摘要： 目的 评估终丝离断术对不同年龄段终丝脂肪浸润/脂肪瘤型脊髓拴系综合征患儿的治疗结局，探索此种类型脊髓拴系综合征的自然病程和治疗策略。方法与结果 回顾分析2017年1月至2022年5月在复旦大学附属儿科医院行终丝离断术治疗的77例终丝脂肪浸润/脂肪瘤型脊髓拴系综合征患儿的临床资料，不同组别［幼儿组（< 3岁）、学龄前组（3~6岁）和学龄组（> 6岁）］患儿入院原因（Fisher确切概率法：P=0.001）、骶尾部皮肤异常者比例（χ²=7.841，P=0.021）、存在临床症状比例（χ²=13.618，P=0.001）及圆锥末端位置（Fisher确切概率法：P=0.039）差异具有统计学意义。与幼儿组相比，学龄组被动检查比例较低（Fisher确切概率法：P=0.021）、有症状就医者比例较高（校正χ²=12.738，P=0.000），骶尾部皮肤异常者比例较低（χ²=6.481，P=0.011）、存在临床症状者比例较高（χ²=13.558，P=0.000）、圆锥末端位置位于L₂及以上者比例较低（Fisher确切概率法：P=0.024）；学龄组存在临床症状者比例亦高于学龄前组（Fisher确切概率法：P=0.034）。术后终丝直径为（2.17 ± 0.70）mm，较术前平均增粗（0.46 ± 0.28）mm，其中26例（81.25%，26/32）术后终丝仍继续增粗。术前9例合并脊髓空洞症2例消失、6例好转、1例持续加重，术后MRI提示脊髓再拴系2例。共随访1.90（1.11，3.10）年，41例术前无症状患儿无新发神经功能缺损症状；35例有症状者幼儿组13例、学龄前组9例、学龄组13例，3组神经功能结局差异无统计学意义（Fisher确切概率法：P=0.246）；失访1例。结论 不同年龄段终丝脂肪浸润/脂肪瘤型脊髓拴系综合征患儿的临床特征不同，年龄越大出现临床症状的风险越高；手术后仍有可能出现神经功能缺损症状。终丝离断术风险低、并发症少，预防性终丝离断术可降低神经功能缺损发生率。

关键词: 神经管缺损, 马尾, 脊髓切断术, 儿童

Abstract: Objective To evaluate the outcome of surgical treatment of tethered cord syndrome (TCS) in children with fatty filum or lipoma of filum at different ages and to explore the natural course and therapeutic strategy of this type of TCS. Methods and Results Total 77 TCS patients with fatty filum or lipoma of filum who underwent surgical division of the filum terminale between January 2017 and May 2022 in Children's Hospital of Fudan University were screened for eligibility. According to age, they were divided into young children group (< 3 years), preschool group (3-6 years) and school-age group (> 6 years). The reasons for admission (Fisher's exact probability:P=0.001), sacrococcygeal skin abnormalities (χ²=7.841, P=0.021), the proportion of children with clinical symptoms (χ 2=13.618, P=0.001), and the position of conus medullaris (Fisher's exact probability:P=0.039) in the 3 groups were statistically significant. Compared with the young children group, the school-age group had a lower proportion of passive examination (Fisher's exact probability:P=0.021), a higher proportion of patients seeking medical attention because of symptoms (adjusted χ²=12.738, P=0.000), a lower proportion of sacrococcygeal skin abnormalities (χ²=6.481, P=0.011), and a higher proportion of patients with clinical symptoms (χ²=13.558, P=0.000), and the proportion of conus medullaris located at or above L₂ probability:P=0.024). The proportion of patients with clinical symptoms in the school-age group was also higher than that in the preschool group (Fisher's exact probability:P=0.034). The average diameter of the filum terminale after surgery was (2.17 ± 0.70) mm, which increased by (0.46 ± 0.28) mm compared with that before operation, and 26 cases (81.25%, 26/32) of the filum terminale continued to increase in diameter. Among the 9 cases with preoperative syringomyelia, 2 cases disappeared, 6 cases improved, and one case continued to worsen. Postoperative MRI 3.10) years of follow-up, no new clinical symptoms were found in asymptomatic patients and 35 symptomatic children had 13 cases in the young children group, 9 cases in the preschool group, and 13 cases in the school-age group. There was no significant difference in exact probability:P=0.246). One patient was lost to follow-up. Conclusions with fatty filum or lipoma of filum are different. The older of the children, the higher of the proportion of clinical symptoms, more than half of the patients still have neurological abnormalities after surgical treatment. Division of the filum terminale is a surgery may reduce neurological damage.

Key words: Neural tube defects, Cauda equina, Cordotomy, Child