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 Table of Contents  
Year : 2017  |  Volume : 6  |  Issue : 2  |  Page : 127-131

Atlantoaxial tuberculosis: Outcome analysis

Department of Neurosurgery, Institute of Neurosurgery, Madras Medical College, Chennai, Tamil Nadu, India

Date of Web Publication19-May-2017

Correspondence Address:
Srihari Sridharan
Institute of Neurosurgery, Madras Medical College, Park Town, Chennai, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijmy.ijmy_55_17

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Background: Tuberculous (TB) spondylitis occurs in <1% of patients with TB. Atlantoaxial involvement is extremely rare amounting to 0.3%–1% cases of TB spondylitis. The management of this entity has been fluctuating with time. Various classifications and management protocols have also evolved. We present our experience in managing this entity in 8 patients. Materials and Methods: Retrospective follow-up study of eight patients with atlantoaxial TB over 2 years. They ranged in age from 14 to 33 years with male preponderance. Four required surgical intervention on initial presentation, 2 were successfully treated conservatively, and 2 had to undergo surgery after a trial of conservative management. All of them were started on antituberculous therapy (ATT) for 18 months. Patients were followed up for 8–29 months. Results: Neck pain and restriction of neck movements were standard presenting features. All patients had pulmonary TB confirmed by sputum examination. Radiological investigations formed an integral part of the evaluation of disease, treatment plan, and prognosis. Improvement in symptoms has been documented in all eight. Conclusion: The involvement of the most mobile spinal segment and the potential cervicomedullary compression makes it a disease of utmost importance. Although ATT remains the mainstay of treatment, surgical intervention is needed for stabilizing the joint and decompressing the cervicomedullary junction. Strict adherence to medical advice and optimum surgical intervention tailored for each patient results in the successful management of the disease.

Keywords: Atlantoaxial tuberculous, craniovertebral junction tuberculous, tuberculosis c1c2

How to cite this article:
Sridharan S, Arumugam T. Atlantoaxial tuberculosis: Outcome analysis. Int J Mycobacteriol 2017;6:127-31

How to cite this URL:
Sridharan S, Arumugam T. Atlantoaxial tuberculosis: Outcome analysis. Int J Mycobacteriol [serial online] 2017 [cited 2021 Jun 14];6:127-31. Available from: https://www.ijmyco.org/text.asp?2017/6/2/127/206609

  Introduction Top

Tuberculosis (TB) infection forms an important part of human morbidity since ages.[1] India had the highest number of TB cases in 2010.[2] Less than 1% of such patients become victims of spinal TB.[3] Craniovertebral junction (CVJ) affection is a rare condition, accounting for 0.3%–1% of all patients with TB spondylitis.[4],[5] Hilton described the first case of atlantoaxial instability due to TB.[6] In this article, we discuss the clinical profile, management, and functional outcome of eight patients with atlantoaxial involvement who were managed in our institute.

  Materials and Methods Top

The data on treated cases of atlantoaxial TB in the Department of Neurosurgery from April 2014 to March 2016 (2 years) were retrieved. All the patients were analyzed on the clinical presentation, radiological features, and mode of management. Functional outcome and radiological evidence were analyzed in long-term follow-up. Follow-up ranged from 9 to 29 months. All the patients were subjected to radiological investigations such as X-ray of neck (flexion and extension), computed tomography scan and/or magnetic resonance imaging (MRI) of CVJ as necessary. The clinical status of the patient was assessed using the Medical Research Council (MRC) grading system and the Nurick grading before treatment commencement and on follow-up. Radiological grading of the disease was based on the Lifeso grading system.[7] Anti-TB treatment was advocated for 18 months, and neck immobilization was advised for a minimum period of 6 weeks using the Philadelphia collar.

  Results Top

A total of 8 patients were treated for atlantoaxial TB. Ranged in age from 14 to 33 years with male preponderance. All the patients had a history of pulmonary TB confirmed by sputum examination and were negative for HIV serology. There was one case of multidrug-resistant TB. The clinical features are tabulated in [Table 1]. Patient profile, duration of symptoms, radiological findings, and the operative procedure done are tabulated in [Table 2]. Neck pain and restriction of neck movements were present in all the patients. History of contact was absent only in patient VII. Bladder disturbance was present in 3 patients. Respiratory distress was present in 3 patients, 2 required postoperative ventilator support and one among them required tracheostomy. Among the total 8 patients, 4 were surgically treated at presentation, 2 were operated after the failure of conservative approach, and 2 patients were successfully treated conservatively. Surgical management included transoral odontoidectomy and posterior stabilization for 2 patients, posterior stabilization alone for three. Eighth patient underwent endoscopic transoral evacuation of pus and diseased C2. Histopathological examination of surgical specimen revealed TB osteomyelitis in 3, granulation tissue in 2, and chronic inflammation in 1. There was no mortality in the sample studied. The clinical status of the patients before and after treatment is listed in [Table 3]. Improvement of a minimum of 1 grade in muscle power and mean improvement in Nurick grade - 3.17–2 (range 0–4) was documented in all surgically treated patients.
Table 1: Clinical features

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Table 2: Patient profile, imaging and management

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Table 3: Functional assessment before and after surgery

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  Discussion Top

Atlantoaxial TB is usually a secondary affliction. The disease starts in the bone which then infiltrates the ligamentous complex and leads to instability.[8] Goel describes three stages of disease.[9] In Stage 1, there is unilateral involvement of cancellous part of facets of C1 or C2. The contralateral joint is affected only in Stage 3 of the disease. Neurological symptoms may present in Stage 2 but is always a feature of Stage 3. Neck pain is the main and sometimes the only presentation. All our patients had neck pain. Although there is the extensive destruction of bone, neurological symptoms are often late to appear. Less than 50% narrowing of the spinal canal produces only mild to moderate deficits. Radiological evidence of bone erosion appears after 50% of the bone has been destroyed.[3] These factors may in part explain the late presentation of patients to medical care especially from a rural setup. If diagnosed late, the combination of diseased bone and lax ligaments can lead to atlantoaxial dislocation, basilar invagination (BI), epidural abscess, and cervicomedullary compression. With nonspecific presentation, can often result in severe morbidity and even sudden death.[10]

Two patients were successfully treated conservatively in this series. Both were young males with a history of pulmonary TB. Conservative approach was undertaken due to the absence of neurological signs and instability. Since the patients were already on anti-TB chemotherapy (patient VI for 1 month and patient VII for 2 weeks), only neck immobilization was added to the management. Tissue diagnosis in patient VI was obtained from the supraclavicular lymphadenopathy. Tissue confirmation was not attempted in patient VII, mainly because of radiological appearance, history of pulmonary TB and 2 weeks' history of chemotherapy. Clinico-radiological response to anti-TB chemotherapy in a susceptible individual with the classic symptomatology can point toward atlantoaxial TB.[3]

Surgical intervention after failure of conservative approach was done in a couple of patients. Worsening neurological status and development of atlantoaxial instability warranted action in both. Patient I [Figure 1] was initially diagnosed as pulmonary TB and started on anti-TB chemotherapy which he defaulted after a month. He then developed neck pain with radiological features of atlantoaxial involvement. Failure of nonoperative management was evident with worsening myelopathy and progressive quadriparesis. After transoral decompression was done, the patient worsened clinically and required ventilation. Occipito-cervical fusion (OCF) was done after 4 weeks when the patient improved. Culture and sensitivity exposed a multi-drug resistant TB for which fluoroquinolone was used. The other patient, (VIII) was diagnosed on imaging and associated sputum positivity. She developed right upper limb monoparesis while on conservative treatment. Subsequent MRI showed BI and complete destruction of the right lateral mass of  Atlas More Details [Figure 2]. Follow-up on 29 months after OCF showed complete recovery. Among other cases, patient IV [Figure 3]a a known case of hypothyroidism had an intramedullary contrast enhancing lesion at the cervicomedullary junction along with atlantoaxial instability. She presented with quadriparesis (right > left). C1 lateral mass and C2 pedicle screw fixation was done. Imaging after 10 months showed a resolving lesion [Figure 3]b. Patient V required tracheostomy and postoperative ventilation after transoral decompression [Figure 4]. OCF was done after a week. Patient II was advocated only transoral endoscopic evacuation of pus and diseased odontoid. Stabilization procedure was not done. The neck was immobilized with hard collar for 2 months [Figure 5].
Figure 1: (a) Preoperative sagittal magnetic resonance imaging of craniovertebral junction showing pre/paravertebral collection from C2 to C5. (b) Postoperative magnetic resonance imaging showing completely resolved collection.

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Figure 2: (a) Magnetic resonance imaging at presentation showing prevertebral collection. (b and c) magnetic resonance imaging and computed tomography scan showing atlanto axial dislocation, basilar invagination, Cord edema, destruction of anterior arch of atlas (right). (d and e) postoperative images.

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Figure 3: (a) Magnetic resonance imaging showing contrast enhancing lesion in the cervicomedullary junction, pre/para-vertebral soft tissue enhancement and collection in atlanto axial interspace. (b) Resolving lesion.

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{Figure 3}
Figure 4: (a) Coronal computed tomography showing erosion of c2. (b) Sagittal magnetic resonance imaging of craniovertebral junction showing pre/paravertebral collection (left), left lateral mass of axis destroyed. (c) Lateral X-ray showing atlanto axial dislocation. (d) Intra operative picture of occipito-cervical fusion.

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Figure 5: (a) Preoperative X-ray. (b) Computed tomography sagittal view showing erosion of anterior arch of atlas. (c) Sagittal magnetic resonance imaging showing pre/para vertebral abscess. (d) Postoperative X-ray. (e and f) Plain and contrast sagittal magnetic resonance imaging showing deficient odontoid process with no collection.

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Clinical presentation of atlantoaxial TB can be diverse and are well described by Edwards et al.[4] Although the spectrum was well observed, lower cranial nerve involvement was not seen in any of the patients presented here. A case of neck pain from an endemic area with a history of contact and radiological evidence should raise the clinical suspicion of atlantoaxial TB. Imaging features alone cannot be relied upon for diagnosing. Elevated erythrocyte sedimentation rate, Montoux test, evening rise of temperature, weight loss add weightage to the diagnosis. Clinical assessment in this series was based on MRC, and Nurick grading as this was a standard assessment tool in all the cases studied. Lifeso grading was used for radiological assessment in which Stage 1 has a minimal bone destruction with intact ligaments and no evidence of anterior displacement of C1 on C2, with or without proximal translocation of the dens. Stage 2 has minimal bone destruction with ligamentous disruption and anterior displacement of C1 on C2 with or without proximal translocation of the odontoid. In Stage 3, there is marked bone destruction with complete obliteration of the anterior arch of C1. Grading systems combining both clinical and radiological parameters are proposed [11] and can be useful in planning management. The radiological picture also includes epidural abscess, increased prevertebral soft tissue shadow, intramedullary lesion, and BI. X-ray of the neck in flexion and extension and contrast enhanced MRI can reveal important information.

Management of this entity has been changing with time. Initial reports were supportive of a purely conservative approach. Then came a period of radical surgery for all cases. Later, a mixed approach was recommended based on clinical and radiological grading. Various grading systems and management protocols have evolved recently.[3],[11] A paper published by Gupta et al.[12] in 2006 reports the outcome of conservative approach and questions the need for surgery. In our series, the decision to operate was based on neurological symptoms and atlantoaxial instability. The patients without these were conservatively treated. The failure of conservative management was considered as the appearance of neurological deficit and atlantoaxial instability. However, the conversion was not even with regard to duration of conservative management. Medical trial was continued for a minimum of 5 months in both patients before surgery was planned. Poor compliance to neck immobilization can be attributed to the failure, but history was not suggestive of this. Arunkumar et al.[10] in their series advise surgery for all patients which would help in early immobilization and rehabilitation.

All the patients in our series had a history of pulmonary TB and were already on chemotherapy. This is in contrast with other studies.[3],[11],[12] The progressive symptomatology of C1 and C2 affliction even after the commencement of chemotherapy could not be explained in these patients except in the patient who had defaulted. One possible explanation could be the simultaneous affection of craniovertebral junction and lungs. If so, it can then represent delayed presentation or even a delay in diagnosis.

Antitubercular chemotherapy forms the mainmast of treatment. We advocated thrice-weekly regimen for 18 months. All major published series recommend a period of 14–24 months for bony TB.[3],[11],[12] Four drug regimen was used. Isoniazid and rifampin were given for 18 months. Ethambutol was given for 12 months. All 4 were given for the first 3 months.

  Conclusion Top

TB has been a constant menace in India with significant morbidity and mortality. Atlantoaxial involvement is usually secondary and thus can represent a group which is diagnosed late. Awareness among people is increasing but is limited to the pulmonary form of the disease. Seeking early medical care may in most cases prevent a medical disease from becoming a surgical one.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Lawn SD, Zumla AI. Tuberculosis. Lancet 2011;378:57-72.  Back to cited text no. 1
Mishra G. Tuberculosis prescription practices in public and private sector in India. Natl J Integr Res Med 2013;4:71-8.  Back to cited text no. 2
Behari S, Nayak SR, Bhargava V, Banerji D, Chhabra DK, Jain VK. Craniocervical tuberculosis: Protocol of surgical management. Neurosurgery 2003;52:72-80.  Back to cited text no. 3
Edwards RJ, David KM, Crockard HA. Management of tuberculomas of the craniovertebral junction. Br J Neurosurg 2000;14:19-22.  Back to cited text no. 4
Lal AP, Rajshekhar V, Chandy MJ. Management strategies in tuberculous atlanto-axial dislocation. Br J Neurosurg 1992;6:529-35.  Back to cited text no. 5
Pandya SK. John Hilton's contributions on atlanto-axial disease – A forgotten chapter in the history of neurosurgery. Neurol India 1970;18:147-57.  Back to cited text no. 6
Lifeso R. Atlanto-axial tuberculosis in adults. J Bone Joint Surg Br 1987;69:183-7.  Back to cited text no. 7
Sinha S, Singh AK, Gupta V, Singh D, Takayasu M, Yoshida J. Surgical management and outcome of tuberculous atlantoaxial dislocation: A 15-year experience. Neurosurgery 2003;52:331-8.  Back to cited text no. 8
Goel A. Tuberculosis of craniovertebral junction: Role of facets in pathogenesis and treatment. J Craniovertebr Junction Spine 2016;7:129-30.  Back to cited text no. 9
Arunkumar MJ, Rajshekhar V. Outcome in neurologically impaired patients with craniovertebral junction tuberculosis: Results of combined anteroposterior surgery. J Neurosurg 2002;97 2 Suppl: 166-71.  Back to cited text no. 10
Teegala R, Kumar P, Kale SS, Sharma BS. Craniovertebral junction tuberculosis: A new comprehensive therapeutic strategy. Neurosurgery 2008;63:946-55.  Back to cited text no. 11
Gupta SK, Mohindra S, Sharma BS, Gupta R, Chhabra R, Mukherjee KK, et al. Tuberculosis of the craniovertebral junction: Is surgery necessary? Neurosurgery 2006;58:1144-50.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

  [Table 1], [Table 2], [Table 3]

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