Division of Pediatric Neurosurgery, Department of Neurosurgery, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.
Department of Neurology and Developmental Neuroscience, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.
Neurosurgery. 2020 Jun 1;86(6):808-816. doi: 10.1093/neuros/nyz378.
Hypothalamic hamartomas (HH) are a challenging pathology that cause gelastic seizures. Magnetic Resonance Imaging-guided Laser Interstitial Thermal Therapy (MRgLITT) offers a safe and effective treatment for HHs via a minimally invasive technique.
To determine how clinical outcome correlates to residual tumor volume and surgical strategy by analyzing radiographic data and reconstructing volumetric imaging.
Clinical and radiographic information of 58 pediatric patients who underwent MRgLITT for HH with at least 6 mo of follow-up were retrospectively reviewed. MR imaging was volumetrically reconstructed to analyze the impact of hamartoma and ablation volumes on outcome. Primary outcome measure was freedom from gelastic seizures.
Eighty-one percent of patients were completely free of gelastic seizures at last follow-up; of 22 patients with secondary nongelastic epilepsy, 15 were free of additional seizures. Postoperative complication rate was low. There was no significant difference in gelastic seizure outcome related to pre- or postoperative hamartoma size. Residual hamartoma percentage in those free of gelastic seizures was 43% compared to 71% in those with continued seizures (P = .021). Larger hamartomas required multiple ablations to achieve seizure freedom.
This large series of patients confirms the safety and efficacy of MRgLITT for pediatric HH and describes morphological considerations that predict success. Our data suggest that complete ablation of the lesion is not necessary, and that the focus should be on appropriate disconnection of the epileptogenic network. We have found that a staged approach to hamartoma ablation allows adequate disconnection of the hamartoma while mitigating risk to surrounding structures.
下丘脑错构瘤（HH）是一种导致发笑性癫痫的具有挑战性的病变。磁共振成像引导激光间质热疗（MRgLITT）通过微创技术为 HH 提供了一种安全有效的治疗方法。
通过分析影像学数据和重建容积成像，确定临床结果与残余肿瘤体积和手术策略的相关性。
回顾性分析了 58 例接受 MRgLITT 治疗 HH 的儿科患者的临床和影像学资料，这些患者至少随访 6 个月。对 MR 图像进行容积重建，以分析错构瘤和消融体积对结果的影响。主要结局指标为无发笑性癫痫发作。
81%的患者在最后一次随访时完全无发笑性癫痫发作；22 例继发非癫痫性癫痫患者中，15 例无额外癫痫发作。术后并发症发生率低。发笑性癫痫发作的结果与术前或术后错构瘤大小无关。无发笑性癫痫发作患者的残余错构瘤百分比为 43%，而持续发作患者的残余错构瘤百分比为 71%（P =.021）。较大的错构瘤需要多次消融才能实现癫痫无发作。
本大量患者系列证实了 MRgLITT 治疗儿童 HH 的安全性和有效性，并描述了预测成功的形态学考虑因素。我们的数据表明，不需要完全消融病变，而应关注适当切断致痫性网络。我们发现，分阶段消融错构瘤的方法可以在减轻对周围结构风险的同时，充分切断错构瘤。
