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Grisel Syndrome
Nontraumatic Atlantoaxila Rotatory Subluxation:
Grisel Syndrome. Case Report and Literature Review
Alecio C. E. S. Barcelos Gustavo C. Pateriota Arlindo Ugulino Netto
Introduction
• Notraumatic rotatory atlantoaxial subluxation
first describesd in 1830 by Charles bell, in
syphillis
• Defines by Grisel in 1950
• Grisel syndrome: atlantoaxial rotatory
subluxation, not triggered by trauma, affecting
Pt with hx of H&N infection
• Rare
• 68% < 12 years
• 90% < 21 years
Case Report
• 7 years old. Boy
• Torticollis, halitosis, cough, odynophagia for 1/52
• No fever
• PE:
– posture in right-sided head rotation
– Subtle flexion
– Ipsilateral SCM spasm
– Nuchal pain
– Hypertrophied tonsils, no abcess
– Normal Neuro exam
• Lab: N crp and WBC
• CT:
– Lt. atlantoaxial facet ant. Dislocation
– ADI normal
• Tx:
– Soft collar
– Analgesia,muscle relaxant,Abx.( ceftriaxone for 14
days)
– Partial relaxation occurred after 24 hrs.
– Larger collar to reduce the flexion
– Clinical reduction after 48 hrs. then Hard collar
– Improved odynophagia
– Reduction was confirmed by radiology.
– MRI| done 21 days later
• Odontoid was slightly shifted to Rt.
• Fluids in C1 C2 facet joints.
• ADI was Normal
• Pt was discharged after 3/52
• He wore hard collar for 6/52
• Return to daily activity including sports
gradually
Discussion
• Grisel syndrome happens after head infection or
otolaryngology procedures
• More in Peds.
– Weak ligaments and joints capsules ( down, marfan)
– Horizontal joint line
– Small supporting muscles.
– Kawasaki Disease is found related to Grisel s.
– More post adenectomy after monopolar catary
comparing to bipolar coagulation.
• KarKos et al :
– Systemic review 71 articles
– 96 Pt with C1 C2 subluxation
– All no hx. of trauma
– 48% had recent URTI
– 40% post neck procedures
– Clinical picture: torticollis,neck pain maily in nape
– Signs:
– Palpable deviation spinous process of C2
– SCM sppasm
– Inability to turn head beyond midline
Pathphysiology
• Unknown
• Grisel emphasize the role of muscle spasm
• Tedesco et al
– Cervical lympadenitis induce irritative spasm of
suboccipital and parvertebral muscles.
• Most accepted theory is by Parke et al.
– Studied vascularization of c spine, cranial base and
peripharyngeal
– Posterior superior veinous drainage is connected to
periodontoidal plexus via pharyngovertebral veins
– This transport septic oxidates
• C1 C2 subluxation can be seen in x-ray
– Asymmetry, increased in lateral mass of C1
– Difficult to obtain optimal view
• Gold standard is CT
• Fundamental issue in diagnosis is hx. Of URTI
• Dx after 3/52 is related to failure of
conservative Tx. Including closed reduction.
And recurrence and permanent deformity.
diagnosis
• Hx.
• Torticollis after few days fron URTI onset
• Anomalous rotation and mild flexion
• Pain with active or passive rotation
• Elevated CRP & WBC in first days.
• Xray:
• CT
Treatment
• Controversial
• Initially conservative
• Waiting period of spontaneous reduction is
not established. However most reduced after
7 days.
• Authors recommends hard collar for 2/52 to
avoi recurrence
• If susequent reduction did not happen, do
closed reduction
• Traction in slight flexion followed by extension
and reverse rotation deformity.
• Jeszenszky manuver
• Wetzel and La Rocca proposed Tx based on
Feilding and Hawkin Classification
• Surgery in failed closed Tx. Or recurrence
• Craniocervical fusion
– In children < 6 years????
– Menezes recommends small craniotomy and upper cervical
laminectomy with interlaminar and occipital rib graft
reinforced by titanium cables.
• > 6 years : screw
fixation
Grisel syndrome

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Grisel syndrome

  • 1. Grisel Syndrome Nontraumatic Atlantoaxila Rotatory Subluxation: Grisel Syndrome. Case Report and Literature Review Alecio C. E. S. Barcelos Gustavo C. Pateriota Arlindo Ugulino Netto
  • 2. Introduction • Notraumatic rotatory atlantoaxial subluxation first describesd in 1830 by Charles bell, in syphillis • Defines by Grisel in 1950
  • 3. • Grisel syndrome: atlantoaxial rotatory subluxation, not triggered by trauma, affecting Pt with hx of H&N infection • Rare • 68% < 12 years • 90% < 21 years
  • 4. Case Report • 7 years old. Boy • Torticollis, halitosis, cough, odynophagia for 1/52 • No fever • PE: – posture in right-sided head rotation – Subtle flexion – Ipsilateral SCM spasm – Nuchal pain – Hypertrophied tonsils, no abcess – Normal Neuro exam • Lab: N crp and WBC
  • 5. • CT: – Lt. atlantoaxial facet ant. Dislocation – ADI normal • Tx: – Soft collar – Analgesia,muscle relaxant,Abx.( ceftriaxone for 14 days) – Partial relaxation occurred after 24 hrs. – Larger collar to reduce the flexion – Clinical reduction after 48 hrs. then Hard collar – Improved odynophagia – Reduction was confirmed by radiology.
  • 6. – MRI| done 21 days later • Odontoid was slightly shifted to Rt. • Fluids in C1 C2 facet joints. • ADI was Normal • Pt was discharged after 3/52 • He wore hard collar for 6/52 • Return to daily activity including sports gradually
  • 7. Discussion • Grisel syndrome happens after head infection or otolaryngology procedures • More in Peds. – Weak ligaments and joints capsules ( down, marfan) – Horizontal joint line – Small supporting muscles. – Kawasaki Disease is found related to Grisel s. – More post adenectomy after monopolar catary comparing to bipolar coagulation.
  • 8. • KarKos et al : – Systemic review 71 articles – 96 Pt with C1 C2 subluxation – All no hx. of trauma – 48% had recent URTI – 40% post neck procedures – Clinical picture: torticollis,neck pain maily in nape – Signs: – Palpable deviation spinous process of C2 – SCM sppasm – Inability to turn head beyond midline
  • 9. Pathphysiology • Unknown • Grisel emphasize the role of muscle spasm • Tedesco et al – Cervical lympadenitis induce irritative spasm of suboccipital and parvertebral muscles. • Most accepted theory is by Parke et al. – Studied vascularization of c spine, cranial base and peripharyngeal – Posterior superior veinous drainage is connected to periodontoidal plexus via pharyngovertebral veins – This transport septic oxidates
  • 10. • C1 C2 subluxation can be seen in x-ray – Asymmetry, increased in lateral mass of C1 – Difficult to obtain optimal view • Gold standard is CT
  • 11. • Fundamental issue in diagnosis is hx. Of URTI • Dx after 3/52 is related to failure of conservative Tx. Including closed reduction. And recurrence and permanent deformity.
  • 12. diagnosis • Hx. • Torticollis after few days fron URTI onset • Anomalous rotation and mild flexion • Pain with active or passive rotation • Elevated CRP & WBC in first days. • Xray: • CT
  • 13. Treatment • Controversial • Initially conservative • Waiting period of spontaneous reduction is not established. However most reduced after 7 days. • Authors recommends hard collar for 2/52 to avoi recurrence
  • 14. • If susequent reduction did not happen, do closed reduction • Traction in slight flexion followed by extension and reverse rotation deformity. • Jeszenszky manuver • Wetzel and La Rocca proposed Tx based on Feilding and Hawkin Classification • Surgery in failed closed Tx. Or recurrence
  • 15. • Craniocervical fusion – In children < 6 years???? – Menezes recommends small craniotomy and upper cervical laminectomy with interlaminar and occipital rib graft reinforced by titanium cables. • > 6 years : screw fixation