GRISEL’S SYNDROME : A CASE REPORT PED23

1. GRISEL’S SYNDROME : A CASE REPORTPED23 I.OMRI, N.BOUCHNAK, L.LAHMAR, H.LOUATI, L. BEN HASSINE, W.DOUIRA, I.BRINI*, I.BELLAGHA Radiology Department *Pediatric Department Children's Hospital BechirHamza, Place BabSaadoun. 1007. JEBBARI. TUNIS. TUNISIA

2. INTRODUCTION

3. Grisel’s syndrome is a non-traumatic atlantoaxial subluxation (AAS) secondary to an inflammatory process in the upper neck. • It may occur secondary to head and neck infection or following routine otolaryngological procedures. • The underlying mechanism is unknown but haematogenous spread of infection from the posterior pharynx to the cervical spine with hyperemia and abnormal relaxation of the atlantoaxial ligaments is a widely accepted theory. • A case of an upper cervical infection is presented to assess etiology, treatment and outcome of Grisel’s syndrome.

4. PATIENTS AND METHODS

5. We report the case of a 2-month-old girl who was referred for evaluation of bilateral cervical masses. Her past medical history was non-contributory. • Atphysicalexamination, the patient was noted to hold her head slightly rotated and bent to the left. • The child underwent first cervical ultrasound. • A cervico-mediastinal MRI was performed as a complement for a better characterization of these masses. It was performed at 1.5 Tesla MR [General Electric, GE healthcare] with and without injection of Gadolinium.

6. RESULTS

7. The cervical ultrasound showed bilateral non compressive jugulo-carotid and spinal lymphadenopathies. Right ones measure 53x27mm and left ones measure 51x27mm . • Three days later, the patient developed quadriparesis and a cervico-mediastinal MRI was performed. • This exam  included  T1-weighted SE sequences both without and with fat saturation, with and without injection of Gadolinium and T2-weighted SE in sagittal and axial planes

8. The MRI helped to highlight: • bilateral necrotic cervical lymphadenopathies . Right ones measure 39x24x15mm and left ones measure 36x35x21mm , • osteitis of clivus and anterior arch of C1 and C2, • epiduritis, • spinal cord compression without any signs of suffering.

9. T1 and T2-weighted axial views showing bilateral necrotic cervical lymphadenopathies ( ), osteitis of anterior arch of C1 and C2 ( ).

10. T2-weighted sagittal views showing bilateral necrotic cervical lymphadenopathies ( ) with extension to prevertebral space, lysis of the anterior arch of C1 and C2 ( ), spinal cord compression ( ) and no signs of suffering.

11. Fat suppression T1-SE after gadolinium administration sagittal and axial viewsconfirms the necroticcharacter of the lymphadenopathiesextending to prevertebralspace ( ), and shows epiduritis ( )

12. DISCUSSION

13. Pathogenesis • The exact etiology of Grisel’s syndrome is controversial. • It is generally accepted that the process is initiated by an infection in the upper cervical region which creates an inflammatory milieu. • Grisel postulated that AAS was caused by muscle spasm; now muscle spasm is thought to be a reaction to adjacent inflammation and the role of this muscle spasm is limited to maintenance of the subluxed position but is not causative . • Hyperemia of the blood supply of bone has been shown to cause resorption.

14. Watson-Jones suggested that resorption of the attachment of the transverse ligament to the atlas mediated by inflammation could lead to AAS. • More recently, Welinder et al provided radiologic evidence for this phenomenon. They followed a Grisel’s patient with serial CT scans and observed distension of the transverse ligament with maintenance of the integrity of its attachments. • The hyperemia following the infection that leads to decalcification of the anterior arch of the atlas and laxity of the transverse ligament is the most widely accepted mechnism responsible for Grisel’s syndrome.

15. Parke et al provided an anatomic explanation for this theory by demonstrating the relationship of the retropharyngeal space to the atlantoaxial space via a previously undescribed pathway, the pharyngovertebral veins. • Interestingly, these veins were also found to have frequent anastamoses with the lymphatics of the retropharyngeal space.

16. Classification • Subluxation of the atlantoaxial joint is graded using the classification system developed by Fieldinget al. • All of the Fielding types have rotary subluxation in common and are differentiated by the degree and direction of atlas displacement. • Fielding classification of AAS • Type I = <3 mm anterior displacement of the atlas. • Type II = 3—5 mm anterior displacement. • Type III = >5 mm anteriordisplacement. • (D) Type IV = posteriordisplacement of the atlas (after Fielding et al. )

17. Type I = <3 mm anterior displacement of the atlas with intact transverse and lateral ligaments, • Type II = 3-5 mm anterior displacement with disruption of the transverse ligament, • Type III = >5 mm anteriordisplacement with disruption of both transverse and lateral ligaments . • Type IV = posterior displacement of the atlas. • Kraft and Tschopp proposed the addition of type 0 to this classification for patients with no subluxation but who have persistent torticollis and may be expected to develop Grisel’s syndrome.

18. Imaging • Early detection of atlanto-axial rotatory subluxation requires a combination of clinical assessment and appropriate radiographic imaging. • Both computed tomography (CT) scanning and magnetic resonance imaging (MRI) are diagnostically used: • CT with three-dimensional reconstruction allows precise determination of the bony detail (bone destruction) and quantification of subluxation (malalignment) • MRI provides more precise details of the situation of the spinal cord and can reveal occult infections in the upper respiratory tract showing soft tissue changes.

19. A radioisotope Tc bone scan is useful during the acute phase of the disease, but is aspecific. • Dynamic studies may be necessary, but they may cause neurological complications. Orthopedic or neurosurgical consultation is advised.

20. Management and prognosis • Early subluxation (Fielding types I and II) is typically not associated with neurologic impairment. • Fielding types III and IV lesions have been reported to have a 15% incidence of neurologic impairment ranging from radiculopathy to paralysis and death . • Fielding and Hawkings in 1978 recommended - traction and immobilization for types I and II - surgical fixation for types III and IV.

21. In a more recent review by Wietzel, the authors suggest treatment of: - type I with soft collar, - type II with hard collar, - type III with halo fixation, - type IV with open fixation. • All studies agree that any patient with neurologic symptoms should undergo immediate open fixation. • Good outcomes for patients with Grisel’s syndrome are linked to early recognition of the disease, prompt medical management of the underlying inflammatory process, and immediate treatment of the AAS with immobilization or fixation as indicated.

22. CONCLUSION

23. Grisel’s syndrome is a rare but dangerous condition that occurs almost exclusively in children and has been associated with upper cervical infections and otolaryngologic procedures. • Making the diagnosis of Grisel’s syndrome is ‘thinking of it’ : a suggestive history of procedures or infections in the head and neck region, an unfavorable evolution of cervical spine problems evolving in torticollis, an immunocompromised patient. • Potential sequelae can be severe. • Early diagnosis and early treatment are fundamental for a better prognosis of Grisel’s syndrome.