摘要
目的研究脊髓发育不良脊髓神经损害的临床病理生理学特点。方法本组共112例患儿,111例施行椎管内手术;单纯末段脊髓缺失1例,未手术。有脊膜修补史46例。依据112例患儿病史、体检以及MRI、尿流和肛肠动力学检查结果及肌电图(EMG)评价并行脊髓手术治疗。术中观察脊髓形态学改变并按照椎管内病变及其脊髓病理解剖改变将其分为终丝栓系(又分为A、B两亚型)、脊髓粘连、脊髓脂肪瘤、囊性占位、脊髓纵裂、静态病变等6型。依据综合评价结果将脊髓损害的病程分为5期,每期给予相应评分并做统计学分析。结果7组脊髓神经损害综合评分的方差分析和临床分期的秩和检验差异都有非常显著性意义(P<0.001或0.05)。直线相关与回归分析表明患儿年龄与脊髓神经损害间(r=0.55,P<0.001)和栓系组圆锥末端位置与首发症状年龄之间(r=0.44,P<0.001)都有显著正相关关系。结论脊髓神经损害是一个由椎管内病变及其脊髓形态学改变引起并进行性加重的动态病理过程。诊治方针应是早期诊断和施行椎管内手术治疗以改善预后,临床分期和病理学分型具有一定临床价值。
Objective To study the clinical pathophysiology on myelodysplasia. Methods One hundred and twelve patients with myelodysplasia were evaluated and diagnosed depending on the disease history, physical examination, MRI, EMG, urodynamics, and then underwent surgical correction. The patients were divided into 6 types depending on the intraspinal anomalies and the morphological changes of spinal cords： tethered spinal cords （including 2 subtypes： A with pathogenesis of terminal filum, B with fibrous adhesions）, lipoma on spinal cord, intraspinal cyst, diastematomyelia and absences of sacral segments of spinal cords and/or their nerve roots. The lesion of myelodysplasia was classified into 5 stages according to the different degrees of impairment. The relationship between the patients＂ age at the onset symptom and morphological types or clinical stages was analyzed. Results There was a significant relationship between the lesion of spinal cord and the patient＇s age. The closely correlation between the levels of conus medullaris and the age at onset was also noted. Conclusions Myelodysplasia is a process of progressive neurological deterioration which results from intraspinal anomalies. It is necessary to manage it as soon as possible to avoid further impairment.
出处
《中华小儿外科杂志》
CSCD
北大核心
2005年第11期586-589,共4页
Chinese Journal of Pediatric Surgery
