OCCLUSIVE HYDROCEPHALUS COMPLICATING TUBEROUS-SCLEROSIS - REPORT OF 2CASES

Two patients-6 and 14 years old-with tuberous sclerosis are presented. Both developed a subependymal giant cell astrocytoma from nodules loc ated near to the foramen of Monro. They caused obstruction of the cere brospinal fluid pathways. Signs of raised intracranial pressure were d etected in both patients, and one of them had also developed infantile spasm-Blitz-Nick-Salaam seizures. Cutaneous stigmata being characteri stic for this entity were observed in both cases, but their mental dev elopment was unaffected. Diagnosis was based on computed tomography. A ngiography did not reveal pathological vessels. The tumours were compl etely excised through transcallosal exposure in both cases. The patien ts have been symptom-free during the follow-up time of 1 and 2 years. Although the incidence of malignant transformation of tuberous scleros is is less than 15% the disease generally has a poor prognosis which c an be ascribed to sudden increase of intracranial pressure caused by o bstruction of cerebrospinal fluid pathways by paraventricular tumours. However, regular follow-up of paraventricular nodules and maintenance of patency of cerebrospinal fluid pathways by microsurgical methods I n patients suffering from mild cerebral disorders offers a better chan ce of survival.