Atlantoaxial dislocation with congenital “sandwich fusion” in the craniovertebral junction: a retrospective case series of 70 patients

BACKGROUND: In the setting of congenital C1 occipitalization and C2–3 fusion, significant strain is placed on the atlantoaxial joint. Vertebral fusion both above and below the atlantoaxial joint (i.e., a “sandwich”) creates substantial instability. We retrospectively report on a case series of “sandwich fusion” atlantoaxial dislocation (AAD), describing the associated clinical characteristics and detailing surgical treatment. To the best of our knowledge, the present study is the largest investigation to date of this congenital subgroup of AAD. METHODS: Seventy consecutive patients with sandwich fusion AAD, from one senior surgeon, were retrospectively reviewed. The clinical features and the surgical treatment results were assessed using descriptive statistics. No funding sources or potential conflict of interest-associated biases exist. RESULTS: The mean patient age was 42.2 years (range: 5–77 years); 36 patients were male, and 34 were female. Fifty-eight patients (82.9%) had myelopathy, with Japanese Orthopaedic Association (JOA) scores ranging 4–16 (mean: 12.9). Cranial neuropathy was involved in 10 cases (14.3%). The most common presentation age group was 31 to 40 years (24 cases, 34.3%). Radiological findings revealed brainstem and/or cervical-medullar compression (58 cases, 82.9%), syringomyelia (16 cases, 22.9%), Chiari malformation (12 cases, 17.1%), cervical spinal stenosis (10 cases, 14.3%), high scapula deformity (1 case, 1.4%), os odontoideum (1 case, 1.4%), and dysplasia of the atlas (1 case, 1.4%). Computed tomography angiography was performed in 27 cases, and vertebral artery (VA) anomalies were identified in 14 cases (51.9%). All 70 patients underwent surgical treatment, without spinal cord or VA injury. Four patients (5.7%) suffered complications, including 1 wound infection, 1 screw loosening, and 2 cases of bulbar paralysis. In the 58 patients with myelopathy, the mean JOA score increased from 12.9 to 14.5. The average follow-up time was 50.5 months (range: 24–120 months). All 70 cases achieved solid atlantoaxial fusion at the final follow-up. CONCLUSIONS: Sandwich fusion AAD, a unique subgroup of AAD, has distinctive clinical features and associated malformations such as cervical-medullar compression, syringomyelia, and VA anomalies. Surgical treatment of AAD was associated with myelopathy improvement and minimal complication occurrence.