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A Review of Os Odontoideum in Children

Abstract

Elvira A Allakhverdieva, Ryan A Grant and Michael Diluna

Study Background: Os odontoideum is an anomaly of the cervical axis characterized by an independent ossicle of variable size separated from the hypoplastic dens that can present as an incidental radiographic finding to severe neck pain, torticollis, myelopathy, as well as seizures and syncope. Diagnosis is primarily made based on clinical presentation and extensive radiologic evaluation. In this review, we discuss the etiology, treatment, and management of this rare condition in the pediatric population and propose a treatment algorithm.
Methods: We performed a comprehensive retrospective review of English literature in PubMed on surgical approaches to treatment and stabilization of Os osontoideum in children.
Results: Asymptomatic children, who are diagnosed with Os odontoideum, but lack overt neurologic deficits, are advised to continue radiologic monitoring with lateral flexion-extension X-rays once a year and MRI evaluation once every five years. For symptomatic patients with significant atlantoaxial instability, neurologic deficits, or myelopathy, surgical treatment is recommended. In cases where the atlantoaxial joint is aligned properly without any kyphotic deformities and vertebral arteries abnormalities, transarticular C1-C2 screw fixation should be considered. If these requirements are not met, C1 lateral mass screws with C2 pars/pedicle screws method are advised. The transoral approach, odontoid screw fixation, and minimally invasive techniques are possible alternatives, but have not been studied enough to be considered standard treatments in pediatric population.
Conclusions: These recommendations are meant to initiate a “best practices” strategy for treatment of Os odontoideum in children. We anticipate further refinement of these recommendations as new research and reports arise in the field of minimally invasive neurosurgery.

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