Journal of Orthopaedics Trauma Surgery and Related Research

Journal of Orthopaedics Trauma Surgery and Related Research

An Official Journal of Polish Society of Orthopaedics and Traumatology

ISSN:1897-2276
e-ISSN: 2449-9145

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Congenital defect of craniovertebral junction in twins with Kniest dysplasia – case report

Author(s): MACIEJ TESIOROWSKI, STANISLAW KWIATKOWSKI, BARBARA JASIEWICZ, WOJCIECH KACKI, ADRIAN RYMARCZYK, PIOTR SEBASTIANOWICZ

Kniest dysplasia is a rare disease from the group of osteochondrodysplasias, rated type II colagenopathies, and like other skeletal dysplasias may be associated with defects of cranio-vertebral junction with atlanto-axial instability. Objects: a case of spontaneous atlantoaxial subluxation with spinal canal narrowing at the level of craniovertebral junction in twins with Kniest dysplasia was presented. Methods: on the basis of clinical examination, radiographs and MRI study, children were qualified for operative treatment - posterior widening of the foramen magnum and occipito-cervical fusion with titanium anchors. During control examination 4 years after primary surgery, patients was very active, without any neurological deficits, x-ray pictures and MRI study revealed widening of the foramen magnum and full fusion in the place of surgery. Conclusion: authors suggestion is that in the case of foramen magnum and spinal canal narrowing, operative treatment with decompression of nervous structures and stabilization should be advised.


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Annals of Medical and Health Sciences Research Journal of Orthopaedics Trauma Surgery and Related Research a publication of Polish Society, is a peer-reviewed online journal with quaterly print on demand compilation of issues published.
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