The options before us were:

1.       Foramen Magnum Decompression (FMD)

2.       Trans-Oral Odontoidectomy

We proceeded with the latter option, as the primary cause of compression was the malpositioned odontoid process of C2.

After a thorough work up, the patient was placed supine after nasopharyngeal intubation was done and mouth opened with a Boyle-Davis Mouth gag. The posterior pharyngeal wall was incised and dissected under C-ram guidance to reveal the anterior arch of C1 and the Odontoid. Both were drilled to expose the dura of the CMJ. Haemostasis was achieved, before closure was done with soluble sutures.

Post op the patient was mobilized immediately and she showed significant decrease in spasticity. Gradual physiotherapy and rehab care showed excellent recovery. She now is ambulant without support, and performs all activities of daily living freely. She is also active in the kitchen and performs her daily chores well (something that was impossible pre-op) and lives a happy functional life.

DISCUSSION

Craniovertebral Instability is a difficult problem to address. No two patients are the same. Tailoring the management to each individual’s needs is important to ensure good recovery and patient satisfaction [5-7]. The most important aspects of CVJ disorders are that, there are 2 main issues that need attention.

Instability [1-11]

The improperly fused elements of the CVJ lead to instability in weight bearing of the head. Such a problem is significant and alarming, which can lead to significant morbidity. However, CVJ instability is not seen in all disorders. Its severity and the need to intervene, needs to be assessed with respect to the pathology of the disease and the cause of the symptoms of the patient. Blindly fusing all CVJ abnormalities will result in patients like the one discussed, where fusion and excellent stability have resulted in no improvement in functional status.

Neurological decompression [4-6,8,11]

The crux of all procedures, aimed at resolving disease at the CVJ, eventually involves reduction of compression (either bony, ligamentous or produced by external agents such as pannus or pus) on the CMJ. Without adequate neurological decompression, there will be no symptomatic relief for the patient and hence no recovery. It is therefore imperative to understand the nature of the disease and the cause of the symptoms of the patient.

Deciding upon the right management strategy requires many stages of deduction to be performed. These include [7-12]:

1.       Neurological history: These help deduce the causative pathology and thereby point in the direction of the plausible problem. For example, traumatic CVJ dislocations will usually have a predominant component of instability rather than compression while rheumatoid arthritis and inflammatory conditions, will lead to pannus formation and predominantly compressive pathology. A good grasp of the causative etiology and the pathological progression helps decide on surgery, and adjuvant therapy [10-12].

2.       Examination: The presence of a Myelopathy definitely implies serious neurological compression, making its release a priority [12-14].

3.       Presence of abnormal anatomy: The existence of a BI, AAD or other related abnormalities implies reduction to normal anatomical position along with adequate decompression [6,8,11,12].

4.       Instability and neurological compression seen on neuro-radiology: The need for a dynamic study of the CVJ is obvious as it detects instability best of all. In certain complicated anatomical situations, CT scan in flexion and extension is helpful to identify the chief offending pathology. An MRI however is essential to detect the status of the CMJ and the cervical cord. No manipulation of the CVJ and CMJ must be done without an MRI. Failure to do this can result in catastrophic destruction of a delicate and vital structure [3,5,8,9,11,13,14].

5.       Presence of previous surgical implants and their implications to management: As discussed in the case report, the presence of the inter laminar fusion wire along with posterior fusion changed the situation completely. Although trans-oral odontoidectomy is frowned upon nowadays, due to a sturdy posterior fusion being present, there was no need to re-stabilize the joint. Hence only decompression was required. The stainless-steel wire caused us much grief, as an MRI of the CMJ was impossible with it in situ. Due to the essential nature of the MRI, it was decided to surgically remove it and then take an MRI before planning definitive management. Such a strategy, although cumbersome and long-winded, will eventually point the correct direction to take, and hence is worth the trouble to both patient and surgeon.

The algorithm for management is well known and is hence not addressed here. Posterior fusion with adequate decompression is the treatment of choice here. The choice of C0-C1-C2 fusion or just C1-C2 fusion depends upon the extent and nature of the instability. Decompresive procedures include laminectomies and Foramen magnum decompressions. These again are to be decided upon studying the MRI and matching the compression to the symptoms experienced by the patents as cause and effect.

Lastly, postoperative physiotherapy is perhaps more important than surgery itself [14]. Rehabilitation experts are the wizards who make noteworthy improvements in the lives of the patients. Surgery alone is useless if not combined with proper rehab support. As with our patient, it was long-term, persistent and assiduous rehab care that led to the excellent results experienced by our patients [14]. This underlines the need of a team approach to these complex conditions, where Neurosurgeons, nurses, physiotherapists, anesthetists, radiologists and technicians combine to give the patient the best result possible.

SUMMARY AND CONCLUSION

Pathologies of the CVJ are complex and do NOT have a 1 size fits all solution. A thorough understanding of the situation coupled with adequate emphasis to BOTH neurological decompression AS WELL AS instability gives the best result overall, and help avoid difficult and inconvenient redo surgeries.