Weiner Howard L, Carlson Chad, Ridgway Emily B, Zaroff Charles M, Miles Daniel, LaJoie Josiane, Devinsky Orrin
Division of Pediatric Neurosurgery, Department of Neurosurgery, New York University Medical Center, New York, New York 10016, USA.
Pediatrics. 2006 May;117(5):1494-502. doi: 10.1542/peds.2005-1206.
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.
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.
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.
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.
结节性硬化症（TSC）与儿童难治性癫痫和发育迟缓相关，通常由皮质结节引起。年轻患者开始发作的癫痫往往难治，且可能导致发育迟缓。早期控制癫痫发作可改善功能预后。既往报道显示，大龄儿童手术切除单个结节/癫痫病灶有一定益处；然而，许多TSC患儿在1岁前癫痫发作就无法得到控制。为了确定可能从手术中获益的患者并使预后最大化，我们对幼儿采用了一种新型手术方法，该方法包括有创颅内监测，通常分3个阶段进行，且常为双侧。
在过去6年中，由一位外科医生连续为110名儿童进行癫痫手术，其中25例（9名男孩和16名女孩）患有TSC。在我们机构首次手术时，他们的中位年龄为4.0岁。这25例患者共进行了31次癫痫手术入院治疗。13例术前癫痫发作不能明确定位的患儿进行了双侧电极植入，22例患者接受了3阶段手术。
初次切除术后6个月或更长时间，21例（84%）患儿为I级，2例（8%）患儿为II级，2例（8%）患儿为IV级。平均随访28个月时，17例（68%）患儿为I级，6例（24%）为II级，2例（8%）为III级。最初因病灶多发而被拒绝作为手术候选者且需要最初双侧电极研究的患儿中有4例现在已无癫痫发作。
这种方法有助于识别患有多个结节的年轻TSC患者的原发性和继发性致痫区。多个或双侧癫痫病灶不一定是手术禁忌证。长期随访将确定这种方法是否有持久效果。
