Sprengel is a rare congenital deformity of the scapula.
Features of the Sprengel deformity:
(1) There is a failure of the scapula to normally descend during embryonic development (congenital elevation).
(2) It may be associated with dysplasia of the scapula.
(3) There is often disability of the shoulder and ipsilateral arm.
(4) It may be associated with the Klippel-Feil syndrome (KFS).
(5) Other skeletal defects may be present (scoliosis, rib anomalies, etc).
It rarely may be bilateral.
Some patients with KFS and the Sprengel deformity have an omovertebral bone, cartilaginous or fibrous band that connects the medial edge of the scapula to a cervical vertebra. (with omo = shoulder). In these patients there is a risk of cervical myelopathy from compression of the cervical spinal cord.
Surgery to correct the shoulder must be done carefully to preserve the dorsal scapular nerve.