Craniovertebral junction database analysis: incidence,classification, presentation,and treatment algorithms

INTRODUCTION: The initial treatment of bony lesions at the craniocervical junction consisted of posterior decompression with enlargement of the foramen and removal of the posterior arch of the atlas and axis vertebra. This was regardless of the site of compression. Needless to say, the results were poor when an irreducible ventral lesion at the craniocervical junction caused compression of the cervicomedullary area. It was predictable that an adverse outcome would occur in approximately 35-40% of patients treated with dorsal decompression with a fixed anterior abnormality at the craniovertebral junction. Thus, it became essential to identify the causes of failure and recognize the pathology as well as attempt to improve the treatment. The need to identify natural history led to a prospective database. This database now lists 5,300 patients who were analyzed. Of these, 2,000 were children and this has been reviewed here. CRANIOVERTEBRAL ABNORMALITIES IN CHILDREN: Review of symptoms showed that children with craniovertebral abnormalities present with failure to thrive, weakness, basilar migraine, dysphagia, sleep apnea, scoliosis, and the cervical central cord syndrome. Torticollis was seen in the very young. IMAGING TECHNIQUES AND TREATMENT FOR CRANIOVERTEBRAL ABNORMALITIES: New imaging techniques were utilized as they became available. Unexplained neurological symptoms and signs associated with craniovertebral abnormalities in children required angiography which was performed with dynamic motion studies. All children with reducible craniocervical abnormalities underwent stabilization. In irreducible abnormalities, decompression was paramount with a fusion to be done immediately. The decompression was accomplished in the direction in which encroachment occurred. TREATMENT ALGORITHM AND CLASSIFICATION: A treatment algorithm was formulated as a result of this database and a classification of the craniovertebral abnormalities; that holds good for the entire spectrum.     

Occipitocervical fixation in the surgical treatment of pathologies of the craniovertebral junction

BACKGROUND AND PURPOSE: New surgical approaches to the tumors of the skull base necessitate removal of certain bone structures of the base. In the case of the transcondylar far-lateral approach, the whole occipital condyle and lateral mass of C1 are removed resulting in iatrogenic instability. Consequently, an occipitocervical fixation is needed. The aim of the study was to assess the usefulness of the CCD cervical system for the occipitocervical fixation in the surgery of the tumors of the craniovertebral junction. MATERIAL AND METHODS: The authors present the application of the CCD cervical system produced by Medtronic Sofamor-Danek in three patients who underwent surgery of tumors of the foramen magnum, clivus or craniovertebral junction. The system consists of two titanium rods, which adjusted to the curvature of the spine and the occiput, are fixed by laminar cervical and occipital hooks, fixed to the trepanation hole edge or the edge of the foramen magnum. It is also possible to use screws driven to the occipital bone if it is more than 7 mm thick. RESULTS: The system fixation is simple and relatively short. Good stabilization was achieved in all patients. No complications caused by the implants have been observed. CONCLUSIONS: The CCD cervical system can be successfully used for the occipitocervical fixation in a lateral transcondylar approach and in the cases of other non-traumatic craniocervical instability. It is especially recommended in cases of unusual thinning of the occipital bone or in osteoporotic states.     

Tactics of surgical treatment for an inflammatory process of C0-C(I)-C(II) segments in the craniovertebral joint instability

Pathological processes of C0-C(I)-C(II) segments, such as the clivus, atlas arch, dens, and body of the C, vertebra pose major problems for diagnosis and treatment. On removal of a sizable pathological focus, there may be instability of the craniovertebral segment, secondary displacement, and spinal compression syndrome. Therefore these patients frequently need stabilization of the craniovertebral transition. Patients with initial instability of the craniovertebral junction and subluxation of C(I)-C(II) segments are an intricate problem. The authors describe a rare case of an inflammatory process of C0-C(I)-C(II) segments with craniovertebtal junction instability. A differential diagnosis was made between chordoma, osteoblastoma, and an inflammatory focus at the above site. The following tactics was undertaken: the first stage was occipitospondylosynthesis with "Vertex" system with osteoplasty under Halo-traction; the second stage included transoral removal of a pathological removal of the dens and body of the C(II) vertebra, left lateral mass of the CI vertebra, and lower clivus. After removal of the pathological focus, there were portions of the abnormally changed bone with rarefaction without tumor tissue. Histologic studies revealed the signs of a chronic inflammatory process. A five-month follow-up showed that neurological symptoms and craniovertebral junction instability regressed. The control computed tomography made 5 months after surgery demonstrated the radical elimination of the pathology and the absence of C, vertebral subluxation. Thus, there is evidence for the tactics of successive operations (a stabilizing operation--posterior occipitospondylosynthesis (desirably under Halo-traction), followed by removal of a pathological focus via transoral access) in pathological processes of the craniovertebral C0-C(I)-C(II) in cases of initial craniovertebral junction instability.     

Fusions at the craniovertebral junction

INTRODUCTION: The surgical management of craniovertebral junction instability in pediatric patients has unique challenges. While the indications for internal fixation in children are similar to those of adults, the data concerning techniques, complications, and outcomes of spinal instrumentation comes from experience with adult patients. Diminutive osseous and ligamentous structures and anatomical variations associated with syndromic craniovertebral abnormalities frequently complicates the approaches and limits the use of internal fixation in children. Cervical arthrodesis in the pediatric age group has the potential for limiting growth potential and causing secondary deformity. Recent advances in image analysis have enabled preoperative planning which is critical to evaluate the size of instrumentation and its relation to the patient's anatomy. Newer techniques have recently evolved and have been incorporated in the management of pediatric patients with requirement for craniocervical stabilization. MATERIALS AND METHODS: Over 750 craniovertebral junction fusions have been reviewed in children. The indications for atlantoaxial arthrodesis were: (a) absent odontoid process, dystopic os odontoideum, absent posterior arch of C1; (b) Morquio's syndrome, Goldenhar's syndrome, Conradi's syndrome, and spondyloepiphyseal dysplasia. The acquired abnormalities of trauma, postinfectious instability, and Down's syndrome completed the indication in children. The indications for occipitocervical fusion were: (a) anterior and posterior bifid C1 arches with instability, absent occipital condyles; b) severe reducible basilar invagination, unstable dystopic os odontoideum, and unilateral atlas assimilation; (c) acquired phenomenon with traumatic occipitocervical dislocation, complex craniovertebral junction fractures of C1 and C2, after transoral craniovertebral junction decompression, cranial settling in Down's syndrome and inflammatory disease such as Grisel's syndrome. Instability was seen in children with clivus chordoma and osteoblastoma. Atlantoaxial fusions were performed mainly with interlaminar rib graft fusion and more recently with the transarticular screw fixation in the older patient. In the teenager, lateral mass screws at C1 and rod fixation were made; C2 pars interarticular screw fixation and C2 pedicle screw fixation. A C2 translaminar screw fixation is described. Occipitocervical fusions were made utilizing rib grafts below the age of 6. A contoured loop fixation was made in children above the age of 7, and recently, rod and screw fixation was also utilized. RESULTS: Abnormal cervical spine growth was not seen in children who underwent craniocervical stabilization below the age of 5. The authors have reserved rigid instrumentation for children above the age of 10 years and dependent on the anatomy.     

Transoral decompression for craniovertebral osseous anomalies: perioperative management dilemmas.

The surgical outcome of 74 patients, who underwent transoral decompression (TOD) for ventral irreducible craniovertebral junction anomalies between January 1989 to September 1997, was studied to evaluate the perioperative complications and problems encountered. The indications for TOD included irreducible atlantoaxial dislocation (n=24), basilar invagination (n=16), and a combination of both (n=35). Following TOD, occipitocervical stabilization using Jain's technique was carried out in 50 (67.5%) and atlantoaxial fusion using Brooks' construct in 18 (24.3%) patients. The pre- and postoperative radiology was compared to assess the adequacy of decompression and stability. The major morbidity included pharyngeal wound sepsis leading to dehiscence (20.3%) and haemorrhage (4%), valopharyngeal insufficiency (8.1%), CSF leak (6.7%) and inadequate decompression (6.7%). Neurological deterioration occurred transiently in 17 (22.9%) and was sustained in 7 (9.4%) patients. The mortality in six cases was due to operative trauma, exanguination from pharyngeal wound (one each), postoperative instability and inability to be weaned off from the ventilator (two each). Of the 47 (63.5%) patients available at follow up ranging from 3 months to 2 years, 26 (55.3%) showed improvement from their preoperative status while 14 (29.8%) demonstrated stabilization of their neurological deficits. Seven (14.9%) of them deteriorated. Though TOD is logical and effective in relieving ventral compression due to craniovertebral junction anomalies, it carries the formidable risks of instability, incomplete decompression, neurological deterioration, CSF leak, infection and palatopharyngeal dysfunction.     

Craniovertebral junction neoplasms in the pediatric population

INTRODUCTION: The incidence of tumors at the craniovertebral junction in the pediatric population is low. Because of the variable pathology and the rarity of these tumors, ideal therapies are only now being defined. MATERIALS AND METHODS: Thirty-eight children with tumors affecting the craniocervical junction were encountered between 1991 and 2006. These comprised neoplasms of osseous origin and neural extramedullary tumors. RESULTS AND DISCUSSION: Chordomas of the clivus and foramen magnum were seen in eight, fibrous dysplasia in four, aneurysmal bone cysts in four, eosinophilic granuloma affecting the atlas and axis vertebra in four, Ewing's sarcoma involving the atlas in two, osteoblastoma in two, neurenteric cysts in four, meningioma in five, schwannoma in two, and plexiform neurofibromas in three. The location of these tumors was predominantly ventral, and a very small number had a lateral or dorsal location. The ventral tumors included chordoma, meningioma, fibrous dysplasia, aneurysmal bone cyst, and osteoblastoma. Plexiform neurofibroma affecting the craniocervical junction was ventral to the clivus and upper cervical spine causing severe kyphosis of the craniocervical region. Pain in the head and neck occurred in 70%. Paresthesias and dysesthesias in the hands were seen in 40% and spastic weakness of extremities in 22%. Cranial nerve palsies were seen in 33%. Twenty-eight percent of children showed dysphagia or dysarthria. The cranial nerves affected were the vagus followed by hypoglossal and glossopharyngeal nerves. This led to dysphagia, slurred speech, repeated aspiration pneumonia, and weight loss. The most common findings for chordomas at the craniocervical junction were isolated hypoglossal nerve palsy. All individuals underwent magnetic resonance imaging, computed tomography, and 3D computed tomography and angiography. Vertebral angiography was used to understand the dynamics of collateral circulation and tumor vascularity. Tumor embolization was performed in chordoma and aneurysmal bone cysts. Our experience and results are presented here.     

齿状突切除术后颅颈连接不稳定的处理

目的探讨术前未能明确诊断，而齿状突切除术后颅颈连接不稳定的诊断和治疗方法。方法回顾性分析术后8例不稳定病例的诊断治疗经过。结果7例经枕下减压，枕颈融合固定取得了满意效果。无并发症及死亡病例。结论齿状突切除术后颅颈连接不稳定可以通过枕下减压，枕颈融合治疗。     

Damaged ligaments at the craniocervical junction presenting as an extradural tumour: a differential diagnosis in the elderly.

An extradural mass at the craniocervical junction causing progressive neurological disability in five elderly patients is described. The lesion, which might be confused with a meningioma or other tumour, is composed of amorphous degenerate fibrocartilaginous material and could be due to degeneration of the ligaments responsible for atlanto-axial stability. Recognition of the condition early is important as the patient's clinical condition will deteriorate without decompression. Anterior transoral removal is relatively simple, unlike surgery for tumours in the area, and will not destabilise the craniovertebral junction. It is likely that a proportion of these lesions are undetected, misdiagnosed or untreated to the detriment of the patient.     

经口咽前路寰枢椎复位钢板置入内固定治疗颅颈交界部畸形的MRI评价

背景：颅颈交界部畸形经口咽前路寰枢椎复位钢板置入内固定治疗过程中,X射线、CT对于颅颈交界部病变的检查只能显示其骨性结构,无法显示脊髓情况。 目的：分析颅颈交界部畸形经口咽前路寰枢椎复位钢板内固定前后的磁共振图像,探讨MRI成像对其内固定术式的选择及效果判断的价值。 方法：选择2009-06/2010-04收治的颅颈交界部畸形患者25例,男9例,女16例,术前均行X射线、CT、MRI检查,采用经口咽前路寰枢椎复位钢板置入内固定治疗方式,置入后6个月内行MRI复查。 结果与结论：所有患者均顺利完成内固定,未出现脊髓血管损伤等严重并发症。25例患者置入后MRI成像显示内固定在位,寰枢椎复位,脊髓受压情况改善,11例随访患者中,原上颈椎局部疼痛、活动受限等情况有9例明显改善或消失。提示MRI能够多方位清晰显示颅颈交界部畸形情况,对脊髓及颅内病变的显示更直接,可为颅颈交界部畸形经口咽前路寰枢椎复位钢板置入内固定手术方式的选择、脊髓受压情况及手术效果的判断、术后恢复提供客观依据。     

Long-term follow up of transoral anterior decompression and posterior fusion for irreducible bony compression of the craniovertebral junction

The aim of this study was to examine the long-term outcomes of treating irreducible craniovertebral junction bony compression via the transoral approach, with a minimum of 4 years of follow-up. Between 1996 and 2005, the transoral transpharyngeal approach was used for 10 patients who underwent anterior decompression and posterior fusion and two who underwent only anterior decompression. All patients were monitored intraoperatively using combined somatosensory/motor evoked potentials. All patients reported substantial or complete resolution of pain. Six out of the eight patients with preoperative myelopathy experienced an improvement of one Ranawat classification level. Solid fusion was achieved in all patients, and no patient had iatrogenic neurological deficits or cerebrospinal fluid leaks. Two patients experienced wound dehiscence requiring resuturing, one experienced velopalatine incompetence, and one experienced wound infection. There was no re-growth of the bony lesion during follow up. Transoral repair provided generally acceptable long-term results for irreducible craniovertebral junction bony compression. Intraoperative spinal cord monitoring may be useful for preventing neurological complications.     

High cervical C3-4 'disc' compression associated with basilar invagination

A 20-year-old male had torticollis and short neck since birth. He presented with symptom of progressive quadriparesis over a two-year period. Investigations revealed basilar invagination with marked rotation in the craniovertebral region and relatively large C3-4 region osteophytes. Serial MRI over two years showed persistent signal opposite C3-4 disc space suggestive of cord compression. Although the cord was humped over the odontoid process, there was no clear radiological evidence that the cord was compromised at this level. During surgery, instability was identified only at the craniovertebral region and not at the level of C3-4. Distraction of the lateral masses of atlas and axis and fixation using interarticular spacers and bone graft and direct screw implantation in the lateral mass of the atlas and pars of the axis resulted in reduction of the basilar invagination and of atlantoaxial dislocation. The patient had marked clinical recovery, despite the fact that no direct procedure was done for C3-4 disc decompression. The case suggests that C3-4 disc changes could be secondary to primary instability at the craniovertebral junction.     

The case of severe craniocervical dislocation due to rheumatoid arthritis

The authors describe a case of craniocervical dislocation secondary to rheumatoid arthritis producing important canal narrowing: ventrally by migrated odontoid and dorsally by posterior arch of C-1 with medullary compression. Symptoms of hyperreflexia, spasticity and left hemiparesis with Babinski sign were present. Surgical procedure: transoral odontoidectomy was performed followed by suboccipital approach, C-1 laminectomy and occipitocervical fixation (Olerud device and bone graft). Outcome with neurologic improvement. CONCLUSIONS: Transoral odontoidectomy combined with occipitocervical decompression and fixation is effective approach for treatment of severe craniocerebral dislocation. Its advantages: ventral and dorsal decompression combined with immediate stabilisation.     

Problems in the selection of surgical approach for the treatment of non-traumatic deformities of the craniocervical junction

Developmental abnormalities or inflammatory disorders provoke deformations and instability of the craniocervical junction. The most dangerous results of these lesions are: sudden brainstem compression or cervical myelopathy. The authors propose the guidelines for surgical management of non-traumatic deformities caused by: a) rheumatoid arthritis of the spine, b) congenital anatomic changes of the occipit and odontoid. Main goals of surgical treatment are decompression and stabilization. The choice of surgical approach and method depends on pathology. It is very important to estimate individual anatomic changes and mobility--possibility of reduction. The authors discuss surgical methods actually used for fusion and decompression of the occipitocervical junction.     

Craniocervical developmental anatomy and its implications

Introduction  Congenital and developmental osseous abnormalities and anomalies that affect the craniocervical junction complex can result in neural compression and vascular compromise and can manifest itself with abnormal cerebrospinal fluid dynamics. An understanding of the development of the craniocervical junction is essential to recognize the pathological abnormalities. Materials and methods  Atlas assimilation, segmentation failures, os odontoideum, basilar invagination, and the various syndromes that affect the craniocervical junction have been analyzed. The natural history provides an added insight into its treatment. Results  Proatlas segmentation abnormalities surrounded the foramen magnum and the posterior arch of C1. Hindbrain herniation was associated in 33 of the 90 children involved. Spastic quadriparesis presented in 80% and lower cranial nerve abnormalities in 33%. Vertebrobasilar dysfunction was observed in 40% and trauma presentation seen in 60% of individuals. Atlas assimilation was present in 550 individuals who were evaluated for craniovertebral junction abnormalities. Hindbrain herniation occurred in 38%. Segmentation failure of C2 and C3 vertebrae compounded the abnormal dynamics resulting in atlantoaxial instability. This was a reducible instability with formation of pannus around the odontoid process until it became irreducible at approximately 14 years of age. Unilateral atlas assimilation caused torticollis in children. Os odontoideum was investigated regarding craniocervical trauma at a young age. Conclusion  The conclusion was that os odontoideum was associated with an unrecognized fracture in children below the age of 5 with a previously normal odontoid structure as observed in our series. Atlas and axis abnormalities were reviewed in this series. This large database has provided an understanding of the natural history of many entities and allowed treatment protocols to be established that have stood the test of time.     

Chiari malformation and odontoid panus causing craniovertebral stenosis in a child with Crouzon’s syndrome

Crouzon's disease is a well-known disorder affecting multiple organ systems, specifically a craniofacial disorder with highly variable penetrance and severity of deformity. Crouzon's patients typically have anomalies of the skull base leading to gross distortion of the cranium and in some cases the cervicocranium. We present a 5-year-old girl with Crouzon's disease who suffered from an acquired Chiari I malformation after insertion of a ventriculoperitoneal shunt and a coexistent ventral odontoid panus. Both these lesions were causing cervicomedullary compression. The literature is controversial on the surgical management of anterior and posterior compression at the craniocervical junction. We review the literature on surgical options for decompression at the craniocervical junction and offer our surgical case as a treatment option for patients in this rare clinical situation.     

Morphometric analysis of human occipital condyle

OBJECTIVE: The human occipital condyle is the unique bony structure connecting the cranium and the vertebral column. The progress in neuroimaging techniques has increased interest for aggressive craniovertebral surgery. Such surgery requires the knowledge regarding anatomical aspects of the craniovertebral junction. The aim of the present study is to analyze the occipital condyle morphometrically. MATERIAL AND METHODS: 404 occipital condyles of 202 dry skulls were used for this study. Twenty-seven parameters were measured, including length, width and height of occipital condyle, the distances between the occipital condyle and hypoglossal canal, as well as some important condyle-related angles. RESULTS: The length, width and the height of the occipital condyle were found to be 23.4, 10.6, and 9.2 mm, respectively. The anterior and posterior intercondylar distances are 21.0 and 41.6 mm, respectively. Sagittal intercondylar angle was 59.3 degrees. The intracranial orifice of the hypoglossal canal was found in the junction of the second and third quarter on the condyle in more than 55% of specimens. The shape of occipital condyles was classified into eight types as follows--type 1: oval-like condyle; type 2: kidney-like condyle; type 3: S-like condyle; type 4: eight-like condyle; type 5: triangle condyle; type 6: ring-like condyle; type 7: two-portioned condyle and type 8: deformed condyle. The most common type was type 1 (50%), whereas the most unusual type was type 7 (0.8%). CONCLUSION: It is concluded that the occipital condyle may present various shapes, length, width, and orientation, requiring a careful radiological analysis before craniovertebral junction surgery.     

Management of cervicomedullary compression in patients with congenital and acquired osseous-ligamentous pathologies.

We present our experience in the diagnosis, surgical management and long-term follow-up of congenital and acquired osseous-ligamentous abnormalities or pathologies of the craniovertebral junction. The purpose of this study was: (i) to determine the incidence and degree of cervicomedullary compression in pediatric and young adult patients with congenital and acquired abnormalities, and (ii) to correlate cervicomedullary compression with other imaging and clinical factors to determine to what extend cervicomedullary compression is successfully treated with a posterior decompressive procedure, transoral decompression, and medical management. Between January 1995 and December 2004, 26 cases were managed in our department. These patients had: rheumatoid arthritis (RA) (3); traumatic injury (2); congenital basilar impression (5, in 2 cases a posteriorly oriented or retroflexed odontoid); infection (10); craniovertebral junction Pott's disease (9); os odonteideum (3); condylus tertius (1); and tumor (2). Six of the patients (23.1%) had syringomyelia. Only three (11.3%) were in the pediatric age group. Symptoms and signs included headache (72%), ataxia (38%), lower cranial nerve dysfunction (54%), quadriparesis (44%), hyperreflexia (76%), Hoffman positivity (72%), achilles clonus (72%) nystagmus (33%) and dysphagia (22%). The mean follow-up time was 44 months (range 3-85). Twelve (46.2%) had undergone posterior fossa decompression; seven (26.6%) had ventral decompression. Seven of the patients (26.6%) had medical management. The major morbidity included pharyngeal wound sepsis leading to dehiscence (3.8%), valopharyngeal insufficiency (3.8%), cerebrospinal fluid leakage (3.8%), postoperative macroglossia (3.8%) and inadequate anterior decompression (3.8%). Transient neurological deterioration occurred in two patients (7.6%). Our management paradigm will result in some neurologic improvements and limit the progression of symptoms. Patients with these pathologies are likely to show a good neurologic outcome when treatment, whether with or without surgery, is administered early in the course of the disease.     

A case of progressive myelopathy with atlas hypoplasia in an infant: controversy in the surgical strategy

Case report The authors report a case of isolated atlas hypoplasia in an 18-month-old infant who experienced delayed motor development confined to the lower extremities, in the absence of other clinical features. Nonetheless, MRI revealed the upper cervical cord to be highly compressed with a high signal intensity lesion apparent at the craniovertebral junction. The patient underwent atlas laminectomy in addition to occipital decompression, resulting in a good neurological recovery.Discussion We speculate on the significance of this unique clinical presentation and discuss surgical strategies for this condition.     

Surgical approaches: postoperative care and complications “transoral–transpalatopharyngeal approach to the craniocervical junction”

INTRODUCTION: The ventral approach to the craniocervical border has been described for decompression of irreducible extradural pathology. The procedures utilized encompass the transoropharyngeal and median mandibulotomy with glossotomy and the transpalatal procedures. This study was aimed to review the utility of the transoral-transpalatopharyngeal approach. CLINICAL MATERIALS AND METHODS: Seven hundred thirty-three patients underwent transpalatopharyngeal approach for decompression of the brain stem and cervicomedullary junction. Of these, 280 were children below the age of 16 years. The main indication was irreducible ventral pathology compressing the brain stem and cervicomedullary junction. Two hundred two children had irreducible basilar invagination, 28 had proatlas segmentation abnormalities, os odontoideum with a dystopic os odontoideum in 30, and spinal tumors in seven (chordoma, fibrous dysplasia, osteoblastoma). Seven patients with Down's syndrome and irreducible bony compression of the ventral cervicomedullary junction were seen. There were six other miscellaneous diagnoses. All children required craniocervical stabilization which was carried out under the same anesthetic as the transoral procedure. OPERATIVE PROCEDURE: The procedure entailed fiber-optic intubation. The patient was placed in cervical traction prior to the anterior procedure. The soft palate was split only in individuals with a short clivus with a high riding clivus-odontoid articulation. Craniocervical stabilization was performed in the prone position under the same anesthetic. RESULTS: There was one retropharyngeal infection postoperatively. No cesium fluoride leaks were encountered. Velopalatine incompetence was seen in five children who already had preoperative brain stem dysfunction. Neurological recovery was the rule. Patients who had preoperative syringohydromyelia had resolution of the syrinx on postoperative magnetic resonance imaging. DISCUSSION: The author's technique is described. Since 1977, the procedure has been performed in 732 patients (280 children) and has evolved into a safe and direct approach to the ventral cervicomedullary junction with minimal morbidity and mortality.     

Craniovertebral Junction Tuberculosis with Atlantoaxial Dislocation : A Case Report and Review of the Literature

Craniovertebral junction (CVJ) tuberculosis is a rare disease, potentially causing severe instability and neurological deficits. The authors present a case of CVJ tuberculosis with atlantoaxial dislocation and retropharyngeal abscess in a 28-year-old man with neck pain and quadriparesis. Radiological evaluations showed a widespread extradural lesion around the clivus, C1, and C2. Two stage operations with transoral decompression and posterior occipitocervical fusion were performed. The pathological findings confirmed the diagnosis of tuberculosis. Treatment options in CVJ tuberculosis are controversial without well-defined guidelines. But radical operation (anterior decompression and posterior fusion and fixation) is necessary in patient with neurological deficit due to cord compression, extensive bone destruction, and instability or dislocation. The diagnosis and treatment options are discussed.