晚发型脊椎骨骺发育不良伴进行性关节病的MRI研究  

MRI study in spondyloepiphyseal dysplasia tarda with progressive arthropathy

作　　者：谭利华[1] 廖二元[2] 肖恩华[1] 马聪[1] 杜万平[1] 李剑[2] 

机构地区：[1]中南大学湘雅第二医院放射科,长沙410011 [2]中南大学湘雅第二医院内分泌科,长沙410011

出　　处：《中华放射学杂志》2005年第2期195-197,共3页Chinese Journal of Radiology

摘　　要：目的研究晚发型脊椎骨骺发育不良伴进行性关节病(SEDTPA)的MRI表现及其病理基础。方法临床及X线检查确诊的SEDTPA姐弟分别行脊椎、双髋关节及膝关节MR检查,患者姐行股骨头置换术,股骨头标本行病理影像对比研究。结果MRI示脊椎后突或侧弯畸形、椎间盘退行性变随年龄增大而加重,部分椎体二次骨化中心前部未发育,骨化椎体呈“倒置的花瓶样”改变或椎体终板呈“台阶样”改变。膝关节、髋关节骺软骨在发病早期有局限性T2WI高信号病变,晚期呈退行性改变。股骨头标本病理学观察示部分区域软骨增生活跃、成熟不足。结论SEDTPA的MRI表现具有一定特征,影像学表现的主要病理基础是软骨增生活跃、成熟不足。Objective To study the MRI features and the cartilaginous pathology of the spondyloepiphyseal dysplasia tarda with progressive arthropathy (SEDT-PA).Methods MRI of spine, bilateral hips, and knees was taken in 2 cases with clinically and radiographically proven SEDT-PA, who were sister and brother and whose parents were healthy and not inbreeding. The sister's femoral heads were resected bilaterally and the tissues were used for pathological study.Results MRI showed that the kyphosis and lateroflexion of the spine, and the degenerative signs of the intervertebral discs became more evident along with the growth of the patients. The anterior annular secondary ossification centers of cartilaginous epiphyses of some vertebral bodies didn′t appear. So the affected vertebral bodies were like “inverted vase” or the end plate like “steps”. Bilateral acetabular cartilage and medial epicondylian cartilaginous epiphyses of the femurs in the young brother showed regional high signal intensity on coronal fat-saturated proton density weighted MR images and degenerative signs on the elder sister. The regional hyperplasia and hypogenesis of the femoral head cartilage could be seen by microscope.Conclusion There are characteristic features on MRI in SEDT-PA and this is due to the regional hyperplasia and hypogenesis of the cartilage pathologically.

关 键 词：脊椎骨 进行性 晚发型 骨骺发育不良 关节病 软骨 MRI 分区 影像 信号 

分 类 号：R445.2[医药卫生—影像医学与核医学]