BACKGROUND 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. OBJECTIVE To determine how clinical outcome correlates to residual tumor volume and surgical strategy by analyzing radiographic data and reconstructing volumetric imaging. METHODS 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. RESULTS 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. CONCLUSION 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.

中文翻译：

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下丘脑错构瘤的分阶段磁共振引导激光间质热治疗：消融体积和形态学考虑分析

背景下丘脑错构瘤 (HH) 是一种具有挑战性的病理学，会导致凝胶性癫痫发作。磁共振成像引导的激光间质热疗 (MRgLITT) 通过微创技术为 HHs 提供安全有效的治疗。目的 通过分析放射影像数据和重建体积成像，确定临床结果如何与残留肿瘤体积和手术策略相关。方法 回顾性分析 58 例接受 MRgLITT 治疗 HH 且至少随访 6 个月的儿科患者的临床和影像学信息。MR 成像被体积重建以分析错构瘤和消融体积对结果的影响。主要结局指标是无凝胶性癫痫发作。结果 81% 的患者在最后一次随访时完全没有凝胶性癫痫发作；在 22 名继发性非凝胶性癫痫患者中，15 名没有额外的癫痫发作。术后并发症发生率低。与术前或术后错构瘤大小相关的凝胶性癫痫发作结果没有显着差异。没有凝胶性癫痫发作的患者的残余错构瘤百分比为 43%，而持续癫痫发作的患者则为 71% (P = .021)。较大的错构瘤需要多次消融才能实现无癫痫发作。结论 这一大系列患者证实了 MRgLITT 对儿科 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，重点应放在适当断开致痫网络上。我们发现，错构瘤消融的分阶段方法可以充分断开错构瘤，同时减轻对周围结构的风险。