Sprengel's deformity: an analysis of surgically and nonsurgically treated patients.

Abstract

Sprengel's deformity is a congenital anomaly of the scapula and can be associated with reduced shoulder mobility and esthetic concerns. Controversies remain regarding its natural history, radiographic findings, and optimal treatment strategy. A total of 74 Sprengel's shoulders in 71 patients presenting to a pediatric referral hospital were retrospectively reviewed including 24 surgically treated shoulders. Median age at initial presentation was 3.8 years. Median age at the final follow-up was 9.6 years. Twenty shoulders were analyzed for range of motion across time. The median composite abduction range was initially 105° and at the last time-point 98°, with a median loss of 15° over 5 years (P = .007). A total of 26% of the nonsurgical shoulders reported pain at the latest follow-up, compared with 1 surgical patient (4%) (P = .028). Older age was independently associated with pain (P < .001); this effect was only seen in nonsurgical patients. Radiographically, the glenoid inclination angle (GIA) independently correlated with the abduction range (P = .001). Surgically treated shoulders underwent a modified Woodward procedure at a median 4.8 years of age. At a median follow-up of 41 months, the abduction range improved in the surgical group from 90° preoperatively to 110° postoperatively, with a median gain of 40° (P < .001). The median GIA changed from -2° to 4° with a median gain of 9° (P = .004). The natural history of untreated Sprengel's deformity may be one of gradual decline in the abduction range, with a proportion of patients developing pain in adolescence. The GIA correlates with the shoulder abduction range. Surgery with a modified Woodward procedure may alter the natural history of the condition by improving motion and reducing risk of pain later in life.