Inflammatory atlanto-axial subluxation (Grisel's syndrome) in children:: clinical diagnosis and management

Background. Grisel's syndrome is a uni- or bilateral subluxation of the atlas on the axis associated with an inflammatory condition of the head or neck. This uncommon entity usually affects children. The condition seems to be secondary to hyperemia and laxity of the atlanto-axial joints. Patients and methods. We report clinical and neuroimaging findings, treatment modalities, and outcomes in 4 children with atlanto-axial rotatory subluxation (AARS) associated with a previous upper respiratory tract infection treated at our Unit during the last 7 years. Results. All children presented with neck pain, head tilt, and reduced neck motion. In 3 patients, the condition was secondary to otitis media and in the remaining patient to viral pharyngitis. The diagnosis was established by clinical signs and symptoms and confirmed by neuroimaging studies, especially by computerized tomography and 3-D reconstructions. Early detection and treatment resulted in a good outcome in all patients. None of the patients required surgical treatment. Conclusions. Grisel's syndrome must be suspected in children with painful torticollis associated with an upper tract respiratory infection. The best results are obtained with early conservative treatment, which prevents chronic changes leading to persistent neck pain and deformity, namely atlanto-axial rotatory fixation (AARF). We emphasize the fact that the diagnosis of Grisel's syndrome is mainly clinical, while the diagnostic confirmation of AARF can only be attained by special neuroimaging techniques.