Sprengel deformity: a rare congenital anomaly of the scapula

The authors report a case of a typical Sprengel deformity, a rare morphological anomaly which affects one or both scapula. The anomaly is more frequently a sporadic occurrence, but there are well-documented familial cases where inheritance has been reported. which appears located in a higher position and angles down towards the spine (Warkany, 1972). The anomaly is also known as scapula alata, undescended scapula, or scapula elevata, but the name “Sprengel deformity” is the most common. The first report of the anomaly of the scapula is credited to Eulenburg (1863), who reported a single case. Twenty years later, Willet & Walsham (1883) reported an anatomical description of two patients, and Sprengel (1891) described a clinical report of four patients with this anomaly, which was later named after him. The anomaly is rarely inherited and is more frequently a sporadic occurrence, but there are welldocumented familial cases where a dominant pattern of inheritance has been reported (Wilson et al., 1997). We report here a girl with a typical Sprengel deformity affecting the right scapula. The girl has been followed up for three years.