Ankylosing spondylitis of the craniovertebral junction: a single surgeon's experience

The objective of this study was to describe a single surgeon's experience managing craniovertebral junction (CVJ) disease due to ankylosing spondylitis.

The authors undertook a retrospective review of the records of patients with CVJ disease due to ankylosing spondylitis who were evaluated and treated by the senior author. Charts were reviewed for symptoms and signs at presentation, radiography results, treatment, and outcome. In addition, some of the patients had pathology reports available for review.

Eight patients with CVJ disease due to ankylosing spondylitis were identified who were evaluated by the senior author in the years 1990-2008. The most common presenting symptoms were neck pain (37.5%), cranial neuropathy (37.5%), and sensory disturbance (62.5%). On examination, the most common findings were limited cervical range of motion (37.5%), weakness (50%), and myelopathy (75%). Radiographic evaluation revealed atlantoaxial subluxation, retroodontoid pannus formation, basilar invagination, and bone erosion. Surgery was offered to all of the patients, 7 of whom underwent operations. In most cases, the treatment was transoral-transpalatopharyngeal decompression followed by occipitocervical fusion. One patient with a reducible lesion underwent dorsal fusion alone. Neurological outcomes were favorable overall.

The incidence of CVJ disease in patients with ankylosing spondylitis varies among reports. These cases are rare in most neurosurgery clinics. It is important to recognize that patients with ankylosing spondylitis are at risk for CVJ disease, similar to patients with rheumatoid arthritis. Principles common to the management of other CVJ pathologies apply to these patients as well.