Grisel’s syndrome: a comprehensive review with focus on pathogenesis, natural history, and current treatment options

Abstract

Introduction Grisel's syndrome (GS) is a rare otolaryngologic condition that involves rotatory subluxation of the atlantoaxial 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. Methods Using standard search engines, we reviewed the most recent literature regarding GS with special attention to its pathogenesis, natural history, and recent treatment options. Results 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. Conclusion GS remains a rare, but potentially lethal children condition. Early diagnosis and treatment is crucial to its management and prognosis.

Microtia Reconstruction and Postsurgical Grisel’s Syndrome

“…3 This syndrome was first described by Sir Charles Bell in 1830 and subsequently reported by Pierre Grisel in 1930. 4 A review by Karkos et al 5 revealed that the most common cause of Grisel’s syndrome is infection of the head and neck, which is associated with 48% of cases in their study, followed by surgery of the head and neck, associated with 31% of the cases. 5 Among the various types of infections, upper respiratory tract infections accounted for 83% of cases, followed by retropharyngeal abscess and otitis media, at 11% and 4%, respectively.…”

Grisel Syndrome Following Adenoidectomy: Surgical Management in a Case with Delayed Diagnosis

“…There are different pathogenetic hypotheses, recently described by Osiro and coworkers in a comprehensive review [8].…”

“…The primary treatment of early detected Grisel's syndrome is conservative [4,5,8] and includes bed rest, external fixation, antibiotic therapy, muscle relaxants, and anti-inflammatory therapy. Time to reduction of atlanto-axial rotatory subluxation, is directly related to failure of medical treatment and to an increased risk of recurrence or permanent neck deformity.…”

“…Early management, consisting of closed reduction and cervical immobilization and medical treatment, is the key factor for a satisfactory outcome [8]. We report the case of a 9-year-old boy who developed Grisel's syndrome after adenoidectomy.…”

Conservative Treatment in Grisel’s Syndrome Following Tonsillectomy

“…Described by Sir Charles Bell in 1830, and named after usually affects the pediatric population because of the anatomic differences in children when compared to adults [6]. There are different pathogenesis hypotheses, recently described by Osiro et al [7]. The most widely accepted theory for Grisel's syndrome is the two-hit hypothesis.…”