Torticollis with Atlantoaxial Rotatory Subluxation in Children: A Clinical Review

 

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Abstract

A small proportion of children with a sudden onset torticollis (“wry neck”) presents with an atlantoaxial rotatory subluxation, usually after mild trauma or recent head or neck infection. Torticollis is a clinical diagnosis and imaging is usually not indicated, though often performed in clinical practice. Atlantoaxial rotatory subluxation on imaging is often a physiological phenomenon in torticollis, and concomitant neurological symptoms are therefore rare. Treatment is primarily conservative, with analgesics, a rigid neck collar, and if needed benzodiazepines to counteract muscle spasms and anxiety. In case of treatment failure or chronic subluxation, cervical repositioning and fixation under general anesthesia may be considered. Surgical treatment is only indicated in a small percentage of patients with chronic refractory subluxation, concomitant cervical fractures, or congenital anomalies. Early diagnosis and treatment are important, since this is associated with a more successful conservative outcome than a prolonged approach.

Disclosures

This article is largely based on a previous published Dutch article in the Dutch magazine “Tijdschrift voor Neurologie en Neurochirurgie.”[19]

Publication History

Received: 02 December 2023

Accepted: 21 April 2024

Accepted Manuscript online:
24 April 2024

Article published online:
15 May 2024

© 2024. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

• References

• 1 Goel A. Torticollis and rotatory atlantoaxial dislocation: a clinical review. J Craniovertebr Junction Spine 2019; 10 (02) 77-87
  CrossrefPubMedGoogle Scholar
• 2 Pang D, Li V. Atlantoaxial rotatory fixation: part 1–biomechanics of normal rotation at the atlantoaxial joint in children. Neurosurgery 2004; 55 (03) 614-625 , discussion 625–626
  CrossrefPubMedGoogle Scholar
• 3 Kinon MD, Nasser R, Nakhla J. et al. Atlantoaxial rotatory subluxation: a review for the pediatric emergency physician. Pediatr Emerg Care 2016; 32 (10) 710-716
  CrossrefPubMedGoogle Scholar
• 4 Roche CJ, O'Malley M, Dorgan JC, Carty HM. A pictorial review of atlanto-axial rotatory fixation: key points for the radiologist. Clin Radiol 2001; 56 (12) 947-958
  CrossrefPubMedGoogle Scholar
• 5 Neal KM, Mohamed AS. Atlantoaxial rotatory subluxation in children. J Am Acad Orthop Surg 2015; 23 (06) 382-392
  CrossrefPubMedGoogle Scholar
• 6 Tubbs RS, Hallock JD, Radcliff V. et al. Ligaments of the craniocervical junction. J Neurosurg Spine 2011; 14 (06) 697-709
  CrossrefPubMedGoogle Scholar
• 7 Mathern GW, Batzdorf U. Grisel's syndrome. Cervical spine clinical, pathologic, and neurologic manifestations. Clin Orthop Relat Res 1989; (244) 131-146
  PubMedGoogle Scholar
• 8 Kawabe N, Hirotani H, Tanaka O. Pathomechanism of atlantoaxial rotatory fixation in children. J Pediatr Orthop 1989; 9 (05) 569-574
  CrossrefPubMedGoogle Scholar
• 9 Fielding JW, Hawkins RJ. Atlanto-axial rotatory fixation. (Fixed rotatory subluxation of the atlanto-axial joint). J Bone Joint Surg Am 1977; 59 (01) 37-44
  CrossrefPubMedGoogle Scholar
• 10 Grisel P. Enucléation de l'atlas et torticollis naso-pharyngien. Presse Med 1930; 38: 50-54
  PubMedGoogle Scholar
• 11 Parke WW, Rothman RH, Brown MD. The pharyngovertebral veins: an anatomical rationale for Grisel's syndrome. J Bone Joint Surg Am 1984; 66 (04) 568-574
  CrossrefPubMedGoogle Scholar
• 12 Samartzis D, Shen FH, Herman J, Mardjetko SM. Atlantoaxial rotatory fixation in the setting of associated congenital malformations: a modified classification system. Spine 2010; 35 (04) E119-E127
  CrossrefPubMedGoogle Scholar
• 13 Wenger KJ, Hattingen E, Porto L. Magnetic resonance imaging as the primary imaging modality in children presenting with inflammatory nontraumatic atlantoaxial rotatory subluxation. Children (Basel) 2021; 8 (05) 329
  PubMedGoogle Scholar
• 14 Chechik O, Wientroub S, Danino B, Lebel DE, Ovadia D. Successful conservative treatment for neglected rotatory atlantoaxial dislocation. J Pediatr Orthop 2013; 33 (04) 389-392
  CrossrefPubMedGoogle Scholar
• 15 Pang D, Li V. Atlantoaxial rotatory fixation: part 3-a prospective study of the clinical manifestation, diagnosis, management, and outcome of children with alantoaxial rotatory fixation. Neurosurgery 2005; 57 (05) 954-972 , discussion 954–972
  CrossrefPubMedGoogle Scholar
• 16 Subach BR, McLaughlin MR, Albright AL, Pollack IF. Current management of pediatric atlantoaxial rotatory subluxation. Spine 1998; 23 (20) 2174-2179
  CrossrefPubMedGoogle Scholar
• 17 Shimazaki T, Yamada K, Sato K. et al. Primary treatment of atlantoaxial rotatory fixation in children: a multicenter, retrospective series of 125 cases. J Neurosurg Spine 2020; 34 (03) 498-505
  CrossrefPubMedGoogle Scholar
• 18 Spinnato P, Zarantonello P, Guerri S. et al. Atlantoaxial rotatory subluxation/fixation and Grisel's syndrome in children: clinical and radiological prognostic factors. Eur J Pediatr 2021; 180 (02) 441-447
  CrossrefPubMedGoogle Scholar
• 19 Kempeneers MA, Buis DR, Feller RE, Roosendaal SD, Slot KM, Wolf NI, Vandertop WP. Torticollis met atlanto-axiale subluxatie bij kinderen. Tijdschr Neurolo Neurochir 2023; 124 (02) 60-67
  PubMedGoogle Scholar
• 20 Greenberg MS. Functional neurosurgery. In: Handbook of Neurosurgery, 8th ed. New York, Verenigde Staten: Thieme; 2016
  Google Scholar
• 21 Larsen K. Atlas joint instability: causes, consequences and solutions. 2017 . Accessed April 26, 2024 at: https://mskneurologycom/atlas-joint-instability-causes-consequences-solutions/
  PubMedGoogle Scholar