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