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 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 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
QUESTIONS 1 of 3 1 2 3 Previous Next (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 4% (62/1768) 2 Sprengel's deformity 93% (1643/1768) 3 Kniest's dysplasia 1% (26/1768) 4 Beckwith-Wiedemann Syndrome 1% (17/1768) 5 Rett syndrome 0% (7/1768) ML 1 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% (128/2932) 2 abduction 79% (2320/2932) 3 internal rotation 4% (125/2932) 4 external rotation 8% (243/2932) 5 extension 4% (104/2932) ML 2 Select Answer to see Preferred Response PREFERRED RESPONSE 2 Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK
Sprengel Deformity in a 9M (C2709) buddhi prasad bhandari Pediatrics - Sprengel's Deformity 10/20/2016 585 11 2