Traumatic childhood atlantooccipital dislocation (AOD) may be overlooked, especially in patients with concomitant closed head injury and mutiple trauma. We diagnosed and treated 4 children with traumatic AOD seen in less than a 2-year period. We found published descriptions of only 15 other survivors of childhood traumatic AOD in the literature. Clinical histories, radio-graphic findings, treatment, outcome, and complications in these 15 children as well as our 4 patients were reviewed. The age distribution of childhood AOD survivors (average age 6.8 years) closely resembles that of pediatric multiple trauma patients. Early diagnosis of traumatic AOD hinges on precise interpretation of the lateral cervical radiograph. Longitudinal AOD was seen most often. Usually these children presented with cranial nerve palsies, major motor deficits, and depressed level of consciousness. Most underwent posterior atlantooccipital fusion. Outcome varied from normal neurological function to prolonged ventilator dependency and delayed demise. AOD must be diagnosed early to avoid attributing potentially reversible neurologic changes to irreversible injuries since closed head injury and high spinal cord dysfunction may be confused clinically and the outcome of a patient with AOD is unpredictable.

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