Acquired Torticollis due to Grisel's Syndrome: Case Report and Follow-Up of Non-Traumatic Atlantoaxial Rotatory Subluxation

Abstract

Non-traumatic atlantoaxial rotatory subluxation is an uncommon entity, with inconsistent presentations. It is also known as Grisel's syndrome (GS), and most commonly follows infectious processes of the upper respiratory tract. A case is described of a non-traumatic rotatory atlantoaxial dislocation in a three-year-old boy. The patient presented with acute torticollis one week after mild upper respiratory infection. Neurological evaluation and lumbar punction were normal. After five days of intractable pain and non-reducible rotational tilt of the head to the left, CT and MR imaging were performed and showed atlantoaxial rotatory subluxation. In addition, MRI demonstrated middle ear and mastoid inflammation. Tilt reduction could be obtained through gentle skull traction under sedation and relaxation for 3 days. Antibiotic treatment was performed. The patient was then placed in a Minerva cast jacket. After seven weeks of immobilization, CT demonstrated regular atlantoaxial alignment. Follow-up after 5.5 years showed the boy neurologically intact and free of clinical complaints. Dynamic cervical radiographs confirmed that the atlantoaxial joints were stable.

Key words

Grisel's syndrome - non-traumatic atlantoaxial rotatory subluxation - torticollis - inflammatory disorders

References

• 1 Bredenkamp J K, Maceri D R. Inflammatory torticollis in children.  Arch Otolaryngol Head Neck Surg. 1990;  116 310-313
• 2 Dekel B, Paret G, Vardi A, Katz M, Barzilay Z. Torticollis: an unusual presentation of spontaneous pneumomediastinum.  Pediatr Emerg Care. 1996;  12 352-353
• 3 Fielding J W, Hawkins R J. Atlanto-axial rotatory fixation.  J Bone Joint Surg (Am). 1976;  58 400-407
• 4 Grisel P. Enucléation de l'atlas et torticollis naso-pharyngien.  Presse Med. 1930;  38 50-53
• 5 Harouchi A, Padovani J P, El Andaloussi M, Refass A. Atlanto-axial dislocations in children.  Chir Pediatr. 1984;  25 136-144
• 6 Herzka A, Sponseller P D, Pyeritz R E. Atlantoaxial rotatory subluxation in patients with Marfan syndrome. A report of three cases.  Spine. 2000;  15 524-526
• 7 Hettiaratchy S, Ning C, Sabin I. Nontraumatic atlanto-occipital and atlantoaxial rotatory subluxation: case report.  Neurosurgery. 1998;  43 162-164
• 8 Hopla D M, Mazur J M, Bass R M. Cervical vertebrae subluxation.  Laryngoscope. 1983;  93 1155-1159
• 9 Isaacson G, Chan K H, Heffner Jr R R. Focal myositis. A new cause for the pediatric neck mass.  Arch Otolaryngol Head Neck Surg. 1991;  117 103-105
• 10 Kraft M, Tschopp K. Evaluation of persistent torticollis following adenoidectomy.  J Laryngol Otol. 2001;  115 669-672
• 11 Mathern G W, Batzdorf U. Grisel's syndrome. Cervical spine clinical, pathologic, and neurologic manifestations.  Clin Orthop. 1989;  244 131-146
• 12 Mezue W C, Taha Z M, Bashir E M. Fever and acquired torticollis in hospitalized children.  J Laryngol Otol. 2002;  116 280-284
• 13 Okada Y, Fukasawa N, Tomomasa T, Inoue Y, Morikawa A. Atlanto-axial subluxation (Grisel's syndrome) associated with mumps.  Pediatr Int. 2002;  44 192-194
• 14 Parke W W, Rothman R H, Brown M D. The pharyngovertebral veins: an anatomical rationale for Grisel's syndrome.  J Bone Joint Surg. 1984;  66 568-574
• 15 Samuel D, Thomas D M, Tierney P A, Patel K S. Atlanto-axial subluxation (Grisel's syndrome) following otolaryngological diseases and procedures.  J Laryngol Otol. 1995;  109 1005-1009
• 16 Schwarz N. The fate of missed atlanto-axial rotatory subluxation in children.  Arch Orthop Trauma Surg. 1998;  117 288-289
• 17 Straussberg R, Harel L, Amir J. Pseudotumor cerebri manifesting as stiff neck and torticollis.  Pediatr Neurol. 2002;  26 225-227
• 18 Welinder N R, Hoffmann P, Hakansson S. Pathogenesis of non-traumatic atlanto-axial subluxation (Grisel's syndrome).  Eur Arch Otorhinolaryngol. 1997;  254 251-254
• 19 Wetzel F T, La Rocca H. Grisel's syndrome.  Clin Orthop. 1989;  240 141-152