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Symptomatic atlantoaxial instability in Down syndrome.
Ali Nader-Sepahi1, Adrian T H Casey, Richard Hayward
1Department of Neurosurgery, Great Ormond Street Hospital for Sick Children, London, United Kingdom.
Journal of Neurosurgery
|October 22, 2005
Summary
This study audited surgical treatment for symptomatic atlantoaxial instability in Down syndrome patients. Younger age and multiple bone issues increase fusion failure risk, suggesting an aggressive surgical approach is recommended.
Area of Science:
- Orthopedics
- Neurosurgery
- Genetics
Background:
- Atlantoaxial instability (AAI) is a significant concern in Down syndrome.
- Symptomatic AAI requires surgical intervention for spinal cord protection.
Purpose of the Study:
- To audit the surgical treatment outcomes for symptomatic AAI in Down syndrome.
- To identify factors contributing to fusion failure after surgical correction.
Main Methods:
- Retrospective review of 12 pediatric Down syndrome patients with symptomatic AAI.
- Analysis of clinical histories, radiological data, and surgical interventions.
- Assessment of fusion success and factors influencing outcomes.
Main Results:
- Os odontoideum was present in 10 patients; 4 required transoral odontoidectomy.
- Successful fusion achieved in 7/12 patients on first surgery.
- Acute spinal cord injury patients showed functional recovery with appropriate management.
Conclusions:
- Congenital craniocervical junction (CVJ) abnormalities are prevalent in Down syndrome.
- Younger age at fusion and multiple osseous anomalies are risk factors for fusion failure.
- Aggressive surgical management is advised for symptomatic CVJ instability in this population.