Journal of Pediatric Rehabilitation Medicine: An Interdisciplinary Approach 6 (2013) 175–180 DOI 10.3233/PRM-130253 IOS Press
175
Grisel’s syndrome: An unusual cause of Torticollis Gerardo L. Ortiz∗ , Ivette Pratts and Edwardo Ramos Physical Medicine, Rehabilitation, and Sports Medicine Department, University of Puerto Rico, San Juan, Puerto Rico
Accepted 29 April 2013
Abstract. PURPOSE: The objective of this paper is to present a case series of patients with diagnosis of torticollis due to Grisel’s syndrome and, in doing so, raise awareness of an unusual condition that could be fatal. A review of the literature is presented regarding diagnosis and treatment. METHODS: Case series of three patients that were 7, 10, and 12 years old with history of tonsillitis or neck surgery consulted to the service of Rehabilitation Medicine. RESULTS: Physical examination showed a fixed head or limitation of movement to neutral position and initially a normal neurological examination. Initial cervical X-rays were not diagnostic. The final diagnosis was made by CT scan or MRI. All 3 patients were treated with anti-inflammatory medications, immobilization, and/or cervical traction or surgery. CONCLUSION: Grisel’s syndrome is a non-traumatic atlanto-axial rotatory fixation (AARF) with or without subluxation following infection or surgery in the head or neck region. This paper presents an unusual cause of torticollis that could be fatal or cause neurological injury if not recognized and treated appropriately. Keywords: Torticollis, Grisel’s syndrome, atlantoaxial rotatory fixation, atlantoaxial subluxation, children
1. Introduction Torticollis is a sign of an underlining disorder, not a diagnosis, that results in tilting of the head to one side and rotation to the other. The most common cause of torticollis is congenital muscular torticollis. There are more than 80 different causes, from which approximately 18% are non-muscular causes, ranging from diagnosis that require no specific treatment to potentially life threatening conditions. The most common non-muscular cause is Klippel-Feil. Other non muscular causes include ocular disorders (4th cranial nerve palsy), central nervous system abnormalities (tumors), obstetrical palsies (Brachial plexus injury), clavicle fracture, C1-C2 rotatory subluxation, and inflamma∗ Corresponding author: Gerardo L. Ortiz, Paseo Las Brisas, Niza street #29, San Juan, 00926, Puerto Rico. Tel.: +1 787 638 8415; Fax: +1 787 754 1478; E-mail: [email protected].
tory conditions [1]. The purpose of this paper is to discuss the presentation and diagnosis of torticollis in 3 patients and to raise awareness about an unusual cause of torticollis that could be fatal or produce neurological deficit.
2. Findings The 1st patient was a 7 year old boy with hemophilia and a history of severe tonsillitis that resolved in one week, except for residual neck pain and the inability to move his neck. He had no previous history of trauma. At physical examination his head was tilted to the right side and rotated to the left; no masses or nodes were palpable and his musculoskeletal and neurological examination was normal. Cervical X-ray (Fig. 1) was suspicious for C1-C2 subluxation and an MRI (Fig. 2) confirmed this diagnosis. Treatment consisted
c 2013 – IOS Press and the authors. All rights reserved 1874-5393/13/$27.50
176
G.L. Ortiz et al. / Grisel’s syndrome: An unusual cause of Torticollis
Fig. 1. Odontoid fracture versus C1-C2 subluxation. With such an X-ray, CT scan is recommended.
Fig. 3. Atlanto-odontoid distance 6 mm with increased C1-C2 spinous process separation.
Fig. 2. Posterior arch of C1 to odontoid process distance of 5–6 mm: C1-C2 subluxation.
Fig. 4. C1-C2 rotatory subluxation with occipitalization of the posterior arch of C1, rupture of transverse ligament.
of one week of Halter traction, anti-inflammatory medications, and immobilization with a cervical collar for six weeks. Two months post hospital discharge the patient presented with no neurological deficits. The 2nd patient was a 12 year old girl who, one week after a tonsillectomy, awoke with pain over the cervical region and was unable to move her neck. Cervical X-ray was negative and non-steroidal antiinflammatory drugs did not improve pain. The pain progressed and within 3 months the patient was unable to move her right upper extremity. She progressed to right hemiparesis within 24 hours. There was no history of trauma or falls, but a history of recurrent tonsillitis was present. On physical examination, the head tilted to the right and rotated to the left. There was neck stiffness with a maximum range of motion of 10◦ . No masses or nodes were palpable and cerebellar and
cranial nerve examinations were within normal limits. On physical examination, a manual muscle test on the right side showed the following: deltoid, 0/5; biceps, 2/5; hand grip, 2/5; iliopsoas, 4/5; quadriceps, 5/5; anterior tibial, 4/5; gastrosoleus, 4/5. Muscle tone was normal and the range of motion was passively full. A sensory examination showed increased pinprick on the right side. Deep tendon reflexes were +2 upper extremity and +3 lower extremity. The Babinski reflex was not present. Repeated X-rays showed an atlanto-odontoid distance of 6 mm (Fig. 3) with a diagnosis of C1-C2 subluxation confirmed by CT scan and MRI (Fig. 4). The treatment consisted of traction followed by fusion and Minerva jacket immobilization. The patient’s neurological symptoms resolved after surgery, with minor limitation of cervical rotation noted on the physical examination one year post surgery.
G.L. Ortiz et al. / Grisel’s syndrome: An unusual cause of Torticollis
The 3rd patient was a 10 year old girl with Attention Deficit Disorder and autism. The patient was admitted due to right periorbital cellulitis, mastoiditis, and retropharyngeal abscess that resolved with antibiotics. The patient was discharged with residual torticollis that persisted for 5 months. At physical examination, her head was tilted to the left and rotated to the right, and the child was unable to move her head passively. The neurological examination was within normal limits. The cervical X-ray was reported as negative but cervical CT scan diagnosed C1-C2 subluxation. The treatment consisted of cervical traction and immobilization with a Minerva jacket. The patient subsequently presented with recurrence of torticollis and subluxation as demonstrated by CT scan and was readmitted for fusion. The patients in all three cases had a history of neck surgery or tonsillitis, and torticollis presented within one week post infection with a position known as “cock robin”. In this position, in which the sternocleidomastoid muscle is contracted on the side opposite the deformity, the head movement is limited to a neutral or fixed position. Cervical X-rays were difficult to interpret and the diagnosis was made by CT scan or MRI in all cases. Two of the patients failed to respond to conservative treatment (rest, antiinflammatory medications, traction, and cervical collar) and ultimately required surgical fusion.
3. Discussion All three patients showed what is known as Grisel’s syndrome, a non-traumatic atlanto-axial rotatory fixation (AARF), with or without subluxation, following infection or surgery in the head or neck region, most commonly tonsillectomy. Aëtius of Amida (5th– 6th century of the Common Era) was the first to indicate the association between inflammation of the vertebra caused by retropharyngeal abscess and vertebral luxation. The syndrome was later described by Rhazes (865-625 CE) quoting from Hippocrates Of The Epidemics [2]. Other terms for torticollis in literature are: malum suboccipitale rheumaticum, distension luxation, Drehungsverrenkung, torticollis nasopharyngien, maladie de Grisel, non-traumatic subluxation, spontaneous hyperemic dislocation, inflammatory dislocation, spontaneous dislocation, dislocation nontraumatique, and le faux torticollis aigu [3,4]. The atlantoaxial joint is the most active joint in the body, reported to move at least 600 times an hour, both
177
Fig. 5. Posterior view of the atlanto-axial joint, to observe the transverse and alar ligaments. (Colours are visible in the online version of the article; http://dx.doi.org/10.3233/PRM-130253)
while awake and asleep. The normal range of cervical rotation is approximately 90◦ to either side; 50% of this motion occurs at C1-C2 [5]. The facet joints between the atlas and the axis are almost horizontal, which allows excellent rotation at the expense of bony stability. The transverse ligament running behind the odontoid prevents anterior motion of the atlas on the axis (Fig. 5). The paired alar ligaments, which run from the posterolateral tip of the odontoid to the occipital condyles, limit rotation of the atlas on the axis. The right alar ligament prevents excessive rotation to the left, and vice versa [5]. During rotation, the ipsilateral mass of C1 rotates back into the spinal canal, narrowing it, but the spinal canal is widest at this level and anatomical studies have shown that approximately 64◦ of right or left atlanto-axial rotation is required before there is sufficient narrowing to cause spinal cord compression [6]. The patient may complain of neck stiffness and pain upon attempted motion, as occurred in all of the patients in this report. Dysphagia is a frequent complaint, and the patients often experience nuchal pain radiating to the head or ears, with the head held immobile in a pathologic position [3].
4. Pathophysiology This syndrome is more commonly seen in children. Several reasons account for this: the facet joints, specifically in C1 and C2, are shallow and more horizontal; the atlanto-occipital joint is less stable; the condyles are smaller; the ligamentous attachments are more lax and easier to disrupt; the uncinate process
178
G.L. Ortiz et al. / Grisel’s syndrome: An unusual cause of Torticollis
is poorly developed, causing excessive lateral bending and rotation; and the head size disproportion present in children (short neck with minimal musculature) [7–9]. The reason that this deformity becomes fixed is not known. Postulated mechanisms include lymphadenitis producing cervical muscle spasm in the presence of ligamentous laxity, hyperemia that produces ligamentous laxity, distention or decalcification of bony attachments of ligaments, and direct infection resulting in rupture of ligaments [5]. Infection causes periligamentous inflammation, which results in distention, laxity, and, consequently, subluxation of the atlantoaxial joint. The connection between the pharingovertebral veins, the peri-odontoidal venous plexus, and the suboccipital venous sinuses may provide a route for hematogenous spread of infection and an anatomical explanation for atlanto-axial hyperemia [8–11].
5. Classification In 1977, Fielding and Hawkins classified four types of AARF, with Type I being the most common, in which the rotation of the atlas on the odontoid occurs without anterior displacement. This was done before CT was available. Some authors use the term atlantoaxial rotatory subluxation (AARS), but Fielding and Hawkins used the term fixation of the atlas on the axis, as may occur with subluxation or dislocation, or when the position of the atlas and axis are within the normal range of rotation (Table 1) [5,12]. Types II-IV compromise problems with the spinal canal that result in cord compression and neurological signs and have a poorer prognosis than Type I [5]. Based on this classification, the 1st and 3rd patients should be classified as Type II, and the 2nd patient classified as Type III, since this child had a atlanto-odontoid displacement of 6 mm by X-ray with neurological compromise.
6. Diagnosis The diagnosis of Grisel’s syndrome is difficult and often delayed. This condition should be suspected if a child presents with torticollis that fails to resolve within 5–7 days, especially if it is preceded by an upper respiratory tract infection (pharingitis, tonsillitis, otitis media, and/or viral syndrome) or surgical procedure. The condition is accompanied by sternocleidomastoid (SCM) muscle spasm to the side on which the head is rotated in attempt to reduce the deformity. The inabil-
ity to rotate the head past midline means that that the deformity is fixed. Neurological findings are usually absent, unless associated with an anterior or posterior atlanto-axial displacement. Radiological findings for these cases are difficult to interpret. Cervical X-rays are limited by the deformity and the child’s uncooperativeness due to the pain. The open mouth view is difficult to perform when the child does not cooperate, and lateral views are difficult to interpret due to torticollis. If a good lateral view is obtained, an atlas-dens interval greater than 4.5 mm (adults 2.5–3 mm, children up to 4 mm) should alert the physician of the possibility of subluxation of C1 on C2. CT scan is the test of choice. Some authors recommend a dynamic test, which is done by rotating the patient’s head to the opposite direction, showing that the atlas and the axis do not rotate independently of each other but as a unit [6–8,11,14]. Other authors recommend performing the CT scan with 3D reconstruction [4,9]. More recently, MRI is becoming very useful in the diagnosis of this condition. With this technique, the surrounding soft tissue structures can be more clearly visualized and possible spinal cord compression identified. It can also reveal occult infections in the upper respiratory airways or effusions into the atlanto-axial joint [13].
7. Treatment The treatment of atraumatic torticollis of less than one week is conservative. Management with rest, soft collar, muscle relaxants, and anti-inflammatory medications usually is enough to improve the condition. In the case of a bacterial infection, antibiotics will be needed to control the infection, as seen in the 3rd patient. If this fails to resolve, a CT scan, traction, and a rigid cervical collar can be considered. If diagnosis of atlanto-axial rotatory fixation is made after one month of the deformity occurring, then traction should be provided until the deformity is corrected. This is followed by a cervical collar for six weeks. Torticollis present for more than three months will usually require surgery [5]. As evidenced in the literature [5,7,14,15], the major predicting factor for the failure of conservative treatment was the duration of the subluxation before initial reduction was attempted. It is for this reason that neurosurgical consultation is paramount in all cases of torticollis; morbidity is significant in those cases in which
G.L. Ortiz et al. / Grisel’s syndrome: An unusual cause of Torticollis
179
Table 1 Fielding and Hawkins classification 1977 Fielding and Hawkins classification:
Type I – Rotatory fixation without anterior displacement of the atlas (most common and occurs within the normal ROM of the joint). Type II – Rotatory fixation with anterior displacement of 3–5 mm, associated with deficiency of the transverse ligament. Type III – Rotatory fixation with anterior displacement > 5 mm, associated with deficiency of the transverse and alar ligaments. Type IV – Rotatory fixation with posterior displacement of the atlas associated with deficient odontoid process (least common).
diagnosis is delayed, with possibly devastating consequences. Indications for fusion include neurological involvement, anterior displacement, failure to achieve or maintain correction, and the presence of the deformity for more than three months. It is important to mention that with the surgery (C1/C2 fusion), 50% of neck rotation is lost [5]. Delays in diagnosis and conservative treatment can result in an increased need for surgical intervention [4,5,7,8,11,14,15]. In this series, the second patient presented with right hemiparesis and the third patient had a persistent torticollis, both of three months duration, which lead to cervical fusion.
Conflict of interest Editorial support was provided by grants from the National Center for Research Resources (U54 RR 026139-01A1) and the National Institute on Minority Health and Health Disparities (8U54 MD 007587-03) from the National Institutes of Health. The authors report no conflict of interest.
References [1]
[2]
8. Conclusion Grisel’s syndrome is an unusual cause of torticollis that can be fatal or can cause neurological deficit if not recognized and treated appropriately. A conservative approach is recommended for torticollis, but if it persists more than one week, AARF should be suspected. Cervical X-ray usually is not diagnostic; MRI is currently the preferred diagnosis. Treatment depends on the timing of diagnosis. However, conservative treatment is limited in success when the diagnosis is delayed. Acknowledgments We want to thank Scott F. Nadler, D.O (R.I.P.), who encouraged us to publish this case series. The authors highly value the help of Amarilys Irizarry Hernandez, Graphic Designer of the Medical Sciences Campus of the University of Puerto Rico, in the development of the artwork.
[3] [4]
[5]
[6]
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[10]
Ballock R, Song K. The Prevalence of Neuromusculoar Causes of Torticollis in Children. Journal of Pediatric Orthopedics, 1996; (16): 500-4. Golzari SEJ, Ghabili K, Sajadi MM, Aslanabadi S. Early decription of Grisel’s syndrome Childs Nerv Syst, 2013; 29: 359-360. Wetzel F, La Rocca H. Grisel’s Syndrome: A Review. Clinical Orthopedics and Related Research, 1988; 141-150. Cekinmez M, Tufan K, Sen O, Caner H. Non-traumatic Atlanto-Axial Subluxation: Grisel’s Syndrome. Two Case Reports. Neurol Med Chir, 2009; 49(4): 172-4. Roche C, O’Malley M, Dorgan J, Carty H. A Pictorial Review of Atlanto-axial Rotatory Fixation: Key Points for the Radiologist. Clinical Radiology, 2001; (56): 947-58. Roche C, King S, Dangerfield P, Carty H. The Atlanto-axial Joint:Physiological Range of Rotation on MRI and CT. Clinical Radiology, 2002; (57): 103-8. Subach B, McLlaughlin M, Albright L, Pollack I. Current Management of Pediatric Atlantoaxial Rotatory Subluxation. Spine, 1998; (23): 2174-9. Sia KJ, Tang IP, King CK, Nasriah A. Grisel’s Syndrome: A Rare Complication of Tonsillectomy. J Laryngol Otol, 2012; 126(5): 529-31. Dagtekin A, Kara E, Vayisoglu Y, Koseoglu A, Avci E, Talas D, Bagdatoglu C. The Importance of Early Diagnosis and Appropriate Treatment in Grisel’s Syndrome: Report of Two Cases. Turk Neurosurg, 2011: 21(4): 680-4. Parke W, Rothman R, Brown M. The Pharingovertebral veins: An Anatomical Rationale for Grisel’s Syndrome. The Journal of Bone and Joint Surgery, 1984; (66-A): 568-574.
180 [11]
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G.L. Ortiz et al. / Grisel’s syndrome: An unusual cause of Torticollis Harma A, Firat Y. Grisel Syndrome: Nontraumatic Atlantoaxial Rotatory Subluxation. J Craniofac Surg, 2008; 19(4): 1119-21. Hicazi A, Acaroglu E, Alanay A, Yazici M, Surat A. Atlantoaxial Rotatory Fixation-Subluxation Revisited. Spine, 2002; (27): 2771-5. Salpietro V, Polizzi A, Granata F, Briuglia S, Mankad K, Ruggieri M. Upper repiratory tract infection and torticulis in children Clin Neuroradiol, 2012; 22: 351-353.
[14]
[15]
Mezue W, Taha Z, Bashir E. Fever and Aquired Torticollis in Hospitalized Children. The Journal of Laryngology and Otology, 2002; (116): 280-4. Karkos PD, Benton J, Leong SC, Mushi E, Sivaji N, Assimakopoulos DA. Grisel’s Syndrome in Otolaryngology: A Systematic Review. Int J Pediatr Otorhinolaryngol, 2007; 71(12): 1823-7.
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175
Grisel’s syndrome: An unusual cause of Torticollis Gerardo L. Ortiz∗ , Ivette Pratts and Edwardo Ramos Physical Medicine, Rehabilitation, and Sports Medicine Department, University of Puerto Rico, San Juan, Puerto Rico
Accepted 29 April 2013
Abstract. PURPOSE: The objective of this paper is to present a case series of patients with diagnosis of torticollis due to Grisel’s syndrome and, in doing so, raise awareness of an unusual condition that could be fatal. A review of the literature is presented regarding diagnosis and treatment. METHODS: Case series of three patients that were 7, 10, and 12 years old with history of tonsillitis or neck surgery consulted to the service of Rehabilitation Medicine. RESULTS: Physical examination showed a fixed head or limitation of movement to neutral position and initially a normal neurological examination. Initial cervical X-rays were not diagnostic. The final diagnosis was made by CT scan or MRI. All 3 patients were treated with anti-inflammatory medications, immobilization, and/or cervical traction or surgery. CONCLUSION: Grisel’s syndrome is a non-traumatic atlanto-axial rotatory fixation (AARF) with or without subluxation following infection or surgery in the head or neck region. This paper presents an unusual cause of torticollis that could be fatal or cause neurological injury if not recognized and treated appropriately. Keywords: Torticollis, Grisel’s syndrome, atlantoaxial rotatory fixation, atlantoaxial subluxation, children
1. Introduction Torticollis is a sign of an underlining disorder, not a diagnosis, that results in tilting of the head to one side and rotation to the other. The most common cause of torticollis is congenital muscular torticollis. There are more than 80 different causes, from which approximately 18% are non-muscular causes, ranging from diagnosis that require no specific treatment to potentially life threatening conditions. The most common non-muscular cause is Klippel-Feil. Other non muscular causes include ocular disorders (4th cranial nerve palsy), central nervous system abnormalities (tumors), obstetrical palsies (Brachial plexus injury), clavicle fracture, C1-C2 rotatory subluxation, and inflamma∗ Corresponding author: Gerardo L. Ortiz, Paseo Las Brisas, Niza street #29, San Juan, 00926, Puerto Rico. Tel.: +1 787 638 8415; Fax: +1 787 754 1478; E-mail: [email protected].
tory conditions [1]. The purpose of this paper is to discuss the presentation and diagnosis of torticollis in 3 patients and to raise awareness about an unusual cause of torticollis that could be fatal or produce neurological deficit.
2. Findings The 1st patient was a 7 year old boy with hemophilia and a history of severe tonsillitis that resolved in one week, except for residual neck pain and the inability to move his neck. He had no previous history of trauma. At physical examination his head was tilted to the right side and rotated to the left; no masses or nodes were palpable and his musculoskeletal and neurological examination was normal. Cervical X-ray (Fig. 1) was suspicious for C1-C2 subluxation and an MRI (Fig. 2) confirmed this diagnosis. Treatment consisted
c 2013 – IOS Press and the authors. All rights reserved 1874-5393/13/$27.50
176
G.L. Ortiz et al. / Grisel’s syndrome: An unusual cause of Torticollis
Fig. 1. Odontoid fracture versus C1-C2 subluxation. With such an X-ray, CT scan is recommended.
Fig. 3. Atlanto-odontoid distance 6 mm with increased C1-C2 spinous process separation.
Fig. 2. Posterior arch of C1 to odontoid process distance of 5–6 mm: C1-C2 subluxation.
Fig. 4. C1-C2 rotatory subluxation with occipitalization of the posterior arch of C1, rupture of transverse ligament.
of one week of Halter traction, anti-inflammatory medications, and immobilization with a cervical collar for six weeks. Two months post hospital discharge the patient presented with no neurological deficits. The 2nd patient was a 12 year old girl who, one week after a tonsillectomy, awoke with pain over the cervical region and was unable to move her neck. Cervical X-ray was negative and non-steroidal antiinflammatory drugs did not improve pain. The pain progressed and within 3 months the patient was unable to move her right upper extremity. She progressed to right hemiparesis within 24 hours. There was no history of trauma or falls, but a history of recurrent tonsillitis was present. On physical examination, the head tilted to the right and rotated to the left. There was neck stiffness with a maximum range of motion of 10◦ . No masses or nodes were palpable and cerebellar and
cranial nerve examinations were within normal limits. On physical examination, a manual muscle test on the right side showed the following: deltoid, 0/5; biceps, 2/5; hand grip, 2/5; iliopsoas, 4/5; quadriceps, 5/5; anterior tibial, 4/5; gastrosoleus, 4/5. Muscle tone was normal and the range of motion was passively full. A sensory examination showed increased pinprick on the right side. Deep tendon reflexes were +2 upper extremity and +3 lower extremity. The Babinski reflex was not present. Repeated X-rays showed an atlanto-odontoid distance of 6 mm (Fig. 3) with a diagnosis of C1-C2 subluxation confirmed by CT scan and MRI (Fig. 4). The treatment consisted of traction followed by fusion and Minerva jacket immobilization. The patient’s neurological symptoms resolved after surgery, with minor limitation of cervical rotation noted on the physical examination one year post surgery.
G.L. Ortiz et al. / Grisel’s syndrome: An unusual cause of Torticollis
The 3rd patient was a 10 year old girl with Attention Deficit Disorder and autism. The patient was admitted due to right periorbital cellulitis, mastoiditis, and retropharyngeal abscess that resolved with antibiotics. The patient was discharged with residual torticollis that persisted for 5 months. At physical examination, her head was tilted to the left and rotated to the right, and the child was unable to move her head passively. The neurological examination was within normal limits. The cervical X-ray was reported as negative but cervical CT scan diagnosed C1-C2 subluxation. The treatment consisted of cervical traction and immobilization with a Minerva jacket. The patient subsequently presented with recurrence of torticollis and subluxation as demonstrated by CT scan and was readmitted for fusion. The patients in all three cases had a history of neck surgery or tonsillitis, and torticollis presented within one week post infection with a position known as “cock robin”. In this position, in which the sternocleidomastoid muscle is contracted on the side opposite the deformity, the head movement is limited to a neutral or fixed position. Cervical X-rays were difficult to interpret and the diagnosis was made by CT scan or MRI in all cases. Two of the patients failed to respond to conservative treatment (rest, antiinflammatory medications, traction, and cervical collar) and ultimately required surgical fusion.
3. Discussion All three patients showed what is known as Grisel’s syndrome, a non-traumatic atlanto-axial rotatory fixation (AARF), with or without subluxation, following infection or surgery in the head or neck region, most commonly tonsillectomy. Aëtius of Amida (5th– 6th century of the Common Era) was the first to indicate the association between inflammation of the vertebra caused by retropharyngeal abscess and vertebral luxation. The syndrome was later described by Rhazes (865-625 CE) quoting from Hippocrates Of The Epidemics [2]. Other terms for torticollis in literature are: malum suboccipitale rheumaticum, distension luxation, Drehungsverrenkung, torticollis nasopharyngien, maladie de Grisel, non-traumatic subluxation, spontaneous hyperemic dislocation, inflammatory dislocation, spontaneous dislocation, dislocation nontraumatique, and le faux torticollis aigu [3,4]. The atlantoaxial joint is the most active joint in the body, reported to move at least 600 times an hour, both
177
Fig. 5. Posterior view of the atlanto-axial joint, to observe the transverse and alar ligaments. (Colours are visible in the online version of the article; http://dx.doi.org/10.3233/PRM-130253)
while awake and asleep. The normal range of cervical rotation is approximately 90◦ to either side; 50% of this motion occurs at C1-C2 [5]. The facet joints between the atlas and the axis are almost horizontal, which allows excellent rotation at the expense of bony stability. The transverse ligament running behind the odontoid prevents anterior motion of the atlas on the axis (Fig. 5). The paired alar ligaments, which run from the posterolateral tip of the odontoid to the occipital condyles, limit rotation of the atlas on the axis. The right alar ligament prevents excessive rotation to the left, and vice versa [5]. During rotation, the ipsilateral mass of C1 rotates back into the spinal canal, narrowing it, but the spinal canal is widest at this level and anatomical studies have shown that approximately 64◦ of right or left atlanto-axial rotation is required before there is sufficient narrowing to cause spinal cord compression [6]. The patient may complain of neck stiffness and pain upon attempted motion, as occurred in all of the patients in this report. Dysphagia is a frequent complaint, and the patients often experience nuchal pain radiating to the head or ears, with the head held immobile in a pathologic position [3].
4. Pathophysiology This syndrome is more commonly seen in children. Several reasons account for this: the facet joints, specifically in C1 and C2, are shallow and more horizontal; the atlanto-occipital joint is less stable; the condyles are smaller; the ligamentous attachments are more lax and easier to disrupt; the uncinate process
178
G.L. Ortiz et al. / Grisel’s syndrome: An unusual cause of Torticollis
is poorly developed, causing excessive lateral bending and rotation; and the head size disproportion present in children (short neck with minimal musculature) [7–9]. The reason that this deformity becomes fixed is not known. Postulated mechanisms include lymphadenitis producing cervical muscle spasm in the presence of ligamentous laxity, hyperemia that produces ligamentous laxity, distention or decalcification of bony attachments of ligaments, and direct infection resulting in rupture of ligaments [5]. Infection causes periligamentous inflammation, which results in distention, laxity, and, consequently, subluxation of the atlantoaxial joint. The connection between the pharingovertebral veins, the peri-odontoidal venous plexus, and the suboccipital venous sinuses may provide a route for hematogenous spread of infection and an anatomical explanation for atlanto-axial hyperemia [8–11].
5. Classification In 1977, Fielding and Hawkins classified four types of AARF, with Type I being the most common, in which the rotation of the atlas on the odontoid occurs without anterior displacement. This was done before CT was available. Some authors use the term atlantoaxial rotatory subluxation (AARS), but Fielding and Hawkins used the term fixation of the atlas on the axis, as may occur with subluxation or dislocation, or when the position of the atlas and axis are within the normal range of rotation (Table 1) [5,12]. Types II-IV compromise problems with the spinal canal that result in cord compression and neurological signs and have a poorer prognosis than Type I [5]. Based on this classification, the 1st and 3rd patients should be classified as Type II, and the 2nd patient classified as Type III, since this child had a atlanto-odontoid displacement of 6 mm by X-ray with neurological compromise.
6. Diagnosis The diagnosis of Grisel’s syndrome is difficult and often delayed. This condition should be suspected if a child presents with torticollis that fails to resolve within 5–7 days, especially if it is preceded by an upper respiratory tract infection (pharingitis, tonsillitis, otitis media, and/or viral syndrome) or surgical procedure. The condition is accompanied by sternocleidomastoid (SCM) muscle spasm to the side on which the head is rotated in attempt to reduce the deformity. The inabil-
ity to rotate the head past midline means that that the deformity is fixed. Neurological findings are usually absent, unless associated with an anterior or posterior atlanto-axial displacement. Radiological findings for these cases are difficult to interpret. Cervical X-rays are limited by the deformity and the child’s uncooperativeness due to the pain. The open mouth view is difficult to perform when the child does not cooperate, and lateral views are difficult to interpret due to torticollis. If a good lateral view is obtained, an atlas-dens interval greater than 4.5 mm (adults 2.5–3 mm, children up to 4 mm) should alert the physician of the possibility of subluxation of C1 on C2. CT scan is the test of choice. Some authors recommend a dynamic test, which is done by rotating the patient’s head to the opposite direction, showing that the atlas and the axis do not rotate independently of each other but as a unit [6–8,11,14]. Other authors recommend performing the CT scan with 3D reconstruction [4,9]. More recently, MRI is becoming very useful in the diagnosis of this condition. With this technique, the surrounding soft tissue structures can be more clearly visualized and possible spinal cord compression identified. It can also reveal occult infections in the upper respiratory airways or effusions into the atlanto-axial joint [13].
7. Treatment The treatment of atraumatic torticollis of less than one week is conservative. Management with rest, soft collar, muscle relaxants, and anti-inflammatory medications usually is enough to improve the condition. In the case of a bacterial infection, antibiotics will be needed to control the infection, as seen in the 3rd patient. If this fails to resolve, a CT scan, traction, and a rigid cervical collar can be considered. If diagnosis of atlanto-axial rotatory fixation is made after one month of the deformity occurring, then traction should be provided until the deformity is corrected. This is followed by a cervical collar for six weeks. Torticollis present for more than three months will usually require surgery [5]. As evidenced in the literature [5,7,14,15], the major predicting factor for the failure of conservative treatment was the duration of the subluxation before initial reduction was attempted. It is for this reason that neurosurgical consultation is paramount in all cases of torticollis; morbidity is significant in those cases in which
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Table 1 Fielding and Hawkins classification 1977 Fielding and Hawkins classification:
Type I – Rotatory fixation without anterior displacement of the atlas (most common and occurs within the normal ROM of the joint). Type II – Rotatory fixation with anterior displacement of 3–5 mm, associated with deficiency of the transverse ligament. Type III – Rotatory fixation with anterior displacement > 5 mm, associated with deficiency of the transverse and alar ligaments. Type IV – Rotatory fixation with posterior displacement of the atlas associated with deficient odontoid process (least common).
diagnosis is delayed, with possibly devastating consequences. Indications for fusion include neurological involvement, anterior displacement, failure to achieve or maintain correction, and the presence of the deformity for more than three months. It is important to mention that with the surgery (C1/C2 fusion), 50% of neck rotation is lost [5]. Delays in diagnosis and conservative treatment can result in an increased need for surgical intervention [4,5,7,8,11,14,15]. In this series, the second patient presented with right hemiparesis and the third patient had a persistent torticollis, both of three months duration, which lead to cervical fusion.
Conflict of interest Editorial support was provided by grants from the National Center for Research Resources (U54 RR 026139-01A1) and the National Institute on Minority Health and Health Disparities (8U54 MD 007587-03) from the National Institutes of Health. The authors report no conflict of interest.
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8. Conclusion Grisel’s syndrome is an unusual cause of torticollis that can be fatal or can cause neurological deficit if not recognized and treated appropriately. A conservative approach is recommended for torticollis, but if it persists more than one week, AARF should be suspected. Cervical X-ray usually is not diagnostic; MRI is currently the preferred diagnosis. Treatment depends on the timing of diagnosis. However, conservative treatment is limited in success when the diagnosis is delayed. Acknowledgments We want to thank Scott F. Nadler, D.O (R.I.P.), who encouraged us to publish this case series. The authors highly value the help of Amarilys Irizarry Hernandez, Graphic Designer of the Medical Sciences Campus of the University of Puerto Rico, in the development of the artwork.
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