The outcome of 56 children (61 shoulders) treated surgically at the Rizzoli Institute between April 1975 and June 2010 for congenital elevation of the scapula is reported. There were 31 girls and 25 boys with a mean age at surgery of 6.4 years (2 to 15). The deformity involved the right shoulder in 20 cases, the left in 31 and was bilateral in five. The degree of the deformity was graded clinically and radiologically according to the classifications of Cavendish and Rigault, respectively. All patients underwent a modified Green procedure combined, in selected cases, with resection of the superomedial portion of the scapula and excision of any omovertebral connection. After a mean follow-up of 10.9 years (1 to 29.3), there was cosmetic improvement by at least one Cavendish grade in 54 shoulders (88.5%). The mean abduction of the shoulder improved from 92° (50° to 155°) to 112° (90° to 170°) and the mean flexion improved from 121° (80° to 160°) to 155° (120° to 175°). The unsatisfactory cosmetic result in seven shoulders was due to coexistent scoliosis in two cases and insufficient reduction of the scapular elevation in the other five. An incomplete upper brachial plexus palsy occurred post-operatively in three patients but resolved within seven months.

We suggest that a modified Green procedure combined with resection of the superomedial portion of the scapula provides good cosmetic and functional results in patients with Sprengel’s shoulder.

We evaluated scapular dysplasia and malposition in 15 patients with the Sprengel deformity using three-dimensional CT (3D-CT). The shape, height-to-width ratio, the areas of both scapulae, the anterior curvature of the supraspinous portion and glenoid version were assessed on scapular posterior, medial and inferior views. The degree of rotation and superior displacement were measured on the trunk posterior view. The omovertebral connection was also assessed and correlated with the operative findings. Most of the affected scapulae had a characteristic shape with a decrease in the height-to-width ratio and were larger than the contralateral scapulae. There was an inverse relationship between scapular rotation and superior displacement. The typical curve of the supraspinous portion of the scapula was seen in only three cases. There was no significant difference in glenoid version. The point of tethering of the omovertebral connection may determine the shape, rotation and superior displacement of the scapula. 3D-CT was helpful in delineating the deformity in detail, and in planning scapuloplasty

Aim: Assessment of unilateral Sprengel’s deformity using CT scan with 3-D reconstruction in order to determine if there are sub-groups in which different procedures maybe appropriate. Materials and Methods: Between 1996 and 2002, 14 consecutive patients with unilateral Sprengel’s deformity were assessed using CT scanning with 3-D reconstruction. Size, shape, elevation and rotation of the scapulae and glenoid were measured. The incidence of omovertebral connections and congenital abnormalities were noted. A spiral CT scanner was used. Results: 3 of the 14 cases had clearly abnormal scapulae with widespread and multiple associated abnormalities. These were classified as the dysplastic group. The other 11 cases had wide variation in the elevation of the scapula or glenoid, the length of the supra-spinous portion and the rotation of the scapula and glenoid. There was only one case of a superior hook. The location of the tether could be determined from the shape of the medial border. It was noted that rotation was variable and elevation was variable. Conclusion: It was felt that no one procedure was appropriate for all scapulae, but the guiding factors included the elevation of the glenoid, the rotation of the glenoid, the location of the tether in addition to the clinical considerations. It was felt that these features were best evaluated using this 3-D CT scanning technique

Introduction and Aims: There is a variety of different procedures advocated to address the problems of cosmesis and limited abduction in Sprengel’s shoulders which are based on an understanding of the morphology of the deformity from two-dimensional images. We have reviewed the morphology of the scapulae using 3D CT to determine whether there are sub-groups in which different treatments would be more appropriate. Method: We assessed 14 consecutive patients (age two to 21 years) between 1996–2002, using a spiral CT scanner with 1.5mm and 3mm slices. These were then reconstructed to give 3D images. These images were then rotated and measured to determine the dimensions, elevation, rotation and shape of the scapula, compared to the normal side. We also looked for the presence of an omovertebral connection and superior hook, and at the range of movement of the shoulder and incidence of associated anomalies. Results: There was marked variation in the elevation of the scapulae. Three of the 14 had a very abnormal, small, high scapula with multiple associated anomalies. We called these dysplastic. The other 11 scapulae were longer (105%, range 64–132%) and narrower (85%, range 50–133%). They were rotated so the glenoid faced caudally. One out of 11 had a superior hook. The location of the tether could be determined by the shape of the medial border. There is a sub-group of Sprengel’s with a dysplastic scapula and multiple associated abnormalities. These may represent a difference in aetiology. Within the non-dysplastic group there was a wide variation in the size, elevation, orientation and length of the supra spinous portion of the scapulae. Conclusion: We believe that there are different subgroups of Sprengel shoulder in whom different procedures are indicated and that CT scanning with 3D reconstruction is essential to adequately plan the procedure