Abstract
Grisel’s syndrome is non traumatic atlanto axial subluxation usually associated with inflammatory conditions of head and neck region. Non traumatic atlanto axial joint subluxation is a rare entity for an unsuspecting otolaryngologist. The condition if missed and not treated properly can have serious implications with possible neurological deficit. We attempt to review the existing literature on the disease condition and also to highlight the proper management of the condition. Retrospective analysis of hospital record of last five years and an internet based literature survey on the current concepts and management of the condition. We have come across five cases of Grisel’s syndrome over the last five years. The clinicopathological and radiological features are described. Grisel syndrome may remain undiagnosed at initial presentation if not suspected. Delayed diagnosis may result in life long morbidity for the patient and the need for surgical intervention. Many of the early cases can be treated successfully by conservative approach.
Keywords: Grisel syndrome, Nontraumatic atlantoaxial subluxation, Torticollis
Introduction
Grisel syndrome was first described in a patient with syphilis by Charles Bell in 1830 and refers to “non traumatic atlantoaxial rotatory subluxation not triggered by trauma, affecting patients with a head and/or neck infections or occurring after otorhinolaryngology procedures” [1]. The condition is rarely encountered in routine otolaryngology practice but is an important condition that requires early diagnosis and prompt management in order to prevent any permanent neurological sequelae. Most of the literature on the condition have been isolated case reports. It is also noteworthy that most of the cases are referred to an otolaryngologist or neurosurgeon at a later stage which often results in neurological deficit. The purpose of this paper is to make us aware of this rare condition which may be missed in the early stage if not suspected.
Materials and Methods
We retrospectively analyzed the hospital records for the diagnosis of Grisel syndrome or nontraumatic atlantoaxial subluxation. Only five cases were detected in the last five years. The clinicopathological profile and the radiological features of the disease were noted and analyzed. We reviewed the existing literature by conducting an internet based search in Medline/Pubmed and Google Scholar.
Results and Observations
We have encountered five cases of Grisel syndrome over the last 5 years in our institute (Table 1). All of the cases were of the pediatric age group with age ranging from 6–12 years. All of them were from the urban population and belonged to the upper middle class society as per the modified Kuppuswamy scale. There were no specific cyclical or seasonal trends in the occurrence of the disease. There was no evidence of clustering of the cases in time and place. The patients presented with an abnormal head posture and neck pain. The onset of neck pain and abnormal head posture were of sudden onset in all the patients and all of them gave a history of waking up to a painful stiff neck in the morning. There was a history of upper respiratory tract infection in all five patients within the preceding week. Two patients (40 %) had fever with ipsilateral tender lymph node swelling at the time of presentation. Lymph nodes involved were unilateral Level II and III group of nodes. There was no history of antecedent trauma in any of the patients. There was no neurological deficit in any of the patients. It is interesting to note that only two cases with tender cervical lymphadenopathy and fever presented primarily to the ENT OPD while the rest were referred to ENT department at least a week after the onset of symptoms. The initial presentation of the three cases were to general physicians and were treated conservatively for symptoms of upper respiratory tract infection. One patient had grade I deformity (Fig. 1) while the rest had grade II deformity (Fig. 2a–c). Four patients were managed conservatively with antibiotics, muscle relaxants and cervical immobilization. One patient refused treatment. Complete recovery was achieved in four cases while the outcome of one case was not known. None of the patients presented with any recurrence of symptoms.
Table 1.
Clinical profile of the patients diagnosed with Grisel syndrome
| SL | Age | Sex | Clinical features | ||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Duration of symptoms | Neck pain | Fever | Sore throat | Coryza | Lymphadenopathy | Abnormal neck posture | Restriction of neck movement | C2 tenderness | Radiological grading | Neurological deficit | |||
| 1 | 12 years | M | 8 days | + | + | − | + | − | + | + | + | II | − |
| 2 | 8 years | M | 10 days | + | + | − | + | − | + | + | + | II | − |
| 3 | 7 years | M | 3 days | + | + | + | + | + | + | + | + | II | − |
| 4 | 6 years | F | 7 days | + | + | − | + | − | + | + | − | I | − |
| 5 | 10 years | M | 4 days | + | + | + | + | + | + | + | + | II | − |
Fig. 1.
Rotational deformity (grade I) in a patient presenting with torticollis
Fig. 2.
a–c 3D reconstruction and coronal scan showing subluxation and rotation of the atlantoaxial joint (grade II deformity)
Discussion
Spontaneous subluxation due to inflammatory conditions of the head and neck region is a rare scenario. The incidence rate of the disease is not known. It is commonly seen in the pediatric age group. Most common cause is the infective cause (48 %) of which upper respiratory tract infection was commonest (83 %) followed by retropharyngeal abscess (11 %), otitis media (4 %) and mumps (2 %) [2]. It has also been known to be associated with tonsillar abscess and adenotonsillitis [3] and there has been report of occurrence of the disease in a patient with Kawasaki disease [4]. In our series also upper respiratory tract infection was seen in all cases with two patients developing acute lymphadenitis of the cervical lymph nodes. There is no known identified pathogen which can be implicated in the pathogenesis of Grisel syndrome. There has been mention of isolation of pathogens like Group B beta hemolytic streptococcus, MRSA, Fusobacterium necrophorum, Mycobacterium tuberculosis and Epstein Barr virus from the primary infection causing the disease [5, 6]. Historically the first case of this syndrome was described in a patient with syphilis [1]. It is not known at present whether any particular pathogen has more tendency to cause the disease than others. Grisel syndrome has also been known to occur after surgical procedures of the head and neck region (40 %) with adenotonsillectomy being the most common (78 %). Though less commonly it can also occur after procedures like pharyngoplasty (2.5 %), otoplasty (2.5 %) tympanomastoidectomy (2.5 %) and grommet insertion (2.5 %) [2].
The mechanism of atlantoaxial subluxation is not known clearly. However it is considered that the subluxation occurs due to laxity of the ligaments around the atlantoaxial joint. This laxity occurs as a result of hyperemia secondary to hematogenous spread of the infection through the pharyngovertebral veins which provide communications of the veins of the posterosuperior portion of the pharynx with that of the periodontoid venous plexus. It is also proposed that cervical lymphadenitis secondary to nasopharyngeal infection results in spasm of the suboccipital and paravertebral muscles causing torticollis [1]. The occurrence of the disease in the pediatric age group may be due to the relatively larger head size, loose ligaments and joints and a relatively shallow and more horizontally placed facet joint. The rich lymphatic and greater number of retropharyngeal lymph nodes may also contribute to the higher incidence of the disease in the pediatric population [3].
Patients usually present with an abnormal head posture. It has been seen in all the cases in this series that the neck symptoms started suddenly on waking up in the morning. It is not known at present whether any abnormal sleep posture predispose the patient to Grisel syndrome. The head posture has been classically described a Cock Robin tilt where the chin is rotated to one side while the neck is flexed to the opposite side. The neck is fixed and any attempt to mobilize the head position is painful and resisted. A tender palpable C2 spinous process is an indicator of subluxation [7]. A history of upper respiratory tract infection or inflammatory pathology of the head and neck region is usually present as is seen in the present series. Cases presenting early may have only rotational deformity of the atlanto axial joint without any subluxation [8]. Fielding and Hawkin’s classified the deformity into four grades [1] where:
Grade I denotes rotational deformity of atlas without any anterior displacement.
Grade II deformity is rotational deformity of atlas with anterior shift of 3–5 mm,
Grade III deformity included rotational deformity of atlas with anterior shift of more than 5 mm.
Grade IV deformity are those with rotation with posterior subluxation of the atlas.
Neurological deficit is usually accompanied by Type III and Type IV rotational deformity and may lead to spinal injury and quadriplegia.
Lateral plain radiograph of the neck may reveal an increased atlanto odontoid distance. An atlanto odontoid distance greater than 5 mm is significant in children [3]. However due to torticollis positioning the neck for lateral view may be difficult and the diagnosis missed. CT scan of the cervical vertebra gives a clear outline of the joints and is sensitive in detecting early subluxation. 3D reconstruction of the images gives a clear picture of the deformity and helps in planning the right management approach. MRI is indicated to rule out neurological involvement and when surgical intervention is planned. A clinical variant of the condition has been described with subluxation of the C2–C3 vertebra and a clinical presentation similar to Grisel’s syndrome [9]. However it is not known whether the variant has any clinical or prognostic significance from its classical counterpart.
The patients in the present series were treated conservatively with antibiotics, anti-inflammatory and muscle relaxants and cervical immobilization with a hard cervical collar. Application of a hard cervical collar may be difficult initially due to torticollis. In such cases initial application of soft collar may be followed with a hard collar once the spasm is relieved. It is recommended to continue the hard collar for a minimum period of two weeks. Participation in sports should be prevented for about six weeks [1]. Majority of Grade I, II and III cases undergo spontaneous reduction with conservative measures [2]. However some cases may require closed reduction and treatment with halter traction [1, 10]. Surgical intervention is recommended with Grade IV subluxation, recurrent subluxation and in failure of conservative approaches. Various surgical techniques are described in the literature each having its advantages and rates of complication. However there is lack of consensus on the optimal surgical technique for individual cases [10].
Grisel syndrome being an uncommon condition is often missed at initial presentation [11]. Though the exact figures are not known it is often seen in our country that people resort to massage and manipulation for painful torticollis. Cervical spine manipulation and massage carry the risk of serious neurological and vascular complications [12]. Osmotherly and Rivette found lack of definite protocol for screening of craniovertebral junction instability among physiotherapists [13]. Manipulation and massage of the cervical spine by physiotherapists and primary care givers should be discouraged as it may aggravate the subluxation and precipitate a neurological deficit. It needs to be emphasized that such patients with painful torticollis should be referred early so that a multidisciplinary care can be instituted. A delay in treatment should be prevented as it has been seen that delayed treatment lead to permanent deformity and neurological complications and increases the need for surgical intervention [14, 15].
Conclusion
Grisel syndrome is an uncommon entity and may often be undiagnosed at initial presentation. The consequences of a delayed diagnosis may lead to a life long morbidity for the patient and the need for surgical intervention. Many of the early cases may be treated successfully by conservative approach. The condition requires a multidisciplinary approach between the otolaryngologists and neurosurgeons or the spinal surgeons for proper management. The primary health care provider must be aware of the condition and immobilize the cervical spine with a cervical collar before referral. Manipulation and application of massage by the primary health care provider should be discouraged.
Compliance with Ethical Standards
Conflict of interest
Soumyajit Das, Suvamoy Chakraborty and Subhajit Das declares that they have no conflict of interest.
Contributor Information
Soumyajit Das, Email: drsoumya_entamch@rediffmail.com.
Suvamoy Chakraborty, Email: drsuvamoy@rediffmail.com.
Subhajit Das, Email: gko.smch@gmail.com.
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