Sprengel deformity is a dysostosis with axial involvement that manifests as unilateral elevation of the shoulder with a hypoplastic scapula. Sprengel deformity may occur as part of Klippel-Feil syndrome, but may also be associated with other abnormalities such as scoliosis, kyphoscoliosis, rib malformations or spina bifida, torticollis, or hypoplasia of the clavicle or humerus.
Origin of the name
The deformity was named after the German surgeon Otto Sprengel (1852–1915)[^1].