Skip to main content

Atlanto-axial rotatory fixation: a serious potential complication of paediatric ENT surgery that requires prompt diagnosis and treatment

  • G James (a1) and D N P Thompson (a1)
Abstract Background:

Atlanto-axial rotatory fixation is a persistent deformity of the C1–2 vertebral relationship caused by subluxation of the articular surfaces, and can occur after positioning for ENT procedures where the head is rotated – for example to access the ear or posterior triangle of the neck. If promptly recognised, it can usually be managed successfully with conservative methods, without long-lasting sequelae, but delayed or inappropriate management may lead to permanent neck deformity, neurological problems and pain.


Case review.

Case report:

Two children with atlanto-axial rotatory fixation following ENT surgery; one child was referred early and managed successfully, and one had delayed referral resulting in permanent severe positional deformity.


Atlanto-axial rotatory fixation is easily missed; there are significant clinical and medicolegal implications if it is not promptly recognised. A suggested management algorithm is presented.

Corresponding author
Address for correspondence: Mr Greg James, Department of Neurosurgery, Great Ormond Street Hospital for Children, London WC1N 3JH, UK Fax: +44 (0)20 7813 8244 E-mail:
Hide All
1 Fielding JW, Hawkins RJ. Atlanto-axial rotatory fixation. (Fixed rotatory subluxation of the atlanto-axial joint). J Bone Joint Surg Am 1977;59:3744
2 Park SW, Cho KH, Shin YS, Kim SH, Ahn YH, Cho KG et al. Successful reduction for a pediatric chronic atlantoaxial rotatory fixation (Grisel syndrome) with long-term halter traction: case report. Spine 2005;30:E444–9
3 Deichmueller CM, Welkoborsky HJ. Grisel's syndrome--a rare complication following ‘small’ operations and infections in the ENT region. Eur Arch Otorhinolaryngol 2010;267:1467–73
4 Casey AT, O'Brien M, Kumar V, Hayward RD, Crockard HA. Don't twist my child's head off: iatrogenic cervical dislocation. Br Med J 1995;311:1212–13
5 Kim B, Iwata K, Sugimoto K, Suzuki S, Ema Y, Tsunobuchi H et al. Significance of prevention and early treatment of postoperative twisted neck: atlantoaxial subluxation after head and neck surgery. J Anesth 2010;24:598602
6 Pang D. Atlantoaxial rotatory fixation. Neurosurgery 2010;66(3 suppl):161–83
7 Tauchi R, Imagama S, Ito Z, Ando K, Muramoto A, Matsui H et al. Atlantoaxial rotatory fixation in a child after bilateral otoplastic surgery. Eur J Orthop Surg Traumatol 2014;30(suppl 1):S289–92
8 Martinez-Lage JF, Perez MM, Cornejo VF, Poza M. Atlanto-axial rotatory subluxation in children: early management. Acta Neurochir (Wien) 2001;143:1223–8
9 Schwarz NN. The fate of missed atlanto-axial rotatory subluxation in children. Arch Orthop Trauma Surg 1998;117:288–9
10 Glotzbecker MP, Wasser AM, Hresko MT, Karlin LI, Emans JB, Hedequist DJ. Efficacy of nonfusion treatment for subacute and chronic atlanto-axial rotatory fixation in children. J Pediatr Orthop 2014;34:490–5
11 Kawabe N, Hirotani H, Tanaka O. Pathomechanism of atlantoaxial rotatory fixation in children. J Pediatr Orthop 1989;9:569–74
12 Crossman JE, Thompson DN, Hayward RD, Ransford AO, Crockard HA. Recurrent atlantoaxial rotatory fixation in children: a rare complication of a rare condition: report of four cases. J Neurosurg 2004;100(3 Suppl Spine):307–11
13 Lee SC, Lui TN, Lee ST. Atlantoaxial rotatory subluxation in skeletally immature patients. Br J Neurosurg 2002;16:154–7
14 Haque S, Bilal Shafi BB, Kaleem M. Imaging of torticollis in children. Radiographics 2012;32:557–71
15 Beier AD, Vachhrajani S, Bayerl SH, Diaz Aguilar CY, Lamberti-Pasculli M, Drake JM. Rotatory subluxation: experience from the Hospital for Sick Children. J Neurosurg Pediatrics 2012;9:144–8
Recommend this journal

Email your librarian or administrator to recommend adding this journal to your organisation's collection.

The Journal of Laryngology & Otology
  • ISSN: 0022-2151
  • EISSN: 1748-5460
  • URL: /core/journals/journal-of-laryngology-and-otology
Please enter your name
Please enter a valid email address
Who would you like to send this to? *



Altmetric attention score

Full text views

Total number of HTML views: 5
Total number of PDF views: 38 *
Loading metrics...

Abstract views

Total abstract views: 207 *
Loading metrics...

* Views captured on Cambridge Core between 25th September 2017 - 19th February 2018. This data will be updated every 24 hours.