Grisel’s Syndrome: Non-traumatic Atlantoaxial Rotatory Subluxation—Report of Five Cases and Review of the Literature

  • Corrado IaccarinoEmail author
  • Ormitti Francesca
  • Spennato Piero
  • Rubini Monica
  • Rapanà Armando
  • Pasquale de Bonis
  • Aliberti Ferdinando
  • Giorgio Trapella
  • Lorenzo Mongardi
  • Michele Cavallo
  • Cinalli Giuseppe
  • Servadei Franco
Part of the Acta Neurochirurgica Supplement book series (NEUROCHIRURGICA, volume 125)


Background: In children, when unresponsive neck rigidity and distress are observed after ear, nose and throat (ENT) surgical treatment or nasopharyngeal inflammation, Grisel’s syndrome should be suspected. This is a rare syndrome involving non-traumatic rotatory subluxation of the atlantoaxial joint. Conservative management with external cervical orthoses and empirical antibiotic, muscle relaxant and analgesic therapy should be the first choice of treatment. Surgical stabilization is indicated when high-grade instability or failure of stable reduction are observed. The instability is graded according to the classification system devised by Fielding and Hawkins. Several recommendations for treatment are available in the literature, but there are no common guidelines. In this paper, the authors discuss the need for prompt diagnosis and treatment considerations.

Case Description: Five children with Fielding type I–III rotatory subluxation are reported. Three patients were treated with a cervical collar, and one patient was treated with skull traction and sternal–occipital–mandibular immobilizer (SOMI) brace application. Surgical treatment was necessary for one patient after failure of initial conservative management. The intervals between the onset of torticollis and radiological diagnosis ranged from 12 to 90 days. A relationship between an increased grade of instability and delayed diagnosis was observed.

Conclusion: In children with painful torticollis following ENT procedures or nasopharyngeal inflammation, Grisel’s syndrome should always be suspected. Cervical magnetic resonance imaging (MRI) allows prompt and safe diagnosis, and a three-dimensional computed tomography (CT) scan provides better classification of the instability. Surgery, which is indicated in cases of high-grade instability or failure of conservative treatment, may be avoided with prompt diagnosis.


Grisel’s syndrome Fielding classification Atlantoaxial instability Atlantoaxial rotatory subluxation 





Atlantodental interval


Computed tomography


Ear, nose and throat


Maximum-intensity projection


Magnetic resonance imaging


Sternal–occipital–mandibular immobilizer


Competing Interests

The authors declare that they have no competing interests.

Compliance with Ethical Standards

No financial support was received for this work.


  1. 1.
    Grisel P. Enucleation de l'atlas et torticollis nasopharyngien. Presse Med. 1930;38:50–3.Google Scholar
  2. 2.
    Golzari SEJ, Ghabili K, Sajadi MM, Aslanabadi S. Early description of Grisel’s syndrome. Childs Nerv Syst. 2013;29:359–60. Scholar
  3. 3.
    Ortiz GL, Pratts I, Ramos E. Grisel's syndrome: an unusual cause of torticollis. J Pediatr Rehabil Med. 2013;6(3):175–80. Scholar
  4. 4.
    Barcelos AC, Patriota GC, Netto AU. Nontraumatic atlantoaxial rotatory subluxation: Grisel syndrome. Case report and literature review. Global Spine J. 2014;4(3):179–86. Scholar
  5. 5.
    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):245–9.PubMedPubMedCentralGoogle Scholar
  6. 6.
    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):342–7.CrossRefPubMedGoogle Scholar
  7. 7.
    Gourin CG, Kaper B, Abdu WA, Donegan JO. Non traumatic atlantoaxial subluxation after retropharyngeal cellulitis: Grisel’s syndrome. Am J Otolaryngol. 2002;23(1):60–5.CrossRefPubMedGoogle Scholar
  8. 8.
    Grobman LR, Stricker S. Grisel’s syndrome. Ear Nose Throat J. 1990;69(12):799–801.PubMedGoogle Scholar
  9. 9.
    Pilge H, Holzapfel BM, Lampe R, Pilge S, Prodinger PM. A novel technique to treat Grisel’s syndrome: results of a simplified, therapeutical algorithm. Int Orthop. 2013;37(7):1307–13. Scholar
  10. 10.
    Wetzel FT, LaRocca H. Grisel’s syndrome. Clin Orthop. 1989;240:141–52.Google Scholar
  11. 11.
    Wilson BC, Jarvis BL, Haydon RC. Nontraumatic subluxation of the atlantoaxial joint: Grisel’s syndrome. Ann Otol Rhinol Laryngol. 1987;96(6):705–8.CrossRefPubMedGoogle Scholar
  12. 12.
    Wilson MJ, Michele AA, Jacobson EW. Spontaneous dislocation of the atlanto-axial articulation, including a report of a case with quadriplagie. J Bone Joint Surg Am. 1940;22:698–707.Google Scholar
  13. 13.
    Kerolus M, Jeans EB, Fontes RB, Deutsch H, Traynelis VC. Atlantoaxial instability of inflammatory origin in adults: case reports, literature review, and rationale for early surgical intervention. Neurosurgery. 2015;76(2):E226–32. Scholar
  14. 14.
    Allegrini D, Autelitano A, Nocerino E, Fogagnolo P, De Cillà S, Rossetti L. Grisel's syndrome, a rare cause of anomalous head posture in children: a case report. BMC Ophthalmol. 2016;16:21. Scholar
  15. 15.
    Ismi O, Ozalp H, Hamzaoglu V, Bucioglu H, Vayısoglu Y, Gorur K (2016) Grisel’s syndrome accompanying a submandibular abscess. Braz J Otorhinolaryngol.
  16. 16.
    Lee JK, Oh CH, Park HC, Yoon SH. Grisel's syndrome induced by Mycobacterium tuberculosis. Korean J Spine. 2015;12(2):84–7. Scholar
  17. 17.
    Shunmugam M, Poonnoose S. Spontaneous atlantoaxial subluxation associated with tonsillitis. Asian J Neurosurg. 2015;10(2):139–41. Scholar
  18. 18.
    Baker LL, Bower CM, Glasier CM. Atlanto-axial subluxation and cervical osteomyelitis: two unusual complications of adenoidectomy. Ann Otol Rhinol Laryngol. 1996;105(4):295–9.CrossRefPubMedGoogle Scholar
  19. 19.
    Bucak A, Ulu S, Aycicek A, Kacar E, Miman MC. Grisel’s syndrome: a rare complication following adenotonsillectomy. Case Rep Otolaryngol. 2014;2014:703021. Scholar
  20. 20.
    Elyajouri A, Assermouh A, Abilkacem R, Agadr A, Mahraoui C. Grisel’s syndrome: a rare complication following traditional uvulectomy. Pan Afr Med J. 2015;20:62. Scholar
  21. 21.
    Feldmann H, Meister EF, Küttner K. From the expert's office. Atlanto-axial subluxation with spastic torticollis after adenoidectomy resp. tonsillectomy in rose position—malpractice of the surgeon or the anaesthesiologist? Laryngorhinootologie. 2003;82(11):799–804.CrossRefPubMedGoogle Scholar
  22. 22.
    Kourelis K, Haronis V, Konandreas I, Kontrafouri A, Asimakopoulos A. Atypical post-adenoidectomy Grisel’s syndrome in Crouzon child with kyphotic skull base. Auris Nasus Larynx. 2015;42(5):416–8. Scholar
  23. 23.
    Nakashima T, Matsuda K, Okuda T, Tono T, Takaki M, Hayashi T. Hanamure case report of atlantoaxial rotatory fixation after cochlear implantation. Case Rep Otolaryngol. 2016;2016:6486271. Scholar
  24. 24.
    Ramdoo K, Hall A, Dimitriadis PA, Singh A. Torticollis following tympanoplasty: an index case with lessons in encountering the unexpected. BMJ Case Rep. 2014;2014:bcr2013201539. Scholar
  25. 25.
    Spennato P, Nicosia G, Rapanà A, Cicala D, Donnianni T, Scala S, Aliberti F, Cinalli G. Grisel syndrome following adenoidectomy: surgical management in a case with delayed diagnosis. World Neurosurg. 2015;84(5):1494.e7–1494.e12. Scholar
  26. 26.
    Fielding JW, Hawkins RJ. Atlanto-axial rotatory fixation. J Bone Joint Surg Am. 1977;59:37–44.CrossRefPubMedGoogle Scholar
  27. 27.
    Ahmed R, Traynelis VC, Menezes AH. Fusions at the craniovertebral junction. Childs Nerv Syst. 2008;24(10):1209–24. Scholar
  28. 28.
    Cekinmez M, Tufan K, Sen O, Caner H. Non-traumatic atlanto-axial subluxation: Grisel’s syndrome. Two case reports. Neurol Med Chir (Tokyo). 2009;49(4):172–4.CrossRefGoogle Scholar
  29. 29.
    de Carvalho M, Swash M. Neurologic complications of craniovertebral dislocation. Handb Clin Neurol. 2014;119:435–48. Scholar
  30. 30.
    Morales LC, Alvarado F, Corredor JA, Rodríguez A. Bilateral C1 laminar hooks combined with C2 pedicle screw fixation in the treatment of atlantoaxial subluxation after Grisel syndrome. Spine J. 2016;16(12):e755–60. Scholar
  31. 31.
    Osiro S, Tiwari KJ, Matusz P, Gielecki J, Tubbs RS, Loukas M. Grisel’s syndrome: a comprehensive review with focus on pathogenesis, natural history, and current treatment options. Childs Nerv Syst. 2012;28:821–5. Scholar
  32. 32.
    Wang JC, Malic C, Reilly C, Verchere C. Microtia reconstruction and postsurgical Grisel's syndrome: a rare cause of torticollis in a child. Plast Reconstr Surg Glob Open. 2014;2(6):e176. Scholar
  33. 33.
    Parke WW, Rothman RH, Brown MD. The pharyngovertebral veins: an anatomical rationale for Grisel’s syndrome. J Bone Joint Surg Am. 1984;66(4):568–74.CrossRefPubMedGoogle Scholar
  34. 34.
    Tedesco B, Grisel P, Desfosses P, Tassin M. Deux nouveaux cas d’énucléation de l’atlas par torticolis nasopharyngien. Bull Soc Pediat. 1930;28:252–62.Google Scholar
  35. 35.
    Battiata A, Pazos G. Grisel’s syndrome: the two-hit hypothesis—a case report and literature review. Ear Nose Throat J. 2004;83:553–5.PubMedGoogle Scholar
  36. 36.
    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):1467–73. Scholar
  37. 37.
    Tschopp K. Monopolar electrocautery in adenoidectomy as a possible risk factor for Grisel’s syndrome. Laryngoscope. 2002;112(8 Pt 1):1445–9.CrossRefPubMedGoogle Scholar
  38. 38.
    Subach BR, McLaughlin MR, Albright AL, Pollack IF. Current management of pediatric atlantoaxial rotatory subluxation. Spine (Phila Pa 1976). 1998;23(20):2174–9.CrossRefGoogle Scholar
  39. 39.
    Viscone A, Brembilla C, Gotti G. The importance and effectiveness of conservative treatment in Grisel's syndrome. J Pediatr Neurosci. 2014;9(2):200–1. Scholar
  40. 40.
    Akbay A, Bilginer B, Akalan N. Closed manual reduction maneuver of atlanto-axial rotatory dislocation in pediatric age. Childs Nerv Syst. 2014;30:1083–9.CrossRefPubMedGoogle Scholar
  41. 41.
    Rinaldo A, Mondin V, Suarez C, Genden EM, Ferlito A. Grisel’s syndrome in head and neck practice. Oral Oncol. 2005;41(10):966–70.CrossRefPubMedGoogle Scholar
  42. 42.
    Hirth K, Welkoborsky HJ. Grisel’s syndrome following ENT-surgery: report of two cases. Laryngorhinootologie. 2003;82(11):794–8. Scholar
  43. 43.
    Kasten P, Zeichen J, Gosling T, Krettek C. Grisel syndrome—a trauma surgery rarity. Unfallchirurg. 2002;105(6):565–8.CrossRefPubMedGoogle Scholar
  44. 44.
    Wright NM. Posterior C2 fixation using bilateral, crossing C2 laminar screws: case series and technical note. J Spinal Disord Tech. 2004;17:158–62.CrossRefPubMedPubMedCentralGoogle Scholar
  45. 45.
    Martinez-Lage JF, Martinez Perez M, Fernandez Cornejo V, Poza M. Atlanto-axial rotatory subluxation in children: early management. Acta Neurochir. 2001;143(12):1223–8.CrossRefPubMedGoogle Scholar
  46. 46.
    Yu KK, White DR, Weissler MC, Pillsbury HC. Nontraumatic atlantoaxial subluxation (Grisel syndrome): a rare complication of otolaryngological procedures. Laryngoscope. 2003;113(6):1047–9.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2019

Authors and Affiliations

  • Corrado Iaccarino
    • 1
    • 2
    Email author
  • Ormitti Francesca
    • 3
  • Spennato Piero
    • 4
  • Rubini Monica
    • 5
  • Rapanà Armando
    • 6
  • Pasquale de Bonis
    • 7
  • Aliberti Ferdinando
    • 4
  • Giorgio Trapella
    • 7
  • Lorenzo Mongardi
    • 7
  • Michele Cavallo
    • 7
  • Cinalli Giuseppe
    • 4
  • Servadei Franco
    • 8
  1. 1.Neurosurgery–Neurotraumatology UnitUniversity Hospital of ParmaParmaItaly
  2. 2.Azienda USL-IRCCS di Reggio EmiliaReggio EmiliaItaly
  3. 3.Department of NeuroradiologyUniversity Hospital of ParmaParmaItaly
  4. 4.Neurosurgery DepartmentAzienda Ospedaliera di Rilievo Nazionale “Santobono-Pausilipon-Annunziata” Children’s HospitalNaplesItaly
  5. 5.Division of Paediatric General and Emergency Care UnitChildren’s Hospital of ParmaParmaItaly
  6. 6.Neurosurgery Unit, Lorenzo Bonomo HospitalAndriaItaly
  7. 7.Neurosurgery DepartmentUniversity Hospital of FerraraFerraraItaly
  8. 8.New YorkUSA

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