Silva Adikarige H D, Lo William B, Mundil Nilesh R, Walsh A Richard
J Neurosurg Pediatr. 2020 Feb 28;25(6):588-596. doi: 10.3171/2019.12.PEDS19231. Print 2020 Jun 1.
The surgical approach to hypothalamic hamartomas (HHs) associated with medically refractory epilepsy is challenging because of these lesions' deep midline or paramedian location. Whether the aim is resection or disconnection, the surgical corridor dictates how complete a procedure can be achieved. Here, the authors report a transtemporal approach suitable for Delalande type I, inferior extraventricular component of type III, and type IV lesions. This approach provides optimal visualization of the plane between the hamartoma and the hypothalamus with no manipulation to the pituitary stalk and brainstem, allowing for extensive disconnection while minimizing injury to adjacent neurovascular structures.Through a 1-cm corticectomy in the middle temporal gyrus, a surgical tract is developed under neuronavigational guidance toward the plane of intended disconnection. On reaching the mesial temporal pia-arachnoid margin, it is opened, providing direct visualization of the hamartoma, which is then disconnected or resected as indicated. Critical neurovascular structures are generally not exposed through this approach and are preserved if encountered.Three patients (mean age 4.9 years) with intractable epilepsy were treated using this technique as part of the national Children's Epilepsy Surgery Service. Following resection, the patient in case 1 (Delalande type I) is seizure free off medication at 3 years' follow-up (Engel class IA). The patient in case 2 (Delalande type III) initially underwent partial disconnection through a transcallosal interforniceal approach and at first had significant seizure improvement before the seizures worsened in frequency and type. Complete disconnection of the residual lesion was achieved using the transtemporal approach, rendering this patient seizure free off medication at 14 months postsurgery (Engel class IA). The patient in case 3 (Delalande type IV) underwent incomplete disconnection with a substantial reduction in seizure frequency at 3 years' follow-up (Engel class IIIC). There were no surgical complications in any of the cases.The transtemporal approach is a safe and effective alternative to more conventional surgical approaches in managing HHs with intractable epilepsy.
由于下丘脑错构瘤（HHs）位于深部中线或中线旁位置，因此对于药物难治性癫痫相关的下丘脑错构瘤进行手术治疗具有挑战性。无论目标是切除还是离断，手术通道决定了手术能够达到的完整程度。在此，作者报告了一种适用于Delalande I型、III型脑室下外侧部分及IV型病变的经颞叶入路。这种入路能够最佳地显示错构瘤与下丘脑之间的平面，而不对垂体柄和脑干进行操作，从而在最大限度减少对相邻神经血管结构损伤的同时实现广泛离断。通过在颞中回进行1厘米的皮质切除术，在神经导航引导下开辟一条手术通道，通向预期离断的平面。到达颞叶内侧软膜 - 蛛网膜边缘后将其打开，直接显露错构瘤，然后根据情况进行离断或切除。通过这种入路一般不会暴露关键神经血管结构，若遇到则予以保留。
三名难治性癫痫患者（平均年龄4.9岁）作为国家儿童癫痫外科服务的一部分接受了该技术治疗。切除术后，病例1（Delalande I型）患者在3年随访时停药无癫痫发作（Engel IA级）。病例2（Delalande III型）患者最初通过经胼胝体穹窿间入路进行了部分离断，起初癫痫发作有明显改善，但随后发作频率和类型恶化。使用经颞叶入路实现了残留病变的完全离断，该患者术后14个月停药无癫痫发作（Engel IA级）。病例3（Delalande IV型）患者进行了不完全离断，在3年随访时癫痫发作频率大幅降低（Engel IIIC级）。所有病例均无手术并发症。
在治疗伴有难治性癫痫的下丘脑错构瘤时，经颞叶入路是一种比传统手术方法更安全有效的替代方法。
