AIMS: to assess etiology, treatment and outcome of Grisel's syndrome.
METHODS: A Medline search was performed using the terms Grisel's syndrome, spontaneous atlantoaxial subluxation, head, neck, ear, nose and throat. A systematic review of the literature was performed. Case series of both adult and pediatric cases were included. Only papers focusing on true non-traumatic atlantoaxial subluxation were included.
RESULTS: Seventy-one papers have been published from 1950 to 2006. Forty-eight of these fulfilled our inclusion criteria, totaling 103 patients for review. The main causes of Grisel's syndrome were infection (48%) and post-adenotonsillectomy (31%). Less common causes included other postoperative cases such as pharyngoplasty and ear operations. Neurosurgical consultation was paramount in all cases. In the majority of cases conservative management in the form of bedrest, antibiotics, muscle relaxants, traction and collar was effective; in a few cases only surgery in the form of arthrodesis was deemed necessary. Morbidity was significant in those cases where diagnosis was delayed, with the most devastating consequence a permanent neurological deficit in one case.
CONCLUSIONS: Grisel's syndrome is a rare but dangerous complication that can go unnoticed in its early phase and can be a major cause of morbidity and mortality following infection or head and neck procedures/interventions. Early recognition of any cervical complication following routine otolaryngological operations together with early neurosurgical consultation is mandatory to prevent devastating consequences.