Various respiratory, musculoskeletal, gastrointestinal, neurological, and urinary complications have been reported in Kawasaki disease. Here, we describe a rare case of atlantoaxial rotatory fixation (AARF) associated with incomplete Kawasaki disease. The case is of a healthy 4-year-old Japanese boy who had a high-grade fever, lymphadenopathy, and torticollis diagnosed with incomplete Kawasaki disease. Intravenous high-dose immunoglobulin and oral aspirin quickly resolved his fever and improved his lymphadenopathy, but torticollis remained. On orthopaedic examination, torticollis was observed with a marked restriction of rotation, and an open-mouth anteroposterior cervical radiograph and a CT scan confirmed rotational dislocation at the dens axis (AARF). Cervical collar fixation was immediately started, and the torticollis gradually normalized within a week. AARF is defined as torticollis due to dislocation or subluxation of the atlantoaxial joint. The diagnosis of AARF is difficult with routine plain cervical radiographs in 2 directions alone, and an additional cervical open-mouth anteroposterior radiograph and a CT scan aid the diagnosis. AARF associated with Kawasaki disease is uncommon, and only 24 cases have been reported in the literature. AARF may occur in Kawasaki disease patients with cervical lymphadenopathy. Still, torticollis is often transient and may not be recognized or ignored by family doctors and paediatricians. Reduction of the atlantoaxial joint can often be achieved spontaneously or with conservative treatment such as a collar or neck traction, but treatment is difficult if the diagnosis is delayed. Therefore, family doctors and paediatricians need to suspect the onset of AARF if torticollis is observed during treatment for Kawasaki disease, perform plain cervical radiographs including open-mouth anteroposterior view and a CT scan of the cervical spine, and have orthopaedists immediately intervene to avoid invasive surgery.
Keywords: Grisel’s syndrome; Kawasaki disease; atlantoaxial subluxation; cervical lymphadenopathy; torticollis.
© The Author(s) 2024. Published by Oxford University Press on behalf of the British Institute of Radiology.