Sprengel's Deformity - Pediatrics

Introduction

Congenital condition with a small and undescended scapula often associated with
- scapular winging
- hypoplasia
- omovertebral connection between superior medial angle of scapula and cervical spine (30-50%)
Epidemiology
- incidence
  - most common congenital shoulder anomaly in children
- Male to female ratio 1:3
- location
  - bilateral in 10-30% of cases
Etiology
- interruption of embryonic subclavian blood supply
  - at level of subclavian, internal thoracic or suprascapular artery
  - in contrast, Poland syndrome is subclavian artery interruption proximal to internal thoracic and distal to vertebral artery
Associated diseases
- Klippel-Feil (approximately 1/3 have Sprengel deformity)
- congenital scoliosis
- upper extremity anomalies
- diastematomyelia
- kidney disease

Anatomy

Osteology
- scapula consists of
  - body
  - spine
  - acromion
  - coracoid process
  - glenoid
Articulations
- AC joint and glenohumeral diarthrodial articulations of the scapula
Muscles
- muscles that insert on medial border of scapula
  - levator scapulae
  - rhomboids major and minor
  - teres major
    - small portion just proximal to inferior angle
  - latissimus dorsi
    - small slip of origin at inferior angle

Presentation

Treatment

Nonoperative
- observation
  - indications
    - no severe cosmetic concerns or loss of shoulder function
Operative
- surgical correction
  - indications
    - severe cosmetic concerns or functional deformities (abduction < 110-120 degrees)
    - best to perform surgery from 3 to 8 yrs of age
    - risk of nerve impairment after the age of 8
  - pre-operative planning
    - MRI or CT to identify omovertebral bar
  - procedures
    - Woodward procedure
      - detachment and reattachment of medial parascapular muscles at spinous process origin to allow scapula to move inferiorly and rotate into more shoulder abduction
      - modified Woodward includes resection of superiormedial border of scapula in conjunction with surgical descent
    - Schrock, Green procedure
      - extraperiosteal detachment of paraspinal muscles at the scapular insertion and reinsertion after inferior movement of scapula with traction cables
    - Clavicle osteotomy
      - in conjunction with above procedures for severe deformity to avoid brachial plexus injury, performed before movement of clavicle.
    - Bony resection
      - extraperiosteal resection of proximal scapular prominence for cosmetic concerns, may be done with other procedures or alone
  - outcomes
    - Woodward and Green procedures can improve abduction by 40-50 degrees