AbstractTuberculosis of the craniovertebral junction (CVJ) is an uncommon condition, particularly in the pediatric population, and may present with severe neurological deficits due to instability and spinal cord compression. Surgical management is especially challenging because of the complex regional anatomy and the proximity of critical neurovascular structures. We report the case of a 14-year-old girl who presented with progressive quadriplegia and respiratory compromise secondary to atlantoaxial tuberculosis. Despite empirical antitubercular therapy, her neurological status continued to deteriorate. Imaging demonstrated extensive destruction of C1–2 with associated spinal cord compression and instability. A staged surgical approach was undertaken, consisting of posterior decompression and stabilization followed by anterior endoscopic transnasal decompression. Histopathological examination and GeneXpert testing confirmed tuberculosis, which was subsequently identified as multidrug-resistant disease. With appropriate antitubercular chemotherapy, the patient exhibited progressive neurological recovery and achieved complete functional recovery at follow-up. Early recognition of CVJ tuberculosis associated with neurological compromise and instability is essential. A multidisciplinary approach incorporating timely combined anterior and posterior surgical intervention, along with optimized antitubercular therapy, can result in excellent neurological and functional outcomes, even in severe presentations.
INTRODUCTIONTuberculosis of the spine accounts for approximately half of all cases of musculoskeletal tuberculosis [1], with cervical spine involvement being relatively rare [2]. Lesions affecting the craniovertebral junction (CVJ) constitute only 0.3%–1% of cases of spinal tuberculosis [3]. These are associated with significant morbidity due to instability and compression of the cervicomedullary junction. Pediatric CVJ tuberculosis is particularly uncommon and poses diagnostic and therapeutic challenges. Mehrotra et al. [4] reported CVJ tuberculosis in 29 out of 229 cases over a 14-year period, highlighting its infrequent occurrence. We present a rare case of atlantoaxial tuberculosis in a child presenting with quadriplegia, managed successfully with a combined posterior and anterior endoscopic transnasal approach.
CASE PRESENTATION1. Clinical PresentationA 14-year-old female presented with progressively worsening neck pain for 2 months, followed by gradual onset weakness of all 4 limbs over 1 month. The weakness progressed to quadriplegia, and the patient developed difficulty in breathing and was unable to maintain a propped-up position. There was no prior history of trauma. Given the epidemiological data regarding tuberculosis in India, antitubercular therapy without tissue diagnosis of TB was initiated [5]. This included isoniazid, pyrazinamide, rifampicin and ethambutol. However, there was no neurological improvement.
Initial magnetic resonance imaging (MRI) performed before referral demonstrated significant C1–2 pathology with spinal cord compression, radiologically suggestive of tuberculosis.
Informed consent was obtained from the patient’s legal guardian for publication of this case report and accompanying images.
2. Radiological Findings - MRI Findings Are Illustrated in FiguresOn presentation to our center, a detailed clinical and neurological evaluation was performed. Repeat imaging, including MRI, computed tomography (CT), and CT angiography, was undertaken to assess the extent of bony destruction, degree of neural compression, instability, and vertebral artery anatomy. Imaging revealed atlantoaxial instability with destruction of the C1 lateral mass and compressive inflammatory granulation tissue at the CVJ.
Preoperative CT scan showed that the distance between the anterior arch of C1 and the anterior cortex of the odontoid process measured approximately 1.2 cm (anterior atlantodental interval), consistent with significant atlantoaxial instability (Figure 1). Preoperative CT angiography demonstrated a narrow-caliber left vertebral artery (V1–V3 segments) with preserved contrast opacification, while the right vertebral artery was normal in caliber and course, findings relevant for surgical planning (Figure 2). MRI screening of the cervical spine revealed that the posteriorly displaced odontoid process caused marked compression of the cervicomedullary junction against the posterior arch of C1 (Figure 3A and B). MRI showed anterior displacement of the atlas in relation to the axis, resulting in severe canal compromise at the C1–2 level with an anteroposterior canal diameter of approximately 4.2–4.6 mm with associated cord signal changes (Figure 3B). Hyperintense signal changes on T2-weighted images were noted within the cervicomedullary junction and upper cervical cord at C1–2, suggestive of compressive myelopathy. Altered marrow signal intensity, appearing hypointense on T1-weighted images and hyperintense on T2-weighted and STIR (short tau inversion recovery) sequences, was seen involving the occipital condyles, C1, and C2 vertebrae (Figure 3C and D). Abnormal soft-tissue signal intensity was identified between the anterior arch of C1 and the odontoid process, extending into the prevertebral and paravertebral spaces (Figure 3E), with an associated inflammatory collection in the retropharyngeal space extending up to the C6 level (Figure 3F). No obvious epidural extension was noted. Given the severity of neurological deficit, mechanical instability, and lack of response to medical therapy, surgical intervention was planned following a multidisciplinary discussion involving spine surgeons, skull base surgeons, anaesthesiologists, and infectious disease specialists.
Prior to presentation, the patient underwent a 3-month course of empirical antitubercular therapy (AKT). During this interval, comprehensive preoperative evaluation was performed, including CT angiography to delineate vertebral artery anatomy, along with CT and MRI of the spine to assess the extent of bony involvement, soft-tissue disease, and neural compression. The case was subsequently discussed in a multidisciplinary team (MDT) meeting to determine the optimal management plan. Following presentation to our unit and completion of the necessary preoperative work-up, surgical intervention was planned and performed on the third day postpresentation.
3. Surgical Technique1) Preoperative planningMDT meeting was conducted including a skull base surgeon, neurosurgeon, radiologist, infectious disease specialist and orthopedic spine surgeon. After a comprehensive review, it was agreed upon that owing to the high degree of bone destruction anteriorly, removing additional anterior tissue before stabilizing from posterior would render the spine more unstable. Hence, a staged single-sitting procedure consisting of posterior stabilization for instability, followed by anterior endoscopic endonasal decompression, was planned. All procedures were performed under general anesthesia with neuromonitoring where feasible.
2) Stage 1: posterior stabilizationPatient positioning: The patient was positioned prone and secured in a Sugita head frame to ensure rigid cranial fixation.
Reduction and exposure: Partial reduction of atlantoaxial instability was achieved and confirmed using intraoperative fluoroscopy (C-arm guidance). A standard posterior midline approach was utilized to expose the CVJ [6].
Decompression and instrumentation: Posterior decompression was performed by removal of the posterior arch of C1. Right side: C1 lateral mass and C2 pars screw fixation using 3.5-mm titanium screws. Left side: Due to significant destruction of the C1 lateral mass, occiput-to-C2 fixation was performed using an occipital keel plate and C2 laminar screw [7]. Rod constructs were applied to achieve rigid stabilization.
Instrumentation: Standard posterior cervical instrumentation set including Kerrison rongeurs, Penfield dissectors, self-retaining retractors, and bipolar/monopolar cautery was used.
3) Stage 2: anterior endoscopic endonasal decompressionPatient repositioning: Following posterior stabilization, the patient was repositioned supine with the head secured in the Sugita frame.
Endoscopic approach: A binasal endoscopic endonasal approach was performed using a 0° rigid endoscope [8]. Initial diagnostic nasal endoscopy was carried out (Figures 4 and 5). Surgical corridor creation included: Out fracturing of the inferior turbinates (Figure 6), limited posterior ethmoidectomy. A vascularized nasoseptal flap (Hadad-Bassagasteguy flap) (Figures 7 and 8) based on the sphenopalatine artery was prepared [9]. Drilling of maxillary crest was performed (Figure 9).
Exposure of the CVJ: Sphenoidotomy performed (Figure 10) via drilling of the sphenoid rostrum and vomer [10]. Identification of key landmarks including internal carotid arteries, eustachian tubes, torus tubarius, and fossae of Rosenmüller. Incision of the posterior nasopharyngeal and pharyngobasilar fascia. An inferiorly based U-shaped nasopharyngeal flap was elevated [11].
Decompression: The longus colli and paravertebral muscles were dissected to expose the inferior clivus, defect on anterior arch of C1 (Figure 11), and body of C2.
Sequential drilling and decompression included: Inferior clivus, anterior arch of C1, Odontoid process. Infective granulation tissue was excised (Figure 12), achieving adequate decompression of the cervicomedullary junction under endoscopic visualization.
Reconstruction and hemostasis: The nasopharyngeal flap was repositioned. The nasoseptal flap was placed over the defect. Defect is packed with Surgicel and Tisseel (Fibrin Glue) to reduce risk of cerebrospinal fluid leak [12] (Figure 13). Gelfoam and Floseal were used for hemostasis (Figure 14).
Specimen collection: Tissue samples were obtained and sent for histopathological examination and GeneXpert testing.
Postoperative course: The patient was extubated immediately following the procedure with no perioperative complications.
Endoscopic equipment: 0° rigid endoscope (Smith & Nephew); high-definition camera system; monopolar cautery; endoscopic burr system (NSK); hemostatic agents: Floseal, Gelfoam; Fibrin sealant: Tisseel.
4. Postoperative Course and OutcomeThe patient was monitored in the intensive care unit for 48 hours. Post operative plain x-ray (anteroposterior and lateral views) of cervicomedullary junction confirmed adequate stabilization with satisfactory alignment (Figure 15A and B). Histopathological analysis and molecular testing confirmed the diagnosis of tuberculosis. The patient was initiated on standard four-drug AKT (isoniazid, rifampicin, pyrazinamide, and ethambutol) [13]. Subsequent testing identified multidrug-resistant tuberculosis, following which treatment was modified in consultation with tuberculosis specialists, and bedaquiline was added to the regimen [14].
The patient demonstrated gradual neurological improvement in the postoperative period. She was discharged on postoperative day 12 following suture removal. At 3-month follow-up, the patient had regained full motor power in all limbs. At 6 months, she was independently ambulant and able to perform all activities of daily living without assistance.
DISCUSSIONCervical spine tuberculosis constitutes approximately 3%–5% of all cases of spinal tuberculosis [15], with involvement of the CVJ being particularly rare, especially in children. Neurological deficits result from a combination of instability, deformity, and compressive granulation tissue.
While AKT remains the cornerstone of management, surgical intervention is indicated in cases with progressive neurological deficits, instability, respiratory compromise, or failure of conservative treatment. We made use of the Spine Instability Scoring System as proposed by Rajasekaran to assess the need for surgical management [16]. This was based on the 4 ‘spine at risk’ signs described: facet subluxation/retropulsion/lateral translation/toppling sign [17] (Table 1).
In our case, the patient score was calculated to be >3 and hence surgical management was planned.
Chatterjee and Das [18] proposed a classification system for CVJ tuberculosis, wherein patients with instability and gross neurological deficit (type I) benefit from early surgical intervention. Combined anterior and posterior approaches have been rarely described in the literature, and reports of anterior endoscopic transnasal decompression are exceedingly scarce.
The transnasal endoscopic approach provides direct access to the CVJ with minimal soft-tissue disruption, avoiding the morbidity associated with traditional transoral approaches. When combined with robust posterior stabilization, it offers effective decompression and stabilization in selected cases.
Traditionally, most cases have been managed with antitubercular therapy and immobilization, with surgical stabilization reserved for patients with instability or progressive neurological deficit. When anterior decompression is required, the transoral approach has historically been the standard surgical corridor for accessing the ventral CVJ.
With advances in endoscopic skull base surgery, the endonasal approach has emerged as a minimally invasive alternative for ventral CVJ lesions, particularly in conditions such as basilar invagination, rheumatoid pannus, and tumors. However, the literature describing the application of this approach in infective pathologies of the CVJ remains extremely limited. To the best of our knowledge, no prior reports have described endoscopic transnasal decompression for atlantoaxial tuberculosis, particularly in the pediatric population. The present case therefore highlights a novel surgical application combining posterior stabilization with anterior endoscopic transnasal decompression for severe neurological compromise.
CONCLUSIONAtlantoaxial tuberculosis in children is a rare but potentially devastating condition. Early diagnosis, vigilant monitoring, and appropriately planned surgical management are critical in patients presenting with neurological compromise and instability. A combined posterior stabilization and anterior endoscopic transnasal decompression, supported by appropriate antitubercular chemotherapy, can result in excellent neurological and functional outcomes.
Compared to previously reported cases of CVJ tuberculosis managed with transoral decompression, our approach offers several advantages including avoidance of oral contamination, reduced postoperative dysphagia, and improved visualization of the ventral CVJ. Furthermore, in contrast to reports where posterior stabilization alone resulted in neurological improvement, our patient presented with severe anterior compressive pathology necessitating direct decompression.
Figure 1.Preoperative computed tomography scan (precontrast helical acquisition) of the craniovertebral junction (from the root of the neck to the vertex of the skull). Anterior displacement of C1 relative to C2 (odontoid process), resulting in significant canal narrowing (anteroposterior diameter: 4.6 mm). Distance between the anterior arch of C1 and the anterior cortex of the odontoid process: 1.2 cm. Figure 2.Preoperative computed tomography angiography scan (precontrast helical acquisition). Reduced caliber of the left vertebral artery (V1, V2, and V3 segments) with preserved contrast opacification. Two calcified plaques are noted in the V1 segment. The right vertebral artery demonstrates normal caliber and contrast opacification. Figure 3.Magnetic resonance imaging (MRI) of the cervical spine. (A) Sagittal T2-weighted image demonstrating anterior displacement of C1 over C2, causing severe compression at the cervicomedullary junction against the posterior arch of C1 vertebra. (B) Sagittal T2-weighted image demonstrating hyperintensity at the cervicomedullary junction and within the spinal cord at the C1–2 level, suggestive of compressive cervical myelopathy with significant canal narrowing at this level (anteroposterior diameter – 4.6 mm). (C) Sagittal T1-weighted hypointensity and T2/STIR (short tau inversion recovery) hyperintensity involving the occipital condyles and the C1 and C2 vertebrae. (D) Sagittal T2-weighted image demonstrating anterior displacement of C1 over C2, resulting in severe compression at the cervicomedullary junction against posterior arch of C1 vertebra. (E) Axial T2-weighted image demonstrating abnormal soft tissue intensity noted between anterior arch of C1 vertebra and odontoid process, pre and paravertebral spaces at this level. No obvious epidural extension of abnormal soft tissue noted. (F) Sagittal T2-weighted image demonstrating anterior displacement of C1 over C2 causing severe compression at cervicomedullary junction against posterior arch of C1 vertebra. Image demonstrating inflammatory collection in retropharyngeal space extending upto C6 level. Figure 15.Postoperative radiographs of the cervical spine and craniovertebral junction. (A) Lateral radiograph demonstrating posterior stabilization with satisfactory alignment of the craniovertebral junction. (B) Anteroposterior radiograph demonstrating posterior stabilization with satisfactory alignment of the craniovertebral junction. Table 1.Spine instability scoring system Table 2.Literature review: surgical approaches for craniovertebral/atlantoaxial tuberculosis
Table 3.Literature review: conservative versus surgical management of atlantoaxial tuberculosis
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