Background: Sprengel’s deformity is a rare congenital anomaly of the scapula, characterized by an elevated position, inferior tilting of the glenoid, and hypoplasia. Due to limited data on the impact of this congenital anomaly, the purpose of this study is to objectively characterize the initial clinical presentation and quantify both the range of motion and patient-reported function in children with Sprengel’s deformity. Methods: Children with Sprengel’s deformity were prospectively enrolled into the multicenter Congenital Upper Limb Differences (CoULD) Registry between 2014 and 2023. Shoulder range of motion and patient-reported outcomes (PROs) data were collected from the patient’s most recent preoperative follow-up and compared with normative values. PROs included the Pediatric Outcomes Data Collection Instrument (PODCI) and PROMIS Upper Extremity, Pain Interference, Depression, Anxiety, and Peer Relations domains. Univariate analysis was performed to compare unilateral versus bilateral presentation, non-syndromic versus syndromic association, and Cavendish grades. Results: In total, 59 patients (52.5% female) were included with a median age of 5.9 years. Most patients presented with unilateral involvement (96.6%) and without an associated syndrome (78.0%). Cavendish grade III was the most common (50.8%) presentation. Average shoulder abduction (113 degrees) and forward elevation (120 degrees) were significantly less than the normal range ( P 0.05). Conclusions: Children with Sprengel’s deformity have decreased shoulder range of motion, specifically abduction and forward elevation. Regardless of bilateral or syndromic presentation, patients generally demonstrated significantly lower physical function and sports abilities, but reported less anxiety and depression than population norms. A higher Cavendish grade was associated with worse transfer and mobility function. Level of Evidence: Therapeutic level II.
Lee et al. (Mon,) studied this question.