International Journal of Pediatric Otorhinolaryngology (2005) 69, 1689—1692

www.elsevier.com/locate/ijporl

CASE REPORT

Grisel’s syndrome: A case report andreview of the literature

Chad Galer a,*, Eric Holbrook b, John Treves c, Donald Leopold a

aDepartment of Otolaryngology, University of Nebraska Medical Center,981225 Nebraska Medical Center, Omaha, NE 68198-1225, USAbMassachusetts Eye and Ear Infirmary, Department of Otolaryngology,981225 Nebraska Medical Center, Omaha, NE 68198-1225, USAc Section of Neurosurgery, University of Nebraska Medical Center,981225 Nebraska Medical Center, Omaha, NE 68198-1225, USA

Received 14 December 2004; accepted 27 March 2005

KEYWORDSAtlantoaxialsubluxation;Grisel’s syndrome;Potential sequelae

* Corresponding author. Tel.: +1 402fax: +1 402 559 8940.

E-mail address: cgaler@unmc.edu

0165-5876/$ — see front matter # 20doi:10.1016/j.ijporl.2005.03.046

Summary Grisel’s syndrome is non-traumatic atlantoaxial subluxation (AAS) sec-ondary to an inflammatory process in the upper neck. It is a rare condition that occursalmost exclusively in children and has been associated with upper cervical infectionsand otolaryngologic procedures. A case of AAS secondary to an upper cervicalinfection is presented. Potential sequelae can be severe; early diagnosis and treat-ment of Grisel’s syndrome can prevent tragic outcome.# 2005 Elsevier Ireland Ltd. All rights reserved.

1. Introduction

Grisel’s syndrome is non-traumatic atlantoaxial sub-luxation (AAS) secondary to an inflammatory processin the upper neck. It was first reported by Bell in1830, but is named after Grisel who published ontwo cases in 1930 [1]. Grisel’s is a rare condition thatoccurs almost exclusively in children and has beenassociated with upper cervical infections and oto-laryngologic procedures. Few cases of this raresyndrome have been reported in the last 35 years.We present the case of a 3-year old girl with Grisel’ssyndrome caused by cervical infection.

559 8007;

(C. Galer).

05 Elsevier Ireland Ltd. All rig

2. Case report

A 3-year old girl was referred for evaluation of sorethroat, neck pain, and fever. She had been seen inthe Emergency Department 3 days previously, wasdiagnosed with tonsillitis and started on a course oforal antibiotics. In the interim, she continued tohave fevers and sore throat. Her past medical his-tory was non-contributory. At physical examination,the patient was noted to hold her head slightlyrotated and bent to the left. Computed tomographyof the neck with contrast was obtained whichrevealed a 2.1 cm � 1.1 cm � 3 cm low density fluidcollection in the prevertebral space superior andposterior to the left palatine tonsil (Fig. 1). Due tothe location of the lesion and the patients goodoverall status, we elected to treat conservatively

hts reserved.

1690 C. Galer et al.

Fig. 1 CT scan showing a 2.1 cm � 1.1 cm � 3 cm lowdensity fluid collection in the prevertebral space. Nosubluxation is evident.

Fig. 2 CTscan obtained 3 days later shows persistence ofthe fluid collection. Rotary subluxation is demonstrated bythe orientation of the bodies of C1 (A) and C2 (B) and theeccentric position of the odontoid process in relation tothe C1 arch (A).

with intravenous antibiotics. A repeat CTscan of theneck was obtained on hospital day 3 and showedpersistence of the fluid collection as well as a C1-C2atlantoaxial subluxation (Fig. 2). At this point, theneurosurgical service was consulted. The patientwas taken to the operating room for peroral needledrainage of the fluid collection and reduction of thesubluxation by cervical traction and placement of ahard cervical collar. Attempts to aspirate the col-lection were unsuccessful. The subluxation was wellreduced and the child was sent home on the sub-sequent day with cervical collar and 4 weeks ofintravenous antibiotics. Repeat CT scan one monthlater revealed normal alignment of the cervicalvertebrae as well as resolution of the fluid collection(Fig. 3).

3. Discussion

The exact etiology of Grisel’s syndrome is contro-versial. It is generally accepted that the process isinitiated by an infection in the upper cervical regionwhich creates an inflammatory milieu. Grisel pos-tulated that AAS was caused by muscle spasm; nowmuscle spasm is thought to be a reaction to adjacentinflammation and the role of this muscle spasm islimited to maintenance of the subluxed position butis not causative [1,2]. Hyperemia of the blood supplyof bone has been shown to cause resorption. Wat-son-Jones suggested that resorption of the attach-

ment of the transverse ligament to the atlasmediated by inflammation could lead to AAS. In acase report by Watson-Jones and Roberts, a patientwith Grisel’s syndrome was found to have a decal-cified anterior arch of the atlas, which reappearedafter reduction and treatment of the inciting infec-tion [3]. However, this was not substantiated in ameta-analysis of post-mortem specimens and roent-genograms of patients with Grisel’s syndrome byPandya [4]. Wittek suggested that the etiologymay be due to distension of the ligaments withmaintenance of the bony-ligamentous attachment[5]. This is supported by experiments in rabbit kneejoints that have shown that a sterile inflammatoryenvironment causes marked weakness and laxity ofthe surrounding ligaments [6]. More recently, Welin-der et al. [7] provided radiologic evidence for this

Report and review on Grisel’s syndrome 1691

Fig. 4 Fielding classification of atlantoaxial subluxa-tion. (A) Type I = <3 mm anterior displacement of theatlas. (B) Type II = 3—5 mm anterior displacement. (C)Type III = >5 mm anterior displacement. (D) Type IV = pos-terior displacement of the atlas (after Fielding et al. [9]with permission).

Fig. 3 Axial CT scan with contrast obtained after treat-ment. Note resolution of abscess and normal anatomicrelationship of C1-C2.

phenomenon. They followed a Grisel’s patient withserial CTscans and observed distension of the trans-verse ligament with maintenance of the integrity ofits attachments. Parke et al. [8] provided an ana-tomic explanation for both theories by demonstrat-ing the relationship of the retropharyngeal space tothe atlantoaxial space via a previously undescribedpathway, the pharyngovertebral veins. Interest-ingly, these veins were also found to have frequentanastamoses with the lymphatics of the retrophar-yngeal space.

Subluxation of the atlantoaxial joint is gradedusing the classification system developed by Fieldinget al. [9] (Fig. 4). All of the Fielding types have rotarysubluxation in common and are differentiated by thedegree and direction of atlas displacement. TypeI = <3 mm anterior displacement of the atlas withintact transverse and lateral ligaments, type II = 3—5 mm anterior displacement with disruption of thetransverse ligament, type III = >5 mm anterior dis-placement with disruption of both transverse andlateral ligaments and type IV = posterior displace-ment of the atlas. Kraft and Tschopp [10] proposedtheadditionof type0 to this classification forpatientswith no subluxation but who have persistent torti-collis and may be expected to develop Grisels syn-drome. Early subluxation (Fielding types I and II) istypically not associated with neurologic impairment;Fielding types III and IV lesions have been reported to

have a 15% incidence of neurologic impairment ran-ging from radiculopathy to paralysis and death [11].

Treatment of atlantoaxial subluxation is tradi-tionally conservative for Fielding types I and II with-out neurologic deficit. Fielding and Hawkings [9] in1978 recommended traction and immobilization fortypes I and II and surgical fixation for types III and IV.In a more recent review by Wietzel, the authorssuggest treatment of type I with soft collar, type IIwith hard collar, type III with halo fixation, and typeIV with open fixation [12]. All studies agree that anypatient with neurologic symptoms should undergoimmediate open fixation. A study of 20 patients withAAS performed at the Children’s Hospital of Pitts-burgh showed a 70% success rate for conservativemanagement with hard cervical collars with or with-out traction [13]. Also of note, there was a lowersuccess rate noted for patients who had AAS forlonger than 3 weeks, which agrees with previouslypublished studies.

Multiple investigators have suggested that Grisel’ssyndrome is a gradually progressive process. Mezueet al. [13] studied 13 patients who presented withfever and torticollis and found that many had rota-tional deformity but did not develop AAS if treatedearly with intravenous antibiotics and benzodiaze-penes. Kraft and Tschopp incidentally found thatsubluxation was progressive in a patient with latediagnosis and treatment. Furthermore, the incidence

1692 C. Galer et al.

of types I and II subluxation reported in modernliterature contrasts sharply with the reports of fataloutcomes often seen in early literature on the topic.This is likely due to the ready availability of effectiveantibiotics as well as the earlier recognition of thedisease. Good outcomes for patients with Grisel’ssyndrome are linked to early recognition of the dis-ease, promptmedicalmanagement of the underlyinginflammatory process, and immediate treatment ofthe AAS with immobilization or fixation as indicated.Grisel’s syndrome often presents as fever and torti-collis in children and has been demonstrated as acomplication of adenoidectomy as well as other oto-laryngologic procedures. Otolaryngologists should beaware of this rare but potentially serious entity.

References

[1] P. Grisel, Enucleation de l’atlas et torticollis nasopharyn-gien, Presse Med. 38 (1930) 50—53.

[2] D. Samuel, D.M. Thomas, P.A. Tierney, P.S. Patel. Atlanto-axial subluxation (Grisel’s syndrome) following otolaryngo-logical diseases and procedures. J. Laryngol. Otol. 109(1995) 1005—1009.

[3] R.Watson-Jones, P.E. Roberts, Calcification, decalcification,and ossification, Br. J. Surg. 241 (1934) 461—499.

[4] S.K. Pandya, Atlanto-axial subluxation, Neurology (India) 20(1972) 13—48.

[5] A. Wittek, Ein Fall bon distensionluxation im atlantoepitro-pheal gelenke, Muchener Medizinsch Wochenschrift 55(1908) 1836—1837.

[6] R.K. Lipmann, Arthropathy due to adjacent inflammation, JBone Joint Surg. 35A (1953) 967—979.

[7] N.R. Welinder, P. Hoffman, S. Hakansson, Pathogenesis ofnon-traumatic atlanto-axial subluxation (Grisel’s syn-drome), Eur. Arch. Otorhinolaryngol. 254 (1997) 251—254.

[8] W.W. Parke, R.H. Rothman, M.D. Brown, The pharyngover-tebral veins: an anatomical rationale for Grisel’s syndrome,J Bone Joint Surg. 66A (1984) 568—574.

[9] J.W. Fielding, R.J. Hawkins, R.N. Hensinger, W.R. Francis,Atlantoaxial rotary deformitie, Orthop. Clin. N. Am. 9 (4)(1978) 955—966.

[10] M. Kraft, K. Tschopp, Evaluation of persistent torticollisfollowing adenoidectomy, J. Laryngol. Otol. 115 (2001)669—672.

[11] F.T. Wetzel, H. LaRocca, Grisel’s syndrome: a review, Clin.Orthop. Relat. Res. 240 (1989) 141—152.

[12] B.R. Subach, M.R. McLaughlin, A.L. Albright, I.F. Pollack,Current management of pediatric atlantoaxial subluxation,Spine 23 (1998) 2174—2179.

[13] W.C. Mezue, Z.M. Taha, E.M. Bashir, Fever and torticollisin hospitalized children, J. Laryngol. Otol. 116 (2002) 280—284.