Clinical Presentation and Patient-reported Function in Children With Sprengel's Deformity

Area of Science:

• Pediatric Orthopedics
• Congenital Anomalies
• Musculoskeletal Disorders

Background:

• Sprengel's deformity is a rare congenital anomaly of the scapula.
• It involves elevated scapula, glenoid hypoplasia, and inferior tilting.
• Limited data exists on its clinical impact in children.

Purpose of the Study:

• To characterize the clinical presentation of Sprengel's deformity.
• To quantify range of motion in affected children.
• To assess patient-reported functional outcomes.

Main Methods:

• Prospective enrollment in the Congenital Upper Limb Differences (CoULD) Registry (2014-2023).
• Collected shoulder range of motion and patient-reported outcomes (PROs).
• Compared data to normative values using univariate analysis.

Main Results:

• 59 children with Sprengel's deformity analyzed; most cases were unilateral and non-syndromic.
• Significant reductions in shoulder abduction and forward elevation observed.
• Lower physical function and sports abilities reported, but less anxiety/depression than norms.

Conclusions:

• Children with Sprengel's deformity exhibit reduced shoulder mobility (abduction, forward elevation).
• Physical function and sports abilities are impaired, irrespective of presentation type.
• Higher Cavendish grades correlate with poorer transfer and mobility function.