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Weiner HL, Ferraris N, LaJoie J, Miles D, Devinsky O.
J Child Neurol. 2004 Sep;19(9):687-9.
Objective
Tuberous Sclerosis Complex (TSC) is associated with medically refractory epilepsy and developmental delay in children usually resulting from cortical tubers, the areas of cortical dysplasia surrounded by reactive gliosis. Seizures that begin in young patients are often refractory and may contribute to development delay. Functional outcome is improved if seizures are controlled at an early age. Previous reports have shown modest benefit from surgical resection of single tubers/seizure foci in older children. However, many children with TSC develop uncontrolled seizures before age one. Moreover, these patients often have multiple potentially epileptogenic tubers, rendering localization challenging, and are therefore rejected as potential surgical candidates. To identify patients who might benefit from surgery, and to maximize outcome, we have utilized a novel surgical approach in young children consisting of invasive intracranial monitoring, which is typically multi-staged and bilateral.
Methods
Of 110 consecutive children who underwent epilepsy surgery by a single surgeon in the last 6 years, 25 (9M; 16F; mean age 3.0 years, range 7 months-16 years) had TSC. Six failed VNS (vagal nerve stimulator) placement and 1 failed prior resection elsewhere. Bilateral electrode placement was performed in 13 children whose seizures could not be lateralized definitively preoperatively. Twenty-two children had 3-stage surgery and three had 2-stage (72 operations). 20/25 patients underwent resection of two or more tubers, and two had bilateral resections. Two children failed initial surgery, requiring re-operation approximately one year postoperatively.
Results
At a mean follow-up of 24 months (range 3 months-6 years), 17 children (68%) are seizure free (Class I), 7 (28%) are > 90% improved (Class II), 1 (4%) is > 50% improved (Class III). 4 of the 5 children initially rejected as surgical candidates because of multifocality, and who required initial bilateral electrode study, are now seizure free.
Conclusions
This approach has been useful in identifying both primary and secondary epileptogenic zones in young TSC patients with multiple tubers. Multiple or bilateral seizure foci are not necessarily a contraindication to surgery. Long-term follow up will determine whether this approach has durable effects. TSC may be a paradigm for understanding the role of surgery in lesional epilepsy of childhood.
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