Vol. 7, Issue 1, Part A (2025)

Odontoid anomalies in the pediatric population, particularly os odontoideum, represent a rare but clinically significant cause of atlantoaxial instability. These conditions pose diagnostic challenges due to the anatomical and developmental complexity of the pediatric cervical spine, often mimicking trauma-related pathology. This narrative review synthesizes current evidence from 15 key studies, focusing on diagnostic strategies, surgical indications, and controversies in the management of both symptomatic and asymptomatic patients. Advanced imaging, including dynamic radiographs and MRI, is essential for assessing instability and neural compromise, yet interpretation is hindered by age-related variability. While posterior C1-C2 fixation offers high fusion rates and neurological recovery in children with demonstrable instability, the timing and necessity of surgery in asymptomatic cases remain debated. The presence of syndromic conditions such as Down or Morquio syndrome further complicates decision-making, given their higher risk of instability and surgical morbidity. Although surgical techniques are generally effective, data on long-term outcomes, growth implications, and functional recovery are limited. This review highlights the urgent need for standardized diagnostic criteria, multicenter outcome data, and evidence-based protocols to guide individualized treatment in this vulnerable population.