The Pain Operations

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Absolute Neutral Position is suprisingly universal

A body floating in space, a fetus in the womb, a dad lounging in his favorite chair, share the feature of weightlessness and represent the absolute neutral position (figure above) of the human which is the position of a relaxed supine quadruped -a dead mouse. Anything else is a stress position, including standing. Repeating motions outside of this relaxed pose or holding those positions away from this absolute neutral for long periods of time is a nidus for injury and pain. That is why most land animals sleep flat on the ground.

The Pain Operations

Operations to relieve pain are often the most gratifying to both patient and surgeon to perform successfully. This circumstance applies to the commonly performed procedures such as spine surgery, endometrial ablations, and varicose vein resections. When the pain is due to a rare set of circumstances, things are not so easy. Typically for rarer pain syndromes, two things need to coincide for the successful operation to happen. First is the patient must suffer while more common and potentially life threatening diseases are ruled out and even treated if these are found. This may take months or years. The second necessary condition is finding a physician who has seen the particular pain syndrome before and understand how to test for it and treat it. That meant the majority of people never get treated, or are shunted into the circle of shame as malingering, drug seeking, and mentally unstable. The opioid epidemic creates double jeopardy for these patients -they can become addicts as their pain is never successfully diagnosed and treated and they get labeled as drug seeking.

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A random list of conditions that cause pain that should be on the mind after the usual things are ruled out. Also, vasculitis, autoimmune disorders, and foreign body reactions

All pain syndromes that can be successfully treated share common features that give you a degree of surety about the diagnosis, but at the end, there is a leap of faith on the part of both patient and practitioner because many of these operations have a failure rate ranging from 5-20 percent. First, the symptoms must be associated with sensory nerves, somatic or visceral. Second, there is a physical mechanism for that nerve to be inflamed from compression, swelling, or irritation that can be accounted for through history, physical examination, and imaging studies. Third, though not a constant, a major nerve trunk will be associated with a blood vessel, typically and artery, that is also affected by compression. Fourth, when swollen veins are the cause of pain, it has to be recognized that at an end stage the organ that the veins drain can also be affected.

The Pain Must Have a Testable Anatomic Basis

The somatic sensory nerves in the periphery are well mapped out and known since even classical times. The described pain should be consistent with a nerve. The best and easiest example is a neuroma that forms in an amputation stump. It triggers pain in its former distribution. It is palpable as a nodular mass. It is visible under ultrasound or cross sectional imaging. And it is easy to turn off temporarily with an injection of lidocaine, either under palpation or image guidance. If you can turn off the nerve and relieve the pain, it is likely that ablating or relieving the nerve of irritation will also relieve the pain. Such is the case in median arcuate ligament syndrome (figure below).Screen Shot 2019-03-03 at 5.34.02 PM.png The celiac plexus is caught under the median arcuate ligament and compressed. It causes a neuropathy that is felt in its visceral sensory distribution and the brain interprets these signals in the typical ways irritation of the stomach is interpreted -as pain, burning, nausea, sensations of bloating, and general malaise. These nerves can be turned off with a celiac plexus block and the effects tested by giving the patient a sandwich. When it works, the patient will say they will have had relief for the first time in years and operation to relieve the ligament compression and ablate the nerve can proceed. Same for many of the diseases listed.

Tight Spaces Impinging Nerves, Arteries, and Veins

Many of the tight spaces involving the nerves have accompanying arteries that are compressed. This results in injury to the artery in the form of intimal hyperplasia, post stenotic dilatation, aneurysm formation, and thromboembolism. Shared tight spaces that cause problems for nerves and arteries have the common features of fixed ligaments, adjacent bones and muscles, inflammation, and motion. These include the thoracic outlet, antecubital fossa, cubital canal, diaphragmatic hiatus at median arcuate ligament, inguinal ligament, popliteal fossa, carpal tunnel, obturator canal, mediastinum, retroperitoneum -basically anywhere nerve, compression, and motion occur. In some instances of median arcuate ligament syndrome, postures and breathing trigger the pain. Holding a child in an arm may trigger pain in neurogenic thoracic outlet. Or sitting while wearing tight jeans may trigger a burning pain in meralgia paresthetica. It is not uncommon to find damaged arteries in median arcuate ligament syndrome, thoracic outlet syndrome, and popliteal entrapment or thrombosed veins in nutcracker syndrome, May-Thurner Syndrome, and Paget-von Schroetter Syndrome. Because nerves are typically difficult to visualize, their compression may only be inferred by testing for compression in their adjacent arteries.

Dilated Veins and Swollen Organs and Visceral Pain

Venous hypertension is most commonly conceived of as varicose and spider veins of the legs and offer a model of pain when applied to other pain caused by venous dissension. The visceral sensory fibers veins and arteries trigger a very intense pain that localizes to the trigger. I have often witnessed this when I manipulate a blood vessel during local anesthesia cases. Visceral pain from swelling has a dull achiness that is localizable to my spider veins after a long day standing like a bruise (below).my spider vein The swelling from varicoceles which I have also had feel nothing less than feeling the aftereffects of getting a kick in the balls -not the immediate sharp pain but imagine about 5 minutes after with the mild nausea, abdominal discomfort and desire not to move too much, and even a little flank pain. Imagine this occurring low in the pelvis with ovarian vein varices in pelvic congestion syndrome. This kind of swollen gonad pain afflicts many women whose pain is so frequently dismissed by male physicians because they have no context -well imagine getting kicked in the balls hard, wait about 5 minutes and that moment stretch it out to whenever you stand for a long period of time (below).

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Actual Slide From Midwest Vascular Surgery Traveling Fellowship talk 2017, Chicago, IL, USA

 

When a limb is swollen from a thrombosis, the veins hurt and is similar to a bone pain from a fracture or a pulled muscle -that is how the brain processes the pain, but when the muscles and skin get tight from edema, the pain is sharp and dire. This is the same kind for pain from a distended left kidney from nutcracker syndrome or a spleen from a splenic vein thrombosis. These conditions can be modeled and predicted based on history and correct differential and confirmed with proper imaging -always.

 

Build a theory of the pain based on a testable proposition and set of nerves

That is the final message. These pain syndrome require some imagination and empathy to map and model. Predictive tests then can be performed on physical examination, functional testing, or imaging. Often, the adjacent artery is the only thing that can be reliably visualized and tested, knowing that it is the nerve that is compressed. Turning off the offending nerve with a block and relieving the pain is the most powerful argument for operating. It is building the argument for an operation that requires these objective data, but at the end, it does require some experience and faith. You have to believe in your patient and the science and when they coincide, you have to act.

POTS+May-Thurner’s Syndrome: Rare Disease Causes Rare Disease?

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The patient is a younger man in his twenties who began having dizzy spells associated with near syncope and tunnel vision. He was previously an athlete and was fit and never had such episodes -he had a resting heart rate typically in the 60’s or lower. Workup for arrhythmias was ultimately positive for POTS -postural orthostatic tachycardia syndrome and he was referred to Dr. Fredrick Jaeger of our Syncope Clinic. Tilt table testing the demonstrated the reported tachycardia over 140bpm while upright rising from 60bpm while supine. A radionuclide hemodynamic study (Syncope Radionuclide Hemodynamic Test) showed 54% of his blood volume pooled in his left lower extremity and lower abdomen with upright posture. Air plethysmography (PHLEBOTEST) showed abnormal refill and fill times in both legs and a duplex of the legs showed deep venous reflux in both legs. MRV revealed narrowing of left common iliac vein by the overlying right common iliac artery (May-Thurner’s Syndrome, MTS), and this was where the patient came to my clinic.

The MRV, shown above and below showed the typical pathoanatomy for MTS, but the patient had no symptoms related to left leg swelling, DVT, or varicosities. He did have a reducible left inguinal hernia which was quite tender.

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After some deliberation, while not promising anything regarding his POTS, I agreed to proceed with treating his pathoanatomy. Discussion with Dr. Jaeger revealed this: normally about 20% of blood volume parks in the legs with standing which is rapidly dissipated with normal calf muscle pump action. In a subset of patients with POTS, there is a 30-40% maldistribution of blood volume into the legs which may or may not drive the autonomic responses leading to POTS. He has never seen a study result showing a 54% distribution.

It made physiologic and anatomic sense to me to proceed with a venogram and intervention, but I confess I was dubious about any affects I might have on the patient’s POTS and I informed him of it. Also, I recommended seeing a general surgeon for his hernia.

Venography showed obstruction of his left common iliac vein as evidenced by the filling of pelvic and lumbar collateral veins.

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Intravascular ultrasound showed the narrowing better and more directly (panel below). The right common iliac artery narrowed the left common iliac vein severely.

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A 22mm Wall Stent was positioned across this and dilated with a 22mm balloon in the IVC and an 18mm balloon in the iliac vein. The resulting venogram showed resolution of the obstruction with collateral veins no longer visualized (below).

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But again, IVUS demonstrated more directly the result (and illustrates the importance of having IVUS available for venous interventions).

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The patient was discharged after procedure on a baby aspirin only. He subsequently underwent laparoscopic inguinal herniorrhaphy and returned to my clinic about a month later. His followup duplex showed a widely patent stent and normal flows in the left iliac venous system.

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Surprisingly -the patient declared that he was cured of his POTS. He said since the stents went in, he has not had any more episodes of near syncope, dizziness, tunnel vision, nor weakness requiring lying down to rest. His wife confirmed that he was a flurry of activity over the holidays that was surprising considering how debilitated he was before. This is astonishing to me.

But it should not be a surprise given this: if the POTS symptoms were the result of autonomic dysregulation, a breakdown of the feedback control loop, there were only several places this could be a problem.

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The pathology, the MTS, explains the POTS in this instance very nicely. Because the problem was in the cardiovascular system part of the diagram which I can fix and not the autonomic nervous system control element, which I can’t fix yet, a solution could be tried. This was not an asymptomatic compression of the iliac vein which we do encounter as an incidental finding. It seems to be POTS caused by MTS, and cured for now by treatment of the MTS.