Cervical vertebrae subluxation

Rotary subluxation of the atlantoaxial joint is an uncommon complication of head and neck surgery and infections. Reported sequelae of this disorder range from torticollis to death from compression of the spinal cord. Two cases of C1-C2 rotary subluxation will be discussed. These complications occurred following otitis media, repair of choanal atresia, and an adenoidectomy. The literature is reviewed.     

Grisel's syndrome following ENT-surgery: report of two cases

BACKGROUND: Non-traumatic atlanto-axial subluxation is a rare complication of upper neck inflammatory processes and head and neck surgery. It is called Grisel's syndrome named after P. Grisel, who first described this condition in 1930. Persistent torticollis following head and neck surgery or upper respiratory tract infections should alert the surgeon to a beginning atlanto-axial subluxation. Due to lax ligaments it especially occurs in children and patients with Down's syndrome. PATIENTS: We present two cases of Grisel's syndrome in children following head and neck surgery with prolonged history, discussing pathogenesis, diagnostic measures and therapy. RESULTS AND CONCLUSIONS: Although there are several theories concerning the actual pathogenesis, it is generally agreed that an inflammatory process is the primary cause of Grisel's syndrome. Therefore, early antibiotic treatment is recommended. Further treatment depends on clinical findings and Fielding classification of the degree of the subluxation and includes muscle relaxations, soft collar or stiff neck, cervical traction or even arthrodesis of C1 and C2. If recognised early and appropriate treatment is applied, the prognosis is excellent. Severe cases can present with degenerative disorders of the cervical spine or even with neurological malfunction.     

Grisel syndrome: a delayed presentation in an asymptomatic patient

Grisel syndrome is a rare condition characterised by atlanto-axial subluxation following an inflammatory process in the head and neck region. It occurs more commonly in children and usually presents with cervical pain and torticollis, in addition to symptoms of the primary infection. We present the case of an asymptomatic 78-year-old man who was incidentally found to have atlanto-axial subluxation on a routine follow-up computed tomography scan, three months following successful treatment of a skull base infection. This case emphasises the importance of appropriate follow-up imaging for patients with skull base infections, even if they respond clinically to medical treatment.     

Fever and acquired torticollis in hospitalized children

Acute torticollis due to non-traumatic atlanto-axial subluxation (AAS) is often seen in children presenting with inflammatory conditions of the upper respiratory tract and the neck. Grisel's syndrome is the eponym given to this condition. These patients may present earlier in the disease process without evident subluxation. Thus, early recognition of the condition with prompt commencement of appropriate conservative treatment could halt the progression into Grisel's syndrome. The purpose of this study is to address the importance of early recognition of inflammatory torticollis that can be treated successfully by conservative methods. A retrospective review was made of the case files and radiological investigations of 13 children with fever and torticollis who were treated in the neurosurgery unit of Hamad General Hospital in Qatar, over a two-year period from July 1996 to July 1998. The children were aged between three and 12 years with a male to female ratio of 3:1. All patients arrived at the hospital within 48 hours of onset of torticollis and almost all had manifestations of upper respiratory tract or head and neck infections. Radiological examination by cervical spine X-rays, computerized tomography (CT) or magnetic resonance images (MRI) revealed that only three cases out of 13 had AAS. All patients underwent conservative treatment that included rest, neck collar, simple analgesics and antibiotics, where appropriate. A muscle relaxant was used in nine cases and Halter traction was applied to the three with AAS. All patients responded well to treatment and none required surgical intervention for AAS. We conclude that the majority of children presenting acutely with inflammatory torticollis have rotational deformity only without AAS. Progression to the latter, i.e. Grisel's syndrome, may be aborted should the diagnosis be made early and conservative treatment initiated in time. On the other hand, delay in diagnosis would deprive these children an opportunity of receiving effective conservative treatment.     

Síndrome de Grisel como secuela de una mastoiditis aguda complicada

Grisel's syndrome is a rare complication of ENT area infections. It consists of a non-traumatic atlantoaxial subluxation after an infectious process. Its characteristic symptom is persistent torticollis despite a resolved infection. The knowledge of this condition helps early diagnosis and treatment. We therefore present the case of a patient with Grisel's syndrome as a sequela of an acute mastoiditis complicated by a Bezold's abscess.     

Evaluation of persistent torticollis following adenoidectomy

Post-operative neck complaints are not an uncommon finding following adenotonsillectomy. However, non-traumatic subluxation of the atlantoaxial joint (Grisel's syndrome) should be considered in cases of persistent neck pain and stiffness. An early diagnosis and adequate treatment of this rare condition is mandatory to prevent potentially serious complications. We describe three cases with persistent torticollis in the post-operative period, discuss the pathogenesis and evaluate the clinical management of these patients.     

Grisel's syndrome: a case report and review of the literature

Grisel's syndrome is non-traumatic atlantoaxial subluxation (AAS) secondary to an inflammatory process in the upper neck. It is a rare condition that occurs almost exclusively in children and has been associated with upper cervical infections and otolaryngologic procedures. A case of AAS secondary to an upper cervical infection is presented. Potential sequelae can be severe; early diagnosis and treatment of Grisel's syndrome can prevent tragic outcome.     

Grisel’s syndrome—a rare complication following “small” operations and infections in the ENT region

The atlanto-axial subluxation (Grisel’s syndrome) is a rare complication following operative procedures and/or infections in the upper aerodigestive tract. Pathogenetically the higher flexibility of the ligaments during the inflammation causes a subluxation between axis and atlas. When the inflammation heals, this can probably result in a fixation in the rotated position. The purpose of the present study was to describe the clinical and radiological characteristics of this rare disease in children. The clinical files of 12 patients with Grisel’s syndrome were examined retrospectively. The clinical files of these patients were reviewed and analyzed along with the results of radiographic and laboratory examinations. The clinical parameters were compared with the patient’s therapy and outcome. Of 12 children (6 males, 6 females, average age of 7.1 years), Grisel’s syndrome developed following surgery in 8 patients, and in another 4 patients following a severe in infection of the upper aerodigestive tract. The duration of complaints differed from 2 days to 6 months. All patients had a torticollis as the first symptom of atlanto-axial dislocation and three children had accompanying cervical lymphadenopathy. All patients were given antiphlogistic therapy either with diclofenac or ibuprofen. Additionally, 11 patients were treated with intravenous antibiotics (amoxicillin, ampicillin, clindamycin or cephalosporins) and 1 child with oral antibiotics. In eight patients a remission during antibiotic therapy occurred. In four cases (2 with Grisel’s syndrome following surgery, 2 following infection), however, the torticollis persisted despite adequate conservative treatment and required reposition of the atlanto-axial joint along with external fixation. In all of them, starting of therapy was delayed. An early diagnosis of Grisel’s syndrome and immediate therapy is most important. Grisel’s syndrome must be taken under consideration in children with acute torticollis following either an infection or operative procedure in the upper aerodigestive tract. Early adequate antibiotic and antiphlogistic therapy is mandatory and leads to a high remission rate.     

Grisel's syndrome in otolaryngology: a systematic review

AIMS: to assess etiology, treatment and outcome of Grisel's syndrome. METHODS: A Medline search was performed using the terms Grisel's syndrome, spontaneous atlantoaxial subluxation, head, neck, ear, nose and throat. A systematic review of the literature was performed. Case series of both adult and pediatric cases were included. Only papers focusing on true non-traumatic atlantoaxial subluxation were included. RESULTS: Seventy-one papers have been published from 1950 to 2006. Forty-eight of these fulfilled our inclusion criteria, totaling 103 patients for review. The main causes of Grisel's syndrome were infection (48%) and post-adenotonsillectomy (31%). Less common causes included other postoperative cases such as pharyngoplasty and ear operations. Neurosurgical consultation was paramount in all cases. In the majority of cases conservative management in the form of bedrest, antibiotics, muscle relaxants, traction and collar was effective; in a few cases only surgery in the form of arthrodesis was deemed necessary. Morbidity was significant in those cases where diagnosis was delayed, with the most devastating consequence a permanent neurological deficit in one case. CONCLUSIONS: Grisel's syndrome is a rare but dangerous complication that can go unnoticed in its early phase and can be a major cause of morbidity and mortality following infection or head and neck procedures/interventions. Early recognition of any cervical complication following routine otolaryngological operations together with early neurosurgical consultation is mandatory to prevent devastating consequences.     

Atlantoaxial subluxation in an adult secondary to retropharyngeal abscess

Atlantoaxial subluxation secondary to a retropharyngeal abscess is well described in children, but very rare in adults. Only two adult cases have been reported in the literature. We present a case of severe atlantoaxial subluxation in an adult secondary to retropharyngeal abscess. His atlas-dens interval was very large, up to 10 mm in flexion. We tried external fixation with a neck collar initially, but for remaining instability, surgical fusion was performed. The mechanism of subluxation is attributed to softening of the ligament allowing greater mobility at the joint. The etiology of this process is speculative.     

The importance of cervical muscles in responses of galvanic body sway test

Body sway in normal subjects was analyzed by means of various methods to study a role of cervical muscles in galvanic body sway test. Galvanic stimulation through the retro-auricular electrode induced an initial response and a deviation response in body sway. When the anodal stimulation was given through the right retro-auricle during standing, a deviation response toward the right side was observed. While keeping a posture weighted on one foot, the stimulation induced a similar response. The stimulation during squatting produced also a deviation response toward the right side. When the head was rotated to the right, the stimulation produced backward responses. When rotated to the left, it produced forward responses. Even without galvanic stimulation, similar responses were also induced by some other method, for example, inclining the head to one side. Galvanic stimulation while sitting resulted in slight but apparent head inclination. The results suggested that cervical muscles played an important role in galvanic body sway test. Initial and deviation responses appeared to be secondarily produced by changes in the cervical muscular tension.     

Relationship between vertebral artery blood flow in different head positions and vertigo

Objective: To identify the vertebral artery blood flow in different head positions in patients with positional vertigo with no specific diagnosis.

Methods: Patients with history of vestibular symptoms associated with changes in head position were enrolled into the study. Healthy volunteers were evaluated as control group. Doppler ultrasonography examination of the cervical segment of the vertebral arteries was performed under three different head positions: (i) supine position, (ii) head hyperextended and rotated to the right side and (iii) head hyperextended and rotated to the left side.

Results: In the study group, right and left vertebral artery blood flow was significantly lower in the ipsilateral hyperextended position compared to standard supine position (respectively p?=?.014; p?=?.001), but did not differ significantly when compared between the standard supine and contralateral hyperextended positions (respectively?=?.959; p?=?.669). In the control group, left and right vertebral artery blood flow did not differ significantly when the head was hyperextended to the right or left sides compared to standard supine position (p?>?.05).

Conclusions: Our data demonstrated that the etiology of vestibular complaints in patients with undiagnosed positional vertigo might be related to impairment in vertebral artery blood flow according to head positions.     

Focal myositis. A new cause for the pediatric neck mass

Focal myositis is an inflammatory pseudotumor of skeletal muscle that may involve the head and neck. It can closely mimic either a neoplastic or infectious process. We present the case of a 7-year-old boy with a 2-week history of a painful, unilateral neck swelling, fever, and torticollis. He failed to respond to antibiotic therapy and required an open neck exploration. An incisional biopsy of the indurated, inflammatory tissue adherent to his sternocleidomastoid muscle showed focal myositis. Focal myositis is an unusual, but important possibility in the differential diagnosis of any neck mass and especially one in a child. Although its cause is unknown, it is a self-limited disease and neither excisional biopsy nor radical resection is justified.     

From the expert's office. Atlanto-axial subluxation with spastic torticollis after adenoid-ectomy resp. tonsillectomy in rose position - malpractice of the surgeon or the anaesthesiologist?

INTRODUCTION: An arbitration board had to decide whether or not there had been a causal connection between an adenoidectomy or resp. a tonsillectomy and an atlanto-axial dislocation and if so whether this was to be considered a case of malpractice. CASE HISTORIES: In two young girls aged 6 and 11 a torticollis had developed 3 resp. 4 days after the operation. In both cases the proper diagnosis was made only after extensive diagnostic procedures including radiology, neurology, neurosurgery and orthopaedics. The findings are presented in detail. In both cases there was a rotary subluxation between cervical vertebrae C1 and C2 due to a retropharyngeal inflammation. Normal function could only be achieved by surgical reposition and the application of a fixateur externe for quite a long period. DISCUSSION: A non-traumatic torticollis is a very rare complication of an adenoidectomy or tonsillectomy, two of the most common surgical interventions in oto-rhino-laryngology. It is known as Grisel's disease because Grisel in 1930 was the first to describe this sequel following nasopharyngitis and tonsillectomy. A detailed review of the literature and a discussion of the underlying pathology is presented. Predisposing factors might be additional local anaesthesia and electro-haemostatis. CONCLUSIONS:: In both cases evidence for malpractice could not be found, neither concerning the intervention itself nor the handling in the postoperative period. The latency of several days between the operation and the manifestation of the torticollis is regarded as proof that intraoperatively there was no malpractice. In cases where the torticollis is present immediately after the intervention, as has been reported in the literature, a traumatic luxation during the operation or positioning of the patient may be taken into consideration. Because of the extreme rareness of the complication it does not seem compulsory to make it part of the preoperative informed consent.     

Monopolar Electrocautery in Adenoidectomy as a Possible Risk Factor for Grisel's Syndrome

Objectives/Hypothesis Grisel syndrome is a nontraumatic atlantoaxial subluxation after an inflammatory process in the upper cervical region. It results from a peripharyngeal infection as well as a rare complication following adenotonsillectomy. After the introduction of monopolar suction electrocautery in adenoidectomy, an increased incidence of Grisel's syndrome has been observed. The purpose of the study was to evaluate monopolar suction electrocautery as a possible risk factor. Study Design Retrospective chart review along with current measurements of monopolar and bipolar electrocautery during adenoidectomy. Methods The charts of 1431 consecutive cases of adenoidectomy were reviewed before (n = 710) and after (n = 721) the introduction of monopolar suction electrocautery for intraoperative hemostasis. Moreover, energy of delivered current of electrocautery was measured in 30 pediatric patients during adenoidectomy. Results Three cases of Grisel's syndrome occurred as a postoperative complication in 721 adenoidectomies (0.4%) after the introduction of monopolar suction electrocautery. In contrast, no Grisel's syndrome was observed in 710 procedures before the use of monopolar electrocautery. Current measurements demonstrate an almost fourfold higher level of electrical energy using monopolar electrocautery compared with bipolar coagulation for bleeding control in adenoidectomy. Conclusion Monopolar suction cautery in adenoidectomy may be considered as a risk factor for Grisel's syndrome.     

Die objektive Darstellung des Torticolliswinkels mittels der Cranio-Corpo-Graphie (CCG)

Summary Cranio-Corpo-Graphy (CCG) is a fotooptical recording procedure for head and body movements. Through a hard hat wearing two light bulbs and through shoulder lights the longitudinal axis of the head and the transversal axis of the shoulders are light traced during head and body movements while stepping or standing. Recording is done by means of a Polaroid camera which allows superexpositions of several tests on the same foto and of a polarcoordinate light projection for calibrating the foto individually. Verging of the head can easily be measured by drawing the longitudinal head axis and the transversal shoulder axis as the so-called torticollis angulogram.This technique has been applied on patients suffering from spasmodic torticollis or from postoperative torticollis after neck dissection.     

Zervikopropriozeptive Provokation von horizontalen und vertikalen Nystagmen bei Probanden

Background

The cervico-ocular reflex (COR) has been confirmed in numerous animal experiments. On the other hand, its clinical assessment is disputed. Anteflexion and retroflexion of the head are among the main movements of the atlanto-occipital joint. We investigated whether these head movements produce neck proprioceptive stimulation in the vestibular system.

Subjects and method

We investigated 50 students under the experimental conditions of strictly cervical provocation. The trunk was rotated, anteflexed, and retroflexed into its end positions for 60 s under video-oculographic control without changing the head–space relationship. During the cervical provocation, horizontal, vertical, or rotational nystagmus within a time window of 5–30 s after the start of the provocation was detected as a target parameter.

Results

The results showed that, compared to the baseline, there was a significant increase in vertical and horizontal nystagmus after the cervical provocation. The horizontal nystagmus reaction, rather than the vertical, was increased significantly by the maximum trunk rotation. In addition, we found a significant amount of vertical rather than horizontal nystagmus under trunk flexion provocation.

Conclusion

Under cervical provocation of test subjects, we were able to elicit horizontal and vertical nystagmus (upbeat nystagmus) via cervico-ocular roots. Due to a neck proprioceptive activation of the vestibular system we interpret our result as a “cervico-tonic provocation nystagmus”.     

Persistent geotropic nystagmus--a different kind of cupular pathology and its localizing signs

CONCLUSION: A persistent geotropic positional nystagmus indicates a dysfunction in the lateral semicircular canal with a cupula of less specific weight than the surrounding endolymph. It is possible to determine the side of the affected cupula by recording the nystagmus pattern in yaw and pitch plane. OBJECTIVES: To identify the clinical features in patients with a persistent geotropic positional nystagmus, establish lateralizing signs and relate the findings to a pathophysiologic mechanism. PATIENTS AND METHODS: Six patients with acute onset vertigo of a peripheral origin and persistent geotropic nystagmus were examined with videonystagmoscopy and the nystagmus characteristics in different positions of the head in yaw and pitch plane were studied. RESULTS: Besides the persistent geotropic nystagmus, a zero zone was found with no nystagmus, beyond which the nystagmus changed direction when the head of the patient in supine position was gradually rotated from side to side. The zero zone was present when the head was turned slightly towards one side and is thought to represent a position where the affected cupula is aligned with the gravitational vertical. With the head bent forwards the nystagmus direction was to the non-affected side and when the head was bent backwards to the affected side.     

Nontraumatic atlantoaxial subluxation (Grisel syndrome): a rare complication of otolaryngological procedures

OBJECTIVE: At the conclusion of the report, the readers should be able to recognize the rare complication of adenotonsillectomy of Grisel syndrome, discuss its pathogenesis, and provide early, effective treatment. STUDY DESIGN: Analysis of the clinical presentation, plain radiographs, computed tomography scans, and magnetic resonance imaging scans of a child with Grisel syndrome after adenotonsillectomy. METHODS: Retrospective study of the case record and a review of the literature regarding the pathogenesis, treatment modalities, outcome, and prognosis after early and delayed treatment of Grisel syndrome. RESULTS: Pathophysiology of atlantoaxial subluxation revolves around the periodontoid vascular plexus that drains the posterosuperior pharyngeal region. Infectious and inflammatory emboli cause synovial engorgement, weakening paraspinal ligaments. Radiological studies play an important role in diagnosis. Treatment consists of cervical immobilization, muscle relaxants, analgesics, and antibiotics. Full resolution is expected with early conservative management. Failure to recognize the syndrome can lead to catastrophic results. CONCLUSIONS: Recognition of Grisel syndrome in addition to other rare complications of adenotonsillectomy requires a high index of suspicion. Early intervention is the critical factor for a positive outcome. However, delayed diagnosis is common and can result in catastrophic consequences, including neurological deficits, cosmetic deformity and, in rare instances, paralysis and death in the short term.     

Persistent geotropic nystagmus—a different kind of cupular pathology and its localizing signs

Abstract

Conclusion. A persistent geotropic positional nystagmus indicates a dysfunction in the lateral semicircular canal with a cupula of less specific weight than the surrounding endolymph. It is possible to determine the side of the affected cupula by recording the nystagmus pattern in yaw and pitch plane. Objectives. To identify the clinical features in patients with a persistent geotropic positional nystagmus, establish lateralizing signs and relate the findings to a pathophysiologic mechanism. Patients and methods. Six patients with acute onset vertigo of a peripheral origin and persistent geotropic nystagmus were examined with videonystagmoscopy and the nystagmus characteristics in different positions of the head in yaw and pitch plane were studied. Results. Besides the persistent geotropic nystagmus, a zero zone was found with no nystagmus, beyond which the nystagmus changed direction when the head of the patient in supine position was gradually rotated from side to side. The zero zone was present when the head was turned slightly towards one side and is thought to represent a position where the affected cupula is aligned with the gravitational vertical. With the head bent forwards the nystagmus direction was to the non-affected side and when the head was bent backwards to the affected side.