![]() We also provide an overview of different management strategies, surgical complications, and postoperative considerations for OO patients. In this review, we discuss epidemiology, embryology, pathophysiology, clinical presentation, and the role of diagnostic radiography in OO. ![]() Treatment recommendations between conservative or operative management thus depend on the severity and type of clinical presentation in OO patients. Although neurological deterioration can occur in OO patients when cervical spine instability is left untreated, predictive factors for disease progression are unclear, especially in asymptomatic patients. In severe cases, the condition can lead to sudden death resulting from paralysis of the respiratory center. At the onset of the disease, compromised joint stability can be accompanied by neural and vascular compression, predisposing patients to sensory disorders, cervical myelopathy, quadriplegia, and other motor disturbances in addition to intracranial symptoms such as cerebellar infarction or brainstem damage. Such symptoms arise from the instability of the joint between the C1 and C2 vertebrae, which can further lead to atlantoaxial dislocation (AAD), occipital-cervical pain, or vertebrobasilar ischemia. Both types present variably, ranging from asymptomatic incidental findings to neurological dysfunction. Orthotopic refers to an ossicle that moves in conjunction with the anterior arch of C1, while dystopic denotes an ossicle that is functionally fused to the basion. The condition was first described by Giacomini in 1886 and is classified as either orthotopic or dystopic. OO has a controversial etiology, natural history, and management, compounded by a paucity of literature. Os odontoideum (OO) is a rare anomaly of the craniocervical junction (CVJ), in which a round ossicle is detached from a hypoplastic odontoid process at the body of the second cervical vertebrae (C2). More large-center studies are thus needed to promote accurate management of OO patients with optimal outcomes. Further understanding of the natural history of OO is critical to form evidence-based guidelines for the management of OO patients. Conflicting studies and the paucity of current literature on OO highlight poor comprehension of the condition. Although multiple studies show different techniques for surgical management involving posterior fusion, the diversity of such cases illustrates how treatment must be tailored to the individual patient to prevent complications. Asymptomatic patients can be managed either conservatively or surgically, while symptomatic patients should undergo operative stabilization. The presentation of OO varies widely from asymptomatic cases to severe neurological deficits. A search using the query “os odontoideum” yielded 4319 results, of which 112 articles were chosen and analyzed for insights on factors such as etiology, clinical presentation, and management of OO. A literature review was conducted using the PubMed, EMBASE, and Scopus databases. By providing an overview of different management strategies, surgical complications, and postoperative considerations for OO, our findings may guide neurosurgeons in providing proper diagnosis and treatment for OO patients. In this literature review, we aimed to investigate epidemiology, embryology, pathophysiology, clinical presentation, and the role of diagnostic radiography in OO. ![]() With a controversial etiology and unknown prevalence in the population, OO may be asymptomatic or present in patients with myelopathic and neurological symptoms. Os odontoideum (OO) is a rare craniocervical anomaly that is characterized by a round ossicle separated from the C2 vertebral body. ![]()
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