Background: Tuberculosis of the craniovertebral junction (CVJ) is extremely rare. However, recent evidence suggests that the incidence of this condition may be increasing. The diagnosis is often difficult despite advances in imaging using magnetic resonance imaging. The transoral approach to the anterior CVJ provides excellent access to this region, has low mortality and morbidity, and enables biopsy of lesions and decompression of the neuraxis. Management of associated atlantoaxial instability, with regard to timing and method of stabilization, is controversial.
Methods: We report 24 cases of CVJ tuberculosis. Prominent manifestations of the disease included neck pain and stiffness, swelling of the retropharyngeal soft tissues, quadriparesis, osteolytic erosions, and atlantoaxial subluxation. Seven patients had acute presentation. All patients received antituberculous drug treatment for 18 months. Four patients were managed conservatively, 5 underwent only transoral biopsy, 9 patients underwent transoral decompression and posterior fusion, and 6 patients underwent only posterior fusion.
Results: Histopathologic analysis of biopsy material revealed abscess only in 5 cases, granulation tissue only in 6, abscess with granulation tissue in 4, granulation tissue with chronic osteomyelitis in 5, and chronic inflammation in 2. All patients improved, with mean improvement in Nurick grading of 1.71. Even patients with spinal cord signal intensity changes on magnetic resonance images showed improvement.
Conclusions: Although CVJ tuberculosis is a rare disease, the outcome of treatment is good. Antituberculous drug therapy remains the mainstay of treatment after confirming the diagnosis. The surgical management options include transoral decompression with or without posterior fusion, depending upon the presence and persistence of atlantoaxial instability.