Klippel-Feil syndrome with sprengel deformity


Sprengel deformity is the most common congenital shoulder deformity. This causes cosmetic and functional problems. There is an associated restricted motion of the scapula and glenohumeral joint. It is associated with other abnormalities of the vertebrae and ribs. The omovertebral bone (os omovertebrale) is present in about 35% of cases.

In this case; no associated omovertebral bone connection between the scapula and cervical spine. Bifid configuration of the right first rib is noted with crowding of the lower ribs. Fusion of the visualized lower cervical vertebral bodies is also seen with Wasp-waist sign seen as block vertebra of C6 and C7. Spina bifida is noted at C6 and C7 posterior arches. D2 and D4 hemivertebra are noted with hypoplasia of the right component of vertebral body. Mild S-shaped cervicothoracic scoliosis is noted with mild deviation of the mediastinum to the right side.

These features raise the possibility of associated Klippel-Feil syndrome.

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