Sprengel's deformity: long-term follow-up study of 22 cases

This study examines the long-term results of 22 patients treated between 1938 and 1974 either surgically or by observation. Fourteen patients (16 scapulas) were treated by observation, seven patients (seven scapulas) were treated surgically by various methods, and one bilateral case was treated conservatively on one side and surgically on the other. The average follow-up was 26 years (range, 10-55) and the average age at follow-up was 32 years (range, 18-67). Shoulder abduction and cosmesis did not change appreciably over the years for the patients that did not undergo surgery, but the surgical patients did demonstrate an average improvement in shoulder abduction of 38 degrees. Seven of eight patients treated surgically reported a subjective improvement in the cosmetic appearance at follow-up, although four patients had an unsightly surgical scar. Pain in the affected shoulder was uncommon at follow-up in either group. We feel that the final result should be assessed from the viewpoint of the functional outcome and cosmetic appearance, independent of the radiographic aspect. Surgical treatment for patients with Sprengel's deformity is indicated when patient and family expectations are either improved cosmesis or improved function.