小儿先天性脊柱侧弯合并椎管内神经畸形的临床研究

目的]探讨小儿先天性脊柱侧弯合并椎管内神经畸形的发病情况和临床特征及其神经畸形与临床表现的相关性.方法]回顾性研究122例先天性脊柱侧弯患儿,对患者的体格检查情况以及全脊柱CT、全脊髓MRI、双下肢肌电图等相关辅助检查结果进行整理分析.结果]122例先天性脊柱侧弯有63例(51.6％)合并脊髓畸形,其中脊髓栓系(TCS) 69.8％(44/63)；脊髓纵裂(SCM)占60.3％ (38/63),包括Ⅰ型脊髓纵裂占68.4％ (26/38),Ⅱ型纵裂占31.6％ (12/38);脊髓空洞27％ (17/63)；脊膜膨出占20.6％ (13/63)；椎管内囊肿占17.5％ (11/63)；Chiari畸形4.8％ (3/63).结论]小儿先天性脊柱侧弯合并椎管内神经畸形的发生率高,并且常表现为多种脊髓畸形同时存在.合并神经畸形的患儿常表现为腰背部中线处皮肤异常,足畸形和双下肢不对称,但对于不同神经畸形其与常见的临床表现具有不同的相关性.

Clinical study of congenital scoliosis with intraspinal deformity in children

Objective]To explore the incidence and clinincal characteristics of pediatric congenital scoliosis(PCS) associated with spinal cord deformity. Methods]The clinical data of 122 patients with PCS were researched retrospectively.Physical examation,spine X-ray,SCT,MRI examiation were analyzed. Results]There were 63 patients with spinal cord deformities.In them,there were tethered cord or low conus in 44 patients(69.8 %),split cord malformation in 38 patients(60.3%),syringomyelia in 17 patients(27%),lipomyelomeningocele in 13 patients(20.6%) and intraspinal cyst in 11 patients(17.5%). Conclusion]There is a high incidence of PCS associated with spinal cord deformity,and several deformities exist stimutaneously.Children with intraspinal deformity usually presents with lower back midline skin abnormalities,foot deformity and asymmetric lower limbs.There is different correlation between intraspinal deformity and clinical manifestations.