Blog- CCI Surgery; a neurologist’s perspective

A subset of the population of those that suffer with hypermobility, mast cell activation, and dysautonomia will develop what has become to be known as myalgic encephalomyelitis (ME) and/or chronic fatigue syndrome (CFS). A small percentage of these patients develop worsening and progressive symptoms thought to be due to cranio-cervical instability (CCI) or atlanto-axial instability (AAI). This instability is thought to cause a microshearing of the cranial-cervical junction where the medulla of the brainstem becomes the spinal cord top, thereby disrupting neural pathways that contribute to the patient’s symptoms and, in fact, making other forms of treatment either ineffective or suboptimal.

I am embarking on a journey with one such patient and am traveling to join one of the few neurosurgeons who have an intimate understanding of not only this sensitive anatomical concern but of the disease state of these patients overall. I will be joining this neurosurgeon during intensive pre-op evaluation as well as the surgery that has helped some in the ME/CFS community begin to heal.

Day 1

I spent the day touring the hospital and meeting with the nurse manager and patient advocate. We have discussion on patient’s needs and the importance that the rest of the staff has an understanding of how these patients suffer. They clearly have an understanding of the patients in this population. They often suffer with tremendous sensitivities to light and sound and can easily experience sensory overload from too much stimulation, even from too much movement or people. They also have exertional intolerance and generalized weakness so become extremely fatigued even with minimal conversation, and with performing basic activities of daily living. I learn of their experience with previous populations admitted for the CCI surgery. We walk through the hospital and discuss where everything and everyone is that may be needed during pre-op and post-op days. The patient is arriving that evening and we make plans for a smooth admission.

I am feeling of hopeful for the potential for this patient but, as always, am cautiously optimistic.

I spend the rest of the afternoon walking the rainy streets of this town. So close to where I grew up. I watch people go about their days of going to work, going to school, taking care of their children, socializing with their friends. I think of all the patients I have seen and currently seen where those every day tasks and events, that many of us take for granted, are no longer possible for them. They may suffer in silence but that is often because they are not found credible by those around them. We all have our journey and our obstacles, some are more visible, but most are not.

A few hours later, I receive word the patient’s air ambulance has landed and they are en route to the hospital. I head back to the hospital to meet the night team on the floor as well as the air ambulance team, the patient, and the patient’s family.

After debriefing the night nursing team on the specific needs of this patient – no noise, no light, very minimal stimuli – I go to the ambulance bay and get an update from the crew. The flight had gone very well and he was stable and in good spirits. He is transferred to his room and hooked to telemetry. Once settled, I communicate with the neurosurgeon that all has gone well and we plan to meet in the morning to begin the invasive traction evaluation. I return to the hotel for (hopefully) sleep.

Day 2

Early this morning I arrived at the hospital to meet the neurosurgeon and his team. After some quick preliminary conversation, we take the patient to the OR. The patient is prepped for invasive traction with a Trippi-Wells tongs device which is screwed into the skull on the sides of the head, in the temporal region. Under short-duration sedation, the tongs are placed. As sedation wears off, traction is placed at 20 lbs and I do a neurological examination and the patient is asked a series of questions regarding his symptoms of fatigue, brain fog, light sensitivity, noise sensitivity, and exertional intolerance. Xrays and subsequent measurements (CXA, Grabb-Oakes, BAI, and BDI) were taken. Traction was then increased to 35 pounds and this process was repeated immediately and then again at approximately 10 minutes later. Pictures and measurements were taken again. Traction was released and the patient was then again asked about symptoms.

With traction, I saw, and met, a patient I had not known. The face had changed and the patient was more alert, conversant, and robust. The patient reported a significant improvement in most of the symptoms. The patient’s neurological examination showed objective improvements as well. The invasive traction trial was a success and surgery is planned to proceed.

In neurology, there are few diseases that can be cured and certainly fewer than can be that dramatic of a positive change. It was emotional for me as a neurologist to see such a drastic change in neurological status of a patient from one, not-so-simple, procedure. I was also excited for the patient and the family to think about the possibilities for a life after CFS.

The rest of the day the patient remained conversant though clearly some symptoms returned. We discussed ME/CFS, Lyme and other chronic infections, TBI and the co-associations. And life in general. It was one of the more detailed and expansive conversations we have had thus far.

I look forward to the surgery scheduled tomorrow.

Day 3

I did not sleep last night due to a combination of excitement and nervousness. Questions ran through my head about various details of the surgery I don’t yet know such as bone grafting, tissue collection and storage, avoidance of nerve damage, confirmation of alignment prior to fusion, and more. I thought of all the details I wanted to understand about this surgical procedure. The patient asked me not to leave the side of the stretcher and I promised I would not and I am hoping I will be allowed to keep my promise. I like to absorb the fears of my patients so they can relax as much as possible.

Headed to the hospital. I have not been up this early since my call days. And I certainly have not been in a surgery this long since residency, unless you count my own brain surgery but I was asleep for that 🙂

Day 4

It took me a day to process the experience yesterday. I met the patient in the room and accompanied the patient and family down to pre-op area where we met with the anesthesiologist, the plastic surgeon, and of course the neurosurgeon. I worked with the patient throguh visualization techniques. Patient was taken to the OR and prepped for surgery with general anesthesia, intubation, and electromyelogram electrodes. Patient was then placed in prone position with neck flexed and held tight by a Mayfield device. The neurosurgeon prepped the skin and made an incision down the back of the neck from the occiput to around C3. Exquisite dissection was made to expose the calvarium, C1 vertebral body, C2 vertebral body, C1-C2 joint, cranial-cervical junction, C2 nerve roots. Once cleared and exposed, 3 pairs of screws were placed and after confirmation by xrays and CT scan, were secured by a single rod on each side and then further affixed with cadaveric bone graft. The plastic surgeon then closed all 4 layers. The patient was cleaned and turned back to a supine position, extubated, and taken to the post-operative recovery unit. As the patient slowly returned from the depths of anesthesia, neuro checks were done, in specific his pupillary response, movement of tongue since the hypoglossal nerve runs through the anatomy where the surgery takes place, movement or his arms and legs. The patient was in extreme pain as the patient returned to consciousness and we made sure the patient was made as comfortable as possible to stay on top of the pain. I brought the patient’s family in to be by the patient’s side. Over the ensuing 24 hours we kept the patient comfortable and trying to help patient start to make some movements to get out of bed. The family and I discussed various ways we can help patient with pain.

I am optimistic for the patients future and I look forward to seeing this patient through the road of recovery.

Stay tuned here for further updates on my experience as a neurologist observing this journey of a patient. I never take any surgery lightly and I offer various treatments and management options before proceeding with recommendation of surgery.

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