Sprengel deformity is the congenital elevation of the scapula. There are varying degrees of severity; it is usually unilateral. There is restricted scapulothoracic motion (especially with abduction) so most of the shoulder motion is through the glenohumeral joint. There is usually associated hypoplasia of the parascapular muscles. Webbing of the neck and low posterior hairline can be associated problems. There is an association with congenital syndromes, such as Klippel-Feil anomaly, so a thorough history and examination are necessary. Mild forms with a cosmetic deformity and mild loss of shoulder motion do not need surgical correction. Severe forms may have a bony connection (omovertebral) between the scapula and lower cervical spine. Moderate and severe forms need surgical repositioning of the scapula in early childhood to improve comesis and function.
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