Sprengel's Deformity



- 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.



Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Thursday, January 3, 2013 2:42 pm