REVIEWGrisel’s syndrome in head and neck practice
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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Laryngorhinootologie
Cited by (26)
Conservative approach for treatment of Grisel's syndrome after resection of lympahadenitis tuberculosis of the neck: A rare case report
2021, International Journal of Surgery Case ReportsCitation Excerpt :Previous study reported persistent neurological complications in up to 15% patients underwent surgical intervention. Moreover, quadriplegia had been documented [15,16]. Orthosis is usually prescribed for children until they reach skeletal maturity.
Grisel's syndrome post otolaryngology procedures: A systematic review
2020, International Journal of Pediatric OtorhinolaryngologyCitation Excerpt :In agreement with Karkos et al. [2], most of the cases were managed conservatively. Surgery is only needed in the case of failure of conservative measurements, recurrence, or if subluxation is not reducible [6]. In our data, we found that 64.8% of cases were treated with medical therapy alone or in combination with other options; a cervical collar was used in 45 cases (83.3%) and surgery in 13 cases (24.1%).
Grisel syndrome, sigmoid sinus thrombosis and rheumatic carditis: Case report of a rare association
2020, International Journal of Pediatric OtorhinolaryngologyCitation Excerpt :Other authors say that hyperemia of the blood supply of bone, following an infections or surgical trauma, leads to decalcification of the anterior arch of the atlas, the decalcification induces laxity of the anterior transverse ligament between the atlas and the axis [14]. Upon physical examination, three signs define the clinical diagnosis of atlanto axial rotator subluxation: palpable deviation of the spinous process of the axis in the same direction of head rotation, spasm of the sternocleidomastoid muscle ipsilateral to the rotation, and inability to turn the head beyond the midline in the direction of the side opposite the lesion [15]. The patient neck is motionless, in subtle rotation and flexion, with the chin in a position opposite to that of the affected side, characterizing the pathologic posture denoted “cock robin posture,” a term coined in reference to the posture of a robin with its head in rotatio [16].
Grisel's syndrome as a sequela of a complicated acute mastoiditis
2013, Acta Otorrinolaringologica EspanolaGrisel's syndrome in otolaryngology: A systematic review
2007, International Journal of Pediatric OtorhinolaryngologyCitation Excerpt :Whatever the cause though, early identification of the atlantoaxial subluxation and – most importantly – neurosurgical consultation is important to prevent significant neurological deficit. Rinaldo et al. concluded that a significant 15% of patients may develop permanent neurological sequelae following NAAS [3]. Diagnosis of NAAS should be based on a high index of suspicion especially for the “non-high risk” groups.
Grisel's syndrome accompanying a submandibular abscess
2020, Brazilian Journal of Otorhinolaryngology