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).
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.