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2018-11-11  Case of the week # 487  © Kacar Alaattin

Case of the week # 487
January 17, 2019 - January 31, 2019

Kacar clinic Balikesir - Turkey

Case report

Sprengel's deformity is the congenital failure of descent of the scapula. Eulenburg first described this in 1863. 
Sprengel described 4 cases in 1891; hence, the anomaly has been called Sprengel's deformity. Other terms for this entity are Sprengel's anomaly, Sprengel's shoulder, congenital high scapula, and undescended scapula. 
In this entity, varying degrees of scapula elevation and scapula hypoplasia occur. In approximately one third of cases, Sprengel's deformity is accompanied by an accessory ossicle, the omovertebral bone, which articulates between the medial border of the scapula and one or more of the cervical vertebrae. In the absence of an omovertebral bone, a fibrous fascial sheath extends from the superior angle of the scapula to the spinous process, the lamina, or the transverse process of one or more lower cervical vertebrae. This fixation accounts for the medial position and elevation of the scapula. We report a case of prenatal ultrasonographic detection of Sprengel's deformity.

Images 1-4; videos 1-5: 2D images showing the omovertebral bone and the elevated scapula.
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