Procaccini Emidio, Dorfmüller Georg, Fohlen Martine, Bulteau Christine, Delalande Olivier
Division of Pediatric Neurosurgery, Fondation Adolphe de Rothschild, Paris, France.
Neurosurgery. 2006 Oct;59(4 Suppl 2):ONS336-44; discussion ONS344-6. doi: 10.1227/01.NEU.0000233900.06146.72.
Hypothalamic hamartomas (HHs) require surgical treatment in patients presenting with refractory epilepsy.
The authors report on a single-center series of 33 patients (24 males, 9 females) who underwent surgery between January 1997 and April 2004. They experienced several types of seizure (gelastic, tonic, partial, atonic, generalized tonic-clonic, dacrystic, infantile spasm, mental retardation, and behavioral and endocrinological abnormalities). Forty-nine interventions were carried out. Every patient, with the exception of the first, underwent hamartoma disconnection (pterional approach, six patients; endoscopy, 15 patients; both, 11 patients). The endoscopic approach was carried out with a frameless stereotactic system to enhance feasibility and efficacy of the disconnecting procedure.
Surgery-related neurological complications occurred in two patients, both after a pterional microsurgical approach. Furthermore, two patients experienced panhypopituitarism and one patient experienced transitory central insipid diabetes. All patients but one showed recovery or considerable improvement of their epilepsy (Engel Class 1, 48.5%; Engel Class 2, 3%; Engel Class 3, 45.5%; mean follow-up duration, 1 yr 7 mo).
According to the proposed classification of sessile HH into four types, the best candidates for endoscopic disconnection are Type 2 and Type 3 HHs. In the present series, 90% of patients affected by Type 2 HH became seizure free and the remaining 10% improved; of those with Type 3 HH at presentation, 35.3% recovered and 60% improved. Neuropsychological and endocrinological test results showed improvement in many patients. Data from our series demonstrate that frameless stereotactic endoscopic disconnection should be considered as the treatment of choice in the presence of favorable anatomic conditions.
对于患有难治性癫痫的患者，下丘脑错构瘤（HHs）需要进行手术治疗。
作者报告了1997年1月至2004年4月间在单中心接受手术的33例患者（24例男性，9例女性）的系列病例。他们经历了几种类型的癫痫发作（痴笑性、强直性、部分性、失张力性、全身强直阵挛性、哭泣性、婴儿痉挛、智力迟钝以及行为和内分泌异常）。共进行了49次干预。除第一例患者外，每位患者均接受了错构瘤离断术（翼点入路6例患者；内镜手术15例患者；两种方法联合11例患者）。内镜手术采用无框架立体定向系统进行，以提高离断手术的可行性和有效性。
两名患者出现了与手术相关的神经并发症，均发生在翼点显微手术之后。此外，两名患者出现了全垂体功能减退，一名患者出现了暂时性中枢性尿崩症。除一名患者外，所有患者的癫痫均有恢复或显著改善（Engel 1级，48.5%；Engel 2级，3%；Engel 3级，45.5%；平均随访时间为1年7个月）。
根据提出的将无柄HH分为四种类型的分类方法，内镜离断术的最佳适应证是2型和3型HHs。在本系列中，2型HH患者中有90%癫痫发作停止，其余10%有所改善；3型HH患者中，35.3%恢复，60%有所改善。神经心理学和内分泌学检查结果显示许多患者有所改善。我们系列的数据表明，在具备有利解剖条件时，应将无框架立体定向内镜离断术视为首选治疗方法。
