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chronic limb threatening ischemia edema iliocaval venous interstitium lymphedema opinion Practice techniques

The Squeeze Play: Managing a Lymphedema Emergency

When I was a young attending at the Allen Pavilion of Columbia Presbyterian Hospital, I was called into an operating room for a stat consult on a patient about to undergo a cholecystectomy. During the case, the IV had infiltrated and a bag of saline had filled the patient’s hand and forearm with saline, causing the hand to look like an inflated glove. The fingers were cool and white and the edema was firm but yielded to touch.

I elevated the hand and firmly squeezed the edema out of each digit, then gently massaged the edema from the hand onto the forearm. From there, I pushed the edema onto the arm. I then wrapped the hand up in an Ace wrap, and suspended it from an IV pole and returned to my case. Later, I returned and the hand was restored, warm, and perfused.

The lymphatics serve to move extracellular fluid (link). They can be overwhelmed much as drainage from a house can be overwhelmed resulting in puddles and ponds (link). This extracellular space has been “discovered” to be a new organ, but vascular surgeons have known about it for some time. Ultrastructurally, it is very close to a sea sponge with lattices of structural protein connecting cells to form tissues. And like a sea sponge, the salty water can be squeezed out or drained using gravity.

In olden times in central Europe, if you had chronic leg ulcers, you went to abbeys that specialized in their care. There, nuns would milk the edema out of your leg swollen typically from parasites and dress the leg and ulcer in linen cloth soaked in special oils. This is how Dr. Paul Gerson Unna came up with his eponymous Unna’s Boot, substituting Zinc Oxide paste which created a bacteriostatic environment.

Professor Paul Gerson Unna

Every year or so, I will be consulted for what I term a lymphatic emergency. A subset of this is phlegmasia. Whatever color you find -alba (white) or cerulea (blue) is really no matter -who really knows which comes first? It is an emergency in that the time clock for arterial ischemia -minutes to an hour for nerves, an hour to 6 for skeletal muscles, 6-12 for skin and bone, are all in play. The instinct is to go right to fasciotomy, but what you are usually doing is releasing the extracellular space, and the muscles are typically fine, even though their compartment pressures were very high.

Take this patient who developed severe upper extremity edema in the recovery phase after a cardiac arrest.

The ICU staff noted the had discoloration about four hours after the arrest. There were no arterial pulses and the forearm and hand were rock hard, the finger tips ice cold. Compartment pressures measured using the arterial line and needle method didn’t drop after the initial flush of saline below 70mmHg. While I could have been justified in performing upper extremity fasciotomy and even trying thrombectomy in a critically ill, coagulopathic patient on multiple pressors, I could just as easily have been on solid ground for saying the life was more valuable than the dominant hand. Both would have been the wrong move.

I performed the nun’s milking maneuver mentioned at the beginning and lacking an Unna’s boot, I compressed and elevated the best I could with double gloving using a small sized glove and ACE wrap.

Notice the edema has segregated into the arm.

In the morning, taking down the dressing, and re-compressing, there was now a radial artery signal and the fingers were a much improved color. The pulse-oximeter waveform was near normal. As an aside -the pulse oximeter uses the same technology as the digital photoplethysmography for generating toe waveforms in the vascular lab -ie. a vascular lab at every bedside! We have collected and are analyzing the data on this for publication.

The pulse oximetry waveform is the same tech as digital photoplethysmography. Cotton cast padding (Webril) and Coban wrap is a good method of compression that avoids the problems with ACE wrapping.

It’s a hard thing to not run off to the operating room in most cases because that is how we are trained, but understanding how a patient got to that point is crucial in deciding if compression alone will work. If they call you from the ER about a patient with a swollen cold foot with diminished signals, you have to figure out the mechanism. Was it arterial occlusion, rest pain, and chronic dependency of the foot that resulted in this? Typically the swelling appears late. Was it heart failure and inability to walk, resulting in the patient sitting all day in a chair that is the cause? Was it pregnancy with a DVT? Was it the deadly sin of sloth? Only in arterial occlusion in a chronic presentation would compression be contraindicate. In this ICU case, the lack of arterial signal is secondary to the swelling, not the cause of it.

Elevation alone does not manage edema well. Only hanging upside down or being in water up to your neck…

Compression is a necessary component of treating lymphedema emergencies because elevation alone may be insufficient, particularly in the leg.

Wrapping a leg is a critically, undertaught skill. Also, never cover the knee cap.

Elastic compression is ubiquitously available as the ACE wrap, but they can shift and move and roll, causing zones of excess and not enough compression. TED hose and compression stockings are definitely helpful in long term management, but with legs, compression needs to go up to the knee joint, or up to the groin, never halfway or the edema will create a line of ischemia at the end of the stocking that blisters when the stocking is removed, and can progress to full thickness necrosis. Cotton cast padding and Coban, or an Unna’s Boot may be the safest in terms of avoiding skin injury.

ACE wrapping is never taught adequately, and for it to work well and avoid injury to the skin, the wrapping has to be reapplied several times a day. It should be a prerequisite for nursing and medical student certification, as edema is the most common vascular disease.

Moving into our new home after four years out of country, I welcome an old friend from storage, but also unfortunately a health hazard, only mitigated by being fully reclinable.
Categories
AIOD aortoiliac occlusive disease (AIOD) Commentary humor opinion PAD

Perfectly Compulsive, Perfectly Smart

Recently, in clinic, my nurse handed me the patient sheet with the comment, “this is for iliac stents.” This caught my attention as “iliac stents” does not make sense as a chief complaint. The patient had been sent with a vascular lab report. It was a duplex scan documenting peak systolic velocities over 300cm/s in the common iliac arteries, appropriately diagnosing 50-99% stenoses. The patient had hip and thigh pain with walking short distances. I could have been excused for just cancelling the visit and booking an angiogram, except that would make me just a technician responding to a request. So I talked to the patient.

The patient was a nice lady over 70 years of age with recent onset of hip and thigh pain with walking 50-100 feet. This was incapacitating her as she was used to living an active and independent lifestyle. Her pulse examination was normal, not an uncommon finding with aortoiliac occlusive disease which manifests as a hemodynamic phenomena best explained as “small pipes.” Except she had never smoked, and had only hypertension and mild hypercholesterolemia. The review of systems was notable for fatigue and arm and shoulder pain. While she had not lost weight, strangely, her jaws hurt when chewing food.

I do not claim any kind of magic skills when it comes to diagnostics, but these other complaints did not fit. And it is not uncommon for someone to have several common conditions. Maybe she had TMJ, shoulder arthritis, early heart failure, and aortoiliac occlusive disease, to fit all of her complaints. Why was I wasting my time diving into nonvascular ephemera when I could be sending her to be scheduled for an aortogram and iliac angioplasty and stent?

I’ve carried with me this notion that all physicians can be mapped on x-y axes with one axis representing degrees of intelligence peaking at perfectly smart. Perfectly smart doctors have seemingly magical skills. While they are not rolling back their eyes while waving their hands over the patient, the handful of perfectly smart physicians I have worked with can quietly listen and digest a case and come up with the diagnosis, no matter how obscure and rare. On the other axis is compulsion, with the perfectly compulsive marching their patients through every test and algorithm to rule out every diagnosis on a exhaustively long differential list.

Intelligence and Compulsion, Written with a Doctor’s Penmanship

Those striving to be perfectly smart hope to bring efficiency to the clinical process -such as for this patient, it would have made sense for efficiency’s sake to move forward with an exercise treadmill ABI test and booking for an aortogram. Those stuck in perfect compulsion never quite reach a diagnosis, even after ordering batteries of tests, but rarely make mistakes, which is the point of perfect compulsion, because if you carpet bomb the diagnostic possibilities, something will hit. They are especially bulletproof to malpractice, particularly when patients choose not to have any more tests out of exhaustion. Their patients are rarely happy having to go through a myriad of tests to paint away the rule-outs while never quite identifying the disease. Those who play around with being perfectly smart get burned by that which are unknown and unfamiliar. They get blindsided. You want to revert to compulsion when you are tired and overloaded. You want to be smart, all the time.

The point of training, which never ends, is you have strive to be both perfectly smart and selectively compulsive, but it’s better to be lucky than good. It was my luck that I recently reviewed temporal arteritis. Every few weeks, I get asked to remove temporal arteries, and choosing not to be just a technician (although admittedly in the workup of TA, we kind of are), I plowed into UpToDate and Pubmed, seeing if there was a way out of doing these procedures -there really is not, except in the requests for temporal artery biopsy in younger patients -go read it yourself. It was here that I refreshed myself on polymyalgia rheumatica, which has as its symptom complex, muscle pain, lethargy, and jaw claudication. Out of duty, and compulsion, I ordered a CTA, because I knew that the patient had risk for atherosclerosis and arteries stiffened by calcium can have elevated velocities without critical stenoses. Out of curiosity, and after a quick call to one of the Clinic’s rheumatologists who order these temporal artery biopsies, I ordered an ESR and CRP.

The CTA came back with calcium at the aortic bifurcation and origins of the common iliac arteries where the outside duplex showed elevated velocities, but only revealed mild disease on the CTA. Both ESR and CRP came back very elevated. I referred the patient to our rheumatologist, and with steroid therapy, all of her symptoms resolved. Without an aortogram or stents.

I sat and thought about this for a while before posting. The patient was quite happy to give her permission. I cannot fault the outside vascular lab for their diagnosis of iliac stenosis because the diagnostic criteria are basically the same as our labs. It has made me think that approaching this case as a revenue opportunity as increasingly happens would not necessarily have been in error if I had performed an aortogram as long as I did not place stents. I can’t imagine the pressures put upon physicians who have put themselves into situations where they are paying for costly angio suites or their own 90th percentile salaries and lifestyles from not over-calling a stenosis and deploying stents, particularly when there is no oversight.

41 percent of my patients with median arcuate ligament syndrome present missing their gallbladders because biliary colic was the diagnosis that was both familiar and vaguely fit the complaints (reference 1). Not much harm can come from taking out a gallbladder, no? We know that a minority of operators harvest a significant share of the Medicare pie when it comes to peripheral interventions (link to terrific OPED, reference 2). Oh, I am sure each of these cases can be “justified.” Pleading justification from limits of knowledge means I proceed to treat what I am familiar and comfortable with -vascular disease, rather than an unfamiliar disease (at least to vascular surgeons) like polymyalgia rheumatica. If I can fail to recognize my ignorance, who can fault the perfectly compulsive? Like a broken clock that can be correct twice a day, someone of poor intelligence but perfect compulsion can be more effective than a greedy hack seeking to be perfectly smart and efficient.

Dunning and Kruger found that those with lower competence overestimate their ability, and those with higher competence underestimate their ability. Medicine is a perfect laboratory of Dunning Kruger. To be effective, you have to be correct and assertive. The problem is you are trained to project that confidence in the early stages of training and career when you are not ready. What patient would seek an unconfident physician? What person truly knows what they don’t know? The hardest step in medicine is both admitting what we don’t know but also applying hard-gained knowledge and experience with audacity. True humility comes from self knowledge and awareness. False modesty is externally directed, but true humility is internally focused. I don’t have a pat answer, but to become perfectly smart, you have to be perfectly compulsive about filling your knowledge and experience base. You have to submit your complications for peer review, you have seek and collaborate with sound partners, and you have to avoid financial traps that bias you to bad behavior. Above all, you have to stay curious.

References

  1. Weber JM, Boules M, Fong K, Abraham B, Bena J, El-Hayek K, Kroh M, Park WM. Median Arcuate Ligament Syndrome Is Not a Vascular Disease. Ann Vasc Surg. 2016 Jan;30:22-7. doi: 10.1016/j.avsg.2015.07.013. Epub 2015 Sep 10. PMID: 26365109.
  2. Sheaffer WW, Davila VJ, Money SR, Soh IY, Breite MD, Stone WM, Meltzer AJ. Practice Patterns of Vascular Surgery’s “1%”. Ann Vasc Surg. 2021 Jan;70:20-26. doi: 10.1016/j.avsg.2020.07.010. Epub 2020 Jul 29. PMID: 32736025.