Grisel’s syndrome—a rare complication following “small” operations and infections in the ENT region

Abstract

The atlanto-axial subluxation (Grisel's syndrome) is a rare complication following operative procedures and/or infections in the upper aerodigestive tract. Pathogenetically the higher flexibility of the ligaments during the inflammation causes a subluxation between axis and atlas. When the inflammation heals, this can probably result in a fixation in the rotated position. The purpose of the present study was to describe the clinical and radiological characteristics of this rare disease in children. The clinical files of 12 patients with Grisel's syndrome were examined retrospectively. The clinical files of these patients were reviewed and analyzed along with the results of radiographic and laboratory examinations. The clinical parameters were compared with the patient's therapy and outcome. Of 12 children (6 males, 6 females, average age of 7.1 years), Grisel's syndrome developed following surgery in 8 patients, and in another 4 patients following a severe in infection of the upper aerodigestive tract. The duration of complaints differed from 2 days to 6 months. All patients had a torticollis as the first symptom of atlanto-axial dislocation and three children had accompanying cervical lymphadenopathy. All patients were given antiphlogistic therapy either with diclofenac or ibuprofen. Additionally, 11 patients were treated with intravenous antibiotics (amoxicillin, ampicillin, clindamycin or cephalosporins) and 1 child with oral antibiotics. In eight patients a remission during antibiotic therapy occurred. In four cases (2 with Grisel's syndrome following surgery, 2 following infection), however, the torticollis persisted despite adequate conservative treatment and required reposition of the atlanto-axial joint along with external fixation. In all of them, starting of therapy was delayed. An early diagnosis of Grisel's syndrome and immediate therapy is most important. Grisel's syndrome must be taken under consideration in children with acute torticollis following either an infection or operative procedure in the upper aerodigestive tract. Early adequate antibiotic and antiphlogistic therapy is mandatory and leads to a high remission rate.

Grisel’s syndrome: a comprehensive review with focus on pathogenesis, natural history, and current treatment options

“…Gourin et al [10] reported that 21 of 78 (26.9%) GS cases occurred post-op, while 14 of 78 (18%) were secondary to URTIs. This finding was corroborated by Deichmueller and his colleagues who reported that 67% of their GS were post-ENT surgery patients, while 33% followed nasopharyngeal infections [6].…”

“…Deichmueller and Welkoborsky [6] described 12 cases of GS in which all the patients had torticollis as the first symptom and three Table 1 Diagnostic algorithm for Grisel's syndrome (used with permission from [6])…”

“…The anatomical route of possible spread of the septic emboli from the pharyngovertebral veins to the periodontoidal vascular plexus, as was first described by Parke, is often used to support this postulation [21]. The diagnosis of GS is clinical, with painful torticollis and aversion to head rotation considered pathognomonic [6]. However, since it can mimic several otolaryngologic conditions, radiographic confirmation of the diagnosis with either CT scan or MRI is often required.…”

Grisel syndrome, acute otitis media, and temporo-mandibular reactive arthritis: A rare association

“…In the presence of an AARS with no prior cervical trauma and only acute otitis media as the trigger factor, lead us to establish Grisel Syndrome diagnosis (Deichmueller and Welkoborsky diagnostic algorithm [6] - Table 1).…”

A diagnose not to miss: Rotatory Atlantoaxial subluxation in emergency service

“…Most of the patients with Grisel's syndrome have a surgery history, mostly otolaryngeal [28,29]. To preclude irreversible neurologic defisits, treatment should be given as soon as possible.…”