GRISEL

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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
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INTRODUCTION
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• 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.

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PATIENTS AND METHODS
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• 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.

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RESULTS
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• 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

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• 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.

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

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• 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.

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• 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.

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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. )

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• 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.

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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.

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• 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.

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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.

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• 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.

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CONCLUSION
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• 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.