Decreased Shoulder Range of Motion

Jonathan Gordon, M.D., Ph.D.

August 26, 2004

Discussion

Sprengel's deformity is a rare congenital malformation of the scapula which, although it may occur sporadically, occurs in 20-30% of patient's with Klippel-Feil syndrome. The origin of this syndrome is uncertain with genetic or in utero fetal insult mechanisms as possibilities. Patients are characterized by congenital fusion of any two cervical vertebrae due to failure of segmentation during development. Other anomalies include scoliosis, renal anomlies including agenesis, horseshoe kidney, and duplicated collecting systems, inner/middle/outer ear deformity and synkinesia of the upper extremities. Patients usually display shortened neck and a low hair line. Sprengel's deformity is the most common congenital abnormality of the shoulder occurring more commonly on the left. It occurs secondary to tethering of the scapula to the cervical spine by either a fibrous band or an osteocartilaginous union (omovertebral bone) which may be seen on xrays. The glenohumeral joint is normal with normal range of motion. Nevertheless, the deformity results in significantly reduced range of motion which involves the scapula. One or both shoulders may be effected. Surgical correction of the shoulder may be performed to improve function.

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