先天性多发性关节挛缩症伴脊柱侧凸的影像学特征

Radiographic characteristics of scoliosis combined with arthrogryposis multiplex congenita

中文关键词:  先天性多发性关节挛缩症  脊柱侧凸  影像学特征

英文关键词:Arthrogryposis multiplex congenita  Scoliosis  Radiographic characteristics

中文摘要:

  【摘要】 目的：探讨先天性多发性关节挛缩症伴脊柱侧凸（arthrogryposis multiplex congenita，AMC）患者的影像学特征。方法：回顾性分析2001年11月~2013年4月我院收治的以脊柱侧凸首诊的41例AMC患者，男21例，女20例，年龄4～28岁（平均15.6±3.9岁）。记录侧凸类型、累及节段数，测量冠状面侧凸主弯Cobb角、骨盆倾斜角和矢状面成角，计算侧凸柔软度，并对合并骨盆倾斜患者的骨盆倾斜角和主弯Cobb角进行相关性分析。结果：本组中7例合并先天性脊柱畸形。单弯37例（90%），其中胸弯21例，胸腰弯16例；胸腰双弯4例（10%）。主弯平均累及节段数为9±1个，平均Cobb角为80°±29°，平均柔软度（20±11）%。22例（54%）合并骨盆倾斜，倾斜角平均为16°±5°；相关分析示骨盆倾斜角与主弯Cobb角存在显著相关性（r＝0.612，P＜0.05）。矢状面上，胸椎前凸20例（49%），胸椎后凸减小5例（12%），腰椎过度前凸24例（59%）。结论：AMC伴脊柱侧凸以单弯为主，畸形较为僵硬，可合并有先天性脊柱畸形，多合并骨盆倾斜，且骨盆倾斜与侧凸严重程度密切关联。典型的矢状面表现为胸腰椎的前凸畸形，而后凸畸形相对少见。

英文摘要:

  【Abstract】 Objectives: To investigate the radiographic characteristics of scoliosis combined with arthrogryposis multiplex congenita(AMC). Methods: From November 2001 to April 2013, a total of 41 AMC patients who visited our hospital initially for scoliosis, including 21 males and 20 females with the age ranging from 4 to 28 years(average, 15.6±3.9 years), was reviewed retrospectively. Curve pattern and levels of involved vertebrae were recorded. The following parameters were measured: the Cobb angle of main curve, flexibility, pelvic obliquity, thoracic kyphosis(TK) and lumbar lordosis(LL). Correlation analysis was performed between pelvic obliquity and coronal curve. Results: 7 cases were associated with congenital vertebral anomalies. Single curve was found in 37 cases(90%), among whom there were 21 with a single thoracic curve and 16 with a single thoracolumbar curve. 4 cases(10%) had thoracic and lumbar curves. The main curve averagely spanned 9±1 levels. The Cobb angle averaged 80°±29°, and the flexibility averaged(20±11)%. There were 22 cases(54%) with the pelvic obliquity ranging from 7° to 27°(average, 16°±5°). Significant positive correlation was found between pelvic obliquity and the Cobb angle of main curve(r＝0.612, P＜0.05). On the sagittal plane, 20 cases(49%) had thoracic lordosis, 5 cases(12%) had decreased thoracic kyphosis, and 24 cases(59%) had lumbar hyperlordosis. Conclusions: Single curve pattern is predominantly seen in scoliosis with AMC. The curves are always rigid. Congenital vertebral anomalies may also be detected in some patients. Single curve with rigid flexibility and pelvic obliquity on the coronal plane, and thoracic and lumbar hyperlordosis on the sagittal plane are the typical features of scoliosis in AMC. Pelvic obliquity strongly correlates with severity of scol?鄄iosis.