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 catarycomparing 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