Fohlen Martine, Lellouch Arielle, Delalande Olivier
Unité de Neurochirurgie Pédiatrique, Fondation Ophtalmologique A de Rothschild, Paris, France.
Epileptic Disord. 2003 Dec;5(4):267-73.
To study the surgical procedures and results on seizures, in 18 patients with refractory epilepsy due to hypothalamic hamartoma.
Eighteen patients aged from 9 months to 32 years underwent surgery between 1997 and 2002. The mean age at seizure onset was 15.5 months. Seventeen patients had gelastic seizures, 14 had partial seizures, two had infantile spasms, 10 had tonic or atonic seizures and three had generalized seizures. The mean seizure frequency was 21 per day. Four patients had borderline intellectual disability and the others were mentally retarded. Five patients had precocious puberty, one had acromegaly and four suffered from obesity. Brain MRI, performed at least twice in each patient showed the hamartoma as a stable, homogeneous interpeduncular mass implanted either on the mammillary tubercle or on the wall of the third ventricle, with variable extension to the bottom. Ictal SPECT, performed in four patients, showed hyperperfusion within the hamartoma in two.
Twenty-six operations were performed in 18 patients. The first patient underwent a total removal of the hamartoma, whereas the following patients underwent a disconnection, either through open surgery (14 patients) or endoscopy (10 patients).
Regarding the seizure outcome with a mean follow up of three years four months (one year to 4.5 years), nine patients are seizure-free, one patient has only brief gelastic seizures and eight are dramatically improved. Surgery was uncomplicated in all but two patients: one had transitory hemiplegia and paresis of the third cranial nerve, the other presented with hemiplegia due to ischemia of the middle cerebral artery territory. The quality of life, behavior and school performance were greatly improved in most of the patients.
Our series illustrates the feasibility and relative safety of disconnecting surgery of hypothalamic hamartomas, with seizure relief in 50% and a dramatic improvement in the others. Endoscopic disconnection seems to be a very safe way to treat the intra-ventricular part of hamartomas.
研究18例下丘脑错构瘤所致难治性癫痫患者的手术方法及癫痫治疗效果。
1997年至2002年间，18例年龄从9个月至32岁的患者接受了手术。癫痫发作的平均起始年龄为15.5个月。17例患者有痴笑性癫痫发作，14例有部分性发作，2例有婴儿痉挛症，10例有强直或失张力发作，3例有全身性发作。癫痫发作的平均频率为每天21次。4例患者有边缘智力障碍，其他患者均为智力发育迟缓。5例患者有性早熟，1例有肢端肥大症，4例患有肥胖症。每位患者至少进行了两次脑部MRI检查，结果显示错构瘤为稳定的、均匀的脚间池肿块，位于乳头结节或第三脑室壁上，底部延伸范围不一。4例患者进行了发作期SPECT检查，其中2例显示错构瘤内有血流灌注增加。
18例患者共进行了26次手术。首例患者接受了错构瘤全切术，其余患者则进行了离断术，其中14例通过开颅手术，10例通过内镜手术。
平均随访三年零四个月（1年至4.5年），9例患者无癫痫发作，1例患者仅有短暂的痴笑性癫痫发作，8例患者有显著改善。除2例患者外，手术均无并发症：1例患者出现短暂性偏瘫和第三脑神经麻痹，另1例患者因大脑中动脉区域缺血出现偏瘫。大多数患者的生活质量、行为和学习成绩都有很大改善。
我们的系列研究表明，下丘脑错构瘤离断术具有可行性和相对安全性，50%的患者癫痫得到缓解，其他患者有显著改善。内镜离断术似乎是治疗错构瘤脑室内部分的一种非常安全的方法。
