Child's Nervous System

, Volume 28, Issue 6, pp 821–825 | Cite as

Grisel’s syndrome: a comprehensive review with focus on pathogenesis, natural history, and current treatment options

  • Stephen Osiro
  • Kevin J. Tiwari
  • Petru Matusz
  • Jerzy Gielecki
  • R. Shane Tubbs
  • Marios Loukas
Review Paper

Abstract

Introduction

Grisel’s syndrome (GS) is a rare otolaryngologic condition that involves rotatory subluxation of the atlanto-axial joint in the absence of trauma. Sir Charles Bell was the first to describe this condition in 1830. However, it was named after Pierre Grisel, a French ENT specialist who in 1930 described three such cases in children.

Methods

Using standard search engines, we reviewed the most recent literature regarding GS with special attention to its pathogenesis, natural history, and recent treatment options.

Results

Although defined over 80 years ago, the exact pathogenesis leading to GS is still not completely understood. Nevertheless, it occurs mainly as a complication of upper respiratory tract infections and/or surgical interventions in the head and neck. Most recent studies postulate that the spread of septic emboli from such infection nidus via the pharyngovertebral veins to the periodontoid vascular plexus could be the most likely cause. The diagnosis of GS is made by both clinical assessment as well as radiologic confirmation with either CT scan or MRI. Our review found out that most authors favor immobilization with a soft collar and antibiotics over surgical correction in the initial management of GS. Surgery is reserved for severe and refractory cases.

Conclusion

GS remains a rare, but potentially lethal children condition. Early diagnosis and treatment is crucial to its management and prognosis.

Keywords

Grisel’s syndrome Atlantoaxial subluxation Infectious process Antibiotic therapy 

References

  1. 1.
    Battiata A, Pazos G (2004) Grisel’s syndrome: the two-hit hypothesis—a case report and literature review. Ear Nose Throat J 83:553–555PubMedGoogle Scholar
  2. 2.
    Bell C, McKay C (1830) The nervous system of the human body embracing papers delivered to the Royal Society on the subject of the nerves. Longman, Rees, and Orme, LondonGoogle Scholar
  3. 3.
    Cekinmez M, Tufan K, Sen O, Caner H (2009) Non-traumatic atlanto-axial subluxation: Grisel’s syndrome. Two case report. Neurol Med Chir 49:172–174CrossRefGoogle Scholar
  4. 4.
    Chute DJ, Reiber K (2008) Three unusual neuropathologic-related causes of sudden death. J Forensic Sci 53:734–738PubMedCrossRefGoogle Scholar
  5. 5.
    Dagtekin A, Kara E, Vayisoglu Y, Koseoglu A, Avci E, Talas D, Bagdatoglu C (2011) The importance of early diagnosis and appropriate treatment in Grisel’s syndrome: report of two cases. Turk Neurosurg 21:680–684PubMedGoogle Scholar
  6. 6.
    Deichmueller C, Welkoborsky HJ (2010) Grisel’s syndrome—a rare complication following “small” operations and infections in the ENT region. Eur Arch Otorhinolaryngol 267:1467–1473PubMedCrossRefGoogle Scholar
  7. 7.
    Derkay C, Kenna M, Pang D (1987) Refractory torticollis: an uncommon complication of adenotonsillectomy. Int J Pediatr Otorhinolaryngol 14:87–93PubMedCrossRefGoogle Scholar
  8. 8.
    Durst F, Staudenmaier R, Pilge H, Lauen J, Prodinger P, Holzapfel K, Pickhard A (2011) Grisel-Syndrom nach Anthelixplastik. HNO: 1–4Google Scholar
  9. 9.
    Galer C, Holbrook E, Treves J, Leopold D (2005) Grisel’s syndrome: a case report and review of the literature. Int J Pediatric Otorhinolaryngol 69:1689–1692CrossRefGoogle Scholar
  10. 10.
    Gourin C, Kaper B, Abdu W, Donegan J (2002) Nontraumatic atlanto-axial subluxation after retropharyngeal cellulitis: Grisel’s syndrome. Am J Otolaryngol 23:60–65PubMedCrossRefGoogle Scholar
  11. 11.
    Grisel P (1930) Enucleation de l'atlas et torticollis nasopharyngien. Presse Med 38:50–53Google Scholar
  12. 12.
    Grobman L, Stricker S (1990) Grisel’s syndrome. Ear Nose Throat J 69:799–801PubMedGoogle Scholar
  13. 13.
    Harth M, Mayer M, Marzi I, Vogl TJ (2004) Lateral torticollis on plain radiographs and MRI: Grisel syndrome. Eur Radiol 14:1713–1715PubMedCrossRefGoogle Scholar
  14. 14.
    Jackson H (1950) The diagnosis of minimal atlanto-axial subluxation. Br J Radiol 23:672–674PubMedCrossRefGoogle Scholar
  15. 15.
    Karkos PD, Benton J, Leong SC, Mushi E, Sivaji N, Assimakopoulos DA (2007) Grisel’s syndrome in otolaryngology: a systematic review. Int J Pediatr Otorhinolaryngol 71:1823–1827PubMedCrossRefGoogle Scholar
  16. 16.
    Lopes DK, Li V (1998) Midcervical postinfectious ligamentous instability: a variant of Grisel’s syndrome. Pediatr Neurosurg 29:133–137PubMedCrossRefGoogle Scholar
  17. 17.
    Martinez-Lage J, Morales T, Cornejo V (1989) Inflammatory C2–3 subluxation: a Grisel’s syndrome variant. Arch Dis Child 88:628–629CrossRefGoogle Scholar
  18. 18.
    Mathern G, Batzdorf U (1989) Grisel’s syndrome. Cervical spine clinical, pathologic, and neurologic manifestations. Clin Orthop Relat Res 244:131–146PubMedGoogle Scholar
  19. 19.
    Pandya S (1972) Atlantoaxial dislocation. Neurol India 20:13–48PubMedGoogle Scholar
  20. 20.
    Panopalis P, Christopoulos S (2005) Grisel’s syndrome: non-traumatic subluxation of the atlantoaxial joint. J Rheumatol 32:1619PubMedGoogle Scholar
  21. 21.
    Parke W, Rothman R, Brown M (1984) The pharyngovertebral veins: an anatomical rationale for Grisel’s syndrome. J Bone Joint Surg Am 66:568–574PubMedGoogle Scholar
  22. 22.
    Pilge H, Prodinger PM (1976) Nontraumatic subluxation of the atlanto-axial joint as rare form of aquired torticollis: diagnosis and clinical features of the Grisel’s syndrome. Spine 36:E747–E751CrossRefGoogle Scholar
  23. 23.
    Richter GT, Bower CM (2006) Cervical complications following routine tonsillectomy and adenoidectomy. Curr Opin Otolaryngol Head Neck Surg 14:375–380PubMedCrossRefGoogle Scholar
  24. 24.
    Rinaldo A, Mondin V, Suarez C, Genden EM, Ferlito A (2005) Grisel’s syndrome in head and neck practice. Oral Oncol 41:966–970PubMedCrossRefGoogle Scholar
  25. 25.
    van der Vis-Melsen MJ (1992) Torticollis nasopharyngealis (Grisel’s syndrome). Eur J Nucl Med Mol Imaging 19:369–370CrossRefGoogle Scholar
  26. 26.
    Welinder N, Hoffmann P, Hakansson S (1997) Pathogenesis of non-traumatic atlanto-axial subluxation (Grisel’s syndrome). Eur Arch Otorhinolaryngol 254:251–254PubMedCrossRefGoogle Scholar
  27. 27.
    Wilson B, Jarvis B, Haydon RI (1997) Nontraumatic subluxation of the atlantoaxial joint: Grisel’s syndrome. Ann Otol Rhinol Laryngol 96:705–708Google Scholar
  28. 28.
    Wittek A (1908) Ein Fall von Distensions luxation im Atlantoepistropheal Gelenke. Mfinch Med Wochenschr 55:1836–1837Google Scholar
  29. 29.
    Yu KK, White DR, Weissler MC, Pillsbury HC (2003) Nontraumatic atlantoaxial subluxation (Grisel Syndrome): a rare complication of otolaryngological procedures. Laryngoscope 113:1047–1049PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2012

Authors and Affiliations

  • Stephen Osiro
    • 1
  • Kevin J. Tiwari
    • 1
  • Petru Matusz
    • 2
  • Jerzy Gielecki
    • 3
  • R. Shane Tubbs
    • 4
  • Marios Loukas
    • 1
    • 3
  1. 1.Department of Anatomical Sciences, School of MedicineSt. George’s UniversityWest IndiesGrenada
  2. 2.Department of AnatomyVictor Babes University of Medicine and PharmacyTimisoaraRomania
  3. 3.Department of AnatomyMedical School Varmia and MazuriaOlsztynPoland
  4. 4.Pediatric NeurosurgeryChildren’s HospitalBirminghamUSA

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