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GRISEL

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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 – PowerPoint PPT presentation

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Title: GRISEL


1
GRISELS 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
2
INTRODUCTION
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.

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.
  • 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.

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 views confirms
the necrotic character of the lymphadenopathies
extending to prevertebral space ( ), and
shows epiduritis ( )
12
DISCUSSION
13
Pathogenesis
  • 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.

16
Classification
  • 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.

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 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.

22
CONCLUSION
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.
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