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CC BY 4.0 · Journal of Child Science 2021; 11(01): e114-e119
DOI: 10.1055/s-0041-1728727
Case Report

Grisel's Syndrome: A Masquerader of Torticollis with a Hidden Atlanto-Axial Dislocation Agenda

Eyrique Goh 
1   Orthopedics and Traumatology Department, Hospital Keningau, Sabah, Malaysia
,
Pathmarooban D.
1   Orthopedics and Traumatology Department, Hospital Keningau, Sabah, Malaysia
,
Alif F. R.
1   Orthopedics and Traumatology Department, Hospital Keningau, Sabah, Malaysia
› Author Affiliations Funding None.
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Abstract

Torticollis is a rather common presentation in pediatrics, with common etiologies. This is a rare presentation of a spinal pathology secondary to an underlying infection of the head and neck region causing anomalous head position masquerading as a torticollis. Painful torticollis, or abnormal head position described as “cock-robin head” position in children, draws attention. Grisel's syndrome (GS) is an inflammatory condition of a nontraumatic atlantoaxial rotatory subluxation or fixation (AARS/F). Diagnosis is derived clinically with radiological confirmation. The aim of this report is to raise attention of positional masquerade during intervals of radiographs, leading to the delay in diagnosis and heighten awareness of this syndrome that is needed to derive the diagnosis. In this case, the patient initially presented to the institution with a radiograph that had shown no overt abnormality and was requested for further detailed imaging, but the child's parents had refused. Upon a revisit to the clinic due to worsening of symptoms, a repeated radiograph was taken, a clear AARS was detected, and proceeded with a computed tomography (CT) cervical imaging to confirm the subluxation. The CT scan showed an overt subluxation with significant narrowing of the cord spaces with a retropharyngeal abscess. Otolaryngology and Spine services were consulted, and a surgical option was offered. However, the parents refused surgery and proceeded conservatively with antibiotics and a cervical halter. Early diagnosis and early treatment is fundamental to a better prognosis. Presence of GS is associated with late recovery in comparison to non-Grisel's causes of AARS/F. A treating surgeon should be aware of this condition and recognize it based on the “cock-robin” head position in absence of a triggering trauma with an underlying inflammatory pathology of the head and neck region. Presence of concomitant infection with AARS/F and Fielding and Hawkins classification types are prognostic factors for late recovery.

Keywords

Grisel's syndrome - atlantoaxial rotatory subluxation - atlantoaxial rotatory fixation - atlantoaxial instability - retropharyngeal abscess - cock robin - torticollis

Note

The usage of the patient's clinical images is consented by the parents for publications and academic purposes. A written consent was granted and documented in the case notes.




Publication History

Received: 25 October 2020

Accepted: 02 March 2021

Article published online:
01 June 2021

© 2021. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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  • References

  • 1 Spinnato P, Aparisi Gomez MP, Molinari M, Mercatelli D, Bazzocchi A. Torticollis after a somersault: a case of Grisel's Syndrome. Indian J Pediatr 2019; 86 (02) 198-199
    CrossrefPubMedGoogle Scholar
  • 2 Sundseth J, Berg-Johnsen J, Skaar-Holme S, Züchner M, Kolstad F. Atlantoaxial rotatory fixation--a cause of torticollis. Tidsskr Nor Laegeforen 2013; 133 (05) 519-523
    CrossrefPubMedGoogle Scholar
  • 3 Das S, Chakraborty S. Grisel syndrome in otolaryngology: a case series with literature review. Indian J Otolaryngol Head Neck Surg 2016
    PubMedGoogle Scholar
  • 4 Spinnato P, Guerri S, Barakat M. et al. Atlantoaxial rotatory subluxation/fixation and Grisel's syndrome in children: clinical and radiological prognostic factors. Eur J Pediatr 2020
    PubMedGoogle Scholar
  • 5 Aladag Ciftdemir N, Eren T, Ciftdemir M. A rare cause of torticollis: Grisel syndrome. J Trop Pediatr 2018; 64 (03) 245-248
    CrossrefPubMedGoogle Scholar
  • 6 Falsaperla R, Piattelli G, Marino S, Marino SD, Fontana A, Pavone P. Grisel's syndrome caused by mycoplasma pneumoniae infection: a case report and review of the literature. Childs Nerv Syst 2019; 35 (03) 523-527
    CrossrefPubMedGoogle Scholar
  • 7 Anania P, Pavone P, Pacetti M. et al. Grisel syndrome in pediatric age: a single-center italian experience and review of the literature. World Neurosurg 2019; 125: 374-382
    CrossrefPubMedGoogle Scholar
  • 8 Munevver SB, Demirok D, Capkin E. et al. Grisel syndrome: a case report. Official Journal of the Turkish league against Rheumatism (TLAR). Arch Rheumatol 2011; 26 (03) 243-247
    PubMedGoogle Scholar
  • 9 Fielding JW, Hawkins RJ. Atlanto-axial rotatory fixation. (Fixed rotatory subluxation of the atlanto-axial joint). J Bone Joint Surg Am 1977; 59 (01) 37-44
    CrossrefPubMedGoogle Scholar