Often, only one shoulder blade (i.e. one side of the body) is affected. However, in some cases both shoulder blades can be affected. Generally, these abnormalities tend to be painless. The degree of elevation and displacement in Sprengel deformity can vary greatly from one person to another and can range from very mild, in which the abnormality cannot be seen when wearing clothes, to severe cases in which the shoulder is noticeably elevated. Milder cases can go undiagnosed until adolescence. The reported range of displacement of the shoulder blade is 2-10 centimeters, or approximately a half an inch to 4 inches.
The main signs and symptoms of Sprengel deformity are limited or restricted movement of the arm and shoulder blade on the affected side as well as the cervical spine. Some affected individuals have neck deformities as well, ranging from mild tilting (torticollis) to severe spine deformity. In severe cases, the neck may be abnormally Short (brevicollis) and webbed.
In approximately 75% of cases, Sprengel deformity is associated with additional abnormalities, most commonly Klippel-Feil syndrome, but also scoliosis, spina bifida, hemivertebrae, rib segmentation abnormalities, clavicular abnormalities, and underdevelopment (hypoplasia) of neck or shoulder muscles.