Myotonic Dystrophy and Cholecystectomy

[Planktonmd]

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Since we are trying to go back to clinical discussions let me present this case:

A few months ago I was contacted by a general surgeon that wanted to perform a cholecystectomy on a 35 Y/O, M, diagnosed 10 years ago with myotonic dystrophy type 1, he is severely disabled, but his mental abilities appear intact.
He has Pharyngeal muscles involvement and history of recurrent aspiration pneumonia.
He also has chronic respiratory failure secondary to the muscle weakness and the recurrent pneumonias, he is not on mechanical ventilation but his PaO2 on 2 liter Oxygen is 55 with a PaCO2 of 50.
He has cardiomyopathy with EF of 20 %, RBBB and first degree AV block.
He also has insulin requiring DM.
The surgery has been canceled once before because the anesthesiologist did not want to touch him but now the surgeon is saying that he has bad chronic cholecystitis with multiple large stones and several acute episodes and needs that surgery very soon.
So, let's talk about preop workup and intraop management.

 

[milrisome]

First, click your heels together and say "there's no place like home" just to make sure it's not a nightmare.

Pre-op work up:
Cardiac: check for h/o arrythmias, current or past signs of CHF, maybe chest Xray but might be pretty ugly anyway; sounds like EKG/echo done already
DM: check BG and lytes, recent insulin treatments, ask about neuropathy/nephropathy
Aspiration/respiratory failure: make sure of NPO status and give reglan and H2 blocker; can't improve baseline status but make sure no ongoing pneumonia

Above all, make sure family and pt aware of risks: likely post-op vent, possible inability to come off vent, risk of aspiration, arrhythmia

Talk to surgeon and make sure he's going to do laparoscopic.

 

[Planktonmd]

First, click your heels together and say "there's no place like home" just to make sure it's not a nightmare.

Pre-op work up:
Cardiac: check for h/o arrythmias, current or past signs of CHF, maybe chest Xray but might be pretty ugly anyway; sounds like EKG/echo done already
DM: check BG and lytes, recent insulin treatments, ask about neuropathy/nephropathy
Aspiration/respiratory failure: make sure of NPO status and give reglan and H2 blocker; can't improve baseline status but make sure no ongoing pneumonia

Above all, make sure family and pt aware of risks: likely post-op vent, possible inability to come off vent, risk of aspiration, arrhythmia

Talk to surgeon and make sure he's going to do laparoscopic.

All that pre op stuff had been done, CXR shows bilateral infiltrates in the bases not different from the CXR 1 year ago.
EKG as I said showed RBBB and 1st degree AV block, no arrhythmias doccumented.
He does have mild renal failure, creatinine = 2.5.
Electrolytes are normal.
Surgeon said most likely he will need to open.

 

[milrisome]

Another useful thing preop might be to see if there's been any discussion of tracheostomy. If it's being considered, now would be a great time as it would help prevent aspiration and improve ventilation postop.

 

[Planktonmd]

So,
Are we going to put this guy to sleep?
Need any invasive monitors?
Any other ideas?

 

[Noyac]

Why in the world would the surgeon need to open this case instead of laproscpic? With this pts respiratory status and the pain from an open chole, I be surprised if even a perfectly working Thoracic Epidural would keep him off the vent. I'd bet he's trach'd shortly after if not during the case. But even if he stays off the vent postop, why open him up?

If this guy can't do it laproscopically then get someone that can. Families need to know this ****.

 

[Planktonmd]

Why in the world would the surgeon need to open this case instead of laproscpic? With this pts respiratory status and the pain from an open chole, I be surprised if even a perfectly working Thoracic Epidural would keep him off the vent. I'd bet he's trach'd shortly after if not during the case. But even if he stays off the vent postop, why open him up?

If this guy can't do it laproscopically then get someone that can. Families need to know this ****.

The surgeon is a good surgeon and he did not want to do it open but because of how chronic the problem was he was expecting a difficult dissection and a possibility to convert to open.
He also said that he wouldn't mind starting open if my anesthetic required it.
So, let's say it's going to be open (because that is what actually happened), what is the plan?

 

[Noyac]

OK fair enough. If this goes open we got problems, mostly respiratory. You could get PFT's and an ABG to assess resp status. CO2 will likely be elevated even with fairly normal PFT's. These pts have a enhanced sensitivity to narcotics which leads to resp depression when used. I'd probably place an thoracic epidural forthe case and use it with some sedation, propofol with ketamine. Don't forget the glyco for the secretions that he will have difficulty clearing. You want to avoid muscle relaxants and narcotics as much as possible. In the epidural, I'd use fentanyl instead of morphine and low dose local (ropiv at 0.1% or less). Epidural morphine has more cephalad spread than the fentanyl. You could consent for trach as well but I probably wouldn't. You can alwasy come back and do it if needed since he will be tubed anyway if he needs a trach and you won't need to relax him.

If I were to go the GA route it would be without relaxants at all.

 

[milrisome]

Ok, here goes: Obviously going to need GETA. Upper abdominal case and post-op respiratory concerns = low-mid thoracic epidural. Place it pre-op and dose it carefully with local anesthetics while watching his response to it since he's obviously at risk for significant hypotension. Make sure the block is working.

Definitely place arterial access. CVP/PAC: yeah, I probably would. He's already got the cardiomyopathy, and you don't want to go overboard and flood his lungs for sure.

Go to OR, gentle induction, NO SUX!!, use short-acting muscle relaxant (try to find mivacurium) or cisatracurium (b/c of CRI) if necessary. Probably need a vasopressor infusion to counteract the epidural. Probably wouldn't use propofol b/c of vasodilation issues. Could try midaz and ketamine, or maybe dexmedetomidine infusion with catechol infusion if necessary. This would be a case where BIS monitoring would be a good idea.

Keep defibrillator close, he's at high risk for issues. Check his BG and treat prn. Type and cross.

Introp, match his losses while watching PCWP. Don't overdo it with fluids.

End of case, get him on the bed, sit him up, see what ventilation is like. If there's any questions leave him on the vent.

 

[Planktonmd]

Question for the residents: Is GETA really necessary in this case?

 

[ruggerdoc123]

Why in the world would the surgeon need to open this case instead of laproscpic? With this pts respiratory status and the pain from an open chole, I be surprised if even a perfectly working Thoracic Epidural would keep him off the vent. I'd bet he's trach'd shortly after if not during the case. But even if he stays off the vent postop, why open him up?

If this guy can't do it laproscopically then get someone that can. Families need to know this ****.

The "****" that "families need to know" is that they are in the hands of an experienced surgeon with good clinical judgement. The surrounding inflammation and thickened, friable gallbladder wall will make it nearly impossible to safely dissect laparoscopically. Please don't imply that the surgeon is misleading the family, or that you know better than he which surgery is indicated. Didn't we have a thread about other specialists (cardiologists) making unwelcome recommendations for which type of anesthesia should be administered?

As well, I would not discount the well-described deleterious effects on hemodynamics from peritoneal insufflation, especially in patients with poor cardiac reserve.

All things being equal, would you rather have a 4 hour laparoscopic case or 90 minute open chole?

Regarding the necessity of GETA- I suppose it's not truly required even if laparoscopy was planned (Keep the pneumo down and use a thoracic epidural).

 

[Noyac]

When is the last time you have seen an open chole? I haven't seen one since finishing residency.

Not all surgeons have good judgement.

You can do a lap chole with an insufflation pressure much less than 15, I've done it and I've done many on pts with poor cardiac reserve.

The last 4 hour lap chole I did was NEVER so that isn't a true option. But all things considered I'd much rather a laproscopic procedure performed on me than an open one if I had very limited respiratory mechanics even if it was twice as long.

 

[Planktonmd]

Ok,
Here is what I did:
Thoracic epidural at T8-T9 in the holding area, A line.
In the OR titrated Lidocaine 2 % + bicarb and epi to get a segmental block of the lower thoracic upper abdominal dermatomes.
I thought about adding some intercostal blocks but decided not to.
Started Neo drip to keep the BP up, and a very low rate Propofol.
After 8cc Lidocaine I had good surgical anesthesia, and surgery (open Cholecystectomy) started.
I kept him talking and told the CRNA to stay at that level of sedation.
No narcotics in the epidural or IV.
We were prepared to convert to general at anytime if his breathing became compromised or if he became uncomfortable, but that did not happen.
The surgery lasted 2 hours.
Post-OP I used Bupivacaine 0.125 % without narcotics in PCEA mode for 3 days.
He was discharged home after 1 week in the hospital.
I am glad no one from the "Society Against Regional Anesthesia" is around any more so I don't have to do allot of explaining. 😀

 

[zippy2u]

What's the problem here: GETA with prop, vec 10mgs, fentanyl 20ccs and Sevo. Throw him over in the ICU with a tube and call it a day. We ain't got no time for regional and all that other high fallutin' stuff. Anesthesia don't manage no vents either---get the surgeon to manage it or have him call the lung flea. Open or L/S, what the Hey-- tell the surgeon to do whatever method in his hands is the quickest. Don't worry about trachs at this stage of the ball game however if it's mentioned by someone else, tell him you'll be happy to provide quality anesthesia for that surgical procedure as well. Of course your job is not complete until you announce to the family that "anesthesia has successfully got him through the surgery but he's still not out of the woods yet." Bonus points for gettin' the hospital's "preacher man" in the ICU to hold hands with the pt's family and sing "We Shall Overcome" or something along those lines with a few choice prayers thrown in to chase the evil spirits away... Regards, ----Zip

 

[Planktonmd]

What's the problem here: GETA with prop, vec 10mgs, fentanyl 20ccs and Sevo. Throw him over in the ICU with a tube and call it a day. We ain't got no time for regional and all that other high fallutin' stuff. Anesthesia don't manage no vents either---get the surgeon to manage it or have him call the lung flea. Open or L/S, what the Hey-- tell the surgeon to do whatever method in his hands is the quickest. Don't worry about trachs at this stage of the ball game however if it's mentioned by someone else, tell him you'll be happy to provide quality anesthesia for that surgical procedure as well. Of course your job is not complete until you announce to the family that "anesthesia has successfully got him through the surgery but he's still not out of the woods yet." Bonus points for gettin' the hospital's "preacher man" in the ICU to hold hands with the pt's family and sing "We Shall Overcome" or something along those lines with a few choice prayers thrown in to chase the evil spirits away... Regards, ----Zip

Yeah,
You can do that too

 

[milrisome]

Ok,
Here is what I did:
Thoracic epidural at T8-T9 in the holding area, A line.
In the OR titrated Lidocaine 2 % + bicarb and epi to get a segmental block of the lower thoracic upper abdominal dermatomes.
I thought about adding some intercostal blocks but decided not to.
Started Neo drip to keep the BP up, and a very low rate Propofol.
After 8cc Lidocaine I had good surgical anesthesia, and surgery (open Cholecystectomy) started.
I kept him talking and told the CRNA to stay at that level of sedation.
No narcotics in the epidural or IV.
We were prepared to convert to general at anytime if his breathing became compromised or if he became uncomfortable, but that did not happen.
The surgery lasted 2 hours.
Post-OP I used Bupivacaine 0.125 % without narcotics in PCEA mode for 3 days.
He was discharged home after 1 week in the hospital.
I am glad no one from the "Society Against Regional Anesthesia" is around any more so I don't have to do allot of explaining. 😀

Hey, if it worked, it was the right thing to do. Obviously I was wrong about needing GETA.

 

[jetproppilot]

What's the problem here: GETA with prop, vec 10mgs, fentanyl 20ccs and Sevo. Throw him over in the ICU with a tube and call it a day. We ain't got no time for regional and all that other high fallutin' stuff. Anesthesia don't manage no vents either---get the surgeon to manage it or have him call the lung flea. Open or L/S, what the Hey-- tell the surgeon to do whatever method in his hands is the quickest. Don't worry about trachs at this stage of the ball game however if it's mentioned by someone else, tell him you'll be happy to provide quality anesthesia for that surgical procedure as well. Of course your job is not complete until you announce to the family that "anesthesia has successfully got him through the surgery but he's still not out of the woods yet." Bonus points for gettin' the hospital's "preacher man" in the ICU to hold hands with the pt's family and sing "We Shall Overcome" or something along those lines with a few choice prayers thrown in to chase the evil spirits away... Regards, ----Zip

Man, ya gotta love the Zipster....lung flea, preacher man....

HAHAHAHAHAHAHAHAHAHAHA

now theres some Copenhagen residue on my laptop screen from laughing

Nice technique, Plank.