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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
    • Male to female ratio 1:3 q
    • 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
  • 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 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
 

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Questions (3)

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

QID: 3044
1

Poland's syndrome

3%

(51/1464)

2

Sprengel's deformity

93%

(1362/1464)

3

Kniest's dysplasia

1%

(20/1464)

4

Beckwith-Wiedemann Syndrome

1%

(16/1464)

5

Rett syndrome

0%

(5/1464)

Select Answer to see Preferred Response

PREFERRED RESPONSE 2

(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? Review Topic

QID: 87
1

adduction

4%

(113/2761)

2

abduction

79%

(2184/2761)

3

internal rotation

4%

(122/2761)

4

external rotation

8%

(228/2761)

5

extension

4%

(103/2761)

Select Answer to see Preferred Response

PREFERRED RESPONSE 2
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