Rosenfeld Jeffrey V, Feiz-Erfan Iman
Department of Neurosurgery, The Alfred Hospital and Monash University, Victoria, Australia.
Semin Pediatr Neurol. 2007 Jun;14(2):88-98. doi: 10.1016/j.spen.2007.03.007.
Multiple treatment options are available for patients with hypothalamic hamartoma (HH) including the frontotemporal approaches, the anterior transcallosal transseptal interforniceal approach, the transventricular endoscopic approach, and stereotactic radiosurgery. Relatively large patient series of the transcallosal resection/disconnection from Royal Children's Hospital in Melbourne and the Barrow Neurological Institute in Phoenix, AZ, show, respectively, that 52% to 54% are 100% seizure free, and 24% to 35% have >90% seizure reduction. However, there appears to be an 8% to 14% risk of persisting memory problems. The surgery should ideally be performed in the early years of childhood before secondary generalized epilepsy develops and developmental delay and behavioral problems are established. Radiosurgery may be a preferable option for higher-functioning adolescent or adult patients with HH. The choice of treatment must be individualized depending on the age and clinical circumstances of the patient and the size and anatomic relationships of the hamartoma. The transcallosal resection of HH is an effective and safe treatment, but there is a small risk of short-term memory impairment. The endoscopic approach is an alternative to the transcallosal approach for smaller HH.
下丘脑错构瘤（HH）患者有多种治疗选择，包括额颞入路、经胼胝体前经中隔穹窿间入路、经脑室内镜入路和立体定向放射外科治疗。墨尔本皇家儿童医院和亚利桑那州凤凰城巴罗神经学研究所关于经胼胝体切除术/离断术的相对大型患者系列研究分别显示，52%至54%的患者癫痫完全缓解，24%至35%的患者癫痫发作减少>90%。然而，持续存在记忆问题的风险似乎为8%至14%。理想情况下，手术应在儿童早期进行，即在继发性全身性癫痫发展以及发育迟缓与行为问题出现之前。对于功能较高的青少年或成年HH患者，放射外科治疗可能是更合适的选择。治疗方案的选择必须根据患者的年龄、临床情况以及错构瘤的大小和解剖关系进行个体化。HH的经胼胝体切除术是一种有效且安全的治疗方法，但存在短期记忆损害的小风险。对于较小的HH，内镜入路是经胼胝体入路的替代方法。
