Grisel’s syndrome: a comprehensive review with focus on pathogenesis, natural history, and current treatment options
Grisel’s syndrome (GS) is a rare otolaryngologic condition that involves rotatory subluxation of the atlanto-axial joint in the absence of trauma. Sir Charles Bell was the first to describe this condition in 1830. However, it was named after Pierre Grisel, a French ENT specialist who in 1930 described three such cases in children.
Using standard search engines, we reviewed the most recent literature regarding GS with special attention to its pathogenesis, natural history, and recent treatment options.
Although defined over 80 years ago, the exact pathogenesis leading to GS is still not completely understood. Nevertheless, it occurs mainly as a complication of upper respiratory tract infections and/or surgical interventions in the head and neck. Most recent studies postulate that the spread of septic emboli from such infection nidus via the pharyngovertebral veins to the periodontoid vascular plexus could be the most likely cause. The diagnosis of GS is made by both clinical assessment as well as radiologic confirmation with either CT scan or MRI. Our review found out that most authors favor immobilization with a soft collar and antibiotics over surgical correction in the initial management of GS. Surgery is reserved for severe and refractory cases.
GS remains a rare, but potentially lethal children condition. Early diagnosis and treatment is crucial to its management and prognosis.
KeywordsGrisel’s syndrome Atlantoaxial subluxation Infectious process Antibiotic therapy
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