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Updated: Jun 30 2023

Sprengel's Deformity

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  • summary
    • Sprengel's Deformity is a congenital condition characterized by a small and undescended scapula often associated with scapular winging and scapular hypoplasia. 
    • Diagnosis is made clinically with a high-riding, medially rotated, triangular-shaped scapula, with associated limitations in shoulder abduction and flexion. 
    • Treatment is observation in the absence of shoulder dysfunction. Operative management is indicated in the presence of severe cosmetic concerns or functional deformities (abduction < 110-120 degrees).
  • Epidemiology
    • Incidence
      • most common congenital shoulder anomaly in children
    • Demographics
      • male to female ratio 1:3
    • Anatomic location
      • bilateral in 10-30% of cases
  • Etiology
    • Associated conditions
      • scapular winging
      • hypoplasia
      • omovertebral connection between superior medial angle of scapula and cervical spine (30-50%)
    • Pathophysiology
      • 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
    • Symptoms
      • often referred for evaluation of scoliosis
    • Physical exam
      • high riding medially rotated scapula
      • loss of long medial border
      • equilateral triangle like shape
      • shoulder abduction most limited due to loss of normal scapulothoracic motion and glenoid malpositioning
      • forward flexion limited as well
  • 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 scapula.
          • 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
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