This is a complex congenital condition which most of the time is associated with other syndromes or congenital defects. An alternative term used to describe this condition is scapula elevata. Patients may have a cosmetic deformity with a "high riding" scapula which is "hypomobile" and there is some degree of scapula winging. In severe forms of the disease there is a omovertebra bone with joint like connection between the cervical spine and the medial border of the scapula.
There are authors who have described good results after surgical intervention when the condition is diagnosed before the age of 6 . The main problem in this clinical condition is restricted range of shoulder motion. Most of the time forward elevation and abduction is limited, due to loss of the scapular motion or scapulohumeral rhythm as described by Codman in the 1930s.
When the loss of motion is minimal then surgery is usually contra-indicated. Associated syndromes such as Klippel Feil syndrome or other syndromes may compromise surgical care of this condition.
The following case illustrates a patient who present with the condition in adulthood and had no treatment before. The fused upper ribs and deformed thoracic cage indicate in this situation the failure of the scapula to decent during development. Associated hypoplasia of the affected scapula is not uncommon.
The forward elevation of the shoulder in the following case was around 100 degrees and for that reason no surgery was offered.
