SPRENGEL’S SHOULDER

Sprengel's shoulder is characterized by a failure of the developing scapula to descend into its normal position (below the T3 level by 12 weeks gestation). This may affect the position, shape and size of the scapula, which may be shortened. This may be isolated or part of a more complex malformation.

• Usually unilateral, but may be bilateral.
• Females to males = 3:1.
• Scapula is elevated and anchored to the spine by an anomalous omovertebral bone, articulating between the medial border of the scapula and one or more of the cervical vertebrae.
• Scapula is hypoplastic with an abnormal shape.
• Additional skeletal malformations:
• Elevated clavicle.
• Scoliosis (30%), hemivertebrae, fused vertebrae.
• Rib anomalies (25%) - fused or missing ribs.
• Spinal dysraphism (75%).
• Associations.
• Klippel-Feil syndrome

REFERENCES

1. Samilson RL. Congenital and developmental anomalies of the shoulder girdle. Orth Clin North Am 1980;11:219.
2. Leibovic SJ, Ehrlich MG, Zaleske DJ. Sprengel's deformity. J Bone Joint Surg 1990;72:1927.