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
QUESTIONS 1 of 4 1 2 3 4 Previous Next (SBQ13PE.81) A 5-year-old patient presents to the orthopedic clinic with shoulder asymmetry and limited abduction. The patient's parents explain this deformity has been present since birth, and now the child is unable to reach overhead and participate in play. On examination, the left scapula is elevated with medial rotation of the inferior pole. Which of the following is the most common associated finding on further imaging? QID: 5231 Type & Select Correct Answer 1 Absent clavicle 24% (496/2062) 2 Spina bifida 3% (57/2062) 3 Cervical spine abnormalities 56% (1152/2062) 4 Horseshoe kidney 9% (177/2062) 5 Proximal humeral deficiency 7% (153/2062) L 4 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ09.231) Which of the following conditions is characterized by failure of the scapula to migrate caudally during fetal development? QID: 3044 Type & Select Correct Answer 1 Poland's syndrome 3% (88/2678) 2 Sprengel's deformity 93% (2494/2678) 3 Kniest's dysplasia 1% (37/2678) 4 Beckwith-Wiedemann Syndrome 1% (32/2678) 5 Rett syndrome 0% (12/2678) L 1 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic (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? QID: 87 Type & Select Correct Answer 1 adduction 4% (138/3308) 2 abduction 79% (2622/3308) 3 internal rotation 4% (139/3308) 4 external rotation 8% (276/3308) 5 extension 4% (119/3308) L 2 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic
All Videos (1) Podcasts (1) Login to View Community Videos Login to View Community Videos 12th International Congress on Early Onset Scoliosis - 2018 Sprengel’s Deformity – Treatment Options - Ilkka Helenius, MD (ICEOS 2018, #139) Pediatrics - Sprengel's Deformity A 2/12/2019 400 views 5.0 (3) Pediatrics⎪Sprengel's Deformity Orthobullets Team Pediatrics - Sprengel's Deformity Listen Now 9:59 min 3/12/2020 368 plays 4.0 (4)
Sprengel Deformity in a 9M (C2709) buddhi prasad bhandari Pediatrics - Sprengel's Deformity E 10/20/2016 882 12 4