Sprengel deformity in Klippel-Feil syndrome

Case contributed by Dalia Ibrahim
Diagnosis almost certain

Presentation

Left shoulder deformity.

Patient Data

Age: 10 years
Gender: Male
Topogram
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Axial
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Axial bone
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Axial lung
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Axial bone
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3D-VR-Bone
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Coronal
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Coronal
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Sagittal
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Info

Elevated left scapula with an omovertebral bar (osseous connection between scapula and cervical spine).

C6-7 block vertebrae.

Case Discussion

A Sprengel deformity, or congenital elevation of scapula, is a complex deformity of the shoulder and is the most common congenital shoulder abnormality.

Sprengel deformity usually coexists with other congenital abnormalities, particularly those involving the vertebrae and ribs. An omovertebral bar (fibrous, cartilaginous and/ or osseous connection between scapula and cervical spine) is often present.

Patients with Sprengel deformity often have one or more of the following abnormalities and conditions:

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