Bilateral Sprengel deformity with Klippel-Feil syndrome

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Deformity of both shoulders.

Patient Data

Age: 6 years
Gender: Female

Elevation of both scapulae (more on the left) with a superomedial angle at C7 level on the right (grade II), and at C4 on the left side (grade III), according to the radiographic Rigault classification.

Bilateral omovertebral bone is seen connecting the scapulae to the cervical spine (incomplet on the right, and complete on the left).

There is congenital fusion of the vertebral bodies of C4, C5, and C6 (sagittal sequence not performed, however, it is clearly visible on the coronal sequences), findings in keeping with associated Klippel-Feil syndrome.

​No significant hypoplasia or atrophy of the shoulder girdle muscles.

Case Discussion

MRI Features of bilateral Sprengel deformity grade II on the right, and grade III (according to the radiographic Rigault classification) with associated Klippel-Feil syndrome

CT or MRI is often necessary to evaluate this deformity as well as the associated anomalies.

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