Han Yong, Chen Min, Wang Hangzhou
Department of Neurosurgery, Children's Hospital of Soochow University, 92 Zhongnan Street, Suzhou, Jiangsu, 215006, People's Republic of China.
Childs Nerv Syst. 2022 May;38(5):903-907. doi: 10.1007/s00381-022-05466-y. Epub 2022 Feb 7.
Lateral meningocele syndrome (LMS) is a rare genetic connective tissue disorder which is associated with meningocele-related neurologic dysfunction. Several patients with LMS have been reported. But, guidelines for screening and treatment of LMS have not been established.
We review the current knowledge of LMS in the article. Then, we describe a boy for whom a genomic analysis which allowed us to make a diagnosis of LMS and to begin monitoring of his condition for possible neurological complications.
It would be difficult to make a diagnosis of LMS on the basis of clinical manifestations alone. The natural history of dural ectasia in patients with LMS needs to be better defined to establish surgical indications. Based upon the current literature, ventriculoperitoneal shunting (V-Ps) has been recommended as the first-line surgical treatment option for patients with symptomatic thoracolumbar meningoceles.
外侧脑脊膜膨出综合征(LMS)是一种罕见的遗传性结缔组织疾病,与脑脊膜膨出相关的神经功能障碍有关。已有数例LMS患者的报道。但是,尚未建立LMS的筛查和治疗指南。
我们在本文中回顾了LMS的现有知识。然后,我们描述了一个男孩,通过基因组分析对其进行诊断,从而能够对LMS进行诊断,并开始监测其病情以防可能出现的神经并发症。
仅根据临床表现很难诊断LMS。需要更好地明确LMS患者硬脊膜扩张的自然病程,以确定手术指征。根据目前的文献,脑室腹腔分流术(V-P分流术)已被推荐作为有症状的胸腰段脑脊膜膨出患者的一线手术治疗选择。