Case: A 6-year-old boy with Hereditary Sensory and Autonomic Neuropathy Type IV (HSAN IV) presented after repetitive self-inflicted head trauma with new-onset headache and neck discomfort. He had a history of self-injurious behavior and was neurologically intact on exam. Imaging revealed a displaced odontoid synchondrosis fracture with C1 to C2 subluxation. Halo immobilization failed due to pin-site complications and behavior. He was successfully managed with staged nonoperative treatment including Minerva casting and cervicothoracic lumbar orthosis bracing. Conclusion: This case highlights the limitations of halo immobilization in patients with pain insensitivity and reports the longest documented follow-up of upper cervical spine injury in HSAN IV.
Benaroch et al. (Thu,) studied this question.