Nontraumatic Atlantoaxial Rotatory Subluxation: Grisel Syndrome. Case Report and Literature Review
27 May 2013
22 November 2013
13 January 2014 (eFirst)
Study Design Case report and literature review.
Objective To describe a case of nontraumatic atlantoaxial rotatory subluxation (Grisel syndrome) and to review clinical and radiologic aspects, physiopathology, and treatment of this lesion. There is no well-established protocol in the management of patients without spontaneous reduction. The authors discuss the available strategies to achieve reduction and when to operate on these patients.
Methods Case presentation of a 7-year-old patient who presented with torticollis ∼1 week after the onset of an upper airway infection. There was no history of head or neck trauma. Computed tomography demonstrated atlantoaxial rotatory subluxation and a normal atlantodental interval.
Results The patient was treated with nonsteroidal anti-inflammatory drugs and antibiotics and by progressively increasing the soft cervical collar height. Clinical reduction of the subluxation occurred after 48 hours. He wore the rigid collar for 6 weeks. At that moment, the patient was completely asymptomatic and follow-up cervical spine radiograph demonstrated an anatomical C1–C2 relation. The patient was instructed to return to daily life activities in a gradual manner.
Conclusions Grisel syndrome should be considered in the differential diagnosis of torticollis, especially in children. The management can be planned according to the classification of Fielding and Hawkins. The initial treatment involves medicines, injury reduction, and cervical spine immobilization. Surgical treatment is indicated only in cases of failure of conservative treatment, recurrences of subluxation, and irreducible subluxations.
- 1 Grisel P. Enucleation de l'atlas et torticollis nasopharyngien. Presse Med 1951; 59: 1647-1648
- 2 Guleryuz A, Bagdatoglu C, Duce MN, Talas DU, Celikbas H, Köksel T. Grisel's syndrome. J Clin Neurosci 2002; 9 (1) 81-84
- 3 Bocciolini C, Dall'Olio D, Cunsolo E, Cavazzuti PP, Laudadio P. Grisel's syndrome: a rare complication following adenoidectomy. Acta Otorhinolaryngol Ital 2005; 25 (4) 245-249
- 4 Rinaldo A, Mondin V, Suárez C, Genden EM, Ferlito A. Grisel's syndrome in head and neck practice. Oral Oncol 2005; 41 (10) 966-970
- 5 Fernández Cornejo VJ, Martínez-Lage JF, Piqueras C, Gelabert A, Poza M. Inflammatory atlanto-axial subluxation (Grisel's syndrome) in children: clinical diagnosis and management. Childs Nerv Syst 2003; 19 (5–6) 342-347
- 6 Gourin CG, Kaper B, Abdu WA, Donegan JO. Nontraumatic atlanto-axial subluxation after retropharyngeal cellulitis: Grisel's syndrome. Am J Otolaryngol 2002; 23 (1) 60-65
- 7 Grobman LR, Stricker S. Grisel's syndrome. Ear Nose Throat J 1990; 69 (12) 799-801
- 8 Samuel DTD, Thomas DM, Tierney PA, Patel KS. Atlanto-axial subluxation (Grisel's syndrome) following otolaryngological diseases and procedures. J Laryngol Otol 1995; 109 (10) 1005-1009
- 9 Youssef K, Daniel S. Grisel syndrome in adult patients. Report of two cases and review of the literature. Can J Neurol Sci 2009; 36 (1) 109-113
- 10 Yamazaki M, Someya Y, Aramomi M, Masaki Y, Okawa A, Koda M. Infection-related atlantoaxial subluxation (Grisel syndrome) in an adult with Down syndrome. Spine (Phila Pa 1976) 2008; 33 (5) E156-E160
- 11 Herzka A, Sponseller PD, Pyeritz RE. Atlantoaxial rotatory subluxation in patients with Marfan syndrome. A report of three cases. Spine (Phila Pa 1976) 2000; 25 (4) 524-526
- 12 Nozaki F, Kusunoki T, Tomoda Y , et al. Grisel syndrome as a complication of Kawasaki disease: a case report and review of the literature. Eur J Pediatr 2013; 172 (1) 119-121
- 13 Tschopp K. Monopolar electrocautery in adenoidectomy as a possible risk factor for Grisel's syndrome. Laryngoscope 2002; 112 (8 Pt 1) 1445-1449
- 14 Karkos PD, Benton J, Leong SC, Mushi E, Sivaji N, Assimakopoulos DA. Grisel's syndrome in otolaryngology: a systematic review. Int J Pediatr Otorhinolaryngol 2007; 71 (12) 1823-1827
- 15 Subach BR, McLaughlin MR, Albright AL, Pollack IF. Current management of pediatric atlantoaxial rotatory subluxation. Spine (Phila Pa 1976) 1998; 23 (20) 2174-2179
- 16 Tedesco B, Grisel P, Desfosses P, Tassin M. Deux nouveaux cas d'énucléation de l'atlas par torticolis nasopharyngien. Bull Soc Pediat 1930; 28: 252-262
- 17 Parke WW, Rothman RH, Brown MD. The pharyngovertebral veins: an anatomical rationale for Grisel's syndrome. J Bone Joint Surg Am 1984; 66 (4) 568-574
- 18 Deichmueller CM, Welkoborsky HJ. Grisel's syndrome—a rare complication following “small” operations and infections in the ENT region. Eur Arch Otorhinolaryngol 2010; 267 (9) 1467-1473
- 19 Diethelm L, Heuck F, Olsson O , et al. Handbuch der medizinischen Radiologie [Encyclopedia of Medical Radiology]. Berlin, Germany: Springer Verlag; 1976: 195
- 20 Phillips WA, Hensinger RN. The management of rotatory atlanto-axial subluxation in children. J Bone Joint Surg Am 1989; 71 (5) 664-668
- 21 Martinez-Lage JF, Martinez Perez M, Fernandez Cornejo V, Poza M. Atlanto-axial rotatory subluxation in children: early management. Acta Neurochir (Wien) 2001; 143 (12) 1223-1228
- 22 Yu KK, White DR, Weissler MC, Pillsbury HC. Nontraumatic atlantoaxial subluxation (Grisel syndrome): a rare complication of otolaryngological procedures. Laryngoscope 2003; 113 (6) 1047-1049
- 23 Fielding JW, Hawkins RJ. Atlanto-axial rotatory fixation (fixed rotatory subluxation of the atlanto-axial joint). J Bone Joint Surg Am 1977; 59 (1) 37-44
- 24 Park SW, Cho KH, Shin YS , et al. Successful reduction for a pediatric chronic atlantoaxial rotatory fixation (Grisel syndrome) with long-term halter traction: case report. Spine (Phila Pa 1976) 2005; 30 (15) E444-E449
- 25 Wetzel FT, La Rocca H. Grisel's syndrome. Clin Orthop Relat Res 1989; (240) 141-152
- 26 Menezes AH. Craniocervical fusions in children. J Neurosurg Pediatr 2012; 9 (6) 573-585