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Epilepsy surgery in young children with tuberous sclerosis: results of a novel approach
Howard L Weiner1, Chad Carlson, Emily B Ridgway
1Division of Pediatric Neurosurgery, Department of Neurosurgery, New York University Medical Center, New York, New York 10016, USA. howard.weiner@med.nyu.edu
Insights
This study shows that a novel surgical approach for young children with tuberous sclerosis complex (TSC) epilepsy can effectively identify and treat seizure foci, leading to significant seizure reduction and improved outcomes in most patients.
Area of Science:
- Pediatric Neurology
- Neurosurgery
- Epileptology
Background:
- Tuberous sclerosis complex (TSC) frequently causes medically refractory epilepsy and developmental delay in children, often originating from cortical tubers.
- Early seizure control is crucial for improving functional outcomes in young patients with TSC.
- Conventional surgical approaches for single tubers have shown limited success in older children, highlighting a need for effective strategies in younger populations.
Purpose of the Study:
- To evaluate a novel surgical approach using invasive intracranial monitoring in young children with TSC to identify and treat epilepsy.
- To determine the efficacy of this approach in managing medically refractory seizures in TSC patients.
- To assess the impact of early surgical intervention on functional outcomes in pediatric TSC.
Main Methods:
- A cohort of 25 children with TSC underwent epilepsy surgery, with a median age of 4.0 years at their first surgery.
- The surgical approach involved invasive intracranial monitoring, including 3-stage surgeries in 22 patients and bilateral electrode placement in 13 children.
- Data from 31 separate admissions for epilepsy surgery were analyzed.
Main Results:
- At 6 months post-surgery, 84% of children achieved Class I seizure control.
- At a mean follow-up of 28 months, 68% of children remained seizure-free (Class I).
- Four children initially deemed ineligible due to multifocality became seizure-free after bilateral electrode study.
Conclusions:
- The described surgical approach effectively identifies primary and secondary epileptogenic zones in young TSC patients, even with multiple tubers.
- Multiple or bilateral seizure foci do not preclude successful surgical intervention in TSC.
- Further long-term follow-up is necessary to confirm the durability of seizure control achieved with this method.
Objective:
Tuberous sclerosis complex (TSC) is associated with medically refractory epilepsy and developmental delay in children and usually results from cortical tubers. Seizures that begin in young patients are often refractory and may contribute to development delay. Functional outcome is improved when 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 1. To identify patients who might benefit from surgery and to maximize outcome, we used a novel surgical approach in young children that consists of invasive intracranial monitoring, which is typically 3-staged and often bilateral.
Methods:
Of 110 consecutive children who underwent epilepsy surgery by a single surgeon in the past 6 years, 25 patients (9 boys and 16 girls) had TSC. At the time of their first surgery at our institution, they were a median age of 4.0 years. A total of 31 separate admissions for epilepsy surgery in these 25 patients were identified. Bilateral electrode placement was performed in 13 children whose seizures could not be lateralized definitively preoperatively, and 22 patients underwent 3-stage surgeries.
Results:
At 6 months or longer after the initial resection, 21 (84%) children were class I, 2 (8%) children were class II, and 2 (8%) children were class IV. At a mean follow-up of 28 months, 17 (68%) children were class I, 6 (24%) were class II, and 2 (8%) were class III. Four of the 5 children who initially were rejected as surgical candidates because of multifocality and who required initial bilateral electrode study are now seizure-free.
Conclusions:
This approach can help to identify 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.
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