CONGENITAL HIGH SCAPULA (SPRENGEL’S DEFORMITY)

Question 1

CONGENITAL HIGH SCAPULA (SPRENGEL’S DEFORMITY)

Answer 1

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Question 2

Definition

Answer 2

Congenital elevation of the scapula also known as Sprengel‘s deformity is an uncommon anomaly which is sometimes associated with abnormalities in the cervical spine. The scapula is elevated from 1 to 4 inches above its normal position.

Question 3

Etiology

Answer 3

Congenital elevation of the scapula results when the scapula fails to descend to normal position from its high level in the early weeks of gestation. The cause of this failure is unknown. It occurs sporadically; however, occasionally, it has an apparent autosomal dominant pattern of inheritance.

Question 4

Epidemiology

Answer 4

x 4x ♀>♂

x The condition can be bilateral in up to 30%.

Question 5

Clinical Manifestation

Answer 5

x Asymmetry of the shoulders is often the 1st evidence of abnormality.
x The affected scapula is small. Usually its vertical length is decreased and its width relatively increased. Its upper portion tends to hook forward.
x The cervical muscles are shortened on the affected side and are changed in direction.
x In most cases, abduction of the affected shoulder is greatly restricted while glenohumeral rotation is not affected.
x In bilateral cases the neck appears shortened.
x In 10% of the cases torticollis is present together with lateral curvature of the spine.

Question 6

Diagnosis/Differential Diagnosis

Answer 6

The diagnosis is made from the physical signs and the radiograph. Congenital elevation of the scapula must be differentiated from paralysis of the serratus anterior muscle and from obstetric paralysis.

Question 7

Prognosis

Answer 7

Without operation no improvement of the deformity is to be anticipated. Surgical treatment is often followed by moderately improved function and appearance.

Question 8

Complication

Answer 8

x Scoliosis

x Defective development of the cervical vertebrae and upper ribs.

Question 9

Medical Management

Answer 9

When motion is greatly limited and the deformity is unsightly, surgical treatment may be indicated. The operation often includes exposing the scapula extraperiosteally, excising its deforming supraspinous portion and the omovertebral bone if present, bringing the scapula down to the desired level, and repositioning the muscle attachments to provide anchorage.