Abstract
The atlas (C1) and axis (C2) have distinct morphologies to support the skull and facilitate head rotation and neck flexion. Congenital defects of C1 posterior arch are rare. We present a case of a 59-year-old man with both an absent C1 posterior arch with concomitant os odontoideum. The patient presented with neck crepitus, moderate neck pain and progressive worsening upper limbs paraesthesia and pain. Computed tomography (CT) revealed non-union between the odontoid process and body of axis as well as absence of C1 posterior arch. An occiput C0-C3 fusion was performed. The patient's symptoms improved significantly, and he is functioning well at 12 months. CT showed solid fusion without implant migration. Concomitant os odontoideum with aplastic C1 posterior arch is rare with limited evidence to guide management. Posterior fixation and fusion may be a potential solution to prevent dynamic compression, thereby preventing further myelopathy and related complications.