What Is the Cause of This Toddler’s Limited Shoulder Movement?

University of Iowa

Dr Wood is assistant clinical professor and pediatric hospitalist in the department of pediatrics at University of Iowa Carver College of Medicine in Iowa City.

Dr Wood has no conflicts of interest or financial sources to disclose.

WILLIAM YAAKOB, MD—Series Editor: Dr Yaakob is a radiologist in Tallahassee, Fla.

sprengel deformity

A 3-year-old boy has had limited range of motion of his right shoulder since 3 months of age. There was no history of trauma, weakness, or paralysis. He has full range of motion of all other joints. His right hand fine motor skills are normal. His development is normal, and he is otherwise healthy. He has been working with physical therapy with minimal improvement.

On examination, the child is active and well-appearing. He has limited abduction of the right shoulder to 110 degrees. There is no joint warmth, erythema, or effusion.

No tenderness to palpation over the clavicle, humerus, or scapula. Scalpula is elevated and internally rotated on examination of the back. The right elbow, wrist, and hand are normal.

What is the cause of this toddler’s limited shoulder movement?

A. Brachial plexus injury.
B. Sprengel deformity.
C. Glenohumeral dislocation.
D. Proximal humerus fracture.
E. Acute deltoid strain.

(Answer and discussion on next page.)

sprengel deformity

Answer: B, Sprengel deformity

The radiograph shows a right-sided Sprengel deformity, with the right scapula elevated and the inferior border rotated medially.

In Sprengel deformity, or congenital elevation of the scapula, the scapula is more superior than normal and often hypoplastic. The condition results from failure of the scapula to descend during embryonic development. It is the most common congenital abnormality of the shoulder.1 Concurrent skeletal anomalies, including scoliosis, rib deformities, and Klippel-Feil syndrome with fusion of the cervical spine, may be present.2,3

The degree of deformity varies from mild to severe depending on the elevation, motion limitation, and hypoplasia. The defect may be cosmetic in mild cases or limit shoulder motion in severe cases. Patients present with impaired shoulder mobility and a palpable or visible bump, which corresponds to the superomedial corner of the scapula.4 Imaging is required to evaluate the deformity and look for other associated abnormalities.3

Surgery is reserved for severe deformities and is preferably performed at 3 years of age for best outcome and decreased risk of brachial plexus injury.2 This patient had an isolated anomaly and underwent a modified Woodward procedure with successful release of his scapula and improved shoulder abduction.