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TUBERCULOSIS OF THE CRANIO VERTEBRAL JUNCTION
S Naique*, M Kandoi**
*Lecturer; **Sr. Registrar, Dept. of Orthopaedics, KEM Hospital, Mumbai.
Although common, tuberculosis of the CV junction rarely causes severe destruction of the atlas. Vital neurologic structures in the vicinity further aggravates the problem. This is a singular case report, revealing the malignant nature of the tubercular destruction causing almost total disappearance of the atlas.INTRODUCTION
Tuberculosis of the cranio-vertebral (CV) junction presents a dilematic problem, because of its neurologically vital structures in the vicinity, potential unstable articulation and grave prognosis, if gone untreated and undetected. The aim of this article is to highlight the malignant nature that tuberculosis of the CV junction can assume, the varying clinical picture, with inconsistent clinico-radiologic correlation. The final outcome may remain unfavourable, owing to the grave nature of the disease and the fatal instability.
CASE HISTORY
A 24 year old, male patient presented with pain and swelling in the submandibular region of 2 months duration. This was associated with high grade fever and difficulty in breathing and deglutition. The patient, also had pain and stiffness of the neck.
On examination, the patient had a soft, fluctuant and tender swelling in the submandibular region. There was tenderness at the CV junction, with torticollis and severe restriction of all neck motion. He had cranial nerve involvement in the form of left sided lower motor neuron facial palsy and hypoglossal nerve palsy. Neurologic examination revealed normal sensorium and signs of early high cord compression in the form of hyperreflexia and upgoing plantars. The tone and power was normal.
Radiograms of CV junction, showed partial destruction of lateral masses with secondary atlanto-axial dislocation, and occipital bone erosion. CT scan, confirmed the above findings and revealed occipital bone destruction with a large soft tissue abscess anteriorly and in the posterior cranial fossa. There was secondary basilar invagination with the atlas lying almost in the posterior cranial fossa.
A preliminary tracheostomy was done to relieve upper airway obstruction. Skull traction was applied and the CV junction immobilized in extension. A four drug (streptomycin, isoniazid, rifampicin and pyrazinamide) anti tubercular therapy was started. Adequate nutrition and tracheostomy care was ensured.
An X-ray at 3 weeks, showed near total destruction of the atlas with increase in the size of the abscess.
In view of the lack of response to the anti-tubercular therapy and instability, the patient was posted for posterior decompression and stabilisation. The patient, however developed respiratory failure and expired.
DISCUSSION
The occipito-cervical junction, a transitional zone between the skull and the spinal column, serves as the most mobile part of the axial skeleton. Bony abnormalities affecting this complex results in dysfunction of the neural structures by compression along the entire circumference, altering the arterial supply, venous drainage and changing the CSF fluid dynamics.
Lymphatic channels play a dominant role in the aetiopathogenesis of any infective process at the CV junction. The synovial pouch lining the occipito-atlanto-axial joints, where the disease begins, receives afferents from the paranasal sinuses, nasopharyngeal and retropharyngeal areas.[1] [2] Retrograde infection may spread to the CV junction resulting in instability or effusion as an inflammatory response.[3] [4] [5]
The lateral masses are the initial to be affected and destruction of these will eventually lead to collapse or weakening of the bony pillars. Depending on the severity of the disease process, varying degree of bony destruction of the atlas may ensue. This bony destruction may manifest as neck pain, torticollis, occipital neuralgia, dysphagia, dyspnoea (retropharyngeal abscess) or even as neurologic dysfunction.[6]
At this stage, the disease if unabated, will cause ligament destruction aggravating the instability at the CV junction. AAD may result and if the prodens interval is more than 5 mm, it signifies destruction of the transverse ligament of the atlas. Varying degree of rotatory malalignment, is commonly associated and needs to be considered during treatment of the condition.
It is very unusual to find the disease advanced to the stage of complete liquefaction and dissolution of the atlas. When this occurs, (as in our case), the instability at the CV junction is severe, and demands urgent immobilization. It also forewarns complete osteo-ligamentous destruction and warrants emergency management. The associated problems of pressure, like dysphagia and asphyxia may assume alarming proportions. This may as in our case necessitate tracheostomy for effective airway ventilation.
As a sequelae, secondary basilar invagination may occur adding a different clinical dimension to the disease. Occipital bone may be involved as an extension of the disease, with softening allowing the spinal column to further telescope into the posterior fossa. The proximity of the lower cranial nerves make them vulnerable to be affected.
As always early diagnosis and treatment should be the goal with avoidance of fatal instability. The surgical option should be considered, in light of the patient’s general condition, associated cranial nerve palsies and neurologic status.
ACKNOWLEDGEMENT
We are grateful to the dean, KEM Hospital, for allowing us to publish this work, carried out in the hospital.
REFERENCES
1. Brasch JC. Cunningham’s Manual of Practical Anatomy Oxford : Oxford University Press. 1958; 258-95.
2. Deoliveira E, Rhoton AL Jr, Peace D. Microsurgical anatomy of the region of the foramen magnum. Surg Neurol 1985; 24 : 293-352.
3. Mitchie I, Clark M. Neurological syndromes associated with cervical and cranio-cervical anomalies. Arch Neurol 1968; 18 : 241-47.
4. Nicolsen JT, Shark HH. Anomalies of the occipitocervical articulations. JBJS (Am) 1968; 50 : 295-304.
5. Park WW, Rothman RH, Brown MD. The Pharyngovertebral veins. An Anatomical rationale for Grisel’s syndrome. JBJS (Am) 1984; 66 : 568-74.
6. Fang D, Leong JCY, Fang HSY. Tuberculosis of the upper cervical spine. JBJS 1983; 65B : 47.
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