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

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New Trends in Craniovertebral Junction Surgery

Part of the book series: Acta Neurochirurgica Supplement ((NEUROCHIRURGICA,volume 125))

Abstract

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.

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Abbreviations

3D:

Three-dimensional

ADI:

Atlantodental interval

CT:

Computed tomography

ENT:

Ear, nose and throat

MIP:

Maximum-intensity projection

MRI:

Magnetic resonance imaging

SOMI:

Sternal–occipital–mandibular immobilizer

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Competing Interests

The authors declare that they have no competing interests.

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Authors and Affiliations

  1. Neurosurgery–Neurotraumatology Unit, University Hospital of Parma, Parma, Italy

    Corrado Iaccarino

  2. Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy

    Corrado Iaccarino

  3. Department of Neuroradiology, University Hospital of Parma, Parma, Italy

    Ormitti Francesca

  4. Neurosurgery Department, Azienda Ospedaliera di Rilievo Nazionale “Santobono-Pausilipon-Annunziata” Children’s Hospital, Naples, Italy

    Spennato Piero, Aliberti Ferdinando & Cinalli Giuseppe

  5. Division of Paediatric General and Emergency Care Unit, Children’s Hospital of Parma, Parma, Italy

    Rubini Monica

  6. Neurosurgery Unit, Lorenzo Bonomo Hospital, Andria, Italy

    Rapanà Armando

  7. Neurosurgery Department, University Hospital of Ferrara, Ferrara, Italy

    Pasquale de Bonis, Giorgio Trapella, Lorenzo Mongardi & Michele Cavallo

  8. New York, USA

    Servadei Franco

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  1. Corrado Iaccarino
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Correspondence to Corrado Iaccarino .

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Editors and Affiliations

  1. Institute of Neurosurgery, Catholic University of Rome, Institute of Neurosurgery, Rome, Italy

    Massimiliano Visocchi

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Iaccarino, C. et al. (2019). Grisel’s Syndrome: Non-traumatic Atlantoaxial Rotatory Subluxation—Report of Five Cases and Review of the Literature. In: Visocchi, M. (eds) New Trends in Craniovertebral Junction Surgery. Acta Neurochirurgica Supplement, vol 125. Springer, Cham. https://doi.org/10.1007/978-3-319-62515-7_40

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  • DOI: https://doi.org/10.1007/978-3-319-62515-7_40

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