Purpose <p>Os Odontoideum (OO) is an anatomical anomaly of the craniocervical junction, which may lead to severe instability, resulting in potentially fatal spinal cord compression. Considering clinical improtance of OO, the aim of this study was to provide a thorough synthesis of data on its epidemiology, specifically regarding its prevalence with neurosurgical considerations.</p> Methods <p>The major electronic databases (Pubmed/Medline, Embase, Scopus, ScienceDirect) were thoroughly searched up to July 18th, 2025. Studies reporting original data on the prevalence of OO were deemed eligible for inclusion. The extracted data subsequently underwent qualitative assessment. A random effects model based on the DerSimonian-Laird approach was employed. The AQUA tool was used to evaluate the quality and risk of bias of the included studies.</p> Results <p>Of the initial 1,638 records, 19 studies (<i>n</i> = 18,521 patients) met the inclusion criteria. Most of the studies revealed “low” risk of bias. The lowest prevalence of OO was found in the general population (0.21%, 95%CI: 0.00-0.72, <i>I</i><sup><i>2</i></sup> = 50%). OO was more commonly found in subjects with craniofacial malformations (1.9%, 95%CI: 0.90–3.24, <i>I</i><sup><i>2</i></sup> = 0%) and among patients undergoing craniocervical junction fixation (10.84%, 95%CI: 5.19–18.11, <i>I</i><sup><i>2</i></sup> = 83%; random-effects meta-regression with Knapp-Hartung adjustment, <i>p</i> = 0.0006). The highest prevalence of OO was observed in a subgroup of patients with both 21 trisomy and craniocervical junction symptoms (30.86%, 95%CI: 14.82–49.49, <i>I</i><sup><i>2</i></sup> = 42%).</p> Conclusions <p>OO is rare among the general population. However, its prevalence is significantly higher in individuals with craniofacial malformations or 21 trisomy. Knowledge of the epidemiology of OO may facilitate earlier and more accurate diagnosis, particularly in high-risk patient populations. However, substantial heterogeneity warrants cautious interpretation. In pursuance of establishing the odds ratio, a greater number of case-control studies should be conducted.</p>

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Prevalence of os odontoideum: a systematic review, meta-analysis and meta-regression with implications for vertebral artery and osseous anomalies

  • Tomasz Klepinowski,
  • Aleksander Osiowski,
  • Weronika Grzyb,
  • Giuseppe Esposito,
  • Maksymilian Osiowski,
  • Marta Klepinowska,
  • Dominik Taterra,
  • Leszek Sagan

摘要

Purpose

Os Odontoideum (OO) is an anatomical anomaly of the craniocervical junction, which may lead to severe instability, resulting in potentially fatal spinal cord compression. Considering clinical improtance of OO, the aim of this study was to provide a thorough synthesis of data on its epidemiology, specifically regarding its prevalence with neurosurgical considerations.

Methods

The major electronic databases (Pubmed/Medline, Embase, Scopus, ScienceDirect) were thoroughly searched up to July 18th, 2025. Studies reporting original data on the prevalence of OO were deemed eligible for inclusion. The extracted data subsequently underwent qualitative assessment. A random effects model based on the DerSimonian-Laird approach was employed. The AQUA tool was used to evaluate the quality and risk of bias of the included studies.

Results

Of the initial 1,638 records, 19 studies (n = 18,521 patients) met the inclusion criteria. Most of the studies revealed “low” risk of bias. The lowest prevalence of OO was found in the general population (0.21%, 95%CI: 0.00-0.72, I2 = 50%). OO was more commonly found in subjects with craniofacial malformations (1.9%, 95%CI: 0.90–3.24, I2 = 0%) and among patients undergoing craniocervical junction fixation (10.84%, 95%CI: 5.19–18.11, I2 = 83%; random-effects meta-regression with Knapp-Hartung adjustment, p = 0.0006). The highest prevalence of OO was observed in a subgroup of patients with both 21 trisomy and craniocervical junction symptoms (30.86%, 95%CI: 14.82–49.49, I2 = 42%).

Conclusions

OO is rare among the general population. However, its prevalence is significantly higher in individuals with craniofacial malformations or 21 trisomy. Knowledge of the epidemiology of OO may facilitate earlier and more accurate diagnosis, particularly in high-risk patient populations. However, substantial heterogeneity warrants cautious interpretation. In pursuance of establishing the odds ratio, a greater number of case-control studies should be conducted.