Grisel's Syndrome Research Articles

Intraoperative Ultrasonography for the Surgical Treatment of Grisel Syndrome of the Adult: Management of A Rare Condition.

This is a narrative review and case report. To review the literature concerning Grisel syndrome physiopathology, diagnosis, and surgical reports, highlighting the decision-making for treatment and its timing. We describe the role of intraoperative US in the management of 2 cases of GS of the adult. GS is a rare nontraumatic post inflammatory C1-C2 rotatory instability usually affecting children; adult cases are even rarer, and the role of surgical treatment is not well defined. Case 1: A 72-year-old man with upper cervical pain and no neurological deficit; radiologic examination revealed C1-C2 spondylitis and epidural abscess. After antibiotic therapy, the patient developed cervical instability. Hence, surgical decompression and C1-C2 stabilization were performed. Case 2: An 82-year-old woman with progressive right hemiparesis. CT and MRI showed C1-C2 spondylitis with retro-odontoid epidural abscess. Atlanto-axial rotatory instability was evident so surgery was achieved. In both cases, intraoperative US was useful for localizing retro-odontoid abscess and allowing safe puncture of the collection, leading to its remarkable radiologic reduction. GS in adults remains a challenge: Patients should be closely monitored concerning neurological and inflammatory status. We describe the successful use of intraoperative US for draining retro-odontoid abscess for the first time, with satisfactory postoperative outcome without need of circumferential approach.

Imaging Assessment of Nontraumatic Pathologic Conditions at the Craniovertebral Junction: A Comprehensive Review.

Nontraumatic pathologic conditions of the craniovertebral junction encompass a range of conditions affecting the complex anatomy of this region without direct physical injury. These conditions include congenital syndromes that predispose individuals to ligamentous laxity, potentially leading to instability. Additionally, rare but noteworthy cases such as Grisel syndrome, a cause of pediatric torticollis, may arise without a traumatic trigger. Inflammatory diseases, including rheumatoid arthritis, ankylosing spondylitis, and crystal deposition, can lead to cervical instability and spinal cord compression. Infections at the upper cervical spine are dominated by tuberculosis, typically transmitted through hematologic or lymphatic routes with characteristic imaging findings. On the other hand, purulent bacterial infections in this area are rare. Furthermore, although tumors involving the structures of the craniovertebral junction are infrequent, they can lead to significant complications, albeit less frequently through cord compression and more commonly via pathologic fractures or subluxation. The craniocervical junction is a complex anatomic region comprising ligaments, bones, joints, and muscles that support the head's weight and enable its wide range of motion. Accurate recognition and understanding of the complex anatomy and the various nontraumatic pathologic conditions at the craniovertebral junction are pivotal for initiating timely and appropriate treatment strategies. ©RSNA, 2024 Test Your Knowledge questions for this article are available in the supplemental material.

Grisel Syndrome and Its Anesthesia Management: A Comprehensive Review

Grisel syndrome is an atraumatic rotatory atlantoaxial subluxation associated with head-and-neck inflammatory conditions. It predominantly affects the pediatric population. Anesthetic management in these patients poses several challenges, and astute management is crucial for a good outcome. We present a case of a young male patient who presented for management of painful torticollis and restricted neck movements. He had a history of inadequate treatment of throat infection for a month and was subsequently diagnosed with Grisel’s syndrome. He underwent occipitocervical fusion. This case report reviews the literature and describes perioperative anesthesia care and concerns in a comprehensive manner in the management of Grisel’s syndrome.

Diagnostic Approach and Treatment Options for Pediatric Cases of Grisel's Syndrome Post Otolaryngology Procedure: A Systematic Review.

Grisel's syndrome is an uncommon cervical spine condition marked by non-traumatic rotational subluxation of the atlantoaxial joint. This systematic review aims to collect potential evidence from relevant studies that reported symptoms, diagnostic methods, and management options among pediatric cases of Grisel's syndrome post otolaryngology procedures, which can aid and guide the diagnosis and management in clinical practice.We conducted both electronic and manual search strategies within the potential databases and included case reports, case series, and articles; however, review papers and correspondence papers were excluded. The post-otolaryngology procedures included adenoidectomy, tonsillectomy, tympanoplasty, cochlear implantation, double opposing Z plasty and pharyngeal flap, and adenotonsillectomy. In this systematic review, we identified and analyzed 20 studies encompassing a total of 24 pediatric patients with Grisel's syndrome following otolaryngology procedures. The patient demographics revealed a fairly even distribution between females (45.83%) and males (50.00%), with ages ranging from 2.5 to 12 years. The most common otolaryngology procedures associated with Grisel's syndrome were adenoidectomy (29.17%) and adenotonsillectomy (33.33%). Clinical symptoms included neck pain (75.00%), torticollis (50.00%), and limited neck mobility (20.83%), while diagnostic confirmation primarily relied on CT scans (50.00%). Treatment strategies varied, with conservative measures being the most frequent choice, followed by surgical interventions in four cases (16.67%). Complications were reported in 20.83% of cases. Due to the rarity of this condition, our findings are limited to case reports only, which may limit the generalizability of results. Grisel syndrome can be effectively managed through conservative treatment, including antibiotics and anti-inflammatory drugsif diagnosed timely. Early diagnosis and prompt management are essential to avoid neurological and fatal complications.This analysis would contribute to improving clinical knowledge and treatment strategies while providing additional insights into this rare condition.

Iatrogenic atlantoaxial rotatory subluxation after thyroidectomy in a pediatric patient: A case report

Atlantoaxial rotatory subluxation (AARS) is an uncommon disease with a greater prevalence among children than adults, and it is mostly associated with trauma. Iatrogenic spinal injury accounts for a low percentage of injuries. However, in AARS, 20%-40% of cases are associated with surgery, and 48% are caused by infection. Here, we describe our experience with a case of iatrogenic AARS after general anesthesia. A 12-year-old girl presented with right-sided torticollis and cervical motion limit. The patient had undergone thyroidectomy 2 mo ago. Computed tomography revealed AARS with bilateral locked facets. Following the failure of repeated external reduction under general anesthesia, the patient underwent an open surgical reduction. The patient gained atlantoaxial alignment without any complications. Follow-up radiographs showed a normal appearance without instability. The cervical spine of children is more predisposed to injury due to anatomical and biomechanical differences. AARS secondary to infection and surgery is known as Grisel's syndrome, which involves non-traumatic AARS. Several cases of AARS after surgery and other procedures with no evidence of inflammation have been reported. Our experience shows that surgery requiring hyperextension of the neck after general anesthesia should also be included as a risk factor. Surgeons and anesthesiologists should be careful not to excessively extend the neck during pediatric surgery. Moreover, clinicians caring for pediatric patients with recent head and neck procedures must be aware of common AARS presentations.

Orthopedic Physical Therapy Treatment for Chronic Nontraumatic Atlantoaxial Rotation Subluxation (Grisel Syndrome): A 6-Year-Old Female Case Report

BACKGROUND: Grisel syndrome (GS) is a rare kind of atlantoaxial joint subluxation (AAJS) in children due to infectious or inflammatory processes of the head and neck region. This case report aims to present the orthopedic physical therapy treatment and outcomes of a case with GS. CASE PRESENTATION: A 6-year-old girl with AAJS, diagnosed 3 months after a severe bout of tonsillitis, was studied. Her physical examination revealed that cervical spine active range of motions was limited. Computerized tomography showed fixation of the atlantoaxial joint consistent with type II AAJS. The patient was treated with orthopedic physical therapy (ie, a combination of myofascial release, manual traction, a home exercise program, and a cephalo-cervicothoracic splint) for 4 weeks. OUTCOME AND FOLLOW-UP: After the treatment, the AAJS decreased, pain and cervical range of motions improved, and a normal posture of the head was regained. At the follow-up periods (in the third and twelfth months), the patient reported no pain during active cervical movements, and the active range of motions was optimal. DISCUSSION: The significance of this case report lies in the successful management of a conservative treatment approach for GS. The findings obtained in this case study suggest that physical therapy could be a successful option for the treatment of chronic GS. JOSPT Cases 2022;2(4):196–203. Epub: 12 October 2022. doi:10.2519/josptcases.2022.10883

Grisel syndrome and peripheral arthritis simultaneously occurred in a 7-year-old Chinese boy with Kawasaki disease.

Grisel syndrome and peripheral arthritis simultaneously occurred in a 7-year-old Chinese boy with Kawasaki disease.

Surgical management of Grisel syndrome in the adult patient: illustrative case.

BACKGROUNDGrisel syndrome describes an infectious soft tissue process that destabilizes the cervical bony elements and ligamentous complexes. This nontraumatic atlantoaxial rotary subluxation occurs in children primarily. This case illustrates a rare case presentation of an adult with Grisel syndrome: infectious destruction of the right atlantoaxial facet joint caused the occiput-C1 vertebra (head) to rotate rightward with lateral horizontal displacement off the C2 vertebra.OBSERVATIONSBecause the infection destroyed the C1 bony arch and atlantoaxial facet joints with epidural extension, the rotated head and atlas pulled the brainstem–cervical spinal cord junction against a fixed odontoid process, resulting in a cord contusion. Because of the highly unstable craniocervical junction, the patient presented with torticollis and left upper extremity weakness.LESSONSTreatment entailed closed reduction under general anesthesia followed by occipitocervical fusion with an occipital plate, C1 lateral mass screws, and C2-C5 pedicle screws. This case describes the unique surgical pearls necessary for occipitocervical fusion of an unstable craniocervical junction, including tips with neuronavigation, trajectories of the cervical pedicle screws, aligning the lateral mass and pedicle screws with the occipital plate, and nuances with occipitocervical distraction.

Mild Grisel Syndrome: Expanding the Differential for Posttonsillectomy Adenoidectomy Symptoms.

Tonsillectomy with or without adenoidectomy is the second most common pediatric surgical procedure in the United States with up to 97% performed as an outpatient.1,2 While it is largely a safe procedure, several complications have been described and are encountered in the emergency department and primary care setting. A 29-month child presented to the emergency department with neck stiffness 10 days after tonsillectomy and adenoidectomy. A computed tomography scan of the neck limited by motion artifact was unrevealing, but a consult to the pediatric otolaryngologist generated concern for Grisel syndrome, the atraumatic rotary subluxation of the atlantoaxial joint. While surgical intervention can be required, the patient had an uncomplicated clinical course and the anomalous neck posture resolved with time and antiinflammatories alone. Keeping a broad differential for posttonsillectomy and adenoidectomy patient concerns is important for the clinician. Serious, life-threatening complications can arise from Grisel syndrome while good functional outcomes can be achieved with timely and appropriate treatment.

Pearls & Oy-sters: Grisel Syndrome Presenting as Pseudodystonia: A Twist in the Neck.

Pseudodystonia is a term that describes abnormal postures, repetitive movements, or both, where clinical analysis, imaging, laboratory, or electrophysiologic investigations indicate that these movements are not consistent with dystonia. Grisel syndrome (GS), characterized by rotatory subluxation of the atlantoaxial joint (AAJ) due to nontraumatic causes, is a cause of pseudodystonia. GS is seen in children less than 12 years of age and should be suspected in patients with acute onset of painful torticollis. We report 2 girls, aged 9 and 6 years, who developed painful torticollis following upper respiratory tract infection. They were thought to have cervical dystonia and referred to a movement disorder specialist for botulinum neurotoxin therapy (BoNT). MRI of the cervical spine showed type I and type II rotary AAJ subluxation, respectively, which confirmed the diagnosis of GS. Short tau inversion recovery hyperintensity was noted suggesting AAJ edema without any bone erosion or cord compression. Abruptness of onset, presence of severe pain, resistance to passive neck movements, fixed postures present equally in rest and action, absence of sensory trick, and persistence in sleep favor pseudodystonia. Both subjects improved with conservative treatment, which included temporary immobilization of the cervical spine and anti-inflammatory drugs. Early identification and treatment is imperative to avoid neurologic complications, like high cervical compressive myelopathy, which can lead to quadriplegia and respiratory distress. Prominent sternocleidomastoid contraction ipsilateral to the rotated chin helps to clinically identify GS.

Grisel Syndrome About A Case

Grisel syndrome, also known as nasopharyntic torticollis is a combination of a torticollis and nasopharingitis, describes first time in 1830 by bell and taken over by grisel in 19305which descibed two observations in two children complicated renopharyngitis since this syndrome bears his name.characterized clinically by sudden probing and radiographically by an enucleation of the atlas innon traumatic dislocation- rotating position . physiopathologically is caused by the inflammatory contracture of the prevertebral muscles whose origin could be a spontaneous or post opérative infection of the NTE area.This acute febrile torticollis is a rare affection mainly affects the child with an estimated age range between 8 months to 12 years, few cases are reported by literature concerning adults.Diagnosis should be realized early in order to avoid neurological restoration which cannot be carried out with certainty that other diagnoses of febril torticollis are eliminatedThe treatment is simple, the prognosis is often favorable with an adequate and early attitude directed towards the causal agent while restoring the spinal stabilit.

Halter Traction for the Treatment of Atlantoaxial Rotatory Fixation.

Atlantoaxial rotatory fixation (AARF) comprises a spectrum of abnormal rotational relationships between C1 (atlas) and C2 (axis). We aimed to evaluate the efficacy and long-term clinical outcomes of halter traction in treating patients diagnosed with primary AARF. We included patients <18 years of age who presented with new-onset painful torticollis, neck pain, and sternocleidomastoid muscle spasm, had an AARF diagnosis confirmed by use of 3-dimensional dynamic computed tomography, received in-hospital cervical halter traction under our treatment protocol, and were followed for ≥12 months. Radiographic and long-term clinical outcomes were analyzed. A total of 43 patients (31 male and 12 female; average age of 7.9 years) satisfied the inclusion criteria. There were 5 acute, 6 subacute, and 32 chronic cases. The mean duration of initial symptoms prior to treatment was 12.1 weeks. Thirty-seven (86.0%) of the patients experienced previous minor trauma, and 6 (14.0%) had a recent history of upper-respiratory infection (Grisel syndrome). The mean duration of in-hospital traction was 17.6 days. The mean follow-up period was 8.5 years. Forty-two (97.7%) of the patients achieved normal cervical alignment after treatment. One patient (2.3%) had recurrence and received a second course of halter traction, with cervical alignment restored without any surgical intervention. No neurological deficits were noted during or after the treatment. No major complications were observed. Normal anatomy and restoration of cervical alignment can be achieved by cervical halter traction in most cases of AARF. Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Nontraumatic Atlantoaxial Rotatory Subluxation: A Rare Complication of COVID-19 in Elderly Patient

Introduction: Nontraumatic Atlantoaxial Rotatory Subluxation (NAAS) or Atlantoaxial Rotatory Subluxation (AARS) without trauma or concomitant bone pathology was first described by Sir Charles Bell in 1830 in a patient with syphilis and pharyngitis, who developed lethal outcome due to spinal compression. The syndrome was named after Grisel who described two cases of pharyngitis and atlantoaxial subluxation in 1951. However, this is an uncommon condition of uncertain etiology characterized by NAAS, usually seen in children secondary to an infection and inflammation in the head and neck region or otolaryngeal procedures. Patients generally complain about neck stiffness and pain, and sometimes dysphagia may occur. Diagnosis is established based on the clinical and radiological findings. The etiopathogenesis and the underlying pathomechanics have not been clearly explained. A hematogenous spread of infection from the posterior pharynx to the cervical spine, according to the recent literature, with hyperemia and abnormal relaxation of the atlantoaxial ligaments is a widely accepted theory. The vascular plexus providing the drainage of poster superior pharyngeal area is responsible. The periodontoid plexus is connected with posterior nasopharyngeal veins via the pharyngovertebral vein. Any infective embolism may spread from superior pharyngeal area to upper cervical joints due to this plexus which does not have any lymph node, thus providing an anatomical explanation for the atlantoaxial hyperemia reported in Grisel's syndrome; however, the clinical picture and complications of COVID-19 are unclear until now and every day new symptoms and findings are reported as early and late complications.&#x0D; Methodology: An 86-year-old male presented to our clinic in KKT, Jeddah from Yanbu (which is 330 km away) with a complaint of neck stiffness in anterior-lateral position after recovery from COVID-19 six months back. The patient was treated at home without admission in hospital. He had no history of trauma. During the physical examination, the patient's neck was stiff, and there was neck pain with palpation and left-sided torticollis. The patient’s weight was 58 kg, height 160 cm, blood pressure 140/75 mmHg with a pulse rate of 72, SPO2 95, and BMI 22.7. There was no sign of fever or any type of inflammation in his body. The patient had previously consulted neurosurgeons at three different hospitals in Jeddah and was advised a medication with a cervical collar for a clinical follow-up. After three weeks of follow-up, they advised him to start physiotherapy for one month with no benefits seen in clinical finding, the patient then came to us. Direct radiogram of the cervical region showed suspicious findings at the atlantoaxial joint. Anterior view of the cervical radiography revealed tilted position of the head over neck, while the lateral view showed no thickening of the parapharyngeal soft tissue. The distance between the axis and dens was within normal values for his age (ADI = 3–4 mm). This confirms our diagnosis of type 1 atlantoaxial subluxation according to Fielding and Hawkins classification.&#x0D; Result: Based on the patient’s history and physical examination, there was no infection or neck operation causing an inflammation in the neck except the COVID-19 six months prior to the neck stiffness. With this case, we would like to highlight that atlantoaxial subluxation should be kept in mind when neck stiffness is seen in elderly patient without history of trauma but with a history of COVID-19 as a rare complication.&#x0D; Conclusion: NAAS is a rare but major complication that can often go unnoticed in its early and late phase, which can be a major cause of morbidity following COVID-19 infections, thus early recognition and diagnosis is mandatory especially in adult and elderly patients recovering form COVID-19.

Conservative approach for treatment of Grisel's syndrome after resection of lympahadenitis tuberculosis of the neck: A rare case report.

IntroductionGrisel's syndrome is a rare condition characterized by nontraumatic rotatory subluxation of the atlantoaxial joint, which was caused by previous inflammation around the head and neck. It is usually seen in children and signed as torticollis. There was no consensus for management, yet early diagnosis and treatment is paramount.Presentation of caseA 5-year-old girl came to outpatient clinic complaining of wry neck 1 day after surgical excision of her TB lymphadenitis and got worsen by time. There was no history of trauma around the neck. Examination under general anesthesia and CT scan revealed acquired severe torticollis consistent with Grisel's Syndrome Fielding type 3 accompanied by TB lymphadenitis, and TB myositis of the neck. Manipulation under general anesthesia and immobilization using Minerva cast followed by Lerman Cervicothoracal Osthosis was conducted.DiscussionThis patient was diagnosed with Grisel's syndrome and underwent conservative treatment consisting of reduction under general anesthesia and immobilization using Minerva cast for 6 weeks. The patient was then applied Lherman Cervical Thoracic Orthosis (CTO) halo brace for another 3 months. Anti-tuberculous drug was given to control tuberculous infection. Eight months follow-up showed neither residual deformity, neck pain, nor movement limitation of the neck.ConclusionGrisel's syndrome has excellent result that is treated with conservative treatment using reduction under general anesthesia and Minerva cast.

Neck pain following adenotonsillectomy: consider Grisel’s syndrome

Adenotonsillectomy is a common surgical procedure in children, and postoperative complications are generally limited to bleeding episodes. Grisel's syndrome, a non-traumatic rotatory subluxation of the atlantoaxial joint, is a less common and poorly understood condition that may occur after otolaryngological surgery or nasopharyngeal inflammation.

Síndrome de Grisel como Complicação de Abcesso Parafaríngeo

INTRODUÇÃO: A síndrome de Grisel é uma entidade rara que corresponde a uma subluxação rotatória atlanto-axial não-traumática, por norma secundária a infeção das vias aéreas superiores ou a cirurgia da cabeça e pescoço. Os pacientesapresentam-se com torcicolo, e um grau de suspeição elevado é necessário para o diagnóstico.CASO CLÍNICO: Criança do sexo masculino, 6 anos, admitida por odinofagia, febre e torcicolo. Exame objetivo revelava flexão cervical com rotação para a direita. Tomografia computorizada cervical demonstrava abcesso parafaríngeo e subluxação anterior atlanto-axial de 3 mm, configurando uma subluxação tipo II de Fielding. A criança foi internada para realização de antibioterapia endovenosa, vigilância e imobilização cervical.DISCUSSÃO: A síndrome de Grisel é um diagnóstico a considerar numa criança com torcicolo e infeção cervical. Os otorrinolaringologistas devem relembrar-se das possíveis apresentações e fatores de risco para esta síndrome. O tratamento conservador é muitas vezes suficiente, e a identificação precoce com imobilização parecem levar a melhores outcomes.

A Systematic review of Grisel's syndrome after otolaryngology procedures

A Systematic review of Grisel's syndrome after otolaryngology procedures

Atlantoaxial rotatory subluxation/fixation and Grisel's syndrome in children: clinical and radiological prognostic factors.

Atlantoaxial rotatory subluxation/fixation (AARS/F) is a rare cause of torticollis in children. The aim of our study is to investigate all possible clinical and radiological prognostic factors in children with AARS/F. We retrospectively reviewed all cervical spine CT scans of children with AARS/F treated in our Hospital over the last 15 years. AARS/F was classified according to Fielding and Hawkins classification and C1-C2 rotation-degree was calculated. Moreover, two orthopedic surgeons reviewed all clinical reports of these children. All patients were conservatively treated (cervical traction/neck collar). An early recovery was considered in patients with complete clinical/radiological healing at 3 months follow-up, while a late recovery was considered in patients with disease persistence or relapse at 3 months follow-up or earlier. Fifty-five patients with diagnosis of AARS/F were included in the study (mean age = 8.5 years old - 25F, 30M). In 9/55 subjects (16.4%), a late recovery was observed. The presence of a concomitant infection or inflammation in the head and neck region (Grisel's syndrome) was significantly associated with a late recovery (p < .001). Also, the type of AARS/F (p = .019), according to the Fielding and Hawkins classification, and C1-C2 rotation-degree (p = .027) were significantly correlated with the recovery time.Conclusion: In patients with AARS/F, the presence of a concomitant infection/inflammation in the head and neck region is the most important prognostic factor and it is associated with a late recovery. The Fielding and Hawkins classification and C1-C2 rotation-degree well correlate with patients' recovery time. What is Known: • Atlantoaxial rotatory subluxation/fixation (AARS/F) is a rare cause of torticollis in children and CT is the most useful imaging tool for diagnosis and classification of AARS/F. • Conservative treatments are effective in the majority of pediatric patients with AARS/F. What is New: • The presence of a concomitant infection/inflammation in the head and neck region associated with AARS/F (Grisel's syndrome) is the most important prognostic factor and it is associated with a late recovery. • C1-C2 rotation-degrees, as well as Fielding and Hawkins classification system, well correlate with patients' recovery time.

Atlantoaxial Rotatory Dislocation: Delayed Diagnose Will Result in More Invasive Treatment Options

The atlantoaxial joint represents the most mobile joint complex within the spine, secured by ligaments and capsules. Integrity of the atlantoaxial joint is crucial with respect to the mobility of the head and the upper spine. Atlantoaxial rotatory dislocation is the most common type of injury within this joint in children and is characterized by a typical position of the head (cock robin position). Nevertheless, this type of injury is frequently overlooked. The purpose of the current study was threefold. First, the characteristics of the patients with atlantoaxial dislocation were identified. Next, we checked if the time to treatment did influence the type of treatment. Finally, we checked if the age of the child at the time of treatment influenced the type of treatment. Forty-four children, who were treated consecutively due to atlantoaxial dislocation at a single spine center between September 1993 and October 2018, are analyzed retrospectively regarding age, sex, symptoms, etiology, time to diagnosis, time to treatment, and outcome. Forty-four children (30 girls, mean age 8.9 years) were included in the study. The cock robin head position was found in all of them, but neurological deficits were not found in any of them. In 21 patients, dislocation was caused by previous infection (Grisel's syndrome), whereas in 19 patients, dislocation was due to minor trauma. In 4 cases, etiology remained unknown. Mean time to sufficient treatment was 178 days. Eighteen patients received closed reduction and immobilization after 57 days at mean. Open reduction followed by temporary fixation was done in 12 patients after a mean time gap of 188 days. Bony atlantoaxial fusion was necessary in 14 children, who were diagnosed after 319 days on average. Invasiveness of treatment was dependent on the time delay between development of dislocation and treatment; a significant difference was found between invasiveness of treatment and time to treatment (Kruskal-Wallis test, p < 0,05). Moreover, older children were treated significantly more often with fusion than younger ones (χ 2, p = 0,002). Young girls are predisposed to incur an atlantoaxial rotatory dislocation, which usually occurs due to minor trauma or infection. The cock robin position is characteristic, but neurological deficits are not common. There is a need for early and sufficient treatment because delayed treatment necessitates more invasive treatment, thus leading to a complete loss of function of the most mobile joint within the spine. Finally, older children are predisposed to more invasive treatment strategies.

Open Reduction and Decompression of Atlantoaxial Subluxation with Basilar Impression Due to Grisel Syndrome Using the Cervical Management Base Unit

Intraoperative manipulation of the craniocervical junction with the Cervical Management Base Unit (CMBU) has been used as an adjunct for achieving optimal anatomic alignment during instrumented fusion procedures in a variety of disease settings. Here, we present our experience using the CMBU as a supplement to achieving a successful reduction and fixation of a reducible craniocervical subluxation with associated basilar impression/medullary compression in the setting of Grisel syndrome. Under fluoroscopy and neuromonitoring guidance, the elevator and axial translation mechanisms of the CMBU safely allowed for presurgical assessment of reducibility and facilitated complete reduction of the deformity with restitution of a normal atlantodental interval, spinolaminar line, and clivoaxial angle. Magnetic resonance imaging acquired 1 month after surgery and antibiotic therapy showed resolution of a large epidural abscess in the region of the dens and no evidence of residual neural impingement. Upright plain films at 9 months showed maintenance of the desired craniocervical alignment. Intraoperative manipulation of the craniocervical junction using the CMBU, when implemented under fluoroscopy and neuromonitoring, can safely facilitate an enduring anatomic correction of craniocervical deformity in the setting of Grisel syndrome. The dynamic utility of the CMBU for translation of the head and neck obviated the need to apply forces directly to hardware-bone interfaces, and its utility may extend to craniocervical disorders of other etiologies, especially those of a reducible nature and in the setting of poor bone quality and joint laxity.