婴儿隐匿性脊髓栓系综合征18例临床特征及治疗方案探讨

Clinical features and treatment options of 18 cases of infantile occult tethered cord syndrome

目的探讨婴儿隐匿性脊髓栓系综合征的临床特征及治疗方法选择。方法回顾性分析2019年3月至2022年3月山西省儿童医院神经外科收治的18例婴儿隐匿性脊髓栓系综合征临床资料,包括手术前后临床症状、尿流动力学检查及影像学资料。18例病儿中,11例仅表现为腰骶部皮肤外观异常,呈色素斑或皮肤凹陷;6例除皮肤外观异常外,合并尿频症状;单侧马蹄足畸形1例。所有病儿术前行腰骶MR检查显示脊髓圆锥位置正常,终丝呈脂肪附着或脂肪瘤化;尿流动力学检查逼尿肌过度活动13例,膀胱顺应性差2例,未见异常改变3例;病儿均在术中电生理监测下行经棘突间隙入路终丝切断术。结果全组18例病儿术后随访6~36个月,术前无症状的11例病儿均无任何新发临床症状,大小便正常,双下肢活动好;6例尿频病儿,5例明显改善,1例变化不明显;1例马蹄足病儿经骨科后续使用ponseti法治疗痊愈。结论婴儿隐匿性脊髓栓系综合征临床症状不典型,多数仅表现为腰骶部皮肤外观异常,易漏诊。需尽早行腰骶MR检查,确诊后及时手术,预后良好。

Objective To investigate the clinical features and treatment options of infantile occult tethered cord syndrome.Methods The clinical data of 18 infants admitted to the Department of Neurosurgery of Shanxi Children's Hospital from March 2019 to March 2022 were retrospectively analyzed,including clinical symptoms,urodynamic examination and imaging data before and after surgery.Among the 18 children,11 showed only abnormal appearance of the lumbosacral skin,pigmented spots or sunken skin;6 cases had frequent urination symptoms in addition to abnormal skin appearance;One case with unilateral horseshoe foot deformity.Preoperative lumbosacral MR examination showed that the spinal cord conus position was normal,and the terminal filament was fat attached or lipomatized;There were 13 cases of detrusor hyperactivity,2 cases of poor bladder compliance,and no abnormal changes in 3 cases.All children in this group underwent transspinous interstitial approach filamentotomy under intraoperative electrophysiological monitoring.Results The 18 children in the group were followed up for 6-36 months after surgery.The 11 children who were asymptomatic before surgery had no new clinical symptoms,normal urine and urine,and good lower limb movement.Among the 6 children with frequent urination,5 cases improved significantly,and 1 case did not change significantly;One child with horseshoe foot was treated with Ponseti method by orthopedics.Conclusions The clinical symptoms of infantile occult tethered cord syndrome are atypical,and most of them are only manifested as abnormal appearance of lumbosacral skin,which is easy to miss the diagnosis.Lumbosacral MR examination should be performed as soon as possible,and timely surgery after diagnosis has a good prognosis.