Congenital abnormalities

Sprengel’s deformity

This is the most common congenital abnormality around the shoulder, and results from a failure of normal descent of the scapula. In the embryo, the scapula forms in the midcervical region, and then descends to its midthoracic position. With the Sprengel deformity, the scapula is high, small and rotated, and in approximately 50 per cent of cases the scapula is connected to the cervical spine by the omovertebral body, a fibrous or bony bar. In addition there may be other congenital deformities, which include rib abnormalities, scoliosis of the thoracic spine, or cervical spine abnormalities including the Klippel—Feil syndrome (congenital fusion of the cervical vertebrae) (Fig. 22.1).

  Treatment

The major problem is usually cosmetic rather than functional, and this is particularly true for unilateral deformities. In these cases excision of the omovertebral body or superior angle is performed. Surgery is occasionally required to improve function, and in these circumstances more complex reconstructive procedures are carried out.