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Journal of Craniovertebral Junction and Spine
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CASE REPORT
Year : 2021  |  Volume : 12  |  Issue : 1  |  Page : 95-98

Report of two cases with omovertebral bone and Klippel–Feil syndrome with craniovertebral junction instability


1 Department of Neurosurgery, K.E.M. Hospital and Seth G.S. Medical College, Mumbai, Maharashtra, India
2 Department of Neurosurgery, K.E.M. Hospital and Seth G.S. Medical College; Lilavati Hospital and Research Centre, Mumbai, Maharashtra, India

Correspondence Address:
Atul Goel
Department of Neurosurgery, K.E.M. Hospital and Seth G.S. Medical College, Parel, Mumbai - 400 012, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcvjs.jcvjs_7_21

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We present a report of two patients having the association of omovertebra, Sprengel's deformity of the shoulder and Klippel–Feil abnormality with craniovertebral junctional instability. Our literature survey did not locate any report of such association. Significance of bone alterations is analyzed. Two young patients presented with neck pain, torticollis, webbed neck, and spastic quadriparesis. In both patients, the investigations revealed basilar invagination, Klippel–Feil abnormality and Sprengel's deformity of the shoulder. Apart from these relatively common associations, both the patients had omovertebral bone that extended from the transverse process of C5 vertebra to scapula. Following atlantoaxial stabilization surgery, the patients rapidly recovered from all symptoms. Musculoskeletal abnormalities at the craniovertebral junction that include Klippel–Feil abnormality, Sprengel's shoulder, and omovertebra are secondary alterations to primary atlantoaxial instability.


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