CASE REPORT - TUBEROUS SCLEROSIS AND ACUTE HYDROCEPHALUS

Tuberous sclerosis complex is a neurocutaneous and autosomal dominant disease characterized by multiple hamartomas in multiple viscera. It results from spontaneous mutation. The genetic anomaly is usually linked to the 9th chromosome. It may be accompanied by early childhood seizures, multiple brain tumors, skin lesions, angiomyolipomas in the kidneys and liver and rhabdomyomas. A careful physical examination, computerized tomography (CT) and magnetic resonance imaging (MRI) scans of the brain are essential in its diagnosis. In this study, we presented a 16 year old girl who was brought to our emergency service room due to acute loss of conscious and then underwent to ventriculo-peritoneal (V-P) shunt procedure after she had been diagnosed as acute hydrocephalus. The patient had been operated for intracranial mass when she was 2 years old and postoperative pathological diagnosis was established to be subepandimal giant cell astrocytoma (SGCA).