Elsevier

Oral Oncology

Volume 41, Issue 10, November 2005, Pages 966-970
Oral Oncology

REVIEW
Grisel’s syndrome in head and neck practice

https://doi.org/10.1016/j.oraloncology.2005.02.005Get rights and content

Summary

Grisel’s syndrome is a rare condition of uncertain etiology characterized by a non-traumatic atlanto-axial subluxation, secondary to an infection in the head and neck region. The authors discuss the history, pathogenesis, terminology, classification, clinical characteristics, diagnosis, treatment, prognosis and prevention of this syndrome.

Introduction

Grisel’s syndrome (GS) is a rare, but important, condition of uncertain etiology described in the otolaryngologic, pediatric, neurosurgical and orthopedic literature. It is characterized by a non-traumatic atlanto-axial subluxation generally associated with head and neck infection or any condition that results in hyperemia and relaxation of the transverse ligament of the atlanto-axial joint.

Making the diagnosis of GS is “thinking of it”: a suggestive history of procedures or infections in the head and neck region, an unexpected unfavorable evolution of cervical spine problems evolving in torticollis, an immunocompromised patient.1

Otolaryngologists should be made aware of this condition and be vigilant of its early presentation so that effective treatment can be given before the development of potentially disastrous complications.

Section snippets

History

In 1930, a French physician, P. Grisel,2 reported on two young girls of 8 and 9 years of age, respectively, who presented with torticollis and fever secondary to adenoidectomy in the former case and to rhinopharyngitis in the latter patient. He accurately described the symptoms, pathogenesis, and radiographic findings of this particular phenomenon which is now known as “Grisel’s syndrome”. Other such cases had been previously reported,2 but the first description dates back to 1830 by Sir

Pathogenesis

Historically, three main theories have been indicated to explain the pathogenesis of GS: (1) a metastatic inflammatory effusion as cause of the ligamentous stretching and subluxation, (2) subluxation as a consequence of muscle spasms, and (3) ligamentous laxity and subluxation as results of the regional hyperemia and consequent decalcification of the attachments of the transverse ligament.4 Although there are several other theories concerning the pathogenesis of GS, it is generally agreed that

Terminology and classification

Many terms in the literature have been coined to describe what now is known as “Grisel’s syndrome”, i.e., malum suboccipitale rheumaticum, distension luxation, Drehungsverrenkung, torticollis nasopharyngien, nasopharyngeal torticollis, maladie de Grisel, Grisel–Bourgeois syndrome, non-traumatic subluxation, spontaneous hyperemic dislocation, inflammatory dislocation, spontaneous dislocation, dislocation nontraumatique, le faux torticollis aigu. It has been proposed also the term “rotary

Clinical characteristics

GS is characterized by its clinical variability, with not typical time frame noted between the onset of the infection and the appearance of the torticollis. This syndrome occurs mostly in children and is a rare complication following otolaryngologic procedures and upper respiratory infections.4 This high incidence is probably related to elastic or lax ligaments as children have. Based on the same pathogenetic prerequisite, GS often affects patients with Down’s syndrome.25, 27 Atlanto-axial

Diagnosis

The diagnosis of GS is based on the association of clinical findings (head tilt with painful and restricted neck movements) with a previous history of an upper respiratory tract infection.34 The usual alternative diagnosis is meningitis. However, the differential diagnosis of torticollis ranges from trauma to drug reactions.35

Three clinical signs define the diagnosis of an atlanto-axial rotary subluxation: (1) the Sudek sign, i.e. the palpable deviation of the spinous process of C2 in the same

Treatment, prognosis and prevention

Considering that the cause of GS is an infectious process, the first therapeutical step is medical. Therefore, the source of the infection has to be identified and appropriate antimicrobial therapy initiated. Broad spectrum antibiotics should be prescribed initially until the organism is identified either by blood culture, local aspiration or biopsy.8

A Type 0 subluxation generally needs no further treatment following the antibiotic therapy; however, a close follow-up is recommended. Patients

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