国家神经系统疾病临床医学研究中心数据管理与共享系统
Data Management And Sharing System of China National Clinical Research Center for Neurological Diseases
Pediatric intramedullary schwannoma with syringomyelia: a case report and literature review.
第一作者：Wang Keda, Keda
通讯作者：Zhao Jizong, Jizong
基本信息
- 负责人：Wang Keda, Keda
- 数据来源： OTHERS
- 论文编号：OTHERS20190712
- 发表时间：2018-11-28
2018-11-28
已发表文章
主要研究背景及目的
方法
We reviewed the clinical course of a 9-year-old boy, who presented with both lower extremity weakness of 6-month duration. Neurophysical examination revealed a decreased sensation below the T10 dermatome. Magnetic resonance imaging (MRI) showed an well-demarcated intramedullary lesion located at the level of T8 vertebra with isointensity on T2WI and hypointensity on T1WI, which was homogeneous enhanced after gadolinium injection. There was associated syringomyelia extending from T7 down to the level of T10. A mild scoliotic deformity was also observed. The lesion was totally resected after an T7-T8 laminoplasty. Histopathological findings were consistent with schwannoma. Postoperative MRI did not reveal the presence of a residual tumor with syringomyelia reducted. By 2 weeks after treatment, the patient had experienced nearly complete recovery. Management with external bracing was performed on this patient for 3 months after surgery to prevent spinal deformity. However, mild spinal kyphosis occurred 5 months after surgery, and a progressive postoperative spinal kyphosis was observed during these 3 years of follow-up. Continued conservative management with observation was performed as there is no association with functional decline and impairment in health-related quality-of-life measures.
结论
Although extremely rare and uncommonly associated with syringomyelia, schwannomas need to be considered in the preoperative diagnosis of solitary intramedullary tumors in children as total resection can be achieved improving surgical outcome; Pediatric patients should be monitored closely for the development of spinal deformity following resection of intramedullary schwannoma, particularly possessing preoperative scoliotic deformity and/or tumor-associated syringomyelia.