Department of Neurosurgery, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.
National Institute of Health Research (NIHR) Global Health Research Group on Neurotrauma, University of Cambridge, Cambridge, UK.
Indian J Pediatr. 2021 Nov;88(11):1086-1091. doi: 10.1007/s12098-020-03637-w. Epub 2021 Jan 27.
To describe the feasibility, safety, efficacy, and complication profile of primary hypofractionated gamma knife radiosurgery (GKRS), and practical nuances of performing the same in pediatric patients.
Three pediatric patients (age range 17-65 mo) underwent primary hypofractionated GKRS in 2-3 consecutive days with interfraction interval of 24 h. All patients had precocious puberty and were on GnRH analogue. Frame based GKRS done with 8.1-9.2 Gy radiation per fraction at 50% isodose in 2-3 fractions targeting the entire hamartoma volume. The mean target volume was 5.67 cc (4.45-7.39 cc). The authors followed these patients for clinical and endocrinological assessment at every 6 mo interval while the repeat MRI done at 6 mo and then annually. The seizure outcome analysis was done using Engel scale.
At a mean follow up of 27 mo (24-30 mo), 2 patients became Engel class 3 while one achieved Engel class 1 control. 2 patients showed halted pubertal growth with no additional hormonal aberration. 2 patients showed significant volumetric reduction (48% and 32%) and patchy necrosis inside the hypothalamic hamartoma (HH). There was no deficit in visual function, memory and cognition. One patient showed reduction in aggressiveness.
Giant HH are exceptionally difficult neurological diseases. Primary hypofractionated GKRS may be an alternative approach as mono/multitherapy with promising results and minimal complication.
描述原发性低分割伽玛刀放射外科（GKRS）的可行性、安全性、疗效和并发症概况，以及在儿科患者中实施该手术的实际细节。
3 名年龄在 17-65 个月的儿科患者在 2-3 天内分 2-3 次进行原发性低分割 GKRS，每次间隔 24 小时。所有患者均患有性早熟，并接受 GnRH 类似物治疗。采用框架式 GKRS，每次分割 8.1-9.2Gy 辐射，在 50%等剂量线处治疗整个错构瘤体积，共 2-3 次分割。平均靶体积为 5.67cc（4.45-7.39cc）。作者在每 6 个月的间隔对这些患者进行临床和内分泌评估，同时在 6 个月和每年进行重复 MRI。使用 Engel 量表对癫痫结果进行分析。
在平均 27 个月（24-30 个月）的随访中，2 名患者达到 Engel 3 级，1 名患者达到 Engel 1 级控制。2 名患者出现青春期生长停滞，无其他激素异常。2 名患者显示出显著的体积减少（48%和 32%）和下丘脑错构瘤（HH）内部的斑片状坏死。视觉功能、记忆和认知没有缺陷。1 名患者表现出攻击性降低。
巨大的 HH 是一种特殊的神经疾病。原发性低分割 GKRS 可能是一种替代方法，可作为单一/多疗法，具有良好的疗效和最小的并发症。
