EC Paediatrics

We report the case of a patient presenting with Sprengel syndrome, also known as congenital elevation of the scapula, a rare developmental anomaly resulting from defective caudal migration of the scapula during embryogenesis. It is usually diagnosed during childhood and clinically manifests as shoulder asymmetry and limitation of shoulder abduction. This condition may occur as an isolated anomaly or in association with other congenital musculoskeletal abnormalities. One of the characteristic associated findings is the presence of an osseous, fibrous, or cartilaginous omovertebral structure between the scapula and the cervical spine, which may limit scapular mobility. Imaging plays a central role in diagnosis and therapeutic planning. Standard radiography is often the first-line examination. Computed tomography (CT), particularly with three-dimensional reconstructions, provides excellent anatomical assessment of the scapula, the cervicothoracic junction, and possible osseous omovertebral connections. Magnetic resonance imaging (MRI) complements CT by enabling detailed evaluation of soft tissues and detection of non-ossified fibrous or cartilaginous bands, while also assessing potential associated spinal cord or muscular abnormalities. Therapeutic management mainly depends on the patient’s age, the degree of functional limitation, and the severity of the deformity according to the Rigault and Pouliquen classification.

Keywords: Sprengel Deformity; Omovertebral Bone; Vertebral Anomalies; Magnetic Resonance Imaging (MRI); Computed Tomography (CT)

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