Régis Jean, Scavarda Didier, Tamura Manabu, Villeneuve Nathalie, Bartolomei Fabrice, Brue Thierry, Morange Isabelle, Dafonseca David, Chauvel Patrick
Department of Functional Neurosurgery, INSERM 751, Timone Hospital, Marseilles, France.
Semin Pediatr Neurol. 2007 Jun;14(2):73-9. doi: 10.1016/j.spen.2007.03.005.
Numerous neurosurgical approaches are available for children presenting with hypothalamic hamartomas (HHs) associated with severe epilepsy. A concern regarding the impairment of short-term memory after resective surgery is promoting the exploration of less invasive alternatives like radiosurgery. Gamma knife radiosurgery (GKS) can lead to a real reversal of the epileptic encephalopathy. Three years after radiosurgery, 60% of the children have an excellent result with complete seizure cessation in 40% and rare nondisabling seizures in 20%, often in association with dramatic behavioral and cognitive improvement. No permanent neurologic complications have thus far been reported. Rare transient cases of poikilothermia have been observed. GKS is clearly the safer approach for these difficult patients. Young patients with severe epilepsy and neurocognitive comorbidity must be treated by using a curative approach as early as possible. Topological type (according to our original classification) is the major feature for selection of the best treatment strategy. Type I HH deeply embedded in the hypothalamus is treated safely and efficiently by GKS. Type II HH can be resected by either endoscopic or transcallosal approaches or treated by GKS depending on the parent's choice and severity of epilepsy. In small type III HH, GKS is the safer procedure because of the very close relationship to the fornix and mammillary bodies. Types V (rarely epileptic) and IV are frequently operable by disconnection. Very large type VI (or mixed type) with a large component above the floor of the third ventricle must be disconnected, and then the upper remnant is best treated by GKS using a staged technique. Overall, when the lesion is sufficiently small, GKS offers a rate of seizure control comparable to microsurgery but with much lower risk. The disadvantage of radiosurgery is its delayed action. Longer follow-up is mandatory for a reliable evaluation of the role of GKS.
对于患有与严重癫痫相关的下丘脑错构瘤（HHs）的儿童，有多种神经外科手术方法可供选择。由于担心切除性手术后短期记忆受损，促使人们探索如放射外科等侵入性较小的替代方法。伽玛刀放射外科（GKS）可导致癫痫性脑病真正逆转。放射外科治疗三年后，60%的儿童效果极佳，其中40%完全停止发作，20%有罕见的非致残性发作，且往往伴有行为和认知的显著改善。迄今为止，尚未报告永久性神经并发症。观察到罕见的短暂性体温调节障碍病例。对于这些难治性患者，GKS显然是更安全的方法。患有严重癫痫和神经认知共病的年轻患者必须尽早采用根治性方法进行治疗。拓扑类型（根据我们原来的分类）是选择最佳治疗策略的主要特征。I型HH深深嵌入下丘脑，可通过GKS安全有效地治疗。II型HH可根据家长的选择和癫痫严重程度，通过内镜或经胼胝体入路切除或采用GKS治疗。对于小型III型HH，由于其与穹窿和乳头体关系非常密切，GKS是更安全的手术方法。V型（很少癫痫发作）和IV型通常可通过离断术进行手术。非常大的VI型（或混合型）且第三脑室底部上方有很大部分的，必须进行离断，然后上部残余部分最好采用分期技术通过GKS治疗。总体而言，当病变足够小时，GKS提供的癫痫控制率与显微手术相当，但风险要低得多。放射外科的缺点是其作用延迟。为了可靠评估GKS的作用，必须进行更长时间的随访。
