Journal of Pediatric Orthopaedics - 2026-07-01 - Journal Article; Multicenter Study
Clinical Presentation and Patient-reported Function in Children With Sprengel's Deformity.
Lee J, Buttrick E, Vuillermin C, Wall L, Samora J, Shah AS, CoULD Study Group
Topics
Key Takeaway
Children with Sprengel's deformity demonstrate mean shoulder abduction of 113° and forward elevation of 120°—significantly below normative values—with Cavendish grade III being the most common presentation (50.8%) and higher grades correlating with worse PODCI Transfer scores.
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Summary
This multicenter CoULD Registry study prospectively characterized shoulder ROM and patient-reported outcomes in 59 children with Sprengel's deformity (median age 5.9 years, 96.6% unilateral, 78% non-syndromic) compared against published normative values. Mean abduction (113°) and forward elevation (120°) were significantly reduced versus norms (P<0.001), and PODCI Upper Extremity Function, Sports, and Global Function scores were all significantly below normative population scores (P<0.05). Paradoxically, PROMIS Anxiety and Depression scores were better than population norms, while Cavendish grade independently predicted PODCI Transfer function (P=0.011).
Key Limitation
The sample size of 59 patients limits statistical power for subgroup analyses (bilateral vs. unilateral, syndromic vs. non-syndromic), and the absence of postoperative follow-up data prevents any conclusion about whether surgical correction restores ROM or PRO scores toward normative values.
Original Abstract
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 <0.001). Children with Sprengel's deformity reported less anxiety and depression than the normative population ( P =0.017 and 0.028, respectively). Patients scored significantly lower on PODCI Upper Extremity Function, Sports, and Global Function modules than the normative population ( P <0.05). PODCI Transfer sub-scores significantly varied by Cavendish classification ( P =0.011). Neither bilateral presentation nor syndromic association significantly affected other PROs ( 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.