Park Jun T, Manjila Sunil V, Tangen Rachel B, Cohen Mark L, Shahid Asim M, Sweet Jennifer A, Tuxhorn Ingrid E, Miller Jonathan P
Section of Pediatric Epilepsy and.
Epilepsy Center and.
J Neurosurg Pediatr. 2016 Jun;17(6):679-82. doi: 10.3171/2015.11.PEDS15495. Epub 2016 Feb 12.
Catastrophic epilepsy in infants, often due to extensive cortical dysplasia, has devastating consequences with respect to brain development. Conventional lobar, multilobar, or hemispheric resection in these infants is challenging, carrying an increased operative risk compared with that in older children. Removing a larger tissue volume versus removing or disconnecting the epileptogenic region does not always guarantee better seizure outcome. The authors describe 2 infants with catastrophic epilepsy who benefited from individually tailored disconnections based on a hypothesized epileptogenic zone following intensive presurgical evaluation. Two infants with catastrophic epilepsy and epileptic spasms underwent leukotomies between 3 and 12 months of age. They were followed up postoperatively for 19-36 months. Both patients had 90%-100% seizure reduction and a significantly improved neurodevelopmental outcome without postoperative complication. Cortical malformation was seen in both patients. Modifications of established surgical disconnection techniques, tailored to each patient's specific epileptogenic zone, optimized seizure and neurodevelopmental outcomes while minimizing the risks associated with more extensive resections.
婴儿灾难性癫痫通常由广泛的皮质发育异常引起，对脑发育具有毁灭性后果。在这些婴儿中进行传统的叶、多叶或半球切除术具有挑战性，与大龄儿童相比手术风险增加。切除更大的组织体积与切除或离断致痫区域相比，并不总是能保证更好的癫痫发作结果。作者描述了2例灾难性癫痫婴儿，他们在经过强化术前评估后，根据假设的致痫区接受了个体化定制的离断手术，从中获益。2例患有灾难性癫痫和癫痫性痉挛的婴儿在3至12个月大时接受了白质切开术。术后对他们进行了19至36个月的随访。两名患者的癫痫发作均减少了90%-100%，神经发育结局显著改善，且无术后并发症。两名患者均存在皮质畸形。针对每个患者的特定致痫区对既定的手术离断技术进行改良，在将与更广泛切除术相关的风险降至最低的同时，优化了癫痫发作和神经发育结局。
