Grisel's syndrome-a rare complication following small operations and infections in the ENT region

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.