Klippel Feil syndrome - Sprengel deformity

Case contributed by Dr Yasser Asiri

Presentation

Shoulder deformity, for assessment.

Patient Data

Age: 2 years
Gender: Female

Right scapula is more elevated as compared to the left with right paracervical bone density suggestive of omovertebral bone and right shoulder Sprengel deformity. Rest of the exam is unremarkable.

CT study better illustrates the elevation of the right scapula with an omovertebral bone extending to the right posterior elements of the C3-C5 cervical vertebrae.  There are multilevel segmentation fusion anomalies of the cervical spine and craniocervical junction, including congenital fusion of C4 and C5 vertebral bodies, C3 and C2 vertebral bodies. The left shoulder appears unremarkable.


Sprengel deformity of the right shoulder with right omovertebral bone and multilevel segmentation fusion anomalies of the cervical spine are consistent with Klippel-Feil syndrome.

Case Discussion

This case shows the combination of Sprengel deformity with omovertebral bone with multilevel segmentation anomalies in patient with Klippel-Feil syndrome.

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Case information

rID: 65133
Published: 3rd Jan 2019
Last edited: 4th Jan 2019
Inclusion in quiz mode: Included

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