1. IN THE NAME OF GOD 7/7/20161sprengel deformity

2. Congenital elevation of the scapula Mohammad reza etemadifar MD St Zahra hospital Isfahan university of medical sciences 7/7/20162sprengel deformity

3. definition first described by Eulenberg in 1863 first described by Eulenberg in 1863 Scapula is: Scapula is: more superiorly than normal more superiorly than normal Misshapen Misshapen Hypoplastic Hypoplastic Other congenital anomalies may be present such as cervical ribs, malformations of ribs, and anomalies of the cervical vertebrae Other congenital anomalies may be present such as cervical ribs, malformations of ribs, and anomalies of the cervical vertebrae 7/7/20163sprengel deformity

4. The deformity develops due to arrest of the caudal migration of the scapula during development The deformity develops due to arrest of the caudal migration of the scapula during development The deformity may cause functional and cosmetic impairment of the shoulder that is related to the extent of elevation of the scapula and severity of pathological condition The deformity may cause functional and cosmetic impairment of the shoulder that is related to the extent of elevation of the scapula and severity of pathological condition Almost always sporadically Almost always sporadically 7/7/20164sprengel deformity

5. pathology Scapula Scapula Abnormally high,rotated Abnormally high,rotated Small Small Smaller vertical diameter Smaller vertical diameter greater width greater width Supraspinatous portion tilted forward Supraspinatous portion tilted forward Omovertebral bone(20 - 30%) Omovertebral bone(20 - 30%) Bony Bony Cartilagenous Cartilagenous Fibrous band Fibrous band 7/7/20165sprengel deformity

6. Muscle defects Trapezius Trapezius Absent Absent Weak Weak Rhomboids and levator scapula Rhomboids and levator scapula Fibrosed and hypoplastic Fibrosed and hypoplastic 7/7/20166sprengel deformity

7. Associated anomalies(up to 75%) Absent or fused ribs Absent or fused ribs Cervical ribs Cervical ribs Klippel feil syndrome Klippel feil syndrome Congenital scoliosis Congenital scoliosis Spina bifida Spina bifida Renal anomalies Renal anomalies 7/7/20167sprengel deformity

8. Clinical features Female>male (3/1) Female>male (3/1) Asymmetry of shoulder Asymmetry of shoulder Left>right Left>right Omovertebral bone may be palpable Omovertebral bone may be palpable Limitation of abduction Limitation of abduction 7/7/20168sprengel deformity

9. 7/7/2016sprengel deformity9

10. X-RAY Scapular elevation Scapular elevation Deformity Deformity The best measurment method: The best measurment method: Glenoid height difference Glenoid height difference 7/7/201610sprengel deformity

11. Treatment Aims Correction of deformity (cosmesis) Correction of deformity (cosmesis) Improve function(shoulder abduction) Improve function(shoulder abduction) 7/7/201611sprengel deformity

12. Factors for decision Severity of deformity Severity of deformity Functional impairment Functional impairment Associated anomalies Associated anomalies Age of patient (3 to 8 ys) Age of patient (3 to 8 ys) 7/7/201612sprengel deformity

13. Treatment options Observation Observation mild cases with minor dysfunction mild cases with minor dysfunction Surgery Surgery More severe caseswith significant cosmetic and functional problems More severe caseswith significant cosmetic and functional problems 7/7/2016sprengel deformity13

14. Surgical operation Putti: muscle release Putti: muscle release Schrocks: resection of supraspinatous portion Schrocks: resection of supraspinatous portion Ober Ober Koeing: sliding osteotomy Koeing: sliding osteotomy Mc farland: sub total scapulectomy Mc farland: sub total scapulectomy Green: muscle release Green: muscle release Woodward: muscle release origin Woodward: muscle release origin Mear technique(scapular neck osteotomy) Mear technique(scapular neck osteotomy) 7/7/201614sprengel deformity

15. Four surgical categories Resection of elevated corner of scapula and omovertebral bone Resection of elevated corner of scapula and omovertebral bone Releasing medial muscle attachments Releasing medial muscle attachments Vertical medial sliding osteotomy Vertical medial sliding osteotomy Transfering muscle attachment from spine Transfering muscle attachment from spine 7/7/201615sprengel deformity

16. Severity of deformity Cavendish classification Cavendish classification Grade 1: not detectable with cloth Grade 1: not detectable with cloth Grade 2: detectable with cloth Grade 2: detectable with cloth Grade 3: 2-5 cm elevation Grade 3: 2-5 cm elevation Grade 4: more than 5 cm elevation Grade 4: more than 5 cm elevation 7/7/201616sprengel deformity

17. 7/7/201617sprengel deformity

18. Preferred surgical method Resection of omovertebral bone Resection of omovertebral bone Resection of supraspinetous portion Resection of supraspinetous portion Muscle release from spine Muscle release from spine Downward transposition of scapula Downward transposition of scapula 7/7/201618sprengel deformity

19. Results of surgery Improved function (abduction) Improved function (abduction) Improved cosmesis( one cavendish grade) Improved cosmesis( one cavendish grade) 7/7/201619sprengel deformity

20. Complications of surgery Brachial plexus palsy (temporary) Brachial plexus palsy (temporary) Scapular winging Scapular winging Regrowth of superior pole Regrowth of superior pole Surgical scar Surgical scar 7/7/201620sprengel deformity

21. Poor prognostic factors for surgery Winging preoperatively Winging preoperatively Cavendish grade 4 Cavendish grade 4 7/7/201621sprengel deformity

22. Thank you for your attention 7/7/2016sprengel deformity22