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Atlanto-axial segmentation defects and os odontoideum in two male siblings with opsismodysplasia
Authors:Ali Al Kaissi  Farid Ben Chehida  Maher Ben Ghachem  Franz Grill  Klaus Klaushofer
Institution:1.Ludwig Boltzmann Institute of Osteology at the Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling, 4th Medical Department,Hanusch Hospital,Vienna,Austria;2.Department of Paediatric Orthopaedic Surgery,Children’s Hospital of Tunis,Jabari,Tunisia;3.Center of Radiology, Department of Imaging Studies,Ibn Zohr Institute,Tunis,Tunisia;4.Paediatric Department,Orthopaedic Hospital of Speising,Vienna,Austria
Abstract:We report two siblings aged 11 and 7 years, respectively, who presented with the clinical and radiographic features of opsismodysplasia (non-lethal type). 3D computed tomography scans of the craniocervical region revealed a split atlas and os odontoideum in both siblings. To the best of our knowledge, this is the first clinical report detailing craniocervical malformations in two siblings with opsismodysplasia.
Keywords:Opsismodysplasia  Os odontoideum  Atlas splitting  Stillbirths  CT scan
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