Neurophysiologic monitoring during cervical traction in a pediatric patient with severe cognitive disability and atlantoaxial instability

BACKGROUND: Surgical management of atlantoaxial instability (AAI) in pediatric patients with Down syndrome is associated with high neurological morbidity. Moreover, Down syndrome cognitive impairment coupled to AAI removes traditional verbal communication to relay evolving symptoms and aid in neurologic examination. It is not clear whether surgical adjuncts can alter clinical outcomes in this vulnerable population. CASE DESCRIPTION: Herein, we report the case of a 6-year-old patient with significant developmental delay and severe AAI that was successfully managed by stabilization with guidance of neurophysiologic investigations in the perioperative phase. CONCLUSION: Perioperative neurophysiologic monitoring is safe, useful, and reliable in pediatric patients with trisomy 21 undergoing cervical traction and occipitocervical instrumented fusion for AAI.