Ng Yu-tze, Rekate Harold L
Division of Neurology, Barrow Neurological Institute and St. Joseph's Hospital and Medical Center, Phoenix, AZ 85013, USA.
Semin Pediatr Neurol. 2007 Jun;14(2):99-105. doi: 10.1016/j.spen.2007.03.008.
The intrahypothalamic subtype of hypothalamic hamartoma (HH) is usually associated with refractory epilepsy, cognitive impairment, and organic behavioral disturbance. It is often a devastating disorder for both patient and family. The gelastic (laughing) seizure is the hallmark seizure type. However, multiple other seizure types can often develop during the course of the disease, and are typically refractory to antiepilepsy drugs (AEDs). Previously it was uncertain if HH tissue was responsible for seizure genesis or whether resection of the HH would result in improvement of the seizures. Recently both of these questions have been answered in the affirmative. Surgical resection using a transcallosal, interforniceal approach has recently been shown to be efficacious and generally safe for the treatment of the refractory seizures. However, even more recently, we have been performing the majority of HH surgical resection by using an endoscopic technique with a transventricular approach. This article presents the details of the operative technique and discusses preliminary outcome data, particularly with comparison to the transcallosal technique. Using an endoscope holder with micromanipulator facilitates endoscopic resection. Linking the endoscope to a system of frameless stereotaxis is essential for successful resection. Forty-four patients age 8 months to 44 years have undergone endoscopic resection. The ideal candidate for endoscopic removal has a hamartoma completely or nearly completely involving one wall of the third ventricle and is 1 cm or less in greatest diameter. Because it is essential to be able to visualize the lesion within the third ventricle for resection, there must be at least 6 mm of space between the top of the lesion and the roof of the third ventricle. Patients with intractable epilepsy caused by HH can be rendered seizure free or show marked improvement in seizure frequency by surgical removal, surgical disconnection, or radiosurgical ablation of the lesion. Which of these options should be recommended for an individual patient is not yet clear. One of the options involves resection or disconnection of the HH with a transventricular endoscopic approach. In selected patients, endoscopic resection of HH is effective in the treatment of intractable epilepsy, with lower complication rates and shorter hospital stays than transcallosal resection.
下丘脑错构瘤（HH）的下丘脑内型通常与难治性癫痫、认知障碍及器质性行为紊乱相关。对患者及其家庭而言，这往往是一种极具破坏性的疾病。痴笑发作是其标志性的发作类型。然而，在疾病过程中常常会出现多种其他发作类型，且通常对抗癫痫药物（AEDs）难治。此前，尚不确定HH组织是否是癫痫发作的根源，也不确定切除HH是否会使癫痫发作得到改善。最近，这两个问题的答案均为肯定。经胼胝体、穹窿间入路的手术切除最近已被证明对治疗难治性癫痫有效且总体安全。然而，更近一些时候，我们大多采用经脑室入路的内镜技术来进行HH的手术切除。本文介绍了手术技术细节，并讨论了初步的结果数据，特别是与经胼胝体技术相比较的情况。使用带有微操作器的内镜固定器有助于内镜切除。将内镜与无框架立体定向系统相连对于成功切除至关重要。44例年龄在8个月至44岁的患者接受了内镜切除。内镜切除的理想候选者是错构瘤完全或几乎完全累及第三脑室的一侧壁且最大直径为1厘米或更小。因为在第三脑室内可视化病变对于切除至关重要，所以病变顶部与第三脑室顶部之间必须至少有6毫米的空间。由HH引起的难治性癫痫患者通过手术切除、手术离断或放射外科消融病变可实现无癫痫发作或癫痫发作频率显著改善。对于个体患者应推荐哪种选择尚不清楚。其中一种选择是经脑室内镜入路切除或离断HH。在选定的患者中内镜切除HH治疗难治性癫痫有效，与经胼胝体切除相比并发症发生率更低且住院时间更短。
