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Sprengel's Deformity

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Topic updated on 02/17/15 12:16pm
Introduction
  • Congenital condition with a small and undescended scapula often associated with q
    • 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
    • location
      • bilateral in 10-30% of cases
  • 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 is the only true diarthrodial articulation 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 q
    • 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 to identify omovertebral bar
      • procedures
        • Woodward procedure
          • detachment and reattachment of medial parascapular muscles at spinous process origin to allow scapula to move inferiorly
          • modified Woodward includes resection of superior pole 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
        • Bony resection 
          • extraperiosteal resection of scapular prominence alone if cosmetic concerns only
      • outcomes
        • Woodward and Green procedures can improve abduction by 40-50 degrees

 

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Qbank (3 Questions)

TAG
(OBQ09.231) Which of the following conditions is characterized by failure of the scapula to migrate caudally during fetal development? Topic Review Topic

1. Poland's syndrome
2. Sprengel's deformity
3. Kniest's dysplasia
4. Beckwith-Wiedemann Syndrome
5. Rett syndrome

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TAG
(OBQ04.26) A 4-year-old boy with Klippel-Feil syndrome has elevation of the left scapula since birth. Spine radiographs shows no evidence of scoliosis. What shoulder motion is likely to be most limited? Topic Review Topic

1. adduction
2. abduction
3. internal rotation
4. external rotation
5. extension

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