Congenital elevation of the scapula is known as Sprengel deformity. It is rare but is the most common congenital anomaly of the shoulder. It is thought to occur due to an arrest in the caudal migration of the scapula during development. Apart from being elevated, the scapula will often appear dysmorphic or hypoplastic.

Several other osseous abnormalities may also be present:

• presence of an omovertebral bone
• vertebral abnormalities (Klippel-Feil syndrome, spina bifida occulta, scoliosis, hemivertebrae, etc)
• rib deformities (aplasia, hypoplasia, bifid, etc)
• clavicular deformity

In addition, the surrounding muscles may also be affected.

Sprengel deformity is commonly unilateral.

Further imaging with CT is recommended for surgical planning and for better evaluation of any associated abnormalities.