Roth Jonathan, Bergman Lottem, Weil Alexander G, Brunette-Clement Tristan, Weiner Howard L, Treiber Jeffrey M, Shofty Ben, Cukiert Arthur, Cukiert Cristine Mella, Tripathi Manjari, Sarat Chandra Poodipedi, Bollo Robert J, Machado Hélio Rubens, Santos Marcelo Volpon, Gaillard William D, Oluigbo Chima O, Ibrahim George M, Jallo George I, Shimony Nir, O'Neill Brent R, Budke Marcelo, Pérez-Jiménez María Ángeles, Mangano Francesco T, Iwasaki Masaki, Iijima Keiya, Gonzalez-Martinez Jorge, Kawai Kensuke, Ishishita Yohei, Elbabaa Samer K, Bello-Espinosa Luis, Fallah Aria, Maniquis Cassia A B, Ben-Zvi Ido, Tisdall Martin, Panigrahi Manas, Jayalakshmi Sita, Blount Jeffrey P, Dorfmüller Georg, Bulteau Christine, Stone Scellig S, Bolton Jeffrey, Singhal Ashutosh, Connolly Mary, Alsowat Daad, Alotaibi Faisal, Ragheb John, Uliel-Sibony Shimrit
Department of Pediatric Neurosurgery, Dana Children's Hospital, Tel Aviv Medical Center, Tel Aviv University, Tel Aviv, Israel.
Division of Neurosurgery, Department of Surgery, Sainte-Justine University Hospital Centre and University of Montreal Hospital Centre, Montreal, Quebec, Canada.
Epilepsia. 2023 Dec;64(12):3205-3212. doi: 10.1111/epi.17796. Epub 2023 Oct 20.
Lennox-Gastaut syndrome (LGS) is a severe form of epileptic encephalopathy, presenting during the first years of life, and is very resistant to treatment. Once medical therapy has failed, palliative surgeries such as vagus nerve stimulation (VNS) or corpus callosotomy (CC) are considered. Although CC is more effective than VNS as the primary neurosurgical treatment for LGS-associated drop attacks, there are limited data regarding the added value of CC following VNS. This study aimed to assess the effectiveness of CC preceded by VNS.
This multinational, multicenter retrospective study focuses on LGS children who underwent CC before the age of 18 years, following prior VNS, which failed to achieve satisfactory seizure control. Collected data included epilepsy characteristics, surgical details, epilepsy outcomes, and complications. The primary outcome of this study was a 50% reduction in drop attacks.
A total of 127 cases were reviewed (80 males). The median age at epilepsy onset was 6 months (interquartile range [IQR] = 3.12-22.75). The median age at VNS surgery was 7 years (IQR = 4-10), and CC was performed at a median age of 11 years (IQR = 8.76-15). The dominant seizure type was drop attacks (tonic or atonic) in 102 patients. Eighty-six patients underwent a single-stage complete CC, and 41 an anterior callosotomy. Ten patients who did not initially have a complete CC underwent a second surgery for completion of CC due to seizure persistence. Overall, there was at least a 50% reduction in drop attacks and other seizures in 83% and 60%, respectively. Permanent morbidity occurred in 1.5%, with no mortality.
CC is vital in seizure control in children with LGS in whom VNS has failed. Surgical risks are low. A complete CC has a tendency toward better effectiveness than anterior CC for some seizure types.
伦诺克斯 - 加斯托综合征（LGS）是一种严重的癫痫性脑病，在生命的最初几年发病，且对治疗具有很强的抵抗性。一旦药物治疗失败，就会考虑采用诸如迷走神经刺激（VNS）或胼胝体切开术（CC）等姑息性手术。尽管作为LGS相关跌倒发作的主要神经外科治疗方法，CC比VNS更有效，但关于VNS后CC的附加价值的数据有限。本研究旨在评估先进行VNS再进行CC的有效性。
这项多国、多中心的回顾性研究聚焦于18岁之前接受CC手术的LGS患儿，这些患儿之前接受过VNS但未能实现令人满意的癫痫发作控制。收集的数据包括癫痫特征、手术细节、癫痫结局和并发症。本研究的主要结局是跌倒发作减少50%。
共审查了127例病例（80例男性）。癫痫发作的中位年龄为6个月（四分位间距[IQR]=3.12 - 22.75）。VNS手术的中位年龄为7岁（IQR = 4 - 10），CC手术的中位年龄为11岁（IQR = 8.76 - 15）。102例患者的主要发作类型为跌倒发作（强直或失张力）。86例患者接受了一期完全胼胝体切开术，41例接受了前胼胝体切开术。10例最初未进行完全胼胝体切开术的患者因癫痫持续发作而接受了二期手术以完成胼胝体切开术。总体而言，分别有83%和60%的患者跌倒发作和其他发作至少减少了50%。永久性发病率为1.5%，无死亡病例。
对于VNS治疗失败的LGS患儿，胼胝体切开术在癫痫发作控制中至关重要。手术风险较低。对于某些发作类型，完全胼胝体切开术比前胼胝体切开术的疗效更佳。
