Volume 2, Issue 1 , Pages 20-22, March 2007
Atlantoaxial subluxation mimicking neck mass
Article Outline
Summary
Non-traumatic atlantoaxial subluxation (AAS) is a rare complication of upper respiratory system infection or head and neck surgery. It is called Grisel's syndrome named after P. Grisel, who first described this condition. AAS without an underlying cause is an extremely rare entiity. We present a case of AAS mimicking anterior in the neck without an underlying cause.
Keywords: Atlantoaxial subluxation, Neck mass, Grisel's syndrome
1. Introduction
Grisel's syndrome is a condition that occurs almost exclusively in children and has been associated with upper cervical infections and otolaryngologic procedures first described by Grisel [1]. AAS without the presence of Grisel's syndrome is even rarer. To our knowledge there have been no reported cases of idiopathic AAS in the medical literature. We present the case of a 11-year-old boy with a neck mass turned out to be a spontaneous AAS.
2. Case report
A 11-year-old boy was referred for evaluation of a left neck mass. He has been using oral antibiotics for a month and there was no improvement in the size of the mass. The mass was anterior to the left sternocleiodomastoid muscle at the level 2 which sizes 3
cm×2cm. It was not tender and had no fluctuation with palpation. His head position was turning towards right side and chin was tilted. The patients head movements were painfull and limited. The remaining physical examination was normal. There was a negative history for adenotonsillectomy or any kind of operations, trauma and infection. In the interim a fine needle aspiration of the mass was performed and a neck CT was ordered. Fine needle aspiration was reported normal, CT revealed a torticollis secondary to AAS while other neck structures were normal (Fig. 1, Fig. 2). At this point the orthopedical service was consulted, reduction of the subluxation by cervical traction and placement of a hard cervical collar was performed and the subluxation was well reduced.
Fig. 1.
Asymmetry of the dens of axis (indicated by arrow).
Fig. 2.
Overlapping of the condylary process (indicated by arrow).
3. Discussion
Grisel's syndrome represents a subluxation of the atlantoaxial joint not associated with trauma or bone diseases and is primarily a disorder of childhood. Altough Grisel postulated that AAS was caused by muscle spasm; at the moment muscle spasm is thought to be a reaction to adjacent inflammation and the role of this muscle spasm is thought to be limited to maintenance of the subluxed position but is not causative [1]. Watson-Jones and Roberts [2] suggested that resorption of the attachment of the transverse ligament to the atlas mediated by inflammation could lead to AAS. Parke et al. [3] provided an anatomic explanation by demonstrating the relationship of the retropharyngeal space to the atlantoaxial space via a previously undescribed pathway, the pharyngovertebral veins thought to be caused by the spread of septic exudates through venous and lymphatic channels that connect the peripharyngeal space and the cervical venous plexus. These veins were also found to have frequent anastamoses with the lymphatics of the retropharyngeal space. Nasopharyngeal inflammation causes hyperaemia that may weaken the transverse and alar ligaments and the articular capsules resulting in atlantoaxial instability [3].
Subluxation of the atlantoaxial joint is graded using the classification system developed by Fielding et al. [4]. All of the Fielding types have rotary subluxation in common and are differentiated by the degree and direction of atlas displacement. Type I=<3mm anterior displacement of the atlas with intact transverse and lateral ligaments, type II=3–5mm anterior displacement with disruption of the transverse ligament, type III=>5mm anterior displacement with disruption of both transverse and lateral ligaments and type IV=posterior displacement of the atlas.
Treatment of atlantoaxial subluxation is traditional conservative for Fielding types I and II without neurologic deficit. Fielding et al. [4] in 1978 recommended traction and immobilization for types I and II and surgical fixation for types III and IV. In a more recent review by Wietzel, the authors suggest treatment of type I with soft collar, type II with hard collar, type III with halo fixation, and type IV with open fixation [5].
The diagnosis of Grisel's syndrome is based on the association of clinical findings (head tilt with painful and restricted neck movements) with a previous history of an upper respiratory tract infections involving pharynx, tonsils and mastoid bones [6] or otolaryngologic procedures such as adenotonsillectomy [7]. Radiographs and computed tomography scans of the cervical spine establish the diagnosis. Early management – consisting of cervical immobilization, medical treatment, and physiotherapy – is considered the key factor for achieving a satisfactory outcome [7], [8].
Non-traumatic ASS is a rare condition. In order to diagnose, one must be aware of such an entity and keep in mind that an infection or a surgical trauma may proceed to Grisel's syndrome. Its presentation varies from neck stiffness to neurologic symptoms such as bladder dysfunction. The most common symptoms are neck stiffness and pain with head movements [9]. Our patient had a negative history for infection, trauma or operation and the differential diagnosis was made against neck mass and deep neck abscess. CT revealed a torticollis secondary to AAS.
A high index of suspicion in evaluating patients with a stiff neck or pain with head movements is essential to make proper diagnosis and intervention. Otolaryngologists should be aware of this rare but potentially serious entity.
References
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- . Atlantoaxial rotary deformitie. Orthop. Clin. N. Am. 1978;9(4):955–966
- . Current management of pediatric atlantoaxial subluxation. Spine. 1998;23:2174–2179
- . Nontraumatic subluxation of the atlantoaxial joint: Grisel's syndrome. Ann. Otol. Rhinol. Laryngol. 1987;96:705–708
- . Atlantoaxial subluxation (Grisel's syndrome) following otolaryngological diseases and procedures. J. Laryngol. Otol. 1995;109:1005–1009
- . Atlanto-axial rotatory subluxation in children: early management. Acta Neurochir. (Wien). 2001;143:1223–1228
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PII: S1871-4048(06)00096-7
doi:10.1016/j.pedex.2006.11.002
© 2006 Elsevier Ireland Ltd. All rights reserved.
Volume 2, Issue 1 , Pages 20-22, March 2007
