Congenital Elevation of the Scapula or Sprengel deformity
Sprengel deformity is a congenital deformity of the scapula with inadequate descent of scapula from cervical to the thoracic area during the third month of the pregnancy.
Scapula lies more superiorly than it should be to the thoracic cage and it is usually hypoplastic and misshapen.
Sprengel deformity usually occurs sporadically, although a familial occurrence has been observed.
Currently assumed, Sprengel deformity is connected with an anomaly in the formation of cerebral ventricles during early pregnancy. This results in leakages of cerebrospinal fluid into the subcutaneous tissues.
The scapula is initially formed at the level of the 5th cervical vertebra and normally migrates downwards during the 3rd month of pregnancy.
This migration fails to occur in a Sprengel deformity.
In Sprengel deformity, scapula remains too high and too small.
Sprengel deformity is associated with other abnormalities in 70 % of cases. Abnormalities include cervical ribs, malformation of the ribs, anomalies of cervical vertebrae (Klippel-Feil Syndrome), scoliosis; rarely one or more scapular muscles are partly or completely absent.
Girls are more frequently affected than boys.
Sprengel deformity is usually unilateral.
The deformity usually varies in severity.
In mild deformity, the scapula is only slightly elevated, a bit smaller and its motion is only mildly limited.
In severe deformity, the scapula is very small and can be so elevated that it almost touches the occiput.
On clinical examination, it reveals a restriction of abduction i.e. scapulothoracic motion restricted.
Neck muscles are shortened on the affected side and sometimes omovertebral bone is palpable.
Radiographs will show the elevated and rotated scapula.
Omovertebral bone can be identified on the axial view of the scapula.
Computed tomography is useful in assessing the relations of the bony abnormality.
Magnetic Resonance imaging is used to assess the spinal cord anomalies.
No treated is indicated in children with mild deformity and mild impairment of function. These children are encouraged to get involved in sports activities such as swimming.
Surgery is indicated for children with severe deformity.
The ideal age for surgical intervention is between 4 and 6 years of age.
Surgery is indicated to improve the cosmoses and shoulder function.
Surgery is performed to bring the scapula inferiorly to its near-normal position.
Earlier the surgery is performed after 3 years of age, the better the results because the operation becomes more difficult as the child grows.
The long term consequences are the restriction of shoulder abduction, cosmetic deformity, and functional limitation of the shoulder.