The rare sprengel deformity: our experience with three cases.

ABSTRACT

Sprengel shoulder is a rare congenital deformity of one or both scapulae that is usually detected at birth. It occurs due to failure of the scapula to descend during intrauterine development and its cause is still unknown. Although the deformity appears randomly most of the time, familial cases have been reported. Sprengel shoulder is often associated with Klippel-Feil syndrome and other congenital skeletal deformities. Anteroposterior X-ray imaging can accurately diagnose Sprengel deformity. However, computed tomography and magnetic resonance scans with three-dimensional reconstruction are nowadays used in everyday practice in order to diagnose concomitant abnormalities, study in detail the anatomy of the affected shoulder(s), and plan appropriate management. We present here our imaging experience from three pediatric cases with Sprengel shoulder and take the opportunity to discuss this rare entity, which is, nevertheless, the commonest congenital defect of the scapula.