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Long-Term Successful Nonoperative Management of a Displaced Pediatric Odontoid Fracture in Hereditary Sensory and
Lee R Benaroch1, Shawn Thirukumar2, Ali Ahmadi Pirshahid1
1Division of Orthopaedic Surgery, Department of Surgery, Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada.
Insights
Hereditary Sensory and Autonomic Neuropathy Type IV (HSAN IV) patients with upper cervical spine fractures may not tolerate halo immobilization. Nonoperative treatment with casting and bracing is a viable alternative for managing these complex injuries.
Area of Science:
- Orthopedic Surgery
- Neurology
- Genetics
Background:
- Hereditary Sensory and Autonomic Neuropathy Type IV (HSAN IV) is a rare genetic disorder characterized by congenital insensitivity to pain.
- Patients with HSAN IV are prone to repetitive self-inflicted trauma, including head and neck injuries.
- Upper cervical spine injuries, such as odontoid fractures and atlantoaxial subluxation, can occur in HSAN IV patients.
Purpose of the Study:
- To report a case of an upper cervical spine injury in a child with HSAN IV.
- To evaluate the effectiveness of nonoperative management for such injuries.
- To highlight the challenges of halo immobilization in patients with pain insensitivity.
Main Methods:
- A 6-year-old boy with HSAN IV presented with a displaced odontoid synchondrosis fracture and C1-C2 subluxation after head trauma.
- Halo immobilization was attempted but failed due to pin-site complications and behavioral issues.
- The patient was successfully managed with staged nonoperative treatment, including Minerva casting and cervicothoracic lumbar orthosis bracing.
Main Results:
- The patient's upper cervical spine injury was successfully treated without surgical intervention.
- Minerva casting and bracing provided stable immobilization and allowed for fracture healing.
- This case demonstrates the limitations of halo immobilization in patients with HSAN IV due to pain insensitivity and behavioral factors.
Conclusions:
- Nonoperative management with casting and bracing can be effective for upper cervical spine injuries in HSAN IV patients.
- Halo immobilization may be contraindicated or challenging in this population.
- This case provides the longest documented follow-up for an upper cervical spine injury in HSAN IV, emphasizing the need for tailored treatment approaches.
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.
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