OBJECTIVECraniovertebral junction tuberculosis (CVJ-TB) is rare and occurs in only 0.3 to 1% of patients with tuberculous spondylitis. In the available literature, the treatment options offered for this entity have ranged from a purely conservative approach to radical surgery without well-defined guidelines. In this study, we attempt to establish the most effective strategy for the management of this condition.METHODSTwenty-five patients with CVJ-TB were treated during the past 8 years. Severe neck pain, restricted neck movement, and myelopathy were the predominant symptoms. The patients were graded according to their disability as follows: Grade I (n = 7), only neck pain with no pyramidal tract involvement; Grade II (n = 8), independent with minor disability; Grade III (n = 1), partially dependent on others for assistance with activities of daily living; and Grade IV (n = 9), completely dependent on others for assistance with all activities of daily living. Nine patients in Grade IV also had severe respiratory compromise. In all patients, lateral radiographs of the CVJ in flexion and extension were used to determine the presence of atlantoaxial dislocation (AAD). Bony destruction, paraspinal abscess, and thecal compression were seen on intrathecal contrast computed tomographic scans (n = 9) and magnetic resonance imaging studies (n = 22). Under the cover of antituberculous therapy (ATT) administered for 18 months, the patients were placed under a management protocol that took into account the patient’s preoperative grade, the presence of mobile or fixed AAD, bony destruction and retropharyngeal abscess formation at the CVJ, and the clinicoradiological response to ATT within 3 months. Thus, 14 patients were kept on conservative management, with their neck movements stabilized with an external orthosis; 4 patients underwent a single-stage transoral decompression and posterior fusion procedure; and 7 patients underwent direct posterior fusion.RESULTSIn a follow-up period that ranged from 6 months to 7 years (mean, 2.5 yr), the patients in Grades I and II maintained their neurological status. The single patient in Grade III improved to Grade II. Seven of the nine patients in Grade IV returned to normal, and one improved to Grade II. Neck pain improved in all patients. The only death in the series occurred as a result of aspiration pneumonitis leading to septicemia in a child in Grade IV with poor respiratory status and multilevel tuberculous involvement who had undergone transoral decompression and posterior fusion for fixed AAD.CONCLUSIONThis study discusses the clinicoradiological presentation as well as the management of CVJ-TB, in which ATT is administered for 18 months. In the patients with minor deficits (Grades I and II), conservative neck stabilization is adopted; in the patients with severe deficits (Grades III and IV) due to significant cervicomedullary compression caused by fixed AAD or bone destruction and granulation, anterior decompression and posterior fusion are performed. Patients with persistent reducible AAD undergo direct posterior fusion. A significant improvement is possible even in poor-grade patients with judicious use of the surgical options and ATT.