Correction in Malrotation of the Scapula and Muscle Transfer for the Management of Severe Sprengel Deformity

Background: The clinical results of surgical procedures for severe Sprengel's deformity have been uncertain. To obtain improved elevation, we consider that it is necessary to realign the lateral border of the scapula for upward rotation. The purposes of the current study were to evaluate the clinical results and range of motion of the scapula after such realignment. Methods: Seven cases of Sprengel's deformity of Cavendish grade 3 or 4 were treated surgically and then clinically evaluated and examined using three-dimensional computed tomography (3D CT). (Two boys and five girls aged 50.9 ± 15.4 months, mean ± standard deviation at the time of operation.) The mean follow-up was 53.1 months (range, 12 to 92 months). After the omovertebral bone and the superomedial side of the scapula were removed, the levator scapulae and rhomboids were reattached to wrap around the scapula at maximum upward rotation to assist in maintaining this position. Cavendish and Rigault grades were used for evaluation of postoperative appearance. The superior displacement and rotation of the scapula were measured on the trunk posterior view using 3D CT. The relationship between improvement in the range of motion and radiologic change were analyzed statistically. Results: The postoperative flexion (97.9° ± 12.9° to 160° ± 11.5°) and abduction (99.3° ± 13.0° to 161.4° ± 15.7°) were significantly improved compared with the mean preoperative values (P < .0001). 3D CT revealed that in all patients the malrotation of the scapula was improved postoperatively. The current study shows that successful realignment of the scapula led to these improved clinical results. Conclusions: Our procedure has advantages not only for recovery of the range of motion but also for reducing the characteristic lump in the web of the neck. However, our procedure has an inherent limitation related to asymmetrical shoulder level and width. 3D CT may be useful for preoperative planning and postoperative evaluation