Malignant Hyperthermia case

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hippuppy

hippuppy

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Earlier last year, two weeks before my oral boards, I had the craziest case of my young career. I was medically supervising a seasoned CRNA for a 50 y/o M for an ex-lap for sigmoid mass. Surgeons felt it could represent cancer or perhaps an abscess from recurrent diverticulitis. Patient was not the best historian but he had never had anesthesia before, rarely went to the doctor and denied family history of anesthesia issues.

We proceed with the case, GA, no epidural(refused) with a standard induction with roc. Initially was running Sevo and the nurse switched over to Des. Case was a long one (8 hrs when it was all said and done).

I had been going in and out every hour and following along on the EMR. About hour 6, I noticed a concerning trend. ETCO2 had increased to 61 despite an MV of 16L and now a temp of 38.8. When I presented to the OR, my nurse was on a break and another CRNA was present. I noticed a new CO2 absorbent on the back table and the Bair was on ambient. I immediately called for back up from one of my physician colleagues. We're not sure exactly but MH was obviously high on my ddx. We switched to a non-triggering anesthetic, called for the MH cart, got an a-line. ABG showed a resp acidosis. Lactic acid of 4.6. K was 5.3. No rigidity, hemodynamically stable. At this point we should have administered dantrolene but he actually began correcting his acid base status with the withdrawal of the des and administration of IV Tylenol. We all thought, "This can't be MH, he got better without dantrolene." My nurse thought it was just a long case and the patient was hot.

His urine was dark already because the dissection of the mass led to the bladder. We took him to the ICU intubated and he continued to improve clinically.

He was extubated on POD1 and doing well. CPK WAS 11k, wow. This was trending down. Ok, perhaps it was MH. I always thought that once it triggered it didn't stop until dantrolene was administered. Variable penetrance with MH makes this not the case.

I followed up with the patient on POD 2, he was on the floor. His mother was there. "The Anesthesiologist always told me I should tell them about this..." My jaw dropped. His mother pulled out of her purse a medic alert bracelet. On it was inscribed MALIGNANT HYPERTHERMIA. Her sister had nearly died 30 years ago from MH and she had this little gym sitting in her bedside table all those years. She had never shared this with her children.

This case brings up multiple issues. Physician-nurse communication and respect issues, the hesitancy to pull the trigger on Dantrolene because of the misconception that MH patients don't get better unless given Dantrolene and much more. He didn't receive sux and perhaps the least soluble Des allowed this to present like it did. Good news is my patient survived and I actually took care of him for his colostomy reversal. Non-triggering anesthetic no less.


Sent from my iPad using Tapatalk
 
Very interesting case. Thanks for sharing it.

Isnt it funny how MH is ingrained in us through teaching, simulations, ITE, etc.. We can recognize it so easily, as you first suspected it with the classic signs and symptoms. Yet it can be hard to accept that something so rare can happen in real life. We look for all the signs that point us away from the true diagnosis to reassure ourselves that a potential disaster can't be occurring.
 
Chloroform4Life said:
We can recognize it so easily, as you first suspected it with the classic signs and symptoms. Yet it can be hard to accept that something so rare can happen in real life. We look for all the signs that point us away from the true diagnosis to reassure ourselves that a potential disaster can't be occurring.
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What you just described sounds exactly like the practice of Emergency Medicine.
 
very nice. i'm not sure I would have thought MH right away. I thought ETCO2 usually climbs much higher than that? also, no rigidity nor metabolic acidosis. but like you said maybe lack of sux and use of des limited the reaction. is dantrolene useful when no muscle rigidity present?
 
leaverus said:
very nice. i'm not sure I would have thought MH right away. I thought ETCO2 usually climbs much higher than that? also, no rigidity nor metabolic acidosis. but like you said maybe lack of sux and use of des limited the reaction. is dantrolene useful when no muscle rigidity present?
Click to expand...

Agree, sounds like a slow rise, but certainly one to keep in mind in such a situation. Nice catch by the OP.

Dantrolene can cause further muscle weakness (although the lit says its for those with muscle disorders, I'm unsure of the general population) so I'm unsure if one should give it if there is no muscle rigidity. DC volatiles, avoid sux, cool the patient.
 
Thanks for posting this. It's collegial to describe cases that perhaps you would have handled differently. What a moment you had when she dropped that MH bracelet post-op. If that hadn't happened how would you have counseled the patient? Not really a slam dunk case....and you point out he had more surgery ahead.
 
For the rare times we've had suspicious cases, we always call the MHAUS hotline. They're much more than just giving you the recipe to follow in an MH case. They will walk through the differential with you, and use their extensive "we've seen/heard this before" to help determine if this probably is or isn't MH, or perhaps something similar. Many cases are pretty atypical - temp doesn't rise much, CO2 doesn't rise much, etc. They're the experts - and they're free!
 
First CO2 was 62.9 on ABG. I did call MHAUS and completed their questionnaire. They did not recommend dantrolene once the patient was recovering. If I hadn't seen the alert bracelet I would have informed him that he should have a non triggering anesthetic as there was a risk of possible MH.
 
Great case. Thanks for sharing.
I think I posted a case like this a few years back,but I can't find it.
DANTROLENE IS NOT SOMETHING TO BE AFRAID OF USING.
I had a case very similar to this in the middle of the night. I didn't give sux and it was a spinal trauma with neuromonitoring so I did TIVA. After the hardware was in I switched over to Des. About an hour later the pt had some muscle rigidity that we didn't really appreciate fully at the time. I was a bit concerned that it could be MH but there were no other signs. I turned off the gas and finished the case. His temp never went above 37.4 and CO2 remained easy to control without having to increase resp support. So at the end of the case I turned the gas back on so I could wake him up quickly since it was 3am and I wanted to go home. When I woke him up his CO2 began to rise really fast. But only to 60. He opened his eyes and I extubated him. Then he had the rigidity again and became unresponsive but everything else was normal. We Went to PACU and his temp was 37.6. And again 2 minutes later it was 38.2. And then 38.6. We packed him in ice and it came down to 37.6. So I didnt treat it. He was awake and fully responsive at this time. Long story short, I sent him to ICU overnight and told them to call if his temp got above 38. They called. I said pack him in ice and I'm on my way. They said, "he is in ice."
I said mix dantrolene and give it. It was pretty impressive how well it worked. It did make him weak but not enough to need to do anything about. I gave it two more times in 24hrs. Should have just started an infusion. He had difficulty walking that 24 hrs but once I stopped having to give dantrolene he was strong again. It never really affected respiratory mechanics. Mostly skeletal muscle weakness.
So, for those of you wondering about giving it or not. Don't think you only need to give it for muscle rigidity. My pt had that but it was fleeting. It was not the reason I gave it. I gave it because of the increasing temps and it worked almost instantly. My advice is don't wait. And don't be concerned about the weakness. It's not only given for rigidity. It will resolve the MH cycle. And you will more than likely be needed more than once.
When I read the original post I knew wha tit was right away because it sounded so much like my case. You don't forget these cases.
 
Noyac said:
Great case. Thanks for sharing.
I think I posted a case like this a few years back,but I can't find it.
DANTROLENE IS NOT SOMETHING TO BE AFRAID OF USING.
I had a case very similar to this in the middle of the night. I didn't give sux and it was a spinal trauma with neuromonitoring so I did TIVA. After the hardware was in I switched over to Des. About an hour later the pt had some muscle rigidity that we didn't really appreciate fully at the time. I was a bit concerned that it could be MH but there were no other signs. I turned off the gas and finished the case. His temp never went above 37.4 and CO2 remained easy to control without having to increase resp support. So at the end of the case I turned the gas back on so I could wake him up quickly since it was 3am and I wanted to go home. When I woke him up his CO2 began to rise really fast. But only to 60. He opened his eyes and I extubated him. Then he had the rigidity again and became unresponsive but everything else was normal. We Went to PACU and his temp was 37.6. And again 2 minutes later it was 38.2. And then 38.6. We packed him in ice and it came down to 37.6. So I didnt treat it. He was awake and fully responsive at this time. Long story short, I sent him to ICU overnight and told them to call if his temp got above 38. They called. I said pack him in ice and I'm on my way. They said, "he is in ice."
I said mix dantrolene and give it. It was pretty impressive how well it worked. It did make him weak but not enough to need to do anything about. I gave it two more times in 24hrs. Should have just started an infusion. He had difficulty walking that 24 hrs but once I stopped having to give dantrolene he was strong again. It never really affected respiratory mechanics. Mostly skeletal muscle weakness.
So, for those of you wondering about giving it or not. Don't think you only need to give it for muscle rigidity. My pt had that but it was fleeting. It was not the reason I gave it. I gave it because of the increasing temps and it worked almost instantly. My advice is don't wait. And don't be concerned about the weakness. It's not only given for rigidity. It will resolve the MH cycle. And you will more than likely be needed more than once.
When I read the original post I knew wha tit was right away because it sounded so much like my case. You don't forget these cases.
Click to expand...

Can always count on you to drop some knowledge bomb. Thanks!
 
This reminds me of a trauma case from residency. We were getting victims from the Boston marathon bombings. As you can imagine, many of these patients were being brought directly into the OR for amputations. One patient had a piece of paper attached to his chest by his wife before he was taken away from the scene in an ambulance. It read "Malignant Hyperthermia". The wife had the wherewithal to do that amidst all that chaos. Undoubtedly saved his life.
 
It'd be interesting to see what your case(s) scored in this:

Larach, MG et al. A Clinical Grading Scale to Predict Malignant Hyperthermia Susceptibility. Anesthesiology; 1994, 80, p771-779

I'm also surprised MHAUS said no to dantrolene as pt was getting better without it as like Noy said I've been taught to give dantrolene if suspicious as there's really no down side.
 
Wow, all interesting cases. The Boston case with the paper sign is simply amazing. I agree these are probably more common then we realize.
 
As a ca3, i was supervising a ca1 case where the etco2 kept going up and up despite increasing the minute ventilation. No other signs of mh. Abg and ck was completely normal. We didn't treat it as mh and pt did fine. Still not sure what that was.

There are only a few conditions with hypermetabolism and co2 productions. MH, NMS, serotonin syndrome, thyroid storm, and high carb TPN. Am I missing some?
 
hippuppy said:
This case brings up multiple issues. Physician-nurse communication and respect issues, the hesitancy to pull the trigger on Dantrolene because of the misconception that MH patients don't get better unless given Dantrolene and much more. He didn't receive sux and perhaps the least soluble Des allowed this to present like it did. Good news is my patient survived and I actually took care of him for his colostomy reversal. Non-triggering anesthetic no less.
Click to expand...

Very cool case. In a situation like that I wouldn't beat yourself up about not giving Dantrolene. When a patient is clinically improving, doing nothing is nearly always an acceptable response.
 
Chloroform4Life said:
As a ca3, i was supervising a ca1 case where the etco2 kept going up and up despite increasing the minute ventilation. No other signs of mh. Abg and ck was completely normal. We didn't treat it as mh and pt did fine. Still not sure what that was.

There are only a few conditions with hypermetabolism and co2 productions. MH, NMS, serotonin syndrome, thyroid storm, and high carb TPN. Am I missing some?
Click to expand...
Let me guess: This was a laparoscopic case with CO2 used to insufflate the abdomen?
 
Planktonmd said:
Let me guess: This was a laparoscopic case with CO2 used to insufflate the abdomen?
Click to expand...

Had that happen to me in residency, CO2 climbing, no other symptoms.

Crepitus Maximus. If the CO2 gets too high and you're unable to breathe it off, just have to pause surgery and desufflate for a bit to get the CO2 under control. Also not having a 5 hour robot hysto is optimal which I was lacking.
 
Planktonmd said:
You said the ABG was normal? meaning there was no hypercarbia on the ABG???
Click to expand...
Thats right. I think its one of the few times ive seen the paco2 smaller that the etco2. Of course the etco2 wasnt that high when we drew abg because we've been compensating with ventilator. We changed co2 absorbent and everything.
 
There doesn't need to be a metabolic acidosis nor muscle rigidity nor "unexplained" tachycardia. Out of context fever and respiratory acidosis is enough to pull the trigger on it's own.
 
drmwvr said:
There doesn't need to be a metabolic acidosis nor muscle rigidity nor "unexplained" tachycardia. Out of context fever and respiratory acidosis is enough to pull the trigger on it's own.
Click to expand...
What about just out of context respiratory acidosis? Because we know fever is usually a late sign.
 
Chloroform4Life said:
What about just out of context respiratory acidosis? Because we know fever is usually a late sign.
Click to expand...


What does "a late sign" even mean and does it matter? You treat hyperthermia when you see it because it's what kills people, not hypercarbia. If a patients temperature is normal during a 3 hour case until the first temp in PAR is 39, does that mean it was a late sign or that the event is just reaching it's full expression? It doesn't matter IMO because that's the point that I get help and treat.
 
drmwvr said:
What does "a late sign" even mean and does it matter? You treat hyperthermia when you see it because it's what kills people, not hypercarbia. If a patients temperature is normal during a 3 hour case until the first temp in PAR is 39, does that mean it was a late sign or that the event is just reaching it's full expression? It doesn't matter IMO because that's the point that I get help and treat.
Click to expand...
Actually, I think it may be considered a late sign sometimes because many times the pt starts off hypothermic and we don't see any real hyperthermia for possibly hours. I would propose that it is the rate of temp increase that we should be concerned with.
As far as the signs go, there are all sort of presentations. The board answer is that hyperthermia is a "late" sign. But as I stated above, that's not really accurate depending on the starting temp. Increased CO2 is usually an early sign but not always. Rigidity can be early late or middle sign. It just depends. I also think that presentations are so varied that we sometimes get confused or we delay our recognition for this reason.
So don't hang your hat on any sort of specific presentation. If the thought enters your mind then be vigilant. And start thinking about giving dantrolene so that you are ready when the diagnosis is more clear.
 
Just as an aside, personally speaking, just the thought of the wrestling match that is the reconstitution of 2.5 mg/kg of dantrolene is enough to make me want to rationalize away whatever might be creating an index of suspicion for MH. It is truly a circus and the OR nurses see to it that it's done with maximum drama.

Mercifully, the new formulation, 250 mg dantrolene reconstituted in 5 mls is now available and worth every penny more in cost that it probably is than Dantrium. You could quietly mix that up and give it before anyone knew what was going on.
 
drmwvr said:
Just as an aside, personally speaking, just the thought of the wrestling match that is the reconstitution of 2.5 mg/kg of dantrolene is enough to make me want to rationalize away whatever might be creating an index of suspicion for MH. It is truly a circus and the OR nurses see to it that it's done with maximum drama.

Mercifully, the new formulation, 250 mg dantrolene reconstituted in 5 mls is now available and worth every penny more in cost that it probably is than Dantrium. You could quietly mix that up and give it before anyone knew what was going on.
Click to expand...
I haven't seen the old stuff in awhile. The newer stuff mixes like Ancef. And every anesthesiologist should be good at that.
 
Thanks for sharing your cases. I had a similar pt encounter that could've gone disastrously. It was a TAH BSO. First case of the day. No surgical history. Apparently denied any family Hx of problems with GA.

Preop RN relayed a nebulous "allergy to propofol" message and when I asked pt about it, she said, "My father had something hyperthermia."
Me: "Oh? What happened? How did he do?"
Pt: "He died from it after surgery."
Me: "Was the term malignant hyperthermia used at any point?"
Pt: "OH YEAH THAT'S IT!"

It was the first time I did an MH case at a new hospital, and unfortunately the hospital did not have any Vapor-Clean (and never carried it). We switched the schedule so a machine could be flushed before that case. Pt did fine. Afterwards I told pt to get an MH bracelet and extensively counseled her and her family members how important it is to communicate MH family history to her healthcare providers.

During residency and afterwards I've been in 8 hospitals in NY and all of them had Vapor-Clean in MH cart. We called nearby hospitals and was told that they have an extra ventilator specifically for MH cases and also no charcoal filters. I've attached recommended washout time for various ventilator machines. So my question is, does your facility carry Vapor-Clean?
Washout Time.png
 
Foodie said:
Thanks for sharing your cases. I had a similar pt encounter that could've gone disastrously. It was a TAH BSO. First case of the day. No surgical history. Apparently denied any family Hx of problems with GA.

Preop RN relayed a nebulous "allergy to propofol" message and when I asked pt about it, she said, "My father had something hyperthermia."
Me: "Oh? What happened? How did he do?"
Pt: "He died from it after surgery."
Me: "Was the term malignant hyperthermia used at any point?"
Pt: "OH YEAH THAT'S IT!"

It was the first time I did an MH case at a new hospital, and unfortunately the hospital did not have any Vapor-Clean (and never carried it). We switched the schedule so a machine could be flushed before that case. Pt did fine. Afterwards I told pt to get an MH bracelet and extensively counseled her and her family members how important it is to communicate MH family history to her healthcare providers.

During residency and afterwards I've been in 8 hospitals in NY and all of them had Vapor-Clean in MH cart. We called nearby hospitals and was told that they have an extra ventilator specifically for MH cases and also no charcoal filters. I've attached recommended washout time for various ventilator machines. So my question is, does your facility carry Vapor-Clean?View attachment 202274
Click to expand...

Is <5ppm the recommended concentration for MH?
 
At the academic center I work, we have two MHAUS members who take call. Both of them have said that the "late sign" of hyperthermia usually comes from older texts/case reports. from what they are finding the hyperthermia occurs pretty quickly, but often times (especially in the past when that thought was derived) the provider was not getting an accurate core temperature, or any temperature at all. What would happen is that they'd hear about or get calls where anesthesiologists were "concerned" about MH due to ETCO2 and MV increases and were using one of those skin temp probes. Yet as soon as they got a core temp the patient would be 38-40C. Sometimes they would get calls because the anesthesiologist wasn't keeping any type of temp, but when other signs started showing up, they'd pop a probe into the nasopharynx/esophagus and WHAM a "late sign" of 40C showed up... ugh...
 
Foodie said:
Thanks for sharing your cases. I had a similar pt encounter that could've gone disastrously. It was a TAH BSO. First case of the day. No surgical history. Apparently denied any family Hx of problems with GA.

Preop RN relayed a nebulous "allergy to propofol" message and when I asked pt about it, she said, "My father had something hyperthermia."
Me: "Oh? What happened? How did he do?"
Pt: "He died from it after surgery."
Me: "Was the term malignant hyperthermia used at any point?"
Pt: "OH YEAH THAT'S IT!"

It was the first time I did an MH case at a new hospital, and unfortunately the hospital did not have any Vapor-Clean (and never carried it). We switched the schedule so a machine could be flushed before that case. Pt did fine. Afterwards I told pt to get an MH bracelet and extensively counseled her and her family members how important it is to communicate MH family history to her healthcare providers.

During residency and afterwards I've been in 8 hospitals in NY and all of them had Vapor-Clean in MH cart. We called nearby hospitals and was told that they have an extra ventilator specifically for MH cases and also no charcoal filters. I've attached recommended washout time for various ventilator machines. So my question is, does your facility carry Vapor-Clean?View attachment 202274
Click to expand...

What is Vapor-Clean..?
 
btbam said:
What is Vapor-Clean..?
Click to expand...

It's a charcoal filter. Place it on inspiratory and expiratory valves to "clean" the machine of any residual vapor. You can use it as part of the rescue treatment or to prepare a machine for a MHS patient quickly.
 
twoliter said:
It's a charcoal filter. Place it on inspiratory and expiratory valves to "clean" the machine of any residual vapor. You can use it as part of the rescue treatment or to prepare a machine for a MHS patient quickly.
Click to expand...

Is this a widely used thing? I've never heard of it and I'm in a big peds center.
 
btbam said:
Is this a widely used thing? I've never heard of it and I'm in a big peds center.
Click to expand...

Both Peds hospitals (in training) I've worked at have them. The adult side didn't have them (though could've gotten them from Peds side pretty easily). Can't tell you much other than that.
 
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