先天性高肩胛症3D-CT形态分析及手术治疗效果评估

Sprengel Deformity: morphometric analysis using 3D-CT and surgical treatment

目的探讨先天性高肩胛症在3D-CT下的形态特征及其临床相关性,并评估手术治疗的效果。方法回顾性分析2011年9月至2016年9月间在上海交通大学医学院附属新华医院儿骨科接受治疗的29例先天性高肩胛症患儿的临床资料。其中,男15例,女14例;手术时年龄(3.69±1.34)岁。所有患儿均为单侧先天性高肩胛症,包括左侧21例,右侧8例。19例患儿行Woodward手术,其中4例行锁骨碎骨术;10例行肩胛骨部分切除术。术前使用飞利浦64排CT机器进行扫描,PhilipsIntellispacePortal软件重建骨组织,屏蔽软组织。运用Ebmdicomviewer进行后期测量,包括肩胛骨的高宽比、肩胛骨升高比率及肩胛骨旋转差异。评估手术前、后患肢上臂外展角度和Cavendish分型的变化。结果29例患儿随访资料完整,随访时间为(46.38±18.05)个月。术前Cavendish分型Ⅲ型19例,Ⅳ型10例;术后有Ⅲ型1例,Ⅱ型17例,Ⅰ型11例;手术前后比较,差异有统计学意义(P=0)。术前上臂外展的活动度为94.14°±9.83°,术后为154.46°±16.97°,手术前后比较,差异有统计学意义(P=0)。术前的3D-CT评估中,有1例伴有Klippel-Feil综合征,4例观察到棘突裂,4例观察到部分脊椎融合,2例有部分肋骨融合,1例并发脊柱侧弯。有12例(41.38%)观察到冈上窝部分弯曲,22例(75.86%)在术前(或术中)可见骨性(或软组织性)肩椎骨存在。患侧与健侧肩胛骨的高宽比分别为1.37±0.23和1.67±0.22,两者比较,差异有统计学意义(P=0)。肩胛骨升高比率(0.31±0.15)与旋转差异(25.52°±12.65°)间存在明显的负相关性。结论术前3D-CT检查可以辅助评估先天性高肩胛症的肩胛骨异常形态。Woodward手术和肩胛骨部分切除手术治疗先天性高肩胛症均可以取得令人满意的临床效果。

Objective To explore the functional and cosmetic outcomes of Sprengel deformity after Woodward procedure and partial scapulectomy and employ three-dimensional-computed tomography (3D-CT) technique for evaluating the morphometric characteristics of Sprengel deformity. Methods From September 2011 to September 2016, 29 patients (15 boys and 14 girls) with Sprengel deformity underwent Woodward procedure (n=19), clavicle osteotomy (n=4) and partial scapulectomy (n=10). The average operative age was (3.69±1.34) years. A Philips 64-row machine was employed with Philips Intellispace Portal software for reconstructing bone tissue and shielding soft tissues. And an Ebm dicom viewer was employed for assessing the values of height-to-width ratio, superior displacement ratio, rotational difference and anterior curvature of supraspinous portion. The functional and cosmetic outcomes were evaluated on the basis of changes in shoulder abduction and the radiographic findings using Cavendish classification. Results During a postoperative follow-up period of (46.38±18.05) months, significant improvement of at least 1 Cavendish grade was documented in all cases except for one child. The postoperative shoulder abduction significantly improved [(94.14±9.83) to (154.46±16.97) degrees, P=0]. On preoperative 3D-CT, the mean height-to-width ratio of affected scapula was significantly smaller than that of contralateral counterpart. An inverse relationship existed between rotational difference and superior displacement ratio. One patient was associated with Klippel-Feil syndrome. There were spinous processes (n=4), partial spinal fusion (n=4), partial rib fusion (n=2), scoliosis (n=1), omovertebral bone (n=22) and anterior curvature of supraspinous portion (n=12). Conclusions 3D-CT is helpful in evaluating pathological anatomy of Sprengel deformity. Both Woodward procedure and partial scapulectomy offer satisfactory cosmetic and functional outcomes.