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At a glance

Grisel Syndrome is a nontraumatic subluxation of the atlantoaxial joint secondary to inflammatory processes (pharyngitis and pharyngeal abscess) and head and neck surgery. Progressive throat, neck pain and stiffness causing pain or radiculopathies are reported. This is a rare disease that usually affects children. The instability of the cervical spine may lead to quadriplegia especially during laryngoscopy and tracheal intubation. Special attention must also be taken to prevent massive bacterial pulmonary contamination due to rupture of a pharyngeal abscess. Other features include upper respiratory tract infections, peritonsillar or retropharyngeal abscesses. This disease can be lethal following acute respiratory failure.

Synonym

Nontraumatic Atlanto-Axial Subluxation.

History

Although first described in 1830 by the Scottish physician Charles Bell, it is named after the French otorhinolaryngologist P. Grisel who described the features in 1930.

Incidence

Rare. Literature suggests approximately 1:100,000,000 per year. Males and females are affected equally. Usually affects children, but may be diagnosed late into adulthood.

Genetic inheritance

Not inherited disorder.

Pathophysiology

Relaxation of the transverse ligament of the atlanto-axial joint. Infection in the head and neck area spreads toward the upper cervical vertebrae. The inflammation causes laxity of the atlantoaxial ligament complex, leading to anterior subluxation of the atlas on the axis. This probably follows rupture of the transverse ligament and may result in spinal cord compression. The cause of infection may be (1) postsurgery: mastoidectomy, tonsillectomy, adenoidectomy, removal of tumors, or choanal atresia repair, (2) contiguous infection: rhinopharyngitis, tonsillitis, abscess (retropharyngeal to alveolar), or ear infections, or (3) other more rare associations, such as acute rheumatic fever or inflammatory bowel disease.

Diagnosis

Usually clinical and it may be missed for months until significant symptoms occur. Radiographs are of minimal value in the first 4 weeks, although flexion-extension views may be suggestive. A CT- and MRI scans usually confirm the presence of rotational dislocation or anterior subluxation. Flexible nasopharyngoscopy is useful.

Clinical aspects

Usually presents with progressive unrelenting throat and neck pain followed by torticollis and subluxation. There is often little systemic reaction. The torticollis is usually acute and often occurs with sleep or minimal motion. Neurologic complications occur in approximately 15% of cases and range from radiculopathy to myelopathy, transient or permanent paraplegia, and even death. This can occur if the atlas becomes dislocated and can occur following only minimal trauma. Treatment includes antibiotics, surgical drainage of pus collections, bony stabilization, and neurologic protection. In the acute phase of the disease, spinal protection often involves spinal traction treatment. Chronic disease may require fixation, which is commonly performed by an anterior approach.

Precautions before anesthesia

Once the diagnosis is made, the major concerns are spinal ...

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