Sprengel deformity

Case contributed by Dalia Ibrahim
Diagnosis certain

Presentation

Left shoulder deformity since birth.

Patient Data

Age: 12 years
Gender: Female
x-ray

Elevated left scapula.

ct

Elevated left scapula with an omovertebral bar (osseous connection between scapula and cervical spine).

Hypoplasia/atrophy of the left shoulder girdle muscles.

ct

Elevated left scapula with an omovertebral bar (osseous connection between scapula and cervical spine).

Case Discussion

A Sprengel deformity, or congenital elevation of scapula, is a complex deformity of the shoulder and is the most common congenital shoulder abnormality.

Sprengel deformity usually coexists with other congenital abnormalities, particularly those involving the vertebrae and ribs. An omovertebral bar (fibrous, cartilaginous and/ or osseous connection between scapula and cervical spine) is often present.

It is also commonly associated with hypoplasia or atrophy of regional muscles, and these associated features can cause further misshaping of the shoulder and limitation of shoulder movement.

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