Mosleh Mohammad Mohsen, Sohn Moon-Jun
Department of Medicine, Graduate School of Medicine, Inje University, 75 Bokji-ro, Busanjin-gu, Busan 47392, Republic of Korea.
Department of Neurosurgery, Neuroscience and Radiosurgery Hybrid Research Center, Inje University Ilsan Paik Hospital, College of Medicine, Juhwa-ro 170, Ilsanseo-gu, Goyang City 10380, Republic of Korea.
Children (Basel). 2024 Aug 9;11(8):961. doi: 10.3390/children11080961.
This review highlights the need for therapeutic guidelines for syringomyelia associated with tethered cord syndrome (TCS) caused by spinal dysraphism (SD). A comprehensive literature review was conducted, selecting twelve articles to analyze common therapeutic strategies. Surgical cord untethering alone has recently become a preferred treatment, with 45 ± 21.1% of patients experiencing remission or improvement, 47 ± 20.4% unchanged and asymptomatic, and 4 ± 8% worsened. Untethering with direct surgical drainage for the syrinx had better outcomes than untethering alone (78% vs. 45%, = 0.05). Terminal syringostomy was beneficial for syrinxes extending to the filum terminale but not for asymptomatic small syrinxes with a syrinx index < 0.4. Syrinx shunting was recommended for symptomatic large syrinxes (>2 cm in length and syrinx index > 0.5). Various shunt procedures for syrinxes are still advocated, mainly for refractory syringomyelia in Chiari malformation, posttraumatic cases, SD, or other causes. Personalized surgical methods that address the root cause of syringomyelia, particularly those improving cerebrospinal fluid flow, offer promising results with minimized complications. Ongoing studies are required to enhance management strategies for syringomyelia associated with TCS, optimize patient outcomes, and reduce the risk of recurrent symptoms.
本综述强调了针对由脊柱裂（SD）引起的脊髓栓系综合征（TCS）相关的脊髓空洞症制定治疗指南的必要性。进行了全面的文献综述，选择了12篇文章来分析常见的治疗策略。单纯手术松解脊髓栓系最近已成为首选治疗方法，45±21.1%的患者病情缓解或改善，47±20.4%的患者病情无变化且无症状，4±8%的患者病情恶化。脊髓空洞症采用直接手术引流同时松解脊髓栓系的效果优于单纯松解脊髓栓系（78%对45%，P = 0.05）。终丝脊髓空洞造瘘术对延伸至终丝的脊髓空洞症有益，但对脊髓空洞指数<0.4的无症状小脊髓空洞症无效。对于有症状的大脊髓空洞症（长度>2 cm且脊髓空洞指数>0.5），建议行脊髓空洞分流术。目前仍提倡针对脊髓空洞症的各种分流手术，主要用于治疗Chiari畸形、创伤后病例、SD或其他原因引起的难治性脊髓空洞症。针对脊髓空洞症病因的个性化手术方法，尤其是那些改善脑脊液流动的方法，有望取得良好效果并将并发症降至最低。需要进行持续研究以加强对TCS相关脊髓空洞症的管理策略，优化患者预后，并降低症状复发的风险。
