Atlantoaxial dislocation due to Os odontoideum in down syndrome: Literature review and case reports.

INTRODUCTION AND IMPORTANCE: Os odontoideum is a rare condition commonly associated with atlantoaxial instability (AAI) and leading to atlantoaxial dislocation. The incidence of Os odontoideum is higher in patients with Down syndrome. Similar to odontoid fractures, atlantoaxial dislocation in patients with Os odontoideum can result in neurological deficits, disability, and even mortality. CASE PRESENTATION: We present two cases of Os odontoideum accompanied by Down syndrome. Both patients were hospitalized due to progressive tetraparesis after falls several months prior. Upon examination, the patients exhibited myelopathy and were unable to walk or stand. MRI revealed spinal stenosis at the C1-C2 level due to atlantoaxial dislocation. C1-C2 fixation using Harms' technique was performed in both cases. One case experienced a complication involving instrument failure, necessitating revision surgery. CLINICAL DISCUSSION: Due to the characteristics of transverse ligament laxity, low muscle tone, excessive joint flexibility, and cognitive impairment, children with both Down syndrome and Os odontoideum are at a high risk of disability and even mortality from spinal cord injury. Most authors recommend surgical management when patients exhibit atlantoaxial instability. Additional factors such as low bone density, cognitive impairment, and a high head-to-body ratio may increase the risk of surgical instrument failure and nonunion postoperatively in patients with Down syndrome. CONCLUSION: Os odontoideum is a cause of AAI in patients with DS. Indication of surgery in the presence of AAI helps to resolve neurological injury and prevent further deterioration. The use of a cervical collar is considered to prevent instrument failure postoperatively.

Augmented Reality in Spine Surgery: A Case Study of Atlantoaxial Instrumentation in Os Odontoideum.

Despite advancement in surgical innovation, C1-C2 fixation remains challenging due to risks of screw malposition and vertebral artery (VA) injuries. Traditional image-based navigation, while useful, often demands that surgeons frequently shift their attention to external monitors, potentially causing distractions. In this article, we introduce a microscope-based augmented reality (AR) navigation system that projects both anatomical information and real-time navigation images directly onto the surgical field. In the present case report, we discuss a 37-year-old female who suffered from os odontoideum with C1-C2 subluxation. Employing AR-assisted navigation, the patient underwent the successful posterior instrumentation of C1-C2. The integrated AR system offers direct visualization, potentially minimizing surgical distractions. In our opinion, as AR technology advances, its adoption in surgical practices and education is anticipated to expand.

Surgical strategies in the management of atlantoaxial dislocation in Down syndrome.

Aims: To study the clinicoradiological features and treatment outcomes of atlantoaxial dislocation (AAD) in Down syndrome. Settings and Design: Retrospective case series. Subjects and Methods: A retrospective chart and radiology review of 9 Down syndrome patients with AAD managed at our center from 2007 to 2018. Statistical Analysis Used: Chi-squared/Fisher's exact test. Results: There were 4 males and 5 females (n = 9). The median age was 14 years (interquartile range [IQR]: 7-15.5). 77.7% (7/9) of patients had severe spasticity (Nurick Grades 4 and 5). The median duration of symptoms was 9 months (IQR: 5-39). The AAD was reducible in all (n = 9) cases. Eight (88.8%) patients had os odontoideum. The mean atlantodental interval (ADI) was 8.5 mm (±2.9). T2W cord hyperintensity was seen in 66.6% (6/9). Posterior C1-2 transarticular fixation was done in 8 and occipitocervical fusion in 1 patient. Follow-up of more than 6 months (7-57 months) was available in 8/9 (88.9%) patients. There was a significant improvement in spasticity (n = 8, mean Nurick Grade 1.7 (±1.1), P = 0.003). Follow-up radiographs (n = 8) showed good reduction and fusion. A preoperative bedbound patient with poor respiratory reserve expired at 10 months following surgery. There were no other complications. Conclusions: Posterior surgical approach for AAD in Down syndrome resulted in good alignment and fusion, with excellent clinical improvement. Patients with elevated PCO2 are poor surgical candidates and require home ventilation facility.

Fatal cervical myelopathy in a child with glutaric aciduria type 1.

We report the case of a Syrian female refugee with late diagnosis of glutaric aciduria type 1 characterised by massive axial hypotonia and quadriplegia who only started adequate diet upon arrival in Switzerland at the age of 4 years, after a strenuous migration journey. Soon after arrival, she died from an unexpected severe upper cervical myelopathy, heralded by acute respiratory distress after a viral infection. This was likely due to repeated strains on her hypotonic neck and precipitated by an orthotopic os odontoideum who led to atlanto-axial subluxation. This case reminds us not to omit handling patients with insufficient postural control and hypotonia with great care to avoid progressive cervical myelopathy.

Os odontoideum and craniovertebral junction instability secondary to dystonia: case series and review of the literature.

INTRODUCTION: Os odontoideum refers to a rounded ossicle detached from a hypoplastic odontoid process at the body of the axis. The aetiology has been debated and believed to be either congenital or acquired (resulting from trauma). Os odontoideum results in incompetence of the transverse ligament and thus predisposes to atlantoaxial instability and spinal cord injury. METHODS/RESULTS: Three cases of children with severe dystonic cerebral palsy presenting with myelopathic deterioration secondary to atlantoaxial instability due to os odontoideum are presented. This observation supports the hypothesis of os odontoideum being an acquired phenomenon, secondary to chronic excessive movement with damage to the developing odontoid process. CONCLUSION: In children with cerebral palsy and dystonia, pre-existing motor deficits may conceal an evolving myelopathy and result in delayed diagnosis of clinically significant atlantoaxial subluxation.

Os odontoideum: database analysis of 260 patients regarding etiology, associated abnormalities, and literature review.

Introduction: Since the first description of os odontoideum in 1886, its origin has been debated. Numerous case series and reports show both a possible congenital origin and origin from the secondary to craniovertebral junction (CVJ) trauma. We conducted a detailed analysis of 260 surgically treated cases to document the initial symptoms, age groups, radiographic findings, and associated abnormalities, aiming to enhance the confirmation of the etiology. A literature search (1970-2022) was performed to correlate our findings. Methods and materials: A total of 260 patients underwent surgical management of a referral database of 520 cases (1978-2022). All patients were examined by plain radiography and myelotomography as needed until 1984, and since then, CT and MRI have been employed. History of early childhood (aged below 6 years) CVJ trauma was investigated, including obtaining emergency department's initial radiographs from the referral and subsequent follow-up. Associated radiographic and systemic abnormalities were noted, and the atlas development was followed. Results: The age of the patients ranged from 4 to 68 years, mostly between 10 and 20 years. There were 176 males and 86 females. Orthotopic os odontoideum was identified in 24 patients, and 236 patients had dystopic os odontoideum. Associated abnormalities were found in 94 of 260 patients, with 73 exhibiting syndromic abnormalities and 21 having Chiari I malformation. Two sets of twins had spondyloepiphyseal dysplasia. Of 260 patients, 156 experienced early childhood trauma /. Among these, 54 initially presented with normal radiographs but later demonstrated anterior atlas hypertrophy. In addition, a smaller posterior C1 arch was observed, leading to the development of os odontoideum. Two children had initial CVJ trauma as documented by MRI, with subsequent classical findings of os odontoideum and atlas changes. Syndromic patients had an earlier presentation. The literature reviewed confirms the multifactorial etiology. Conclusions: The early presentation and associated abnormalities (such as Down syndrome, Klippel-Feil syndrome, Chiari I malformation, spondyloepiphyseal dysplasia, Morquio syndrome, and others) along with case reports documenting familial, hereditary, and twin presentations strongly support a congenital origin. Likewise, surgical complications are more prevalent in syndromic patients (40%) compared to 15% in other cases, as reported in the literature. The documentation of normal odontoid in early childhood trauma cases followed by the later development of os odontoideum provides evidence supporting trauma as an etiological factor. This process also involves vascular changes in both the atlas and the formation of os odontoideum. Associated abnormalities exhibit an earlier presentation and are only seen in cases with a non-traumatic origin.

Novel Radiographic Parameters for Posterior Atlantoaxial Dislocation Secondary to Os Odontoideum and Its Clinical Significance.

OBJECTIVE: To explore correlations of 3 novel radiographic parameters with myelopathy induced by posterior atlantoaxial dislocation (PAAD) secondary to os odontoideum (OO) and assess their reproducibility. METHODS: Of the 51 patients with OO with PAAD enrolled in this study, 28 developed PAAD-induced myelopathy (myelopathy group), and the other 23 patients had no myelopathy (control group). Neurologic function was evaluated by the neurologic function rating system and the Japanese Orthopaedic Association score system. Three novel radiographic parameters (OP [median sagittal diameter of the spinal canal from the posteroinferior edge of the ossicle to the anterosuperior edge of the spinous process of C2]/C4 SAC [space available for spinal cord] ratio, C1 posterior inclination angle, and posterior dislocation index) were measured by lateral cervical dynamic radiography. Their correlations with neurologic function were analyzed, and their reproducibility was assessed by the intraclass correlation coefficient (ICC). In addition, receiver operating characteristic curve analysis was performed. RESULTS: A significant correlation was observed between the OP/C4 SAC ratio and the neurologic function (P < 0.01), and between the C1 posterior inclination angle and the neurologic function (P < 0.01). Furthermore, their interobserver and intraobserver reliability was excellent (ICC ≥ 0.912). Receiver operating characteristic curve analysis showed that the optimal threshold value relating to myelopathy of the OP/C4 SAC ratio and C1 posterior inclination angle was 0.93 and 20°, respectively. CONCLUSIONS: The OP/C4 SAC ratio and the C1 posterior inclination angle seem to be 2 effective and objective radiographic parameters for relating myelopathy in patients with OO with PAAD. When the OP/C4 SAC ratio is <0.93 and/or the C1 posterior inclination angle is >20°, the risk of developing myelopathy should be highly suspected in patients with OO with PAAD.

A young man with Guillain-Barré syndrome, with a stroke-like presentation and physical findings suggestive of cervical myelopathy.

We describe the case of a 44-year-old gentleman with hypertension and asthma presenting to the emergency department after noticing right upper-extremity weakness upon awakening. Brain imaging did not reveal a stroke. Initial neurological examination pointed to cervical myelopathy with radiculopathy as well as possible underlying length-dependent peripheral neuropathy as there was right arm strength of 4/5 and there were brisker (3+) reflexes all over except at the right biceps reflex and both ankle reflexes. Cervical spine magnetic resonance imaging (MRI) showed myelomalacia at the C2 level and an os odontoideum (OO). Os odontoideum is a chronic condition that occurs due to the failure of the center of ossification of the dens to fuse with the body of C2. By the next day after a few hours of sustaining a fall, weakness progressed to quadriparesis, without a sensory level on examination, followed by urinary retention. This situation was attributed to a possible cervical cord contusion due to the fall in the presence of OO, with other possibilities being spinal cord hemorrhage, infarct and transverse myelitis. However, repeat scanning of the cervical spine (MRI) did not reveal any acute cord changes. The initial examination for common causes of peripheral neuropathy did not reveal any findings. Finally, the diagnosis of Guillain-Barré syndrome (GBS) was considered, and treatment was initiated with intravenous immunoglobulin. Cerebrospinal fluid analysis was normal. The diagnosis was confirmed using electromyography. Our patient's initial presentation of monoparesis and progression in an asymmetric descending manner was unusual for GBS. His initial presentation mimicked a stroke, and the later progression masqueraded as cervical myelopathy secondary to a chronic cervical cord lesion. The presence of a cervical cord lesion (upper motor neuron) concealed the expected areflexia in GBS. The presence of OO on spine imaging, absence of expected areflexia in GBS, and progression to paraparesis after the fall sidetracked the direction of the initial investigation and led to a relative delay in diagnosis. Nonetheless, appraising the diagnostic data in the clinical context led to an appropriate diagnosis. We emphasize the importance of reconciling the available clinical and diagnostic information to reach the correct diagnosis.

Advances in surgical treatment for atlantoaxial instability focusing on rheumatoid arthritis: Analysis of a series of 67 patients.

AIM: An estimated 88% of rheumatoid arthritis (RA) patients experience various degrees of cervical spine involvement. The excessive movement of the atlantoaxial joint, which connects the occiput to the upper cervical spine, results in atlantoaxial instability (AAI). AAI stabilization is usually achieved by C1 lateral mass-to-C2 pedicle screw-rod fixation (LC1-PC2 fixation), which is technically challenging in RA patients who often show destructive changes in anatomical structures. This study aimed to analyze the clinical results and operative experiences of C1-C2 surgery, with emphasis on the advancement of image-guided surgery and augmented reality (AR) assisted navigation. METHODS: We presented our two decades of experience in the surgical management of AAI from April 2004 to November 2022. RESULTS: We have performed surgery on 67 patients with AAI, including 21 traumatic odontoid fractures, 20 degenerative osteoarthritis, 11 inflammatory diseases of RA, 5 congenital anomalies of the os odontoideum, 2 unknown etiologies, 2 movement disorders, 2 previous implant failures, 2 osteomyelitis, 1 ankylosing spondylitis, and 1 tumor. Beginning in 2007, we performed LC1-PC2 fixation under C-arm fluoroscopy. As part of the progress in spinal surgery, since 2011 we used surgical navigation from presurgical planning to intraoperative navigation, using the preoperative computed tomography (CT) -based image-guided BrainLab navigation system. In 2021, we began using intraoperative CT scan and microscope-based AR navigation. CONCLUSION: The technical complexities of C1-C2 surgery can be mitigated by CT-based image-guided surgery and microscope-based AR navigation, to improve accuracy in screw placement and overall clinical outcomes, particularly in RA patients with AAI.

Transtubular Transoral Approach for Irreducible Ventral Craniovertebral Junction Compressive Pathologies: Surgical Technique and Outcome.

Introduction: To investigate the use of a tubular retractor to provide access to the craniovertebral junction (CVJ) sparing the soft palate with the aim of reducing complications associated with traditional transoral approach but yet allowing adequate decompression of the CVJ. Materials and methods: Twelve consecutive patients with severe myelopathy (JOA-score less than 11) from ventral CVJ compression were operated between 2014-2020 using a tubular retractor assisted transoral decompression. Results: All patients improved neurologically statistically (p=0.02). There were no posterior pharynx wound infections or rhinolalia. There was one case with incomplete removal of the lateral wall of odontoid and one incidental durotomy. Conclusions: A Tubular retractor provides adequate access for decompression of the ventral compression of CVJ. As the tubular retractor pushed away the uvula, soft palate and pillars of the tonsils as it docked on the posterior pharyngeal wall, the traditional complications associated with traditional transoral procedures is completely avoided.

Extraoral anterior approach for the treatment of os odontoideum: illustrative case.

BACKGROUND: Os odontoideum is typically treated with instrumented fusion through a posterior cervical approach. When this approach fails, limited options for revision are available. Occipitocervical fusion and transoral anterior fusions have been utilized in the past but are associated with high morbidity and complications. OBSERVATIONS: Here the authors report a case of os odontoideum that was treated with an anterior cervical extraoral approach after failed posterior instrumented fusion. They discuss the challenges that can be encountered with the failure of fusion and the limited options when it comes to approach and fixation of os odontoideum. LESSONS: To the authors' knowledge and based on a review of the literature, this case represents the first use of an anterior extraoral prevascular approach to the high cervical spine to address os odontoideum. They demonstrate that this approach can be utilized as a reasonable alternative to transoral surgery and should be considered in cases where additional or alternative fixation is desired without the morbidity and complications associated with occipitocervical fusion or a transoral approach, especially in a younger patient population.

The Relationship Between Cervical Sagittal Balance and Type of Atlantoaxial Dislocation Secondary to Os Odontoideum.

OBJECTIVE: To analyze the effect of cervical sagittal balance on the direction and type of atlantoaxial dislocation. METHODS: Data of 55 patients seen at our hospital for atlantoaxial instability/dislocation caused by os odontoideum were reviewed. Radiographic variables, including T1 slope (T1S), C1-C2 angle, C2-C7 angle, C1-C2 sagittal vertical axis (SVA), C2-C7 SVA, and atlanto-dens interval (ADI), were measured preoperatively. Patients were divided into three groups according to ADI: anterior atlantoaxial dislocation, atlantoaxial instability, and posterior atlantoaxial dislocation. Differences within and between groups in radiographic variables and relationships between the investigated variables were analyzed. RESULTS: ADI was strongly negatively associated with C1-C2 angle (r = -0.805, P < 0.05); whereas ADI had a medium-strength positive relationship with C2-C7 angle (r = 0.425, P < 0.05) and a medium-strength negative relationship with C2-C7 SVA (r = -0.411, P < 0.05). However, ADI was not significantly correlated with T1 slope (r = -0.092, P > 0.05). CONCLUSIONS: The type and direction of atlantoaxial dislocation is closely associated with cervical sagittal balance. C2-C7 SVA is an important factor in assessing the direction of atlantoaxial subluxation secondary to os odontoideum. ADI decreases with increasing C2-C7 SVA. The larger the C2-C7 SVA, the more likely the atlantoaxial dislocation is to be posterior.

Axial superior facet slope may determine anterior or posterior atlantoaxial displacement secondary to os odontoideum and compensatory mechanisms of the atlantooccipital joint and subaxial cervical spine.

OBJECTIVE: To introduce novel parameters in determining directions of os odontoideum (OO) with atlantoaxial displacement (AAD) and compensations of cervical sagittal alignment after displacement. METHODS: Analysis was performed on 96 cases receiving surgeries for upper cervical myelopathy caused by OO with AAD from 2011 to 2021. Twenty-four patients were included in the OO group and divided into the OO-anterior displacement (AD) group and the OO-posterior displacement (PD) group by displacement. Seventy-two patients were included as the control (Ctrl) group and divided into Ctrl-positive (Ctrl-P) group and Ctrl-negative (Ctrl-N) group by axial superior facet slope (ASFS) in a neutral position. ASFS, the sum of C2 slope (C2S) and axial superior facet endplate angle (ASFEA), was measured and calculated by combining cervical supine CT with standing X-ray. Cervical sagittal parameters were measured to analyse the atlantoaxial facet and compensations after AAD. RESULTS: Atlas inferior facet angle (AIFA), ASFS, and ASFEA in Ctrl-P significantly differed from OO-AD.C0-C1, C1-C2, C0-C2, C2-C7, C2-C7 SVA, and C2S in Ctrl-P significant differed from the OO-AD group. C2-C7 SVA and C2S in Ctrl-N significantly were smaller than the OO-PD group. C1-C2 correlated with C0-C1 and C2-C7 negatively in the OO group. Slight kyphosis of C1-C2 in OO-AD was compared with lordosis of C1-C2 in Ctrl-P, inducing increased extension of C0-C1 and C2-C7. Mildly increased lordosis of C1-C2 in OO-PD was compared with C1-C2 in Ctrl-N, triggering augmented flexion of C0-C1 and C2-C7. CONCLUSION: ASFS was vital in determining directions of OO with AAD and explaining compensations. ASFS and ASFEA could provide pre- and intraoperative guidelines. KEY POINTS: • ASFS may determine the directions and compensatory mechanisms of AAD secondary to OO. • ASFS could be achieved by the sum of ASFEA and C2S.

MR documented craniocervical ligamentous injury at age 18 months: delayed formation of OS odontoideum. Complex management issues. Case-based review.

OBJECTIVE: There are two separate theories regarding the genesis of os odontoideum: congenital and post-traumatic. Trauma documentation in the past has been the presence of a normal odontoid process at the time of initial childhood injury and subsequent development of the os odontoideum. True MR documentation of craniocervical injury in early childhood and subsequent os odontoideum formation has been very rare. METHODS: An 18-month-old sustained craniocervical ligamentous injury documented on MRI with transient neurological deficit. Chiari I abnormality was also recorded. Subsequent serial imaging of craniocervical region showed the formation of os odontoideum and instability. He became symptomatic from the os odontoideum and the Chiari I abnormality. The patient underwent decompression and intradural procedure for Chiari I abnormality and occipitocervical fusion. Postoperative course was complicated by the failure of fusion and redo. He later required transoral ventral medullary decompression. He recovered. RESULTS: This is an MR documented craniocervical ligamentous injury with sequential formation of os odontoideum with accompanying changes in the atlas. Despite a subsequent successful dorsal occipitocervical fusion, he became symptomatic requiring transoral decompression. CONCLUSIONS: Os odontoideum here is recognized as a traumatic origin with the presence of congenital Chiari I abnormality as a separate entity. The changes of the anterior arch of C1 as well as the os formation were serially documented and give credence to blood supply changes in the os and atlas as a result of the trauma. The recognized treatment of dorsal occipitocervical fusion failed in this case requiring also a ventral decompression of the medulla.

Congenital atlanto-occipital dislocation in a patient with Down syndrome: a case report.

Down syndrome, also known as trisomy 21, is associated with congenital cervical spine abnormalities, including atlantoaxial instability with or without os odontoideum, atlanto-occipital instability, and hypoplasia of the atlas. Herein, we report a case of Down syndrome complicated by congenital atlanto-occipital dislocation. The patient presented with severe cervical myelopathy at 13 years of age after a 10-year follow-up. Radiography and computed tomography revealed os odontoideum protruding into the foramen magnum and congenital anterior atlanto-occipital dislocation. Additionally, a bifurcated internal occipital crest with a thinned central portion of the occipital bone was noted. Magnetic resonance imaging revealed kyphotic alignment of the spinal cord with severe compression at the foramen magnum level. As the neurological impairment was partially improved by halo vest immobilization, we performed in situ O-C2 fusion with an iliac autograft and decompression of the foramen magnum and posterior arch of C1. An improvement was observed immediately after surgery. Two years after surgery, radiography and computed tomography showed solid O-C2 segment fusion. The accumulation of similar cases is essential for determining the prognosis or optimal treatment for this rare congenital condition.

Analysis of surgical strategies and efficacy in the treatment of Os odontoideum with atlantoaxial dislocation.

BACKGROUND: There are many classification systems for atlantoaxial dislocation (AAD). Among these systems, the definitions of irreducible AAD remain vague, and its treatments are not unified. OBJECTIVE: To explore the surgical strategies and efficacy for the treatment of os odontoideum (OO) with AAD. METHODS: The clinical data of 56 OO patients with AAD who underwent surgery from January 2017 to June 2021 were retrospectively analyzed. AAD was classified into four types, Type I and type II were treated with posterior fixation and fusion. Type III received posterior fixation and fusion after irreducible dislocations were converted to reducible dislocations by translateral mass release or transoral release. Type IV required transoral release for conversion into reducible dislocations before posterior fixation and fusion. The operation time, blood loss, and complications were recorded. The preoperative and postoperative neurological function changes were assessed using the Japanese Orthopedic Association (JOA) score. Postoperative fusion status was assessed by X-ray. RESULTS: There were 40 cases of type I-II, 14 cases of type III, and two cases of type IV AAD. The operation times of single posterior fixation and fusion, combined translateral mass release and combined transoral release were 130.52 ± 37.12 min, 151.11 ± 16.91 min and 188.57 ± 44.13 min, the blood loss were 162.63 ± 58.27 mL, 235.56 ± 59.94 mL, 414.29 ± 33.91 mL, respectively. One patient with type III died, one with type III underwent revision surgery due to infection, and three patients with type I had further neurological deterioration after operation. fifty-five patients were followed up for 12-24 months. The follow-up results showed that enough decompression was achieved and that fixation and fusion were effective. The JOA score increased from 9.58 ± 1.84 points preoperative to 13.09 ± 2.68 points at 3 months after operation, 14.07 ± 2.83 points at 6 months and 14.25 ± 2.34 at 12 months after operation, all significant differences compared with preoperative results (P < 0.05). CONCLUSION: OO patients with irreducible AAD can be treated by translateral mass release or transoral release combined with posterior fixation and fusion, while some of those with bony fusion can be treated by transoral release combined with posterior fixation and fusion.

Atlantoaxial Instability with Persistent Second Intersegmental Artery.

Understanding the anatomy of the vertebral artery is essential while manipulating the craniovertebral joint during surgery. Its anomalous course in congenital atlantoaxial dislocation makes it more vulnerable to injury. Preoperative dedicated computed tomography (CT) angiography helps identify the artery's position and plan for surgical procedure. A 13-year-boy presented with neck pain and spastic quadriparesis for 1 year. Radiological imaging of the craniovertebral junction revealed atlantoaxial instability with basilar invagination. His CT angiography of neck and brain vessels revealed an anomalous course of the vertebral artery due to a persistent second intersegment artery. He underwent posterior atlantoaxial fixation after mobilization of the vertebral artery. His clinical condition significantly improved after surgery. We report a case of management of an atlanto axial dislocation with persistent second intersegment artery and describe the role of vertebral artery mobilization during surgery.

Os odontoideum: A comprehensive review.

Os odontoideum (OO) is a rare craniocervical anomaly that is characterized by a round ossicle separated from the C2 vertebral body. With a controversial etiology and unknown prevalence in the population, OO may be asymptomatic or present in patients with myelopathic and neurological symptoms. In this literature review, we aimed to investigate epidemiology, embryology, pathophysiology, clinical presentation, and the role of diagnostic radiography in OO. 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. A literature review was conducted using the PubMed, EMBASE, and Scopus databases. 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. The presentation of OO varies widely from asymptomatic cases to severe neurological deficits. Asymptomatic patients can be managed either conservatively or surgically, while symptomatic patients should undergo operative stabilization. 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. Conflicting studies and the paucity of current literature on OO highlight poor comprehension of the condition. Further understanding of the natural history of OO is critical to form evidence-based guidelines for the management of OO patients. More large-center studies are thus needed to promote accurate management of OO patients with optimal outcomes.