Overview
Closed subluxation of the atlantoaxial joint, often referred to as Grisel's syndrome when non-traumatic and associated with upper cervical infections or otolaryngologic procedures, involves abnormal rotation and displacement between the atlas (C1) and axis (C2) without complete dislocation. 245Diagnosis
Clinical Presentation: Torticollis as the primary symptom, often accompanied by neck pain and restricted neck movement. 24
Imaging: Radiographs and MRI are essential for confirming subluxation and assessing cervical alignment and spinal cord compression. 12
Laboratory Tests: Elevated inflammatory markers may support an infectious etiology. 23Management
Conservative Treatment: Bed rest, cervical collar immobilization, antibiotics (if infection is present), and muscle relaxants are typically first-line. 34
Surgical Intervention: Arthrodesis (fusion) may be necessary in cases of persistent subluxation, neurological deficits, or failure of conservative management. Techniques include C1 transposterior arch lateral mass screws combined with C2 pedicle screw and rod fixation for pediatric cases. 13Special Populations
Pediatrics: Specific fixation techniques like C1 transposterior arch lateral mass screws combined with C2 pedicle screw and rod fixation show promising long-term outcomes with no reported subaxial cervical spine degeneration in a 10-year follow-up. 1
Comorbidities: Early diagnosis and treatment are crucial to prevent severe sequelae, especially in cases with delayed diagnosis or neurological involvement. 34Key Recommendations
Early Diagnosis and Aggressive Conservative Management: Initiate bed rest, cervical immobilization, and appropriate antibiotics if infection is suspected. (Evidence: Moderate 3)
Surgical Intervention for Persistent Cases: Consider arthrodesis when conservative measures fail or neurological deficits are present. Techniques such as C1-C2 fusion should be tailored to pediatric patients to preserve spinal development. (Evidence: Weak 1)
Neurological Monitoring: Regular neurological assessments are essential, especially in delayed presentations, to prevent permanent deficits. (Evidence: Expert opinion 4)References
1 Zheng G, Yuan B, Zhao Y, Guo Q, Li F, Xu Z et al.. C1 Transposterior Arch Lateral Mass Screws Combined With C2 Pedicle Screw and Rod Fixation for Pediatric Atlantoaxial Subluxation: A Minimal 10-Year Follow-up Outcome Analysis. Operative neurosurgery (Hagerstown, Md.) 2024. link
2 Deichmueller CM, Welkoborsky HJ. Grisel's syndrome--a rare complication following "small" operations and infections in the ENT region. European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery 2010. link
3 Karkos PD, Benton J, Leong SC, Mushi E, Sivaji N, Assimakopoulos DA. Grisel's syndrome in otolaryngology: a systematic review. International journal of pediatric otorhinolaryngology 2007. link
4 Galer C, Holbrook E, Treves J, Leopold D. Grisel's syndrome: a case report and review of the literature. International journal of pediatric otorhinolaryngology 2005. link
5 Battiata AP, Pazos G. Grisel's syndrome: the two-hit hypothesis--a case report and literature review. Ear, nose, & throat journal 2004. link