Savateev A N, Golanov A V, Saushev D A, Osinov I K, Kostyuchenko V V, Dalechina A V, Melikian A G, Vlasov P A, Mazerkina N A, Makashova E S
Moscow Gamma-knife Center, Moscow, Russia.
Burdenko Neurosurgical Center, Moscow, Russia.
Zh Vopr Neirokhir Im N N Burdenko. 2022;86(4):14-24. doi: 10.17116/neiro20228604114.
Hypothalamic hamartoma (HH) is a dysplastic lesion fused with hypothalamus and followed by epilepsy, precocious puberty and behavioral disorders. Up to 50% of patients become free of seizures after surgery, but various complications occur in 1/4 of cases. Radiofrequency thermocoagulation, laser interstitial thermal therapy and stereotactic radiosurgery (SRS) are alternative treatment options.
To define the indications for SRS in patients with HH and to clarify the irradiation parameters.
Twenty-two patients with HH and epilepsy underwent SRS at the Moscow Gamma-knife Center. A retrospective analysis included 19 patients with sufficient follow-up data. Median age of patients was 11.5 years (range 1.3-25.8). The diameter of irradiated HHs ranged between 5.5 and 40.9 mm. In 8 (36%) cases, the volume of hamartoma exceeded 3 cm. Mean prescribed dose was 18±2.0 Gy, mean prescribed isodose - 48±4.2%. Median follow-up period was 14.8 months (range 3.4 - 96.1).
Three (15.8%) patients were free of seizures. One patient (5.3%) improved dramatically after treatment with compete resolution of generalized seizures and experienced only rare emotional seizures (Engel IB). Eleven (57.8%) patients reported lower incidence of seizures. Severity and incidence of seizures were the same in 4 patients (21.1%). The best results were achieved in mean target dose over 20-22 Gy, minimal target dose over 7-10 Gy, covering by the prescribed dose of at least 70-80% of hamartoma volume, as well as in patients with the prescribed dose of 12 Gy delivered to almost entire volume of tumor. None patient had any complications after SRS.
SRS is safe regarding neurological, endocrine or visual disturbances. Careful patient selection for SRS makes it an effective option for HH-related epilepsy. The best candidates for SRS are children with seizures aged over 1 year, hamartoma <3 cm and area of fusion with hypothalamus <150 mm.
下丘脑错构瘤（HH）是一种与下丘脑融合的发育异常病变，可导致癫痫、性早熟和行为障碍。高达50%的患者术后癫痫发作停止，但四分之一的病例会出现各种并发症。射频热凝、激光间质热疗和立体定向放射外科（SRS）是替代治疗选择。
明确HH患者SRS的适应证并阐明照射参数。
22例患有HH和癫痫的患者在莫斯科伽玛刀中心接受了SRS治疗。回顾性分析纳入了19例有足够随访数据的患者。患者的中位年龄为11.5岁（范围1.3 - 25.8岁）。照射的HH直径在5.5至40.9毫米之间。8例（36%）病例中，错构瘤体积超过3立方厘米。平均处方剂量为18±2.0 Gy，平均处方等剂量线为48±4.2%。中位随访期为14.8个月（范围3.4 - 96.1个月）。
3例（15.8%）患者癫痫发作停止。1例患者（5.3%）治疗后显著改善，全身性癫痫发作完全缓解，仅偶尔出现情感性癫痫发作（Engel IB级）。11例（57.8%）患者报告癫痫发作发生率降低。4例患者（21.1%）癫痫发作的严重程度和发生率相同。平均靶剂量超过20 - 22 Gy、最小靶剂量超过7 - 10 Gy、处方剂量覆盖至少70 - 80%的错构瘤体积以及处方剂量12 Gy覆盖几乎整个肿瘤体积的患者取得了最佳效果。SRS治疗后无一例患者出现任何并发症。
SRS在神经、内分泌或视觉干扰方面是安全的。仔细选择SRS患者使其成为治疗HH相关癫痫的有效选择。SRS的最佳候选者是年龄超过1岁、错构瘤<3厘米且与下丘脑融合面积<150毫米的癫痫患儿。
