Sprengel Deformity
On this page
Direct answer
Sprengel deformity is a congenital elevation of the scapula, caused by failure of its normal caudal descent during embryonic development. The affected scapula is small, high and rotated, which limits shoulder abduction and produces a visible neck-shoulder asymmetry. It is often associated with an omovertebral bone, and surgical correction by the Woodward or Green procedure is considered when the cosmetic or functional deficit is significant.
What you must remember
- Cause: the scapula fails to descend normally in early embryonic life, and the muscles attached to it are often underdeveloped.
- Omovertebral bone: a bony or fibrous band joins the superior scapular border to the cervical spine in a substantial proportion of cases.
- Associations: Klippel-Feil features, scoliosis, cervical ribs and renal anomalies are recognised, so they are screened for at diagnosis.
- Clinical picture: an elevated shoulder, a short-appearing neck with webbing, and limited abduction and forward flexion.
- Severity: graded by the height of the scapula and the degree of muscle hypoplasia.
- Woodward procedure: detaches the trapezius and rhomboids from the spinous processes, excises the omovertebral bone, lowers the scapula and reattaches the muscles lower.
- Green procedure: detaches the muscles from the scapula itself, resects its supraspinous part, lowers the scapula and reattaches the muscles.
- Timing: surgery is considered in early childhood once the deformity is significant, with the aim of improving both function and appearance.
Choosing between the Woodward and Green procedures
A 5-year-old girl has a high right scapula, a visible neck web and limited overhead reach, with an omovertebral bone seen on radiograph. The plan weighs function and cosmetic result before any operation is chosen.
- Characterise the deformity: measure the height of the scapula and the degree of muscle hypoplasia, since both determine severity and the scale of surgery needed.
- Image the cervical spine: radiographs or CT define the omovertebral bone and any fusion of cervical vertebrae, because these change the surgical plan.
- Assess shoulder function: the range of abduction and elevation is measured, and a significant limitation strengthens the case for surgery.
- Choose the procedure: the Woodward procedure detaches the muscles from the spinous processes and excises the omovertebral bone, whereas the Green procedure detaches them from the scapula and resects its supraspinous part.
- Screen for associated anomalies: scoliosis, renal anomalies and Klippel-Feil features are looked for at diagnosis, since they alter both management and follow-up.
- Plan rehabilitation: post-operative physiotherapy aims to regain abduction and avoid stiffness, with long-term review of function and appearance.
Where the exam tries to catch you
Examiners test the anatomy of the deformity and the logic behind the two operations.
- Trap: calling a high scapula an acquired deformity. Correct: Sprengel deformity is congenital, arising from failed descent and usually noticed in infancy or early childhood.
- Trap: ignoring the omovertebral bone. Correct: it shapes the surgical approach, and its excision is an integral part of the Woodward procedure.
- Trap: operating for appearance alone with no functional limit. Correct: surgery is considered for a significant cosmetic or functional deficit, so the decision weighs both.
Frequently asked questions
What causes Sprengel deformity?
Sprengel deformity results from failure of the scapula to descend normally during embryonic development, leaving it high and hypoplastic. The muscles attached to the scapula are often underdeveloped as a result.
What is an omovertebral bone?
An omovertebral bone is a bony or fibrous connection between the superior border of the scapula and the cervical spine, commonly found in Sprengel deformity. It limits scapular movement and is excised as part of the Woodward procedure.
What is the difference between the Woodward and Green procedures?
The Woodward procedure detaches the trapezius and rhomboids from the spinous processes, excises the omovertebral bone, lowers the scapula and reattaches the muscles. The Green procedure instead detaches the muscles from the scapula and resects its supraspinous part before lowering it.
Which conditions are associated with Sprengel deformity?
Klippel-Feil features, scoliosis, cervical ribs and renal anomalies are recognised associations of Sprengel deformity. These anomalies are screened for at diagnosis because they change management and prognosis.