- Discussion:
- uncommon congenital anamoly which arises from interruption of normal caudal migration of the scapula;
- deformity is characterized by elevation & medial rotation of inferior scapula;
- involved scapula is both smaller and more cephalad than normal;
- in 30% of pts, the scapula is attached to the cervical spine by an omovertebral bone, cartilage, or fibrous tissue,
which, when present, can severely limit scapulothoracic motion;
-
associated anomalies:
-
congenital scoliosis, cervical ribs,
torticollis, renal abnormalities, & muscular hypoplasia, especially involving the trapezius;
- Clinical Presentation:
- deformity tends to be painless & many patients are not diagnosed until adolescence;
- due to scapular asymmetry, some pts are mistakenly thought to have scoliosis;
- minor asymmetries commonly seen between right & left scapula should not be designated Sprengel's deformity.
- look for loss of shoulder abduction & foward flexion;
- if an omovertebral bone is present, abduction of the shoulder is commonly limited to less than 90 deg;
- Non Surgical Treatment:
- because passive stretching exercises advocated in the past are not successful, treatment is primarily surgical;
- Surgical Treatment:
- timing:
- surgery is indicated for children between 3-8 yrs of age w/ sig deformities, both functional and cosmetic;
- pts older than 8 yrs of age are not good candidates for scapular displacement procedures;
- options:
- detachment of medial & superior scapular muscles, repositioning scapula caudad, &
subsequently reattaching the muscles to lowered scapula.
- Woodward Procedure:
- procedure has 80% satisfactory functional and cosmetic results.
- incr shoulder abduction following surgery ranges from 34-60 deg.
- child's age at operation and differing methods of measurement play largest role in accounting for these differences;
- younger patients obtain better motion and postoperative correction.
- caudad displacement of scapula is reported to be 1.9 vertebra body heights in one series and 4 cm in another;
- technique:
- involves resection of omovertebral bone and division of vertebral attachments of
trapezius,
rhomboids, and levator scapula;
- scapula is subsequently rotated & translated caudally;
- detached muscle origins are then sutured to more inferior vertebral spinous processes.
- 3 wks of postoperative immobilization are required;
- osteotomy of clavicle may be required to prevent compression of N/V structures against first rib;
- postoperative improvement in shoulder abduction is maintained, although some loss of scapular
translation can occur in first four months postoperatively.
- one third of patients will have widening of their surgical scars, which can be cosmetically disturbing;
Sprengel deformity
Congenital elevation of the scapula. Surgical correction by the Woodward procedure.
Sprengel's deformity: long-term follow-up study of 22 cases.
Sprengel's deformity: long-term follow-up study of 22 cases.
