Neurosurgical aspects in the treatment of children with tuberous sclerosis complex

: Tuberous sclerosis complex (TSC) is a genetic syndrome, characterized by seizures, mental retardation, and a typical facial rash. Many patients have early onset seizures (usually during infancy) which are refractory to medical treatment. It has been shown that the neurocognitive decline in TSC patients is associated with the severity of seizures. The cerebral pathologies in TSC include - tubers (associated with seizures), benign tumors (subependymal giant cell astrocytomas - SEGAs), and intraventricular subependymal nodules (SENs). Until recent years, TSC patients with refractory seizures were only rarely considered to be suitable for epilepsy surgery. Over the past few years, several pre-surgical and surgical techniques have enabled patients to undergo epilepsy surgery with a high rate of seizure control. These techniques include invasive monitoring, mapping and resection of epileptogenic foci. The overall seizure outcome is around 75% seizure freedom. Additionally, benign brain tumors unique for TSC (SEGAs) present a treatment challenge. These tumors are typically located adjacent to the foramen of Monro. Without treatment the tumors may obstruct the CSF pathways leading to hydrocephalus. Current treatment options include surgical approaches, as well as medical treatment with mTOR inhibitors. The combination of medical and surgical treatments may decrease morbidity especially in partially resected tumors, multifocal tumors, or as neoadjuvant treatment in selected cases. In the current paper we review the neurosurgical aspects in the treatment of patients with TSC.