A 12-year-old Asian boy with left hemifacial microsomia and associated left microtia underwent a first-stage Nagata-type reconstruction of the left ear with right rib cartilage graft. Before the surgery, the patient had unremarkable range of motion in the neck with no head-and-neck pain, infection, or mass. Surgical positioning was typical for microtia reconstruction with a shoulder roll, the head prepped and draped fully, and mostly turned to the right on an Acton gel donut. Postoperatively, the patient presented on the ward with a stiff neck, with head tilted and turned to the right side, and limited voluntary mobility (Fig. 1). It was felt to be a protective response to pain and increased pain medication, and mobilization was encouraged. The stiffness persisted even after discharge home. A physiotherapist was consulted who suspected Grisel’s syndrome. A computed tomography (CT) scan revealed a C1–C2 subluxation and Grisel’s syndrome was confirmed (Fig. 2). Treatment with a course of anti-inflammatories, relaxed neck positioning, and collar use proved unsuccessful, and the patient was admitted to hospital for halter traction and splinting. Two months after the initial onset of the torticollis, the torticollis slowly resolved.
A 3-month postsurgical CT scan demonstrated some persistent dysplasia of the anterosuperior surface of the left C2 lateral mass, potentially facilitating anterior subluxation of the C1 lateral mass (Fig. 3). It was unclear if the change was a congenital asymmetry or secondarily resulted from the prolonged subluxation.
Since the resolution of the torticollis, the patient has been followed for 2 years and has resumed full activities with no recurrence. The second stage of ear reconstruction has not been undertaken.
The list of causes of acquired torticollis in children and infants is extensive: traumatic, infectious (retropharyngeal infection), neoplastic (intraspinal or posterior fossa tumors), neurologic, ocular (fourth nerve palsy head tilt), and medication-induced (antipsychotics). Although Grisel’s syndrome is most common in the pediatric otolaryngologic population, due to its potential for permanent neurologic dysfunctions, paralysis, or death,1,2 all surgeons who operate in the craniofacial region should be familiar with it to ensure timely diagnosis and management.
Grisel’s syndrome is the nontraumatic subluxation of the C1–C2 joint.3 This syndrome was first described by Sir Charles Bell in 1830 and subsequently reported by Pierre Grisel in 1930.4 A review by Karkos et al5 revealed that the most common cause of Grisel’s syndrome is infection of the head and neck, which is associated with 48% of cases in their study, followed by surgery of the head and neck, associated with 31% of the cases.5 Among the various types of infections, upper respiratory tract infections accounted for 83% of cases, followed by retropharyngeal abscess and otitis media, at 11% and 4%, respectively.5 Of the head and neck operations, adenotonsillectomy was associated with 78% of the cases, whereas pharyngoplasty and otoplasty were associated with 15% and 2.5% of the cases observed.5
To date, the pathogenesis of Grisel’s syndrome is still unclear. Normally, the transverse ligament of the atlas contributes largely to the stability of the C1–C2 joint.1 Anatomically directly adjacent to the pharynx, some feel pharyngeal inflammation may mediate a laxity of this ligament and also provide a small amount of intra-articular inflammatory fluid that may act as a lubricant for the atlantoaxial joint to more easily sublux.4 Although there is less likely to be a firm skeletal block to reduction, strong muscle spasm maintains the subluxed position.1 Currently, the most accepted theory is that the pharynx and upper respiratory tract share lymphatic drainage with the C1––C2 joint, and any inflammatory process may therefore lead to C1–C2 joint instability.1,4–6 In our noninflammatory surgical case, and the reported surgical cases related to nonpharyngeal pathology, preexisting cervical skeletal anomalies and/or aggressive rotational positioning must be considered potential causes. In our case, we did not note any difficulty achieving intraoperative positioning or operate in extreme rotation.
The diagnosis of Grisel’s syndrome is made upon mostly on clinical assessment and confirmed with radiologic imaging. Grisel’s syndrome usually presents as painful torticollis.3 Rotation of the neck is limited and passive attempts to reposition can cause pain.3 Due to the potentially serious complications of Grisel’s syndrome, early radiographic imaging and management is crucial. A transoral x-ray can be diagnostic but is not usually included in our standard c-spine views. The anteroposterior view would reveal the asymmetry between the C1–C2 facet joints, whereas the lateral view can sometimes show an increased distance in the atlantodens interval7,8 or show a relative oblique tilt to the C1 vertebral ring (Fig. 2). CT can provide 3D evidence of rotator subluxation of the C1–C2 joint.7,9
There is currently no consensus treatment for Grisel’s syndrome,4 but the first line of treatment is generally conservative, including antibiotics and anti-inflammatories. In some cases, muscle relaxants and a cervical collar may also be used.4 In patients who do not improve through nonoperative management, gentle constant traction and/or repositioning under general anesthesia and/or halo fixation may be necessary.4,10 In cases where reductions cannot be achieved in that fashion, or where subluxations recur, surgical options, including arthrodesis,2 may be considered.4
Grisel’s syndrome is a cause of acquired torticollis in children and infants and is most often encountered in otolaryngological practices. It is rare enough that most nonotolaryngologists will be unaware of the diagnosis; however, all surgeons who operate on the head and neck regions of pediatric patients may encounter such condition. Due to the potentially severe consequences that may arise without timely diagnosis and management, it is crucial for practitioners to be familiar with the diagnosis and hold a high index of suspicion to recognize, diagnose, and manage this condition.
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2. Fielding JW, Hawkins RJ, Hensinger RN, et al. Atlantoaxial rotary deformities. Orthop Clin North Am. 1978;9:955–967
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5. Karkos PD, Benton J, Leong SC, et al. Grisel’s syndrome in otolaryngology: a systematic review. Int J Pediatr Otorhinolaryngol. 2007;71:1823–1827
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8. Salpietro V, Polizzi A, Granata F, et al. Upper respiratory tract infection and torticollis in children: differential diagnosis of Grisel’s syndrome. Clin Neuroradiol. 2012;22:351–353
9. Pilge H, Prodinger PM, Bürklein D, et al. Nontraumatic subluxation of the atlanto-axial joint as rare form of acquired torticollis: diagnosis and clinical features of the Grisel’s syndrome. Spine (Phila Pa 1976). 2011;36:E747–E751
10. Deichmueller CM, Welkoborsky HJ. Grisel’s syndrome—A rare complication following “small” operations and infections in the ENT region. Eur Arch Otorhinolaryngol. 2010;267:1467–1473