Sprengel deformity is an uncommon condition in which the scapula (shoulder blade) on one side is higher in the neck than the other side. Normally, during prenatal development, both scapula begin high in the neck and slowly descend toward their normal resting position in the upper back. If, for unclear reasons, one scapula does not descend, a Sprengels deformity results causing two issues:
1) functional issues related to decreased motion of the arm. Typically, the patient will have decreased forward flexion (raising the arm in front until straight above the head) and decreased abduction (moving the arm away from body in the plane of the body).
2) appearance issues related to the high scapula. Essentially, it looks like a large bump in the neck.
When a Sprengel deformity is identified, associated conditions should be considered including Klippel Feil, scoliosis, cervical ribs, and torticollis.
Treatment depends on the severity of the condition- specifically how much the limited motion and abnormal appearance bother the patient/ family. Therapy may be beneficial in improving motion but ultimately if a big enough problem, surgery is the best way to improve motion and appearance.
My preferred treatment is a modified Woodward procedure in which we removed the upper portion of the scapula (i.e., the prominence) and bring the scapula down to a more normal resting position. The scapula is not normally sized and so we have to be careful not to overcorrect or bring the scapula down too far. Depending on age, we may also perform an osteotomy (i.e., cutting) of the clavicle to reduce the risk of nerve injury during the procedure. The preferred age of the surgery has gradually become younger and we prefer surgery between 3-8 years of age. Younger patients may have a better outcome as judged by the improvement in motion. Older patients can also have a very good outcome with improvement in motion and appearance.
|Sprengel deformity at rest. Notice elevated shoulder on left.|
|Sprengel deformity with limited arm elevation.|
|Xray of Sprengel Deformity. Note bump in left shoulder compared to right. That is the high shoulder blade.|