Os odontoideum (OO) is a rare anomaly of the odontoid process first described by Giacomini in 1886. There is considerable debate about the origin of this anomaly, whether congenital or acquired, though a growing body of evidence favors the latter.Using PubMed, we reviewed the literature on OO with regards to its etiology, clinical presentations, diagnostic modalities, and management. Manuscripts cited in reviews were also searched manually.Because the medical literature on this condition is limited, our understanding of the natural history and management of OO is still vague. The management guidelines for asymptomatic OO are preliminary. Therefore, we need more large-center studies to investigate this condition further.
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