先天性脊柱裂的诊断和治疗  

Diagnosis and treatment of congenital spinal bifida

作　　者：齐翔[1] 邹哲伟[1] Qi Xiang;Zou Zhewei(Department of Neurosurgery,Affiliated Children s Hospital,Capital Medical University,Beijing 100045,China)

机构地区：[1]首都医科大学附属北京儿童医院神经外科

出　　处：《临床小儿外科杂志》2019年第2期91-94,共4页Journal of Clinical Pediatric Surgery

基　　金：国家重点研发计划(编号:2016YFC1000806)

摘　　要：先天性脊柱裂是最常见的神经管畸形,近些年对脊柱裂的病因研究取得了诸多进展,但是一些复杂类型脊柱裂即使通过手术治疗,术后仍会出现严重的肢体活动障碍和大小便功能障碍等并发症。通过对病因学研究以及孕期B超检查,可以提前预防和早期诊断,有效降低了先天性脊柱裂的出生率。脊柱裂的手术治疗应遵循以下原则:应在保护神经组织的前提下,尽量切除病变组织,最大程度地松解脊髓拴系;应在神经电生理监测下进行,手术前后需要通过泌尿系统B超和尿动力学检查进行评估,对手术后出现的下肢畸形应及时行矫形手术,这样可以提高脊柱裂的总体治疗效果。但目前对于最佳手术年龄、隐匿性脊髓拴系综合征和无症状脊髓拴系的手术指征等尚存在争议。因此,对于先天性脊柱裂患儿,早期诊断、早期治疗,手术前进行全面评估以及术后定期随访,及时处理并发症,能明显提高其总体治疗效果。Congenital spinal bifida is the most common type of neural tube defects.Much progress has been made in recent years.However,dysknesia and defecating dysfunction still occur postoperatively in some complicated patients.An early diagnosis of pregnancy is made through etiological studies and prenatal ultrasound examinations.Basic surgical principles are protecting spinal and nerve roots and releasing tethered cord sufficiently.Intraoperative neuroelectrophysiological monitoring is necessary.Pre/post-operative urodynamic and ultrasound examinations of urinary system are essential evaluations.Lower limb deformities related with neural tube defects should be treated timely to improve patient quality-of-life.This article mentions the controversies regarding operative age and intra-operative onset of occult or asymptomatic tethered cord syndrome.For congenital spinal bifida patients,early diagnosis and treatment,comprehensive evaluations,long-term follow-ups and timely treatment of complications are essential for better outcomes.

关 键 词：椎管闭合不全 诊断 治疗 

分 类 号：R729[医药卫生—儿科] R651.2[医药卫生—临床医学]