant improvement observed in mental perf ormances as a consequence of the surgical treatment.