Surgical management strategies for atlantoaxial instability/dislocation in down syndrome
Abstract Objective To evaluate surgical strategies and clinical outcomes for atlantoaxial instability/dislocation (AAI/AAD) in Down syndrome (DS) patients. Methods A retrospective review was conducted on 12 DS patients with AAI/AAD treated between March 2018 and June 2024. Surgical plans were tailor...
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| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
BMC
2025-07-01
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| Series: | Journal of Orthopaedic Surgery and Research |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13018-025-06105-3 |
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| Summary: | Abstract Objective To evaluate surgical strategies and clinical outcomes for atlantoaxial instability/dislocation (AAI/AAD) in Down syndrome (DS) patients. Methods A retrospective review was conducted on 12 DS patients with AAI/AAD treated between March 2018 and June 2024. Surgical plans were tailored based on reducibility: reducible cases underwent posterior atlantoaxial/occipitocervical fusion (n = 8), while irreducible cases received transoral anterior release combined with posterior fixation (n = 4). Outcomes were assessed through complications, radiographic parameters (anterior atlantodental interval, ADI), and neurological status (JOA score). Results The cohort (5 males, 7 females) aged 5–28 years (mean 11.3 ± 6.4) completed 1–6 year follow-up. Clinical presentations included myelopathy (75.0%, 9/12) and neck pain (25.0%, 3/12). Radiographic anomalies included os odontoideum (66.7%, 8/12) and odontoid fracture (8.3%, 1/12). Postoperative ADI significantly decreased from 8.95 ± 3.19 mm to 3.40 ± 0.81 mm (P < 0.05), with JOA scores improving from 10.92 ± 4.40 to 15.50 ± 2.43 (P < 0.05). Complications occurred in 25.0% (3/12): one surgical site infection managed by debridement, one dural tear repaired intraoperatively, and one hardware displacement requiring revision. Median fusion time was 6 months (range 4.5–16). All patients demonstrated neurological improvement except one with residual lower limb weakness. Conclusion DS patients with AAI/AAD frequently present with os odontoideum and spinal cord compromise, necessitating surgical intervention. Reducible dislocations may be effectively managed with posterior fusion alone, whereas irreducible cases require combined anterior–posterior approaches. Early surgical intervention is recommended for DS patients exhibiting os odontoideum with AAI/AAD due to elevated risk of progressive myelopathy. |
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| ISSN: | 1749-799X |