Colectomy and von Willebrands

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Noyac

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69 yo female with diverticular disease with colovesical fistula has a history of severe bleeding post knee scope and lap chole. Found to have vWD. She has h/o CAD with stent in 2002, PHTN with systolic press of 60, mod-sev AR, EF 70%, polycythemia vera with hepatosplenomegaly and portal HTN. Oh and IDDM on steroids for Pyoderma gangrenosum. Allergies are MS (makes me crazy), codiene, and NSAID's.

Whats your concerns and how do you approach this case? This was my case today so things are fresh in my mind and therefore I am at an advantage.

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How bad is CAD? What kind of vWD? is IDDM contolled or does she have multiple neuropathies with bad PVD?
 
How bad is CAD? What kind of vWD? is IDDM contolled or does she have multiple neuropathies with bad PVD?

Good questions. CAD is mild now after stent placement and she has fair exercise tolerance. She can climb a flight of stairs (4 mets) without much difficulty. Type II vWD, very important. IDDM is well controlled but she is now on steroids and sugars are more difficult to control. No neuropathies. Oh, and she is a nurse.
 
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Admit.
Consult Heme. Yes, its elective, consult HEME. Get her VW levels up to normal with Cryo. DDAVP 1hr preop.
2 Cryo on hold (she's gonna get em any hoots), 8 units prbc, 1 packed plates.
Central line (milrinone if needed for pulm htn, ilioprost for the fancy people but not a good idea in a BLEEDER, nitric oxide is a waste, ntg can be helpful)

A-line for obvious reasons.

Insulin ggt for outcome.

Screw the NGT. Let surgery put the f'n thing in.

You know what scares me the most though? Its that Pulmonary HTN. Why the hell is it so high? Is the AR that bad? How's her right heart look on that echo? Thats what scares the hell out of me.

Have cards check her out again for the pulm htn. Since this is not an emergency I want to know if there is anything we can do to get it down. Starling curve optimization, sildafinil?, she need a right heart cath?
 
Screw the NGT. Let surgery put the f'n thing in.

You know what scares me the most though? Its that Pulmonary HTN. Why the hell is it so high? Is the AR that bad? How's her right heart look on that echo? Thats what scares the hell out of me.

Have cards check her out again for the pulm htn. Since this is not an emergency I want to know if there is anything we can do to get it down. Starling curve optimization, sildafinil?, she need a right heart cath?

No NGT needed this is a sigmoid colectomy.

Her PHTN has gotten worse (from systolic of 33 to 60) in the past 1-2 years. Her AR is pretty bad but not awful and the R heart looks good, good squeeze and mild atrial enlargement. The last cardiac study was 6 weeks ago I don't see any reason to repeat.
 
69 yo female with diverticular disease with colovesical fistula has a history of severe bleeding post knee scope and lap chole. Found to have vWD. She has h/o CAD with stent in 2002, PHTN with systolic press of 60, mod-sev AR, EF 70%, polycythemia vera with hepatosplenomegaly and portal HTN. Oh and IDDM on steroids for Pyoderma gangrenosum. Allergies are MS (makes me crazy), codiene, and NSAID's.

Whats your concerns and how do you approach this case? This was my case today so things are fresh in my mind and therefore I am at an advantage.

Concerns:
-coagulopathy, to little = bleeding, to much = stent thrombosis any test for response to desmopressin? if positive give less than 1 hour per-op 8mcrg/kg i think it is and repeat every 4h or else it's factor 8 concentrate and platelets

-PHTN pretty high but if MET=4 i guess she's managing it save the viagra for the husband ;)

-polycythemia vera: if her crit is super high i'd do a pre-op normodilution get a liter of blood out for later

Thoracic epidural so you don't have to deal with the allergy crap, blast her with insulin as needed
 
Guess I better read up on it again because I don't remember the different types no matter how many times I go over it.

Types 1, 2A 2B 2M 2N and 3

Type 2B and 3 don't respond to to DDAVP. Also DDAVP can be dangerous in thrombocytopenics. It causes a binding or something b/w plts and the glycoprotein leading to a worsening of thrombocytopenia. Also DDAVP is for mild vWD but is helpful along with other treatments like cryo etc.

Remember DDAVP is a used to treat Diabetes Insipidus and you need to watch te Na.

I was at an advantage b/c I knew that she didn't respnd to DDAVP in the past.
 
Concerns:
-coagulopathy, to little = bleeding, to much = stent thrombosis any test for response to desmopressin? if positive give less than 1 hour per-op 8mcrg/kg i think it is and repeat every 4h or else it's factor 8 concentrate and platelets

-PHTN pretty high but if MET=4 i guess she's managing it save the viagra for the husband ;)

-polycythemia vera: if her crit is super high i'd do a pre-op normodilution get a liter of blood out for later

Thoracic epidural so you don't have to deal with the allergy crap, blast her with insulin as needed

Polycythemia was well controlled. Don't remember the numbers but they were normal.

How many of you would do a regional technique on her?
 
Consult Hematology and give factor VIII + VW factor according to their recommendations.
Have some factor VIII + VW ready.
Have some amicar ready.
Have some PRBC's ready.
Start A line.
Induce using: Remifentanyl + Etomidate + Roc.
Intubate smoothly.
Start central line and monitor CVP keeping it at base line.
Run Remifentanyl drip, maintain baseline BP and avoid bradycardia.
Give a longer acting narcotic at the end and extubate deep to avoid bucking.
She will do fine :)
 
Yes plank something like that.

This pt received Humate p (Factor VIII and vWB) b/4 surgery. Would anyone do an epidural on her?

What about little bleeders at the site of the sigmoid colon resection? Thrombin anyone?
 
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This pt received Humate p (Factor VIII and vWB) b/4 surgery. Would anyone do an epidural on her?

Yes if you supplement her, her coag is as good as normal but i don't practice in a litigious environment or you can do a duramoroh spinal and with ketamine she shouln't need too much opiates...
 
Yes if you supplement her, her coag is as good as normal but i don't practice in a litigious environment or you can do a duramoroh spinal and with ketamine she shouln't need too much opiates...

I agree. I offered her an epidural since hte Humate P was to be continued for many days post-op. She refused b/c she is a nurse and "knows better". So then offered her at least a spinal with some duramorph but she refused that as well. Oh well. Its your body.

As far as hyperalgesia and tolerance. I believe it and therefore, rarely use remi esp'ly in cases that will have post-op pain. NSAID's I would not use in this pt at all.

I also am begining to believe that large doses of narc's intraop can lead to greater pain post-op and increased narcotic requirements. The jury is still out on this but the studies are surfacing.
 
Yes if you supplement her, her coag is as good as normal but i don't practice in a litigious environment or you can do a duramoroh spinal and with ketamine she shouln't need too much opiates...
What test are you going to use to make sure her coagulation has become as good as normal?
 
What test are you going to use to make sure her coagulation has become as good as normal?

The short answer is none :)
If she doesn't have an other factor disorder supplementing her will give her a coag as good as anyone. You can do a bleeding time which will tell you if something is grossly abnormal.

So Plank she refuses a spinal what does that tell you about her future reaction when you're going to give her morphine? Will she tolerate hydromorphone better? What would you give her?
 
The short answer is none :)
If she doesn't have an other factor disorder supplementing her will give her a coag as good as anyone. You can do a bleeding time which will tell you if something is grossly abnormal.

So Plank she refuses a spinal what does that tell you about her future reaction when you're going to give her morphine? Will she tolerate hydromorphone better? What would you give her?

So, How do we know that we gave enough factors to do a Neuraxial anesthetic safely?

The only way to know how someone is going to react to a drug is to give it and see what happens.
So, one possibility is Hydromorphone PCA plain and simple.
 
Before doing neuraxial in type 2 or 3, even after Humate you should get VWF antigen and assay (activity) level- in the few case reports out there for njeuraxial anesthesia usually they shoot for levels near 100% (although surgery will proceed at levels of 30-50%). I don't think anyone knows what level is safe enough for not forming an epidural hematoma. Those tests take a while to get back. There is also TEG (not sensitve enough for assesing plt fxn) and the PFA-100 (platelet function analyzer- basically an automated bleeding time). In this case probably not worth all the effort for post-op pain. However, in labor this is a valid concern.
 
Before doing neuraxial in type 2 or 3, even after Humate you should get VWF antigen and assay (activity) level- in the few case reports out there for njeuraxial anesthesia usually they shoot for levels near 100% (although surgery will proceed at levels of 30-50%). I don't think anyone knows what level is safe enough for not forming an epidural hematoma. Those tests take a while to get back. There is also TEG (not sensitve enough for assesing plt fxn) and the PFA-100 (platelet function analyzer- basically an automated bleeding time). In this case probably not worth all the effort for post-op pain. However, in labor this is a valid concern.

Very good. The TEG may be good enough and even if the plt fxn is not well analyzed b/c it isn't plt fxn that is impaired.

When these pts present for labor and delivery it usually isn't an issue since the pregnant state will increase the vWB numbers and activity.
 
I had a type I VWD present in labor for VBAC. I drew the VWF labs (took 2 days to get back) and her levels did increae above normal. VWF level increases 200-300% in 2-3 trimester ( but its good for people to realize that these levels can drop quickly after delivery so it's best to get that epidural out asap.) So, after talking about it with my attending we drew a TEG- which had that nice short, fat hypercoaguable shape of pregnancy, and since it was type 1- I put the epidural in without any problems. She ended up getting sectioned. I was nervous though, her arm was all bruised up from the bp cuff. I did a presentation on the case and I could't find any examples of TEGs in type 2 or 3. I don't know if I would do the epidural in a type 2 or 3 based on a normal TEG. VWD is pretty interesting.
 
Seems this post has lost steam so I will end it with how I did the case. I was not very concerned with bleeding so I offered a spinal with IT MS but as I mentioned she didn't want that. She likes her Dilaudid. I felt the spinal MS would have been beneficial with her recovery but obviously not necessary. I didn't really want to have a cath in her and worry about pulling it 3 days later with the 2 periods (placement and removal) of risk as opposed to 1 (spinal). I took her to the OR gently put her to sleep with 200 mcg Fentanyl about 5 minutes b/4 intubation. I used 80 mg propofol and 50mg roc and tubed her. BP dropped literally only 5 pts from baseline. Then placed an A-line and a 16g PIV. Her Humate P was given 5 hrs earlier b/c its peak is at 5 hrs. I used Des around 4.5 - 6.0 ET. I added Mg and Ketamine 200mg which ran for the duration of the case. I also considered a Lidocaine drip for post-op pain but figured it was unnecessary since I couldn't continue it post-op. The case lasted about 2 hrs and I gave 1L IVF. Her pelic veins were quite engorged most likely due to her hepatoslenomegaly. We placed thrombin on the site of resection w/c was somewhat bloody but not out of the ordinary. She did fine. I find vWB very interesting I guess.
 
one thing to keep in mind also when talking about PA pressures is their relationship to systemic pressures. It makes a difference if her PA was 70 and her systolic is 80 as opposed to systolic being 180.
 
Seems this post has lost steam so I will end it with how I did the case. I was not very concerned with bleeding so I offered a spinal with IT MS but as I mentioned she didn't want that. She likes her Dilaudid. I felt the spinal MS would have been beneficial with her recovery but obviously not necessary. I didn't really want to have a cath in her and worry about pulling it 3 days later with the 2 periods (placement and removal) of risk as opposed to 1 (spinal). I took her to the OR gently put her to sleep with 200 mcg Fentanyl about 5 minutes b/4 intubation. I used 80 mg propofol and 50mg roc and tubed her. BP dropped literally only 5 pts from baseline. Then placed an A-line and a 16g PIV. Her Humate P was given 5 hrs earlier b/c its peak is at 5 hrs. I used Des around 4.5 - 6.0 ET. I added Mg and Ketamine 200mg which ran for the duration of the case. I also considered a Lidocaine drip for post-op pain but figured it was unnecessary since I couldn't continue it post-op. The case lasted about 2 hrs and I gave 1L IVF. Her pelic veins were quite engorged most likely due to her hepatoslenomegaly. We placed thrombin on the site of resection w/c was somewhat bloody but not out of the ordinary. She did fine. I find vWB very interesting I guess.

Why the ketamine?

You only give 1 liter of IVF for a colectomy? Damn slick.

I wouldn't put an epidural in this woman. Its not worth it. It'll get lost on the floors and the litigation to benifit ratio is wayyy off here.

I did appreciate the pregnancy story above with the VWF. I haven't encountered that and I really like what you guys did. Unfortunately we don't have TEG available here. Is TEG still useful in a VWF setting or are there too many confounding factors?

In addition, are those labs truely needed before you place an epidural (I suppose you would need a starting place to estimate the dose needed for appropriate response, but I'm having some nerdish fun here)? Or could you consult heme and have the humate/cryo going and then place a neuraxial?

Who says that if you send your labs off that two days later you know the parturient's vWF is still at the same level? Or do you just continue that humateP stuff until a few days post-op/SVD?
 
Why the ketamine?

You only give 1 liter of IVF for a colectomy? Damn slick.

I've been playing with ketamine for about a year now. It does seem to add some benefit (narcotic sparing post-op) but not huge. I also use Mg++ 2 gm for the same reason. I try to give 2-5 mg/kg ketamine during the case and I stop it at least 30 b/4 the end of the case. There are other ways of using it but this is how I am trying it.

I try to limit my crystalloids in GI cases like this to decrease post op ileus issues. I am not always able to limit it this much but she was a small woman and blood loss is minimal in these cases. The bowel prep usually hinders this technique somewhat.
 
Why the ketamine?

You only give 1 liter of IVF for a colectomy? Damn slick.

I wouldn't put an epidural in this woman. Its not worth it. It'll get lost on the floors and the litigation to benifit ratio is wayyy off here.

I did appreciate the pregnancy story above with the VWF. I haven't encountered that and I really like what you guys did. Unfortunately we don't have TEG available here. Is TEG still useful in a VWF setting or are there too many confounding factors?

In addition, are those labs truely needed before you place an epidural (I suppose you would need a starting place to estimate the dose needed for appropriate response, but I'm having some nerdish fun here)? Or could you consult heme and have the humate/cryo going and then place a neuraxial?

Who says that if you send your labs off that two days later you know the parturient's vWF is still at the same level? Or do you just continue that humateP stuff until a few days post-op/SVD?

In my personal opinion: VW = No epidural.
 
Great thread. Definitely made me think more about VWF than I have since intern year. Now I like using ketamine in select cases, especially in the opoid tolerant pt. but I do try to avoid it in cases of significant PHTN because we are taught that "ketamine increases PAP" . Has anyone actually seen ketamine significantly increase PAP?
 
Great thread. Definitely made me think more about VWF than I have since intern year. Now I like using ketamine in select cases, especially in the opoid tolerant pt. but I do try to avoid it in cases of significant PHTN because we are taught that "ketamine increases PAP" . Has anyone actually seen ketamine significantly increase PAP?

Good call. I think the inplication comes from larger doses like induction doses but I don't recall it being much of a problem. And especially not at these doses. Basically, anything that increases sympathetic drive can increase PAP. With this low dose you don't get much if any symp discharge.
 
yeah, I havent seen much of that clinical effect at lower doses either. I especially like using sub-induction doses of ketamine in combo with versed for induction on those low EF pts for CABG. Seems to provide a very stable induction and I havent seen any significant tachycardia from the sympathetic discharge.
 
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