Abstract

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.

Keywords: Anti-tubercular therapy, Atlanto-axial dislocation, Craniovertebral junction, Kyphotic deformity, Tuberculosis

INTRODUCTION

The incidence of tuberculosis (TB) in the spine is <1%.[ 2 ] Isolated involvement of the craniovertebral junction (CVJ) (i.e., including the atlas, axis, and basiocciput) is even less frequently encountered and may involve a 0.3–1% risk of spondylodiscitis.[ 4 , 5 ] To prevent long-term neurological deficits the timely diagnosis and surgical management (i.e., decompression/fusion) of TB at the CVJ level is critical along with adjunctive anti-tubercular therapy.[ 3 - 7 ]

CASE 1

History and presentation

A 25-year-old Asian male presented with 2 months of severe neck pain (i.e., focally tender on exam), a progressive bilateral quadriparesis; 3/4 bilateral motor function, bilateral diffuse hyperreflexia/Hoffman’s Signs, Babinski responses, and a definite C2 sensory level (American Spinal Injury Association, ASIA D). Cervical X-rays showed an osteolytic C1 lesion with instability of the Atlanto-Axial dislocation (AAD). The magnetic resonance imaging (MRI) confirmed CVJ instability while also demonstrating a pre-vertebral collection contributing to marked anterior cord compression [ Figure 1 ]. Lab studies showed an elevated erythrocyte sedimentation rate (ESR) of 110 mm, and C-reactive protein (CRP) of 68. The chest X-ray was normal, there were no palpable lymph nodes, and the tuberculin test was negative.

Figure 1:

(a) X-ray cervical spine lateral view showing increased atlanto dental interval (13 mm) with radio dense shadow in between. (b) Magnetic resonance imaging cervical spine in sagittal T1-weighted image and contrast showing prevertebral retro-oropharyngeal abscess. (c) Immediate postoperative cervical X-ray days showed beautiful correction.

Surgery

Prone skeletal tong traction corrected the AAD (Atlanto-Axial-Dens) malalignment. At surgery, following the occiput to C2 exposure, the C1 lateral masses were clearly eroded. With minimal distraction, pus immediately extruded from the prevertebral space; it was washed and followed by an occipito-cervical fusion (i.e., including the use of C2/C3 lateral mass screws). He wore a rigid cervical collar for 1 month, followed by a soft collar for 2 months. Within 2 postoperative weeks, he could walk independently, and at 2 postoperative months was neurologically intact.

CASE 2

History and presentation

A 16-year-old female presented with severe neck pain for 1-month, low grade fever, and 2 weeks of progressive spastic quadriparesis (motor 4/5) with sensory loss to pin from C2 to C4 (ASIA D). The cervical X-rays and computed tomography (CT) scan showed an osteolytic C2 lesion (i.e., involving the C2 body and pedicle) with kyphosis and Atlano-Axial instability. The MRI showed an extradural mass anterior to C1/2 compressing the cord spinal cord; it extended into the retropharyngeal space [ Figure 2 ]. Her ESR was 90 mm in the 1^st h and her CRP was 65. The chest X-ray was normal, and the tuberculin test was negative.

Figure 2:

(a) X-ray cervical spine lateral view showing increased atlanto dental interval (16 mm) with radio dense shadow in between, (b and c) computed tomography (CT) cervical spine lateral (b), coronal (c) view showing osteolytic lesion at C2 body. (d and e) Magnetic resonance imaging (MRI) cervical spine in sagittal T2-weighted image (T2WI) (d) and T1-weighted image (e) showing prevertebral retrooropharyngeal abscess. (f and g) Postoperative cervical X-ray after 15 days, (h) CT cervical spine sagittal after 1 month, and (i) MRI of the cervical spine in sagittal T2WI after 3 months showed complete resolution of abscess, correction of kyphotic deformity.

Surgery

Surgery was performed in two stages. The first was transoral decompression; the second was posterior stabilization [ Table 1 ]. Her motor exam markedly improved within 10 postoperative days. Further, the histopathology, postoperative antibiotic TB treatment regimen, use of Pyridoxine, cervical collar use, and entire postoperative course were similar to case 1 [ Table 1 ].

Table 1:

Craniocervical junction TB (Summary of three cases).

CASE 3

History and presentation

A 68-year-old male presented with severe neck pain for 1 month, respiratory distress, quadriplegia/sphincter dysfunction, a C2 complete sensory level, and bladder-bowel dysfunction (ASIA A). After immediate placement in skeletal (tong) traction, his motor exam improved to the 2/5 level. Cervical X-rays of the CVJ confirmed atlanto-axial instability. The MRI revealed destructive changes involving the C2 and C3 intervertebral discs/vertebral bodies along with an extradural anterior C1–C3 retropharyngeal collection that markedly compressed the cord. The CT confirmed the destruction of dens [ Figure 3 ].

Figure 3:

X-ray cervical spine dynamic view showing C1/2 instability, (b) Computed tomography (CT) cervical spine in sagittal view, showing partial erosion of C1 lateral mass and C2 body, (c, d, e) Magnetic resonance imaging (MRI) of the cervical spine, contrast enhancing extradural mass at C1–3 levels causing obliteration of the anterior CSF column and prevertebral collection of abscesses. (f) Postoperative X-ray of the cervical spine at 1 month, showing spinal fixation between C1 lateral mass, C2 pedicle, and C3 lateral mass with screws and rods, (g) MRI of the cervical spine at 6-month complete resolution of abscess and significant decompression of cord.

Surgery

After 2 days of skeletal (tong) traction, through a midline posterior occiput-C2 incision, the C1/2 lateral joint space was identified and pus was spilling out from it anteriorly. Granulation tissue was debrided lateral to the C2, and C3 lateral masses. The screw was placed from C1 to C3. Postoperatively, the treatment with a collar for 3 months, the antibiotic regimen, the diagnostic studies, and the postoperative lab studies were all similar than the prior cases [ Table 1 ].

Histopathology and antibiotic treatment

Histopathology showed granulomatous inflammation suggestive of TB. The GeneXpert (A cartridge based nucleic acid amplification test rapid diagnostic test for TB detection as well as Rifampicin resistance in direct smear negative cases) was positive for TB. The patient was given 18 months of anti-tubercular therapy; 3 months of 4 drugs followed by 15 months of 2 drugs. He was also given, pyridoxine tablets for 18 months.

Neurodiagnostic follow-up

X-rays were done on postoperative days 1, 7, and 15 and every month thereafter looking for progressive infection/deformity, and/or pseudarthrosis [ Figure 1 ]. Complete blood count and CRP were also followed on post-operative day 1, 15 days later, and at 1 month intervals screening for disease progression.

DISCUSSION

Spinal TB rarely involves the cervical spine (i.e., 3–5%) and even more infrequently occurs at the CVJ.[ 7 , 8 ] When diagnosed with dynamic X-rays, MR, and CT studies, TB typically results in the destruction of vertebra/discitis at multiple contiguous levels. The resultant instability must be immediately stabilized, the patients should undergo emergent spinal cord as well as paraspinal/retropharyngeal decompression/abscess debridement.[ 10 , 11 , 13 , 14 ] Within some cases CT angiography and/or MR angiography of the cervical spine are warranted to evaluate the course of the vertebral arteries and avoid a vascular catastrophe during surgical decompression/fusion.[ 3 , 4 , 9 , 10 , 12 - 14 ] Many authors recommend posterior fusion and fixation with or without anterior decompression, and with or without irreducible AAD [ Table 2 ].[ 1 , 2 , 4 ] Here, we presented three cases of CVJ TB who underwent early diagnosis and management with decompression and CVJ stabilization followed by appropriate long-term 18-month postoperative anti-TB therapy.

Table 2:

Reported series of tuberculous CVJ TB.

CONCLUSION

In three patients with TB at the CVJ patients underwent early diagnosis (X-rays, MR, and CT) and surgical decompressions/ fusions to prevent further neurological deterioration and offer the potential for neurological recovery.

Declaration of patient consent

Patients’ consent not required as patients’ identities were not disclosed or compromised.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation

The author(s) confirms that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

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