Upper airways infection and torticollis in children: thinking to Grisel

Vol 1 - No. 1 - February 2012
Bi-monthly Journal of Pediatrics - ISSN 2240-791X
Upper airways infection and torticollis in children: thinking to Grisel Sindrome
Salpietro V1, Granata F2, Briuglia S1, Sancetta F1, Alterio T1, Randazzo A1, Mallamace R1, Chirico V1, Colavita L1, Manti S1, Loddo I1, Centorrino R1, David E2,
Ruggieri M3, Mankad K4
1Department of Pediatrics, Unit og Genetic and Pediatric Immunology, University of Messina, Italy
2Department of Radiology, University of Messina, Italy
3Department of Formative Processes, University of Catania, Italy
4Great Ormond Street Hospital, London, UK
Grisel syndrome or atlantoaxial non-traumatic subluxation is a rare entity, poorly described in pediatric literature, although it is a pathology that usually appears in young children [1]
[2].
The typical presentation is a torticollis with a previous surgical antecedent (mainly a surgery of the ear, nose, and throat area like adenoidectomy) or after an upper tract respiratory
infection. A prompt diagnosis is essential for a successful evolution, and the treatment in most cases is conservative.
We report a case of an 8-year-old girl with a typical evolution of this unusual complication.
Patient C.R. was the third born to healthy Italian parents and the full term product of a normal pregnancy with normal delivery.
At birth weight, length and head circumference were within the 50th percentile.
The neonatal period was uneventful.
Family and past histories were unremarkable; there was no traumatic antecedent.
Developmental milestones were normal.
During the previous four months she had experienced repeated bouts of fever and cough.
She was first referred to the our paediatric emergency room for persistent fever and painful cervical and limb movements; she was treated with homeopathic drugs including atropa
belladonna compounds.
Three days after hospital admission the fever remitted and torticollis and unbalanced gait ensued; the painful cervical movements were still present.
Upon general examination her weight, length and head circumference were within the 50th percentile.
The remaining of her general examination was otherwise negative except for reddening of the pharynx.
Neurological examination revealed a cooperative, alert and fairly oriented girl with normal tendon reflexes, unbalanced gait, neck stiffness and restricted and painful neck
movements; there was also left torticollis.
Full ophthalmologic examination including fundoscopy was normal as were ECG, EEG and cardiac and abdominal ultrasonography.
Routine blood and urinalysis including white blood cell count, erythrocyte sedimentation rate and C-reactive protein were within normal ranges.
A computed tomography (CT) study of the head was reported as normal.
A magnetic resonance (MR) study of the head and spine revealed increased fluids within the occipito-atlanto-axial joint spaces.
Three days after the MR study of the head and spine her lower limbs tendon reflexes decreased and were unrecordable.
The child was managed concervatively for eight weeks with a soft neck collar and tapering non-steroidal anti-inflammatory agents (ibuprofen 25 mg/Kg/die for 7 days).
Over few days torticollis reverted to normal.
At her last follow-up control the general examination was unremarkable and neurological examination revealed an otherwise normal gait, tendon reflexes and muscular tone and
strength had reverted to normal.
A control MR study of the brain and spine revealed almost complete disappearance of the previous signal abnormalities.
The patient had fever and strep throat, after she developed stiff neck.
Stiff neck should be due to a mechanism “Grisel type” by contiguity of inflammation from posterior pharynx to cervical plexus chain involving atlas transverse ligament and atlo-axial
joint.
Fig. 1 MRI examination at clinical onset
Coronal High-Resolution FSE T2 weighted image (a) and Sagittal SE T2-weighted image (b) (a) A slight enlargement of joint distance between left lateral mass of C1 and C2 body
is present (red line) (a, b) Note the hyperintense intra-articular fluid collection (arrows)
Fig. 2 Follow-up MRI examination
Coronal High-Resolution FSE T2 weighted image (a) and Sagittal SE T2-weighted image (b) (a, b) Note the disappearance of the intra-articular fluid collection (arrows)
References
[1] Ortega-Evangelio G , Alcon JJ , Alvarez-Pitti J , Sebastia V , Juncos M , Lurbe E. Eponym : Grisel syndrome. Eur J Pediatr. 2011 Aug;170 (8):965-8. Epub 2011 May 24.
[2] Harth M, Mayer M, Marzi I, Vogl TJ, Lateral torticollis on plain radiographs and MRI: Grisel syndrome Eur Radiol. 2004 Sep;14 (9):1713-5. Epub 2004 Feb 14.
www.theChild.it bimestrale di divulgazione scientifica dell'Associazione Pediatrica di Immunologia e Genetica
Legge 7 marzo 2001, n. 62 - Registro della Stampa Tribunale di Messina n. 3/09 - 11 maggio 2009
Direttore scientifico Carmelo Salpietro - Direttore responsabile Giuseppe Micali - Segreteria redazione Basilia Piraino - Piera Vicchio
Direzione-redazione: UOC Genetica e Immunologia Pediatrica - AOU Policlicnico Messina