(Febrile torticollis of the child)

Non-traumatic rotatory atlantoaxial subluxation due to acquired laxity of the cervical ligaments resulting from an infection of the adjacent structures (e.g. pharyngeal abscess). Has also been observed following ENT or maxillofacial surgery, or local arthritis. A few cases of C2-3 joint involvement have been reported.

Signs: painful torticollis  with contralateral sternocleidomastoid muscle spasm .

Risk of neurological complications ranging from paresthesia to spinal cord compression with quadriplegia. The risk is increased in case of preexisting ligament hyperlaxity: trisomy 21, Marfan syndrome.

Diagnosis: anteroposterior Xray, CT or MRI.  Atlas-axis  distance greater than  4.5 mm in children

Fielding's classification:

-        type I: rotation of the atlas on the axis without increased the odonto-atloid interval

-        type II: rotation of the atlas on the axis with anterior subluxation of the atlas (distance of 3 to 5 mm between the atlas and the odontoid process )

-        type III: rotation of the atlas on the axis with anterior subluxation of the atlas (greater than 5 mm distance between the atlas and the odontoid process)

-        type IV: rotation of the atlas on the axis with posterior subluxation of the atlas

Treatment: immobilization with a neck brace in moderate cases (Fielding type I or II), cervical traction or arthrodesis in severe cases (Fielding type III or IV).

Anesthetic implications: 

-         to be kept in mind in case of a postoperative painful stiff neck (especially after  ENT or maxillofacial surgery) or in the context on ENT infection 

-         avoid excessive hyperextension of the neck or excessive rotation of the neck under G.A.

-        risk of spinal compression at intubation: maintain the cervical spine in line with a light linear traction; use  videolaryngoscopy or fibroscopy

-         in case of retropharyngeal abscess, intubation is delicate (risk of rupture) and frequently difficult.

References : 

-         Bocciolini C, Dall’Olio D, Cunsolo E, Cavazutti PP, Laudadio P. 
Grisel’s syndrome: a rare complication following adenoidectomy. 
Acta Otorhinolaryngol Ital 2005; 25: 245-9.

-         Martinez-Lage JF, Morales T, Fernandez-Cornejo V. 
Inflammatory C2-3 subluxation : a Grisel’s syndrome variant. 
Arch Dis Child 2003 ; 88 : 628-9.

-        Yu KK, Weissler MC, Pillsbury HC. 
Nontraumatic atlantoaxial subluxation (Grisel syndrome) : a rare complication of otolaryngological procedures. 
Laryngoscope 2003 ; 113 : 1047-9.

-         Dasen KR. 
Atlantoaxial rotatory subluxation after a pediatric tonsillectomy. 
Anesth Analg 1999 ; 89 : 917-9.

-         Casey ATH, O’Brien M, Kumar V, Hayward RD, Crockard HA. 
Don’t twist my child’s head off: iatrogenic cervical dislocation. 
BMJ 1995; 311: 1212-3. 

-        Stilwell PA, Fine D, Roberts J, Goh L.
A pain in the neck: Grisel’s syndrome.
Arch Dis Child 2019; 106: 610

-        Cucuzza ME, D’Ambra A, Evola FR, Greco F, Smilari P.
Grisel syndrome, sigmoid sinus thrombosis and rheumatic carditis: case report of a rare association.
Int J Pediatr Otorhinolaryngol 2020; 19: in press

-        Reddy KN, Baig SM, Batra M, Colodner K, Madubuko U, Korban A, Shah SP.
Grisel’s syndrome : a case report on this rare pediatric disease and its anesthetic challenges.
BMC Anesthesiology 2020 ; 20 : 255. 20:255. doi./10.1186/s12871-020-01176-7

Updated: October 2020