Sprengel deformity

Sprengel deformity, also known as congenital high scapula or congenital elevation of the scapula, is a rare congenital musculoskeletal anomaly in which one or both shoulder blades (scapulae) are abnormally elevated and underdescended from their normal position. During fetal development, the scapula normally descends from the neck region to its final position along the upper back; in Sprengel deformity, this descent is incomplete. The condition is the most common congenital anomaly of the shoulder and is more frequently unilateral, with the left side affected more often than the right. It is also more common in females. The primary clinical features include a visibly elevated and often smaller scapula on the affected side, restricted shoulder abduction and limited range of motion of the shoulder joint, asymmetry of the neck and shoulder line, and cosmetic concerns. In approximately one-third of cases, an omovertebral bone — an abnormal bony, cartilaginous, or fibrous connection between the scapula and the cervical spine — is present, which further restricts scapular movement. Sprengel deformity frequently occurs in association with other congenital anomalies, including Klippel-Feil syndrome (fusion of cervical vertebrae), scoliosis, rib anomalies, spina bifida occulta, and renal abnormalities. It may also be seen as part of broader syndromic conditions. Treatment depends on the severity of the deformity and the degree of functional limitation. Mild cases may require only observation and physical therapy to optimize shoulder range of motion. More severe cases, particularly those with significant functional impairment or cosmetic concerns, may benefit from surgical intervention. The Woodward procedure, which involves surgical repositioning of the scapula by releasing and reattaching the muscles that anchor it, is the most commonly performed corrective surgery, typically undertaken in early childhood (ages 3–8 years) for optimal outcomes. Excision of the omovertebral bone, when present, is usually performed concurrently. Early diagnosis and multidisciplinary management involving orthopedic surgeons, geneticists, and rehabilitation specialists are important for achieving the best functional and cosmetic results.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Sprengel deformity