Craniovertebral Junction Tuberculosis Research Articles

Surgical management of craniocervical junction tuberculosis with atlanto-axial dislocation – A case report and review of the literature

Background: There are few guidelines on how to best manage craniovertebral junction (CVJ) tuberculosis (TB). Certainly, timely tissue diagnosis, immobilization of the neck, and decompression of CVJ with appropriate stabilization are the mainstays of treatment for TB at the CVJ. Case Description: Three patients, ages 16–68, presented with CVJ TB with atlanto-axial dislocation responsible for progressive quadriparesis/plegia. Based on X-rays, magnetic resonance, and computed tomography studies, patients underwent timely decompressions and fusions followed by antitubercular drug treatment. Conclusion: Early diagnosis, proper decompression with fusion, treated with anti-TB drug for proper period were keys to managing TB involving the craniocervical junction in these three patients.

Pathogenesis and Staging of Craniovertebral Tuberculosis: Radiographic Evaluation, Classification, and Natural History.

Retrospective cohort. To radiographically evaluate Craniovertebral junction (CVJ) tuberculosis infection pathogenesis and to propose a modification to the Lifeso classification. A cohort of patients with radiologically or microbiologically identified CVJ tuberculosis treated at a single tertiary referral center in a TB endemic area was queried for characteristics about clinical presentation, treatment, and radiographic evidence of bone destruction and abscess formation were included. Disease was classified according to the Lifeso grading system and bony lesions were classified as either type 1 (preservation of underlying structure) or type 2 (damage of underlying structure). 52 patients were identified (mean age 28.5 ± 13.4yr, 48% male; 14% with a prior history of tuberculosis). All presented with neck pain at presentation, 29% with rotatory pain, and 37% with myelopathy. Comparison by Lifeso type showed Lifeso III lesions had longer symptom durations (P = .03) and more commonly had periarticular or predental abscess formation (P < .05), spinal cord compression (P < .01), and more commonly involved the C2 body and atlanto-dental joint. Underlying bony destruction was more common for lesions of the lateral atlantoaxial joints and atlanto-dental joints in Lifeso III cases than in either Lifeso I or II (all P < .05). The radiologic findings of the present series suggest CVJ TB infection may originate in the periarticular fascia with subsequent invasion into the adjacent atlanto-dental and lateral atlantoaxial joints in later disease. To reflect this proposed etiology, we present a modified Lifeso classification to describe the radiologic pathogenesis of CVJ TB.

Management of CVJ tuberculosis: the changing paradigm.

The literature seems fractured for the management of craniovertebral junction (CVJ)-tuberculosis (TB). Presently, non-surgical management has been in vogue for neurologically intact patients. On the contrary, severely disabled cases of CVJ-TB continue to attract discussion, tilted towards surgical intervention. We present our experience with the non-surgical management of CVJ-TB tailored to their neurological status. Authors managed 37 cases (2004-2019; age 1-57 years, mean 36 years) of CVJ-TB, of which eighteen (18/37, 48.6%) were severely disabled (Nurick grade ≥ 3) with a mean follow-up of 84 months (48-192 months). Irrespective of the clinical status and radiological findings, all patients were managed on medical management only. Needle aspiration established pathology in 23 (62.2%) cases, while 9 (24.3%) cases required drainage of an abscess. All patients received 18 months of anti-tubercular therapy (ATT). In patients with Nurick grade ≥ 3 and documented AAD, we applied halo vest for 12 months to achieve cervical immobilization. Only hard cervical collar for 3 months was prescribed in patients with no documented AAD. All minimally disabled cases (Nurick grade ≤ 2, n = 19) responded favorably (n = 18) to ATT, except for an infant, who succumbed to irreversible hypoxic brain damage due to the obstructed aero-digestive gateway. Of the severely disabled cases (Nurick grade ≥ 3, n = 18), 16 cases had favorable outcomes with only external orthosis (12) and 18-month ATT. One patient succumbed to multiple cerebral infarcts, while one required realignment surgery at CVJ due to fusion in malaligned position. The authors conclude that the disability grading of CVJ-TB is pertinent only for assessing the functional disability of patients at presentation, with minimal relevance in deciding its management strategy. Irrespective of neurological disability, almost all patients respond favorably to external immobilization and ATT.

Craniovertebral junction tuberculosis with Subluxation

Tuberculosis of the Craniovertebral junction is a rare and potentially dangerous condition. Neurological complications are more serious and common than with that of other vertebrae. Hence the need for early recognition and appropriate treatment. Management of craniovertebral junction tuberculosis has been an ever debatable topic of discussion, varying from purely conservative to surgical interventional protocols with no proper consensus. Presenting here 10 cases of occipitocervical tuberculosis with destruction of the occipital condyle with subluxation with or without retropharyngeal abscess formation, managed surgically by posterior occipito-cervical stabilization and fusion with a review of literature

Tuberculous Spondylitis of the Craniovertebral Junction.

Craniovertebral junction tuberculosis is rare, accounting for 0.3 to 1% of all tuberculous spondylitis cases. MR imaging is the modality of choice to detect bone involvement, abscess formation and subligamentous spreading of the pus, to differentiate from other lesions affecting the craniovertebral junction, and to determine the efficacy of treatment. Given the fact that surgical treatment of patients with craniovertebral junction tuberculosis has been associated with a high mortality rate ranging up to 10% and recurrence rate ranging up to 20%, conservative is the standard of treatment for most patients.This article presents a patient with craniovertebral junction Mycobacterium tuberculosis infection diagnosed with CT-guided biopsy. A halo vest was applied and antituberculous treatment with rifampicin, isoniazid and ethambutol was initiated. At 6-month follow-up, the patient was asymptomatic; CT of the cervical spine showed healing of the bony lesions. The halo vest was removed and physical therapy was recommended. Antituberculous treatment was continued for a total of 18 months, without any evidence of infection recurrence

Craniovertebral tuberculosis in children: experience of 23 cases and proposal for a new classification.

The aim of this study was to review cases of paediatric patients with craniovertebral junction (CVJ) tuberculosis with a view to try and stratify the cases into different groups which would help plan treatment and hence develop a protocol for treatment of a fairly uncommon condition still widely seen in the developing world. Twenty-three cases of paediatric craniovertebral tuberculosis had their clinical features and radiology reviewed. The treatment plan in each case was analyzed according to their presentation. The results of treatment after 1 year were assessed in each case. The clinical presentations varied from neck pain with hypoglossal nerve palsy to frank spastic quadriparesis, and frank instability at the atlanto-axial junction was seen in five out of 23 patients. Cold abscesses were seen in 18/23 cases, and severe torticollis was the presentation in 6/23. We graded the children with this condition into three groups: 1) those with instability and gross neurodeficit who required early operative intervention (1 needed trans-oral decompression with posterior fixation and 4 required posterior fixation) 2) those who had severe torticollis and large cold abscesses who were treated with trans-oral aspiration of cold abscess followed by neck immobilization (6/23) and 3) those who did not have significant neck muscle spasm or torticollis and who were treated with immobilization alone (12/23). Only one child in group 3 required delayed intervention for instability which developed after completion of the course of anti-tubercular medication. We concluded that children with craniovertebral tuberculosis should be treated according to their clinical presentation coupled with evidence of radiological instability. Those with gross deficit and instability need early stabilization, those with minimal deficit and no instability but severe pain or torticollis need aspiration of the cold abscess with external immobilization, whereas those without deficit, instability or severe pain may be managed by external orthoses alone. Of course, medical treatment for tuberculosis is necessary in each case.

Pediatric cranio-vertebral junction tuberculosis: management and outcome

Tuberculosis (TB) of the cranio-vertebral junction (CVJ) is a rare condition, accounting for 0.3% to 1% of all cases of spinal TB. Early diagnosis and treatment are important in preventing long-term neurological sequelae. Management protocol of this rare site of TB is yet to be conclusively established. This holds particularly true for pediatric age group in which this condition is infrequently encountered. A total of 29 consecutive pediatric patients presented to the Department of Neurosurgery at Sanjay Gandhi Post-Graduate Institute of Medical Sciences, Lucknow, from January 1997 to October2011 with clinical and/or radiological features suggestive of CVJ TB. A clinical grading system to evaluate the neurological status was developed, and all patients were evaluated using this scoring system. Patients were radiologically evaluated with computed tomography (CT) of CVJ and magnetic resonance imaging (MRI) with gadolinium enhancement. These cases were managed according to their grade and followed up. Out of a total of 29 cases, 18 were females and 11 males. Age range was 4 to 18years with mean age 9 ± 3.8years. The follow-up period ranged from 2months to 7.5years with mean follow-up of 2.7years. Eleven cases were of grades 1 and 2, and 18 cases were of higher grade (grades 3 and 4). Predominantly conservative approach was utilized in cases with better clinical status, and grade (grades 1 and 2) and surgical intervention was needed in the more severe grades. All cases had significant improvement at the last follow-up. One needs to have a high index of suspicion of CVJ TB if one encounters a case with neck pain, neck restriction, and raised erythrocyte sedimentation rate. CT CVJ and MRI with gadolinium contrast enhancement are the investigations of choice for both establishing a diagnosis and planning the management. For cases with mild neurological deficit, conservative approach would work for majority of cases, and for severe cases, initial conservative approach may be tried, failing which surgical intervention would be needed.

Tuberculosis of the craniovertebral junction

Tuberculosis (TB) of craniovertebral junction (CVJ) occurs in 1-5% of cases of TB spondylitis. This can be a life-threatening condition due to mass effect of infective process or resultant instability. Surgical indications for TB of CVJ are not clear from literature. We have reviewed all the patients with TB of CVJ admitted at our center between 2005 and 2010. There were 15 patients including 10 males and 5 females. Average age was 38years and average duration of symptoms was 8months. All patients were started on multidrug antituberculous chemotherapy and skull traction. Those patients who failed to respond in 4-6weeks and had persistent instability or neurological deficit were offered surgery. Rest was treated conservatively by immobilisation or traction. All five patients who were surgically treated had occipitocervical fusion (OCF) with titanium screws and plate/rod construct combined with posterior decompression if needed. Only one patient needed anterior surgery in addition to OCF at a later stage. All patients improved neurologically whether they were treated surgically or conservatively. Only difference was that surgically treated patients had earlier pain relief, mobilisation, neurological improvement and lesser complications. We recommend that all patients with TB of CVJ with instability and neurological compromise, who fail to respond to 4-6weeks of antituberculous chemotherapy and skull traction should be offered occipitocervical fusion with or without posterior decompression. Anterior surgery will be needed only in those few cases who do not improve neurologically after OCF.

Structural Odontoid Lesions in Craniovertebral Tuberculosis

A retrospective chart review. To describe the presentation and the rationale for management of pathological odontoid fracture and complete odontoid destruction in craniovertebral junction tuberculosis (CVJ TB). Presentation of CVJ TB ranges from minor osteomyelitic changes to severe structural damage leading to instability. Structural damage to the odontoid process is poorly characterized in the literature. Inadequate knowledge about the radiological presentations has led to controversy in the management of CVJ TB. The cohort consisted of 15 consecutive patients with CVJ TB, with structural damage to the odontoid process in the form of either odontoid fracture (n = 7) or complete odontoid destruction (n = 8). These patients presented with pain, neurological deficit, torticollis, dysphagia, or respiratory distress. The cause of neurological deficit was craniocervical instability characterized as anterioposterior (n = 15), rotatory (n = 4), and vertical (n = 6). Displacement reduced anatomically in 13 patients. Apart from antibiotics, all patients were treated surgically by either C1-C2 fusion (n = 7) or occipitocervical fusion (n = 8). Average duration of follow-up was 3.6 years (range, 1.5-8 yr). All patients achieved normal neurological status. No complications were noted, except for 1 case, who had a loss of reduction after the use of Hartshill rectangle for occipitocervical fusion. Postoperative computed tomographic scan showed nonunion of odontoid fracture in 2 of 4 patients. No patient of odontoid destruction, of the 5 investigated, revealed structural reformation of the dens. CVJ TB can severely damage the odontoid process, resulting in atlantoaxial dislocation. In these patients, surgery restores and maintains the craniocervical alignment and has a predictable outcome compared with conservative therapy. Pathological odontoid fractures have the potential to go into nonunion. Odontoid process once destroyed completely is rarely restored after antibiotic therapy.

Os Odontoideum Complicating Craniovertebral Junction Tuberculosis

A case report and review of literature. To present a rare case of os odontoideum complicating craniovertebral junction (CVJ) tuberculosis (TB) and discuss the treatment outcome. CVJ is a rare site of predilection for Mycobacterium tuberculosis. The treatment protocol for this entity has periodically undergone swings between conservative treatment and radical surgery. To the best of our knowledge, os odontoideum complicating TB of this region has not been reported before in the literature. We report on a 17-year-old patient with CVJ TB in setting of dystopic os odontoideum. The patient presented with fever, cervical pain and weakness of all the four limbs, and was treated with antitubercular chemotherapy along with cervical traction, followed by halo-vest. Patient made uneventful recovery with conservative form of treatment and was asymptomatic at 36 months of follow-up. This case has the originality of being the first report of CVJ TB with dystopic os odontoideum. High index of suspicion by the clinician must be practiced to make the appropriate diagnosis.

The Results of Nonoperative Treatment of Craniovertebral Junction Tuberculosis: A Review of Twenty-six Cases

There is scarce information on craniovertebral junction tuberculosis even in developing countries. The pendulum of treatment in craniovertebral junction tuberculosis has periodically vacillated between nonoperative management and radical surgery. We performed a retrospective review of prospectively collected clinical and radiographic data on twenty-six consecutive patients with craniovertebral junction tuberculosis who were treated nonoperatively in our institution. The patients who had weakness of the limbs, pyramidal signs, or evidence of atlantoaxial dislocation and/or basilar invagination were immediately managed with immobilization with Crutchfield tongs traction (Group A), followed by halo-vest application. Patients without a neurological deficit, pyramidal signs, or atlantoaxial dislocation or basilar invagination were treated with cervical immobilization with early application of a halo vest (Group B). The common presenting features were neck pain, restriction of neck movement, and spastic weakness of limbs. A retropharyngeal purulent fluid collection and osseous involvement of the dens and lateral mass of the atlas were the common radiographic findings. Twelve patients were partially or completely dependent on others for activities of daily living, and marked ligamentous and bone destruction with displacement at the atlantoaxial level was seen in eight patients at the time of presentation. The twenty patients in group A had cervical traction for a mean of 5.9 weeks, and a halo vest was applied for a mean of 6.9 months with antitubercular therapy for eighteen months. The mean follow-up period was 25.2 months (range, eighteen to forty-two months). All of the patients were independent in activities of daily living at the time of the last follow-up. In the Indian subcontinent, the disease process of craniovertebral junction tuberculosis is usually quite advanced at the time of presentation. On the basis of our study, patients with craniovertebral junction tuberculosis can be managed successfully with nonoperative treatment even with advanced involvement of bone or soft tissues at this spinal level. Therapeutic Level IV. See Instructions to Authors for a complete description of levels of evidence.

Odontoid process pathologic fracture in spinal tuberculosis

Craniovertebral junction tuberculosis is a rare lesion in which treatment remains controversial. Options range from conservative treatment to surgery, independently of any associated neurological threat. We here report the first case of pathologic odontoid fracture in a context of spinal tuberculosis, complicated by unusual neurological evolution. The patient presented with non-contiguous multifocal tuberculosis, of which there have previously been only 6 reported cases.

Craniovertebral junction tuberculosis: a case report and review of the literature

Craniovertebral junction tuberculosis (CVJ TB) is a rare disease, potentially causing significant neurological deficits and even death. We report on a 80-year-old woman presenting with CVJ TB without pulmonary involvement. The diagnosis was made by biopsy of the cervical lymph node showing granulomatous caseation necrosis. Despite extensive erosion of the clivus, C1, and C2, and spinal cord compression, the patient was effectively managed with antituberculous drug therapy and conservative neck stabilization. Neck pain resulting from cervical spondylosis is common in elderly people. However, even if there is no obvious pulmonary involvement, CVJ TB should be considered in the differential diagnosis, especially in patients with painful neck stiffness. The most useful method available for evaluating this region is a combination of CT scan and MRI study. CVJ TB can be managed conservatively, except for a selected few cases, regardless of the extent of bony destruction.

CRANIOVERTEBRAL JUNCTION TUBERCULOSIS

To establish a scoring system and management algorithm for patients with diagnosed craniovertebral junction tuberculosis. The specific goals were: 1) to avoid new neurological deficits; 2) to avoid morbidity and compromise in the quality of life associated with prolonged, rigid, cumbersome external immobilization, such as with a sternal occipital mandibular immobilizer brace and halo traction; and 3) to prevent sudden death. All patients diagnosed with craniovertebral junction tuberculosis were studied prospectively over a 3-year period. The initial severity of the disease was evaluated with clinicoradiological grading, and patients were divided into 3 grades. Overall performance status was assessed with disability scoring. Patients with Grade 1 and 2 severity were managed conservatively. Grade 3 patients underwent transoral decompression and posterior fixation. The patients' neurological recovery was evaluated every 4 weeks with disability scoring, along with x-rays, for the initial 3 months and every 2 months thereafter. Of 71 patients, there were 27 Grade 1, thirty-six Grade 2, and 8 Grade 3 patients. Children and young adults comprised 70% of the study population. All Grade 3 patients underwent early surgery. Five Grade 1 and 2 patients (8%) required delayed surgery for reducible atlantoaxial dissociation. The remaining 58 patients (82%) were effectively managed conservatively. The mean follow-up duration was 18.5 +/- 6.2 months. There was no mortality. Use of our proposed scoring system and management protocol allowed both speedy recovery and early mobilization. All patients had good clinicoradiological outcomes regardless of the grade.

An Unusual Case of Craniovertebral Junction Tuberculosis in an Infant

We present, to the best of our knowledge, the first reported case of craniovertebral junction tuberculosis with complete quadriplegia in an infant. To describe report of an unusual case and discuss the difficulties and peculiarities of diagnosis, management, and follow-up of craniovertebral tuberculosis in an infant. Spinal tuberculosis is prevalent in areas where tuberculosis is endemic, and its incidence is on the rise in developed nations with the rising incidence of HIV/AIDS. Although common in children and young adults, spinal tuberculosis is rare in infants. Again, craniovertebral tuberculosis is one of the rarest forms of spinal tuberculosis. The infant presented to us at the age of 9 months with Grade IV quadriplegia. Among many of the clinical differential diagnoses, craniovertebral tuberculosis was suspected only on MRI and proved after fine needle aspiration cytology demonstrated granulomas. The patient was subjected to transoral debridement, immobilized with help of a pair of pillows by either side of the head and multidrug antitubercular treatment was started, which continued for duration of 12 months. At the last follow-up of 1 year, the patient had recovered fully and caught up with the milestones suitable for her age. Craniovertebral tuberculosis is difficult to diagnose and treat in infants. A high index of suspicion is essential for a prompt diagnosis and treatment, which is all the more crucial in this age group.

Craniovertebral Tuberculosis

Retrospective review of 13 cases with craniovertebral tuberculosis treated conservatively. To evaluate the results of conservative treatment of craniovertebral tuberculosis and compare with the literature. Craniovertebral tuberculosis is a rare entity even in endemic countries, and there is no consensus in the literature regarding conservative or surgical management for the same. Reports range from radical surgery to totally conservative approach. We report our experience in treating such patients conservatively. A retrospective review of 13 patients diagnosed with craniovertebral tuberculosis was performed. All patients were treated conservatively with cervical traction for initial 3 months followed by a brace along with multidrug antitubercular drugs for 18 months. All patients responded favorably to conservative treatment. Follow-up averaged 43 months (range, 16-65 months). No patient deteriorated neurologically. All patients had symptomatic improvement. Failure to reduce atlantoaxial dislocation/lateral subluxation of the dens completely was seen in 2 cases. We think that all patients with craniovertebral junction tuberculosis can be managed adequately using conservative means regardless of the extent of bony destruction with a good patient outcome. Surgery should be reserved for only a selective few where diagnosis is in doubt and there is initial severe or progressive neural deficit with/without respiratory distress in presence of documented mechanical compression and documented dynamic instability following conservative treatment.

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.

Craniovertebral Junction Tuberculosis: Is Surgery Required?

Craniovertebral Junction Tuberculosis: Is Surgery Required?

Occipitocervical contoured rod stabilization: Does it still have a role amidst the modern stabilization techniques?

The occipitocervical contoured rod (CR) stabilization for use in craniovertebral junction (CVJ) pathologies is an effective and economical technique of posterior fusion (PF). The various indications for CR in CVJ pathologies are discussed. Retrospective analysis. Fifty-four patients (mean age: 31.02+/-13.44 years; male: female ratio=5.75:1) who underwent CR stabilization are included. The majority had congenital atlantoaxial dislocation (AAD; n=50); two had CVJ tuberculosis; one each had rheumatoid arthritis and C2-3 listhesis, respectively. The indications for CR fusion in congenital AAD were associated Chiari 1 malformation (C1M) (n=29); occipitalized C1 arch and/or malformed or deficient C1 or C2 posterior elements (n=9); hypermobile AAD (n=2); and, rotatory AAD (n=3). Contoured rod as a revision procedure was also performed in seven patients. Most patients were in poor grade (18 in Grade III [partial dependence for daily needs] and 15 in Grade IV [total dependence]); 15 patients were in Grade II [independent except for minor deficits] and six in Grade I [no weakness except hyperreflexia or neck pain]. Twenty-four patients improved, 18 stabilized and six deteriorated at a mean follow-up (FU) of 17.78+/-19.75 (2-84) months. Six patients were lost to FU. In 37 patients with a FU of at least three months, stability and bony union could be assessed. Thirty-one of them achieved a bony fusion/stable construct. Contoured rod is especially useful for PF in cases of congenital AAD with coexisting CIM, cervical scoliosis, sub-axial instability and/or asymmetrical facet joints. In acquired pathologies with three-column instability, inclusion of joints one level above the affected one by using CR, especially enhances stability.

Unusual presentations of craniovertebral junction tuberculosis: a report of 2 cases and literature review

Background CVJ tuberculosis is a described entity requiring challenging ways of management. Severe neck pain, causing restricted neck movements and torticollis, is a characteristic presentation of neurologically asymptomatic suboccipital Pott's disease. Case Description Two patients with unusual CVJ tuberculosis form the basis for the present communication. The first patient presented with tubercular otitis media, causing progressive erosion of the petrous part of temporal bone, and destruction of the occipital condyle, along with the lateral mass of atlas, leading to CVJ instability. This is a first report of such a presentation, according to our knowledge. Detailed bony architectural destruction demonstrable on CT scan has been described. The second patient, with CVJ tuberculosis, presented with skull base syndrome and with multiple cranial nerve palsies. Both patients were managed without surgical intervention and showed clinical and radiological recovery. Conclusion In such patients with unusual clinical presentations, histopathologic examination is necessary to arrive at a correct diagnosis. The management of patients with tubercular involvement of CVJ remains controversial. In the present communication, both the patients were managed successfully with full dose of antitubercular drugs and immobilization.