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