Title: GRISEL
1GRISELS 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 Bechir Hamza, Place Bab
Saadoun. 1007. JEBBARI. TUNIS. TUNISIA
2INTRODUCTION
3- Grisels 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 Grisels syndrome.
4PATIENTS 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. - At physical examination, 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.
6RESULTS
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.
9T1 and T2-weighted axial views showing bilateral
necrotic cervical lymphadenopathies ( ),
osteitis of anterior arch of C1 and C2 (
).
10T2-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.
11Fat suppression T1-SE after gadolinium
administration sagittal and axial views confirms
the necrotic character of the lymphadenopathies
extending to prevertebral space ( ), and
shows epiduritis ( )
12DISCUSSION
13Pathogenesis
- The exact etiology of Grisels 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
Grisels 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 Grisels 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.
16Classification
- Subluxation of the atlantoaxial joint is graded
using the classification system developed by
Fielding et 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 lt3 mm anterior displacement of the
atlas. - Type II 35 mm anterior displacement.
- Type III gt5 mm anterior displacement.
- (D) Type IV posterior displacement of the atlas
(after Fielding et al. )
17- Type I lt3 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 gt5 mm anterior displacement 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 Grisels syndrome.
18Imaging
- 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.
20Management 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 Grisels 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.
22CONCLUSION
23- Grisels 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 Grisels 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 Grisels
syndrome.