Prenatal ultrasonographic diagnosis of Sprengel's deformity

Abstract

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 1 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 1 or more lower cervical vertebrae. This fixation accounts for the medial position and elevation of the scapula. I report a case of prenatal ultrasonographic detection of Sprengel's deformity.