Recent Case

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pgg

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I haven't posted one in a while. Nothing tricky, just a rare condition, fun case.

43 year old male with no prior medical or surgical history admitted for chest pain and headache after collapsing during exercise. He described about a year of exertional angina and palpitations. BP on admission was 200/110. Labs of note were Cr 2.4, Hb 19.5, glucose 366. Peak troponin of 7.50, BNP 306. Negative drug screen. Prior to transfer to our institution he was diagnosed with polycythemia vera and was phlebotomized 300 mL. Reportedly they felt his MI might have been related to hyperviscosity. Family history of early cardiac events.
Admitted to our ICU for hypertensive crisis, NSTEMI, ARF, still with the working diagnosis of polycythemia vera. BP controlled with hydralazine and clonidine. Renal ultrasound was done (presumably looking for renal artery stenosis) which showed a right adrenal mass, and followup CT showed a 4x4x5 cm adrenal mass. Eventually the word pheochromocytoma shows up in the chart. Confirmatory labs sent out.

Around hospital day 5 we get consulted re: scheduling the adrenalectomy. BPs have been controlled for several days now, and they say he's optimized.

Questions for students and residents.
1) How do you optimize a pheo patient for surgery?
2) How do you know when they're optimized?
3) How long does it take to optimize them?
4) What if you don't optimize them?
5) This patient had an MI. What cardiac workup does he need, if any?
6) How long after his MI should the tumor resection be delayed? Should it be delayed at all?

These can be fun cases intraop, but most of the important work is preop preparation, so I'll leave it there.
 
CA-1 here. Haven't done one yet but did see one in the preop clinic. Will give it a go.

Questions for students and residents.
1) How do you optimize a pheo patient for surgery?
Find out what is the predominant catecholamine secreted. Control the BP with alpha blocker FIRST and add beta blockade later to prevent unopposed alpha and hypertensive crises.
2) How do you know when they're optimized?
Have the patient monitor their BP and HR at home with a log. Validate and make medication dosing changes based on this log and your in office verification of BP and HR. Sufficient blockade will likely result in orthostasis. The "polycythemia" is likely just intravascular volume depletion from the catecholamine surge. They should have some of this replaced prior to the procedure.
3) How long does it take to optimize them?
I would think a week or two.
4) What if you don't optimize them?
They are at risk for intraoperative hypertensive crises and death.
5) This patient had an MI. What cardiac workup does he need, if any?
I think at minimum this patient needs and echo to evaluate his systolic and diastolic function. He is at risk for cardiomyopathy from the prolonged catecholamine surge. It's unclear whether is exercise induced NSTEMI was due to cardiomyopathy and demand ischemia or to some underlying CAD. I would call a cardiac colleague to discuss possible angiography to help me further investigate.
6) How long after his MI should the tumor resection be delayed? Should it be delayed at all?
I would delay for at least 8 weeks. He is at baseline increased risk for perioperative mortality given his pheo. The recent MI increases this risk. Provided he is well optimized waiting 8 weeks while maintaining close follow up should not be an issue.
 
CA-1 here. Haven't done one yet but did see one in the preop clinic. Will give it a go.

Questions for students and residents.
1) How do you optimize a pheo patient for surgery?
Find out what is the predominant catecholamine secreted. Control the BP with alpha blocker FIRST and add beta blockade later to prevent unopposed alpha and hypertensive crises.
2) How do you know when they're optimized?
Have the patient monitor their BP and HR at home with a log. Validate and make medication dosing changes based on this log and your in office verification of BP and HR. Sufficient blockade will likely result in orthostasis. The "polycythemia" is likely just intravascular volume depletion from the catecholamine surge. They should have some of this replaced prior to the procedure.
3) How long does it take to optimize them?
I would think a week or two.
4) What if you don't optimize them?
They are at risk for intraoperative hypertensive crises and death.
5) This patient had an MI. What cardiac workup does he need, if any?
I think at minimum this patient needs and echo to evaluate his systolic and diastolic function. He is at risk for cardiomyopathy from the prolonged catecholamine surge. It's unclear whether is exercise induced NSTEMI was due to cardiomyopathy and demand ischemia or to some underlying CAD. I would call a cardiac colleague to discuss possible angiography to help me further investigate.
6) How long after his MI should the tumor resection be delayed? Should it be delayed at all?
I would delay for at least 8 weeks. He is at baseline increased risk for perioperative mortality given his pheo. The recent MI increases this risk. Provided he is well optimized waiting 8 weeks while maintaining close follow up should not be an issue.

Save some questions for the rest of the class!
 
Pent Sux Tube
 
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Good answers

CA-1 here. Haven't done one yet but did see one in the preop clinic. Will give it a go.
Find out what is the predominant catecholamine secreted. Control the BP with alpha blocker FIRST and add beta blockade later to prevent unopposed alpha and hypertensive crises.

Endocrine started him on phenoxybenzamine, then later added metoprolol. They plan on adding metyrosine in the 4 days prior to surgery.

He also got a nuclear scan looking for active mets or ectopic tumor ... none, just the single adrenal mass.

Have the patient monitor their BP and HR at home with a log. Validate and make medication dosing changes based on this log and your in office verification of BP and HR. Sufficient blockade will likely result in orthostasis. The "polycythemia" is likely just intravascular volume depletion from the catecholamine surge. They should have some of this replaced prior to the procedure.

Right, after the presenting crisis is controlled, these patients can usually go home. This patient was a social admit, no home to go to. He had a very good BP log courtesy of diligent ward nurses bothering him for supine and standing BPs all the time.

They gave him salt tablets with meals to expand his intravascular volume. Not polycythemia vera at all ... we spent some time mocking the guys who gave him the dark ages leech treatment for a pheo.

I would think a week or two

Most sources will say minimum 10-14 days, and there's no reason to rush. It's not just comfortably controlling BP ... it's volume re-expansion, and time for the downregulated catecholamine receptors to get back to normal.

Mortality is something like 15-30% in untreated patients. Sufficiently optimized, it's probably under 3%.

A bad day would've been this guy showing up in the ER a month earlier with a fracture needing surgery.

I think at minimum this patient needs and echo to evaluate his systolic and diastolic function. He is at risk for cardiomyopathy from the prolonged catecholamine surge. It's unclear whether is exercise induced NSTEMI was due to cardiomyopathy and demand ischemia or to some underlying CAD. I would call a cardiac colleague to discuss possible angiography to help me further investigate.

Echo is almost normal, mild concentric LVH, normal LV function, EF 60-65%, normal valves. Cardiology thinks the NSTEMI was all demand related, but because of the family history, they get a cath. Totally clean. They recommend no further treatment.

I would delay for at least 8 weeks. He is at baseline increased risk for perioperative mortality given his pheo. The recent MI increases this risk. Provided he is well optimized waiting 8 weeks while maintaining close follow up should not be an issue.

Does a demand NSTEMI / troponin leak in a patient with a clean cath and a normal echo need to wait? There's nothing to revascularize. This patient's cardiac function is completely normal. But,
2014 ACC/AHA Guideline on Perioperative Cardiovascular Evaluation and Management of Patients Undergoing Noncardiac Surgery said:
MACE after noncardiac surgery is often associated with prior CAD events. The stability and timing of a recent MI impact the incidence of perioperative morbidity and mortality. An older study demonstrated very high morbidity and mortality rates in patients with unstable angina. A study using discharge summaries demonstrated that the postoperative MI rate decreased substantially as the length of time from MI to operation increased (0 to 30 days = 32.8%; 31 to 60 days = 18.7%; 61 to 90 days = 8.4%; and 91 to 180 days = 5.9%), as did the 30-day mortality rate (0 to 30 days = 14.2%; 31 to 60 days = 11.5%; 61 to 90 days = 10.5%; and 91 to 180 days = 9.9%). This risk was modified by the presence and type of coronary revascularization (coronary artery bypass grafting [CABG] versus percutaneous coronary interventions [PCIs]) that occurred at the time of the MI. Taken together, the data suggest that ≥60 days should elapse after a MI before noncardiac surgery in the absence of a coronary intervention. A recent MI, defined as having occurred within 6 months of noncardiac surgery, was also found to be an independent risk factor for perioperative stroke, which was associated with an 8-fold increase in the perioperative mortality rate.

10 days after he started his alpha blocker therapy he met criteria for adequate control. The criteria on openanesthesia.org are typical:
Treatment Regimen
- At least 10-14 days (there is no rush to remove these slow growing tumors)
- Alpha blockade (phenoxybenzamine) in all patients
- Beta blockade (propranolol) if arrhythmias/tachycardia persist

Goals on DOS
- No reading > 165/90 within 48 hours of surgery, including 1 hour of stressful readings (ex. PACU, SAS)
- Orthostatic hypotension should not be lower than 80/45
- No EKG ST or T changes
- PVCs less than one every 5 minute

Pheos are slow growing tumors, but they are tumors, and some are malignant. Do we need to wait 50 more days before resecting it? Should we?
 
I'll get to the point and finish this off.

One reason I posted this case, besides the fact that pheos are cool, was to give a concrete example of a case for which the ACC/AHA guidelines were appropriately disregarded. They are guidelines, not standards. Sticking to the letter of the AHA/ACC guidelines in a patient with a normal heart and no CAD makes about as much sense as sticking to the letter of ACLS when running an intraoperative code. Both sets of guidelines are based on patients and data that are quite different than the patient in front of you. They're useful for framing your thoughts and making some risk estimates, but you still have to think.

This patient had no coronary disease, and no evidence of cardiomyopathy from the chronic elevated catecholamine exposure. He carried the diagnosis of NSTEMI, but there's a world of difference between a recent MI in a patient with coronary disease, and a troponin leak from a heart that was healthy but overworked and flogged with catecholamines. I judged that there was nothing to be gained, from the standpoint of cardiac optimization, by delaying. For his part, the cardiologist agreed.

We did the case 12 days into his alpha blockade. He was, as are all pheos, extremely labile. The vasoactive drugs we gave ranged from 300 mcg/min of nitroglycerin plus boluses of nitroprusside just prior to ligation of the adrenal vein, to norepinephrine 14 mcg/min + 4 mcg/min epinephrine + 0.5 U/min vasopressin after ligation. He did well and was discharged on POD 2.
 
I'll get to the point and finish this off.

One reason I posted this case, besides the fact that pheos are cool, was to give a concrete example of a case for which the ACC/AHA guidelines were appropriately disregarded. They are guidelines, not standards. Sticking to the letter of the AHA/ACC guidelines in a patient with a normal heart and no CAD makes about as much sense as sticking to the letter of ACLS when running an intraoperative code. Both sets of guidelines are based on patients and data that are quite different than the patient in front of you. They're useful for framing your thoughts and making some risk estimates, but you still have to think.

This patient had no coronary disease, and no evidence of cardiomyopathy from the chronic elevated catecholamine exposure. He carried the diagnosis of NSTEMI, but there's a world of difference between a recent MI in a patient with coronary disease, and a troponin leak from a heart that was healthy but overworked and flogged with catecholamines. I judged that there was nothing to be gained, from the standpoint of cardiac optimization, by delaying. For his part, the cardiologist agreed.

We did the case 12 days into his alpha blockade. He was, as are all pheos, extremely labile. The vasoactive drugs we gave ranged from 300 mcg/min of nitroglycerin plus boluses of nitroprusside just prior to ligation of the adrenal vein, to norepinephrine 14 mcg/min + 4 mcg/min epinephrine + 0.5 U/min vasopressin after ligation. He did well and was discharged on POD 2.
So you're saying that, had you waited more, the patient would have been equally unstable?

Friendly question: What would be your answer in court, had the patient died?

Also, from my standpoint, the problem is equally that these "troponin leaks" can be as dangerous and myocardium stunning as MIs. A healthy heart is not healthy anymore after swimming in toxic catecholamines for years. There is a reason we give beta-blockers in CHF.

Why risk it? The lawyers will stick those guidelines where you wouldn't want them to. That's the problem with having them in the first place.

Of course, you were the one who saw the patient, so your judgment is probably right, but it seems tough to defend in court. You will not be judged by your peers.

To exemplify: I recently cancelled an elective outpatient surgery under MAC for a preop sugar of 360 in an untreated asymptomatic diabetic. Reason: patient far from optimized for the procedure. Much smaller periop risk than in your case, so one of us must have been wrong. Who?
 
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Most pheos are benign, most don't metastasize ... most of them. I think if you're going to delay this case for two months, you need a better reason than a set of guidelines based on data from patients completely unlike this one. He didn't have any evidence of cardiomyopathy, normal echo, normal coronaries. We did of course all discuss this, including input from cardiology, endocrine, and the surgeon. What does delay achieve, except a bigger tumor?


I think elective outpatient surgery in a previously undiagnosed and untreated [diabetic] patient is very different.


[edit for clarity]
 
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It was a simple less than 30 minute procedure, under MAC. Anesthetic risk per se was minimal.

I postponed it because I have seen sugar jumps even during these, and I was not in the mood of discharging an untreated diabetic with a sugar of 400 (or a better looking number for just a few hours until my insulin would have been metabolized), who might slip into a coma after a nice big post-NPO feast. Besides the healing and infectious risks. But I had no evidence of being right, in this case, except that the patient could have been easily optimized a lot for her surgery. Which the surgeon unhappily accepted.

Now one could argue the same for jumping into pheo surgery after just 10 days, especially with a troponin bump. I need to look up the recommendations, but I remember a much more conservative number.
 
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Our uro and I delayed a case for pheo due to + urine tox for cocaine and meth. Brought her back a month later and took out a monster of a pheo that had grown substantially... unknown to us she had developed mets everywhre and subsequently died despite removal of the tumor + chemo. Sad but true. Aggressiveness is there and unpredictable. Nice case pgg. Brings up some good points.
 
Great points regarding taking a patient back for surgery post-MI who had a clean cath.

I recently had a T4 paraplegic with a ureteral stone starting to get septic. Had an MI a week earlier on the table trying to get it out electively. Case abandoned. I think it was related to autonomic hypereflexia but the anesthesia record wasn't clear and surgeons aren't very helpful with that stuff.

He was getting sicker and I went against cards recommendation and told the surgeon I would do it but the surgeon didn't want the liability. Cards said to wait 8 weeks. Whatevs. I am guessing he didn't do too well. Need to get an update on him. He was 450 lbs too. That didn't help.
 
Great points regarding taking a patient back for surgery post-MI who had a clean cath.

I recently had a T4 paraplegic with a ureteral stone starting to get septic. Had an MI a week earlier on the table trying to get it out electively. Case abandoned. I think it was related to autonomic hypereflexia but the anesthesia record wasn't clear and surgeons aren't very helpful with that stuff.

He was getting sicker and I went against cards recommendation and told the surgeon I would do it but the surgeon didn't want the liability. Cards said to wait 8 weeks. Whatevs. I am guessing he didn't do too well. Need to get an update on him. He was 450 lbs too. That didn't help.
Wonder why they didn't have IR place a nephrostomy tube
 
It was a simple less than 30 minute procedure, under MAC. Anesthetic risk per se was minimal.

I postponed it because I have seen sugar jumps even during these, and I was not in the mood of discharging an untreated diabetic with a sugar of 400 (or a better looking number for just a few hours until my insulin would have been metabolized), who might slip into a coma after a nice big post-NPO feast. Besides the healing and infectious risks. But I had no evidence of being right, in this case, except that the patient could have been easily optimized a lot for her surgery. Which the surgeon unhappily accepted.

Now one could argue the same for jumping into pheo surgery after just 10 days, especially with a troponin bump. I need to look up the recommendations, but I remember a much more conservative number.

Even if you postpone the case, you still have to discharge the patient. Did you treat the hyperglycemia?
 
Even if you postpone the case, you still have to discharge the patient. Did you treat the hyperglycemia?
Good point. I did not treat it. The patient was going to see her PCP the same day.
 
Great points regarding taking a patient back for surgery post-MI who had a clean cath.

I recently had a T4 paraplegic with a ureteral stone starting to get septic. Had an MI a week earlier on the table trying to get it out electively. Case abandoned. I think it was related to autonomic hypereflexia but the anesthesia record wasn't clear and surgeons aren't very helpful with that stuff.

He was getting sicker and I went against cards recommendation and told the surgeon I would do it but the surgeon didn't want the liability. Cards said to wait 8 weeks. Whatevs. I am guessing he didn't do too well. Need to get an update on him. He was 450 lbs too. That didn't help.

Would be good to hear the follow up.
 
Excellent topic.
Not sure if this is still the epidemiology, but classic teaching for pheo is that it's the 10% tumour:
10% bilateral
10% extra-adrenal
10% malignant
10% recurrent
10% familial
10% associated with MEN IIA or IIB
 
Good point. I did not treat it. The patient was going to see her PCP the same day.

Sorry to get off thread but......

For cases like cataracts and trigger fingers where patients present with hyperglycemia in the 300-400 range, I typically give insulin reg 10units Iv in Preop, do the case and then recheck the sugar in pacu before discharging them. Usually the sugar is better. And I tell the patients to follow up ASAP with their primary. Haven't had any issues so far.
 
Sorry to get off thread but......

For cases like cataracts and trigger fingers where patients present with hyperglycemia in the 300-400 range, I typically give insulin reg 10units Iv in Preop, do the case and then recheck the sugar in pacu before discharging them. Usually the sugar is better. And I tell the patients to follow up ASAP with their primary. Haven't had any issues so far.
My group's cutoff is 300, so I tend to have a reason not to respect it, even for something minor (it creates a precedent). In this case, the patient had been told repeatedly she had 300+ values, she just chose not to have the diabetes treated.

Of course the sugar will be lower after insulin, the question is for how long? For IV insulin, that's like a couple of hours. It's like giving bicarb before taking the CABG patient to the cardiac ICU: the numbers will look better, but it doesn't really change much, or for long. At least that's how I see it, no offense.

I had a similar case years ago, and I agreed to the minimal surgery. Postop I had to chase a sugar of 450 in an ASC PACU, while running between cases. Not the best option.

I know it's debatable (among us, but not in court), and the patient would likely be fine. I am just tired of cutting corners for elective surgeries in patients who don't give a crap about their own health. If anything happens, being nice to the patient is not a valid defense, even if the patient assumes the risks. Actually, we live in a paternalistic system where the patient is not allowed to assume avoidable risks, as if s/he were a minor.

The other day I sent a TSH of 25 packing (she would have been a GA). I researched it the day before and I couldn't find proof in the literature that the level was safe (most texts will mention a TSH of 5-10). Had the thyroid disease been unknown to the patient or PCP? No, it had been a longstanding battle to fix the TSH. Had she been sent to an endocrinologist for better diagnosis and management? Yes. Did she go? No. But I am the bad guy for going by the book and not assuming the risks?
 
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