Posterior occipitocervical instrumented fusion for atlantoaxial instability in a 27-month-old child with Down syndrome: illustrative case

BACKGROUND: Upper cervical spine instability is one of the most serious orthopedic problems in patients with Down syndrome. Despite the recent advancement of instrumentation techniques, occipitocervical fusion remains technically challenging in the very young pediatric population with small and fragile osseous elements. OBSERVATIONS: A 27-month-old boy with Down syndrome was urgently transported to the authors’ hospital because of difficulty in standing and sitting, weakness in the upper limbs, and respiratory distress. Radiographs showed os odontoideum, irreducible atlantoaxial dislocation, and substantial spinal cord compression. Emergency posterior occipitoaxial fixation was performed using O-arm navigation. Improvement in the motor paralysis of the upper left limb was observed from the early postoperative period, but revision surgery was needed 14 days after surgery because of surgical site infection. The patient showed modest but substantial neurological improvement 1 year after the surgery. LESSONS: There are several clinical implications of the present case. It warns that Down syndrome in the very young pediatric population may lead to rapid progression of spinal cord injury and life crisis. This 27-month-old patient represents the youngest case of atlantoaxial instability in a patient with Down syndrome. O-arm navigation is useful for inserting screws into very thin pedicles.