Sprengel deformity

Case contributed by Dr Jose Antonio Habana

Presentation

Chest radiograph done to rule out pneumonia. Further history revealed chronic neck and left shoulder pain.

Patient Data

Age: 50 years
Gender: Female
X-ray

The left scapula is displaced upward, in keeping with a Sprengel deformity. A curved osseous structure is seen between the left scapula and the spine (omovertebral bone). There is mild dextroscoliosis of the upper thoracic spine. Spina bifida occulta noted in T2. The left 1st to 4th ribs as well as the left clavicle are hypoplastic.

Case Discussion

Congenital elevation of the scapula is known as Sprengel deformity. It is rare but is the most common congenital anomaly of the shoulder. It is thought to occur due to an arrest in the caudal migration of the scapula during development. Apart from being elevated, the scapula will often appear dysmorphic or hypoplastic.

Several other osseous abnormalities may also be present:

In addition, the surrounding muscles may also be affected.

Sprengel deformity is commonly unilateral.

Further imaging with CT is recommended for surgical planning and for better evaluation of any associated abnormalities.

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