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Inflammatory atlanto-axial subluxation (Grisel's syndrome) in children: clinical diagnosis and management

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Abstract

Background

Grisel's syndrome is a uni- or bilateral subluxation of the atlas on the axis associated with an inflammatory condition of the head or neck. This uncommon entity usually affects children. The condition seems to be secondary to hyperemia and laxity of the atlanto-axial joints.

Patients and methods

We report clinical and neuroimaging findings, treatment modalities, and outcomes in 4 children with atlanto-axial rotatory subluxation (AARS) associated with a previous upper respiratory tract infection treated at our Unit during the last 7 years.

Results

All children presented with neck pain, head tilt, and reduced neck motion. In 3 patients, the condition was secondary to otitis media and in the remaining patient to viral pharyngitis. The diagnosis was established by clinical signs and symptoms and confirmed by neuroimaging studies, especially by computerized tomography and 3-D reconstructions. Early detection and treatment resulted in a good outcome in all patients. None of the patients required surgical treatment.

Conclusions

Grisel's syndrome must be suspected in children with painful torticollis associated with an upper tract respiratory infection. The best results are obtained with early conservative treatment, which prevents chronic changes leading to persistent neck pain and deformity, namely atlanto-axial rotatory fixation (AARF). We emphasize the fact that the diagnosis of Grisel's syndrome is mainly clinical, while the diagnostic confirmation of AARF can only be attained by special neuroimaging techniques.

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Acknowledgement

The authors wish to thank Mr. Saturnino Espín, of the Hospital's Unit of Photography, for preparing the illustrations.

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Authors and Affiliations

  1. Regional Service of Neurosurgery, Virgen de la Arrixaca University Hospital, 30120, El Palmar, Murcia, Spain

    Víctor J. Fernández Cornejo, Claudio Piqueras & Máximo Poza

  2. Unit of Pediatric Neurosurgery, Virgen de la Arrixaca University Hospital, 30120, El Palmar, Murcia, Spain

    Juan F. Martínez-Lage

  3. Section of Pediatric Radiology, Virgen de la Arrixaca University Hospital, 30120, El Palmar, Murcia, Spain

    Amparo Gelabert

Authors
  1. Víctor J. Fernández Cornejo
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  2. Juan F. Martínez-Lage
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  3. Claudio Piqueras
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  4. Amparo Gelabert
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  5. Máximo Poza
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Corresponding author

Correspondence to Juan F. Martínez-Lage.

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Fernández Cornejo, V.J., Martínez-Lage, J.F., Piqueras, C. et al. Inflammatory atlanto-axial subluxation (Grisel's syndrome) in children: clinical diagnosis and management. Childs Nerv Syst 19, 342–347 (2003). https://doi.org/10.1007/s00381-003-0749-6

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  • DOI: https://doi.org/10.1007/s00381-003-0749-6

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