- Hi, I'm Mollie. I'm from Arkansas. I'm the director of our HHT Center there. I'm going to give you a quick eight minute overview of a fairly complicated disease with a bunch of different kinds of vascular malformations. Does this work or do I have to click this?
Okay, I have no disclosures. HHT is what it's now called. Back in medical school you may have learned it as Osler-Weber-Rendu. This is a patient's hand with telangiectasias on it. When you're looking at telangiectasias,
if you compress them and they disappear then it's a telangiectasia. So you push on it, it blanches. You let it go, it comes back. That's helpful because the older you get the more pink spots you get.
This is the old diagnostic criteria. This is applicable for certain genetic mutations but not as applicable for the other genetic mutations. There are four criteria. Spontaneous, recurrent epistaxis, telangiectasias in classic locations,
visceral AVMs, the common locations are liver, lung, GI tract, brain, and then a first degree family member because it's an autosomal dominant disease. Epistaxis, we're going to sort of just cut right to the chase. This is the most common problem that patients present with.
It generally occurs when they're about 12 years old. Sometimes earlier, sometimes way later. There is a tendency to treat this either with cautery or embolization. Don't do that. You'll make it worse.
I'll show you a picture. I've had two or three patients that have been embolized with fairly disastrous outcomes. What we generally recommend is significant hydration. We use K-Y Jelly because it's a water-based lubricant instead of a petroleum product.
I don't know that it matters so much. You can use antibiotic ointment, you can use Vaseline, you can use saline spray, whatever. We use lots of oral therapy. Doxycycline, 100 milligrams BID.
In real severe cases we recommend Avastin and there is a limited amount of data with Pazopanib at a very, very low dose of 50 milligrams per day. So that is where I would start. This is a patient who underwent serial embolizations at an outside hospital.
Is it going to pause? It's not going to pause. After the tiny telangiectasias in his nose were embolized over and over and they used PVA and a couple other different embolic bead type things, it ended up turning into a real AVM.
So what was tiny little telangiectasias, I think because of the angiogenesis process, turned this into a real AVM and I've seen that happen in three separate patients that have been repeatedly embolized with HHD, so please don't do that.
Pulmonary AVMs are super common in HHT patients. It's a pretty straightforward thing to embolize most of the time. There are some exceptions to this rule. You want to embolize as distal as possible. There have been a couple of papers about
embolizing the venous sac itself. That hasn't shown to necessarily be better or worse, but you definitely want to get as close to the venous sac part of it as you can. It's a Yakes type I in his classification system. For the most part you have to be careful
about treating children and treating them too early because it will recruit systemic feeders, and then you take a low pressure pulmonary system and turn it into a high pressure systemic system and it's more likely to rupture and cause some other problems.
So in general we don't treat children until they're symptomatic or it's a significant impact. We screen people with echo bubble for this. The important thing to tell your cardiologist if you're going to screen is that they have to wait the full 10 to 15 beats
because it'll be an extra cardiac shunt and not a PFO which is what they're usually used to looking for on their echo bubble studies. There is a good classification system where you count the maximum number of bubbles in the ventricle on the echo
to see whether you think you should move to a CT scan, so don't just CT all these people. It's too much radiation and you're not going to find stuff that you want to treat anyway. This is an example of a pulmonary AVM in one patient. This is a different patient.
You can see it has a pretty big venous aneurysm sac. This is that same patient with the ... There it is. This is an Amplatzer 4 device and then a bunch of Nester coils behind it. Sorry, did I just shine the laser right on your head?
I'm sorry. My new favorite thing to embolize pulmonary AVMs with is the microvascular plug, and the reason it's my new favorite thing is because it just has two little dots on it on the post-op CTA imaging or CT imaging,
so you don't have a bunch of artifact from coils and other devices in there. The standard is the Nester coil which is the fuzzy coil down here at the bottom. You do need follow up imaging on these patients, at six months is when I usually get my first
because they will recanalize and develop new feeders and things like that. This is a picture of a recanalized one in the right upper lobe. See if it'll go again. I don't have too much time
to tell you about technique for that. I usually use the white LuMax set. It's an eight French system. It has a little angle tip catheter in it. You want to anticoagulate your patients just a little bit, 3,000 units of heparin,
especially if they have low iron because there is some increased risk of DVT in these patients that have chronic anemia from their nosebleeds and GI bleeds, which is sort of an odd thing. So a little bit of anticoagulation will help you
keep from clotting off their IVC, which I have also seen happen. Liver AVMs are extremely common. Up to 70% of patients with HHT have liver AVMs. Generally they are small and inconsequential, although the larger they get the more problems they cause.
These are the three main problems they cause. High output heart failure, biliary necrosis or biliary cirrhosis, and then portal hypertension. The fistula can be arterial portal, arterial hepatic venous or portal hepatic venous,
so you have to take good pictures and figure out what you're doing with these. Don't embolize them. (laughs) This is a celiac angiogram of a patient who had a big liver AVM with a aneurism. She also had a big pelvic AVM.
We started her on Avastin. Avastin has been shown to work fairly well for this in bridging to transplant or just in reducing their high output heart failure. This is a patient that I just saw. His cardiac output is 12 liters per minute.
This is his CTA. An outside facility decided to put an aortic cuff to cover his celiac artery. - [Man] Nice. - So all that did was piss off my transplant surgeon. Please don't mess with the liver AVMs.
We've started him on Avastin and then we'll do a follow up right heart cath in six months or so and see whether his cardiac output is reduced or not. In summary, epistaxis and livers get medical management. Pulmonary AVMs in adults that are symptomatic,
you should treat. Then brain AVMs, which are also common in HHT patients, you can just use your standard treatment whether that's Onyx embolization plus surgery or radiotherapy or whatever it is, depending on the size and location of the lesion.
The other common brain lesions in HHT, the most common is a developmental venous anomaly. You don't really do much with those. Then sometimes people will have teeny tiny telangiectasia type AVMs. We generally get a four vessel,
take some really pretty pictures, and then leave them alone unless they become symptomatic. I have one patient who had a telangiectasia that was a seizure focus, so she did undergo treatment for that, but in general the brain treatment stuff is the same
as non-HHT patients. That's what I have, thanks. - [Man] Maybe there are questions, one question? - [Man] One question. - [Man] There is one thing. Many times we will get patients with HHT
and they have (muffled speaking) and they have no internal axis. - [Mollie] Yeah. - [Man] After repeated embolization. What we've found (muffled speaking) I've now done it several times,
haven't published it yet, but the offending agent is a capillary venous malformation of the nasal mucosa. I've done a direct puncture into it and alcohol it and it stops their epistaxis. So the malformation that was primarily (person coughs)
but after just being starved from arterial post capillary type lesion and I've found that works very well. - [Mollie] I would say that's great for someone with lots of experience with neuro stuff. There is an ENT out in the country
who's doing Sotradecol direct injection and he's blinded a person because he's not watching. - [Man] Wow. - [Mollie] So, just pay attention to the intracran-- - [Man] He probably does it in his office. - [Mollie] The internal-external carotid connections.
Don't forget about those. - [Man] Yeah, do not forget. - [Man] Okay, thank you.
- This talk is a brief one about what I think is an entity that we need to be aware of because we see some. They're not AVMs obviously, they're acquired, but it nevertheless represents an entity which we've seen. We know the transvenous treatment of AVMs is a major advance in safety and efficacy.
And we know that the venous approach is indeed very, very favorable. This talk relates to some lesions, which we are successful in treating as a venous approach, but ultimately proved to be,
as I will show you in considerable experience now, I think that venous thrombosis and venous inflammatory disease result in acquired arteriovenous connections, we call them AVMs, but they're not. This patient, for example,
presented with extensive lower extremity swelling after an episode of DVT. And you can see the shunting there in the left lower extremity. Here we go in a later arterial phase. This lesion we found,
as others, is best treated. By the way, that was his original episode of DVT with occlusion. Was treated with stenting and restoration of flow and the elimination of the AVM.
So, compression of the lesion in the venous wall, which is actually interesting because in the type perivenous predominant lesions, those are actually lesions in the vein wall. So these in a form, or in a way, assimilate the AVMs that occur in the venous wall.
Another man, a 53-year-old gentleman with leg swelling after an episode of DVT, we can see the extensive filling via these collaterals, and these are inflammatory collaterals in the vein wall. This is another man with a prior episode of DVT. See his extensive anterior pelvic collaterals,
and he was treated with stenting and success. A recent case, that Dr. Resnick and I had, I was called with a gentleman said he had an AVM. And we can see that the arteriogram sent to me showed arterial venous shunting.
Well, what was interesting here was that the history had not been obtained of a prior total knee replacement. And he gave a very clear an unequivocal history of a DVT of sudden onset. And you can see the collaterals there
in the adjacent femoral popliteal vein. And there it is filling. So treatment here was venous stenting of the lesion and of the underlying stenosis. We tried an episode of angioplasty,
but ultimately successful. Swelling went down and so what you have is really a post-inflammatory DVT. Our other vast experience, I would say, are the so-called uterine AVMs. These are referred to as AVMs,
but these are clearly understood to be acquired, related to placental persistence and the connections between artery and veins in the uterus, which occurs, a part of normal pregnancy. These are best treated either with arterial embolization, which has been less successful,
but in some cases, with venous injection in venous thrombosis with coils or alcohol. There's a subset I believe of some of our pelvic AVMs, that have histories of DVT. I believe they're silent. I think the consistency of this lesion
that I'm showing you here, that if we all know, can be treated by coil embolization indicates to me that at least some, especially in patients in advanced stage are related to DVT. This is a 56-year-old, who had a known history of prostate cancer
and post-operative DVT and a very classic looking AVM, which we then treated with coil embolization. And we're able to cure, but no question in my mind at least based on the history and on the age, that this was post-phlebitic.
And I think some of these, and I think Wayne would agree with me, some of these are probably silent internal iliac venous thromboses, which we know can occur, which we know can produce pulmonary embolism.
And that's the curative final arteriogram. Other lesions such as this, I believe are related, at least some, although we don't have an antecedent history to the development of DVT, and again of course,
treated by the venous approach with cure. And then finally, some of the more problematic ones, another 56-year-old man with a history of prior iliofemoral DVT. Suddenly was fine, had been treated with heparin and anticoagulation.
And suddenly appeared with rapid onset of right lower extremity swelling and pain. So you see here that on an arteriogram of the right femoral, as well as, the super selective catheterization of some of these collaterals.
We can see the lesion itself. I think it's a nice demonstration of lesion. Under any other circumstance, this is an AVM. It is an AVM, but we know it to be acquired because he had no such swelling. This was treated in the only way I knew how to treat
with stenting of the vein. We placed a stent. That's a ballon expanded in the angiogram on your right is after with ballon inflation. And you can see the effect that the stenting pressure, and therefore subsequently occlusion of the compression,
and occlusion of the collaterals, and connections in the vein wall. He subsequently became asymptomatic. We had unfortunately had to stent extensively in the common femoral vein but he had an excellent result.
So I think pelvic AVMs are very similar in location and appearance. We've had 13 cases. Some with a positive history of DVT. I believe many are acquired post-DVT, and the treatment is the same venous coiling and or stent.
Wayne has seen some that are remarkable. Remember Wayne we saw at your place? A guy was in massive heart failure and clearly a DVT-related. So these are some of the cases we've seen
and I think it's noteworthy to keep in mind, that we still don't know everything there is to know about AVMs. Some AVMs are acquired, for example, pelvic post-DVT, and of course all uterine AVMs. Thanks very much.
(audience applause) - [Narrator] That's a very interesting hypothesis with a pelvic AVMs which are consistently looking similar. - [Robert] In the same place right? - [Narrator] All of them are appearing at an older age. - [Robert] Yep.
Yep. - This would be a very, very good explanation for that. I've never thought about that. - Yeah I think-- - I think this is very interesting. - [Robert] And remember, exactly.
And I remember that internal iliac DVT is always a silent process, and that you have this consistency, that I find very striking. - [Woman] So what do you think the mechanism is? The hypervascularity looked like it was primarily
arterial fluffy vessels. - [Robert] No, no, no it's in the vein wall. If you look closely, the arteriovenous connections and the hypervascularity, it's in the vein wall. The lesion is the vein wall,
it's the inflammatory vein. You remember Tony, that the thing that I always think of is how we used to do plain old ballon angioplasty in the SFA. And afterwards we'd get this
florid venous filling sometimes, not every case. And that's the very tight anatomic connection between those two. That's what I think is happening. Wayne? - [Wayne] This amount is almost always been here.
We just haven't recognized it. What has been recognized is dural fistula-- - Yep. - That we know and that's been documented. Chuck Kerber, wrote the first paper in '73 about the microvascular circulation
in the dural surface of the dural fistula, and it's related to venous thrombosis and mastoiditis and trauma. And then as the healing process occurs, you have neovascular stimulation and fistulization in that dural reflection,
which is a vein wall. And the same process happens here with a DVT with the healing, the recanalization, inflammation, neovascular stimulation, and the development of fistulas. increased vascular flow into the lumen
of the thrombosed area. So it's a neovascular stimulation phenomenon, that results in the vein wall developing fistula very identical to what happens in the head with dural fistula had nothing described of in the periphery.
- [Narrator] Okay, very interesting hypothesis.
- Hi, thank you so much for having me. I would like to share with you today the importance of preparatory endovascular treatment for surgical resections. Particularly, I'd like to talk to you about this case of intra-lipomatous capillary venous malformation. This is the case that I was able to share with Wayne Yates
as well as our vascular surgeon, colleague, Chris Morin. The only disclosure I have is I'm a plastic surgeon. So LS is a 14-year-old girl who came to us. She presented initially with purplish discoloration in her lumbosacral area. As she grew, the area grew with her
until she was about 12 years old when the lesion began to grow tremendously. This particular mass caused her severe pain on a daily basis. She also suffered from frequent bleeding episode and extreme malodor.
The only treatment that she had in her native country of Cambodia was metronidazole powder. Her case was sent to multiple physicians all around the world, and ultimately, she came to our institution in September 2014. What she was found to have is an intra-lipomatous
capillary-venous malformation, and this is malformation where there is a synergistic epigenetic inducement of cellular growth and proliferation. What happened is that these adepocytes became grossly enlarged, became pathologic,
and then started to stimulate and grow until there is massive tissue necrosis which then caused frequent bleeding and bleeding complications. The tumor, in fact, is really a vascular tumor, and there is pathological lipid as well as vascular cellular anatomy.
This is our patient and a tumor located in a lumbosacral area. As you can see, that is quite large, and it spans her entire width of her torso, and here is a posterior oblique view of her tumor. So histology for this basically show that there is hyper,
I'm so sorry, hypercellular vessels as well as abnormally enlarged adepocytes, and then in between, you have these dense intracellular structures that are very close in proximity to both the vascular and the fat cells. Here she is prone on the operating table
and the view of the tumor from above. So before we could resect this, a lot of endovascular treatments went into work, and this is important because in this particular area, I don't have another way to control bleeding. I can't put a tourniquet on it,
and I needed to be able to get better control in terms of arterial inflows as well as venous occlusion. So here is her pre-op MRI showing the mass, pre-op angio. So after her mass was resected, you can see evidence where the endovascular treatments help us significantly. There's embolized veins as well as arteries.
The defect was closed with three flaps, and despite our best efforts, she was left with a small defect that we couldn't really close, so she underwent dressing changes, and we thought, "Okay, this is going to be great." Six weeks later, she still didn't heal.
She still had bleeding. So what happened? We resected the tumor. The margins appeared to be clean. We didn't really see any other vascular tissue at the periphery.
The wound continued to be open. It was clean. The patient unfortunately began to bleed for the same area. So on a re-study, we found recannalizations of her embolized vessels as well as residual recurrent lumbar vascularity.
So going back for some more endovascular treatments, this time around, I believe Dr. Yates exhausted our institutional supply of onyx and glue for this case. We tried basically to cut down some more pelvic vascularity and doing the second resection, any visible residual fat as well as deep fascia were completely excised.
We also chased out vessels all the way to the paraspinous vessels, anything that we could see. Everything was taken down deep. Here is a closeup view of what was left, and the defect was still quite large, and fortunately, we were able to close over
a couple of JP drains. Pathology from the second surgery basically showed close proximity of fat as well as vascular cells, both of which appear to be abnormal. There's definitely evidence on pathology of the communication on a cellular levels
between fat and vascular cells. After the second surgery, the patient underwent multiple repeated alcohol and embolizations again, to cut down the pelvic vascularity. Here she is with the pre-op MRI, post-op, post-op, and post-op angio.
So even after all of that, the patient still continued to have residual small wound on her right superior gluteal area. She underwent dressing changes, multiple would debridements, and ultimately another right superior gluteal flap clsoure
and continued endovascular treatments, and ultimately, she is healed, and here is her pre-op arteriogram showing large filling of the tumors from the arteries and veins. No oblique view from the internal iliac. And post op.
And another post op. So where we are now it's about three years post treatment, she's doing very well to the best of our knowledge, there's been no evidence of recurrence. Have we cured her? I think only time will tell.
She was able to return back to Cambodia three years ago and is living a relatively normal life. Thank you so much. (audience applauds) - [Male Doctor] I think this case has illustrated one doctor is not enough for these patients.
We need a team to go out with the surgeons, anesthesia, pediatrics, and we really have to have these surgeons that are tenacious. I think that's the word here. ` - [Male Speaker] Yeah, yeah. So is there any name for this entity which is you know,
published or whatever? Because this name is somewhat new in the literature as far as I know it. - [Male Doctor] It is called intralipomatous capillary venal to mouth injections. - [Male Speaker] Okay.
- [Male Doctor] It's not an easy shot because of intense vascularity and you see those massive veins, but it was, trans arterially it was large particles and coils. - [Male Speaker] Yeah. - [Male Doctor] The veins, I had to do direct puncture,
and fill with Onyx and blue, I used everything, 'cause the vein bleeding is worse than the arterial. - [Male Speaker] Okay, okay. But it looks like a tumor, like a real vascular tumor. Or is it not, is it a mel or measurement issue. - [Male Doctor] That stimulates the fat cells,
I've got several of these, I've got to write 'em up. - [Male Speaker] Yes, you have to write it up. - [Male Doctor] Nothing described, get alerted. - [Male Speaker] Yes, you have to write it up.
- Alright, thank you. It's a pleasure to be with you again and I just think that this is wonderful intellectual stimulation that we all get here. I'm honored to have once again the longest titled topic to speak on, so in summary I'll talk about the timing
and characteristics of surgical intervention in head and neck malformations. So the big idea is that there's a paradigm shift. The surgeon needs to defer early excision of the vascular malformation, and the interventional radiologist needs to think
sclerosis versus simply pre-surgical embolization. This paradigm shift means that surgery can be limited to contouring and repositioning of tissues, nips and tucks, rather than bloody, deforming operations. The Yakes Center reported in 2016 about 7,000 patients, 1,500 of them, or about 22%,
involved a head and neck lesion. So it's out there, it's very common. It does address some problematic anatomies. I think of life, liberty, and the purpose of happiness, so airway, AB shunting, brain, speech, hearing, vision, and mastication, speech,
those sorts of things, and you can see various malformations involving airways and eyes and speech and hearing. The types of surgery involved in this normal treatment is total excision, sometimes subtotal excision, contour correction depending on what's already been done,
repositioning and then secondary operations as needed, so this is kind of a normal course of surgical treatment. This traditional timing is that it's done early. So you've seen some cases where there's been embolization and then surgery to resect. Later on there can be a middle stage I would call,
where there's a resection of the nidus trying to remove the vascular malformation but it's usually following 24 to 48 hours of embolization. The late treatment is after embolization is complete and there's reconstructive procedures. So these are the various timings throughout.
The traditional approach of E and E, I'll call it, is illustrated here in McCarthy's textbook on plastic surgery from 1990, and it shows a patient with a vascular malformation. It was treated with some sclerosis, not sure what kind, serial excisions, and then eventually at age 22,
she had embolization of the maxillary artery, and then a hemimaxillectomy, and you can see the defect there 48 hours after treatment, which means it was left open. Then over the next years she had tube flaps from the arms and buccal mucosal grafts to finally get the reconstruction
which all things said is a pretty good outcome. That's the traditional approach. An example I have of the traditional approach is this young girl who was diagnosed with an infantile hemangioma, here she is three months later.
You can see how large it's become, the proctosis. And she was treated with serial injections of steroids three times here up to age one, and then two more injections through age two. And here she is at age four. And then beginning age five she had expanders placed
in her neck and cheek, they dehisced multiple times, they were replaced multiple times, and finally at age five she had a resection. The diagnosis and pathology was a venous malformation.
So 22 operations later at age six this is what she looks like. I see her at age 13, she was referred to me by the ophthalmologist, because of the position of the cheek. So I performed a mask face lift,
lifting the lateral canthus, suspending the cheek tissue, leveling the commissure and in between that time she had seen actually Dr. Yakes, you probably won't remember her, but you had said yes, it's probably a venous malformation, we could have treated it through injections.
This is her after at age almost 16 where I've performed a LeFort osteotomy, a Mandibular osteotomy, re-suspension of the cheek, rhinoplasty, leveling the nose, and correcting her bite. And this is her with ten year follow up. So you can see a traditional approach has given her
many, many operations over these years. A recent MRI showed that there was no recurrence. But I want to talk about a paradigm shift. So Sisyphus here, if he could roll the rock down the hill, we'd make things a lot easier. So that's what we're talking about.
So the paradigm shift is that thorough sclerosis of the lesion, yes, there's going to be multiple treatments, maybe 30, maybe 50, but that legion is going to be controlled and now it's going to be converted to a fibro fatty tissue,
so that the surgery degree construct is now functional and aesthetic in a very predictable manner. And then of course continued monitoring for recurrence. So this is a patient with a Mandibular malformation, bleeding around the gums. You can see she's undergone sclerosis with Dr. Yakes and
after seven years of treatment, the lesion's been cleared. There's no need for surgery. So otherwise she would have gotten a Mandibular resection. Yep. And then this is a case Dr. Yakes showed earlier, with retrobulbar vascular malformation
that was completely cleared. She had a little ectropium but didn't want any surgery. She was not symptomatic. And then this case is a patient with a vascular malformation who was treated with alcohol injections. She has a large cheek, it's fibro fatty,
there are no symptoms. I don't remember how many treatments she received, but when she came to see me, she was complaining of the assymetry. So in these cases what I've done in many of them now is performed liposuction because it's fibro fatty tissue,
it doesn't really bleed, so I can do a traditional Tumescent procedure where I am injecting a small dose of lidocaine with one to one million epinephrine, allow it to have some vasoconstriction, and then I'm contouring the procedure.
So here she is at 24 hours, and you can see the improvement after that. So in conclusion the surgical treatment of vascular malformation is undergoing a paradigm shift so that the lesions are now only going to treat the residual deformity, any residual functional anomalies,
and surgery is not for the excision of the vascular lesion itself. And this will make surgery more effective in achieving I think better outcomes with less trauma to the patient emotionally and psychologically and socially. Thank you.
- Talk to you a little bit about again a major paradigm shift in AVMs which is the retrograde vein approach. I mean I think the biggest benefit and the biggest change that we've seen has been in the Yakes classification the acknowledgment
and understanding that the safety, efficacy and cure rate for AVMs is essentially 100% in certain types of lesions where the transvenous approach is not only safer, but easier and far more effective. So, it's the Yakes classification
and we're talking about a variety of lesions including Yakes one, coils and plugs. Two A the classic nidus. Three B single outflow vein. And we're talking now about these type of lesions. Three A aneurysmal vein single outflow.
Three B multiple outflows and diffuse. This is what I personally refer to as venous predominant lesions. And it's these lesions which I think have yielded the most gratifying and most dramatic results. Close to 100% cure if done properly
and that's the Yakes classification and that's really what it's given us to a great degree. So, Yakes one has been talked about, not a problem put a plus in it it's just an artery to vein.
We all know how to do that. That's pulmonary AVM or other things. Yakes two B however, is a nidus is still present but there is a single outflow aneurysmal vein. And there are two endovascular approaches. Direct puncture, transarterial,
but transvenous retrograde or direct puncture of the vein aneurism with the coil, right. You got to get to the vein, and the way to get to the vein is either by directly puncturing which is increasingly used, but occasionally transvenous. So, here's an example I showed a similar one before,
as I said I think some of these are post phlebitic but they represent the archetype of this type of lesion a two B where coil embolization results in cure, durable usually one step sometimes a little more. In the old days we used to do multiple
arterial injections, we now know that that's not necessary. This is this case I showed earlier. I think the thing I want to show here is the nature of the arteriovenous connection. Notice the nidus there just on this side of the
vein wall with a single venous outflow, and this can of course be cured by puncture, there's the needle coming in. And interestingly these needles can be placed in any way. Wayne and I have talked about this.
I've gone through the bladder under ultrasound guidance, I've gone from behind and whatever access you can get that's safe, as long as you can get a needle into it an 18 gauge needle, blow coils in you get a little tired, and you're there a long time putting in
coils and guide wires and so on. But the cures are miraculous, nothing short of miraculous. And many of these patients are patients who have been treated inappropriately in the past and have had very poor outcomes,
and they can be cured. And that a three year follow-up. The transcatheter retrograde vein is occasionally available. Here's an example of an acquired but still an AVM an acquired AVM
of the uterus where you see the venous filling on the left, lots of arteries. This cannot be treated with the arterial approach folks. So, this one happened to be available
and I was having fun with it as well, which is through the contralateral vein in and I was able to catheterize that coil embolization, cured so. Three A is a slightly different variant but it's important it is different.
Multiple in-flow arteries into an aneurysmal vein wall. And the important identification Wayne has given us is that the vein wall itself is the nidus and there's a single out-flow vein. So, once again, attacking the vein wall by destroying the vein, packing
and thrombosing that nidus. I think it's a combination of compression and thrombosis can often be curative. A few examples of that this was shown earlier, this is from Dr. Yake's experience but it's a beautiful example
and we try to give you the best examples of a singular type of lesion so you understand the anatomy. That's the sequential and now you see single out-flow vein. How do you treat this?
Coil embolization, direct puncture and ultimately a cure. And that's the arteriogram. Cured. And I think it's a several year follow-up two or three year follow-up on this one.
So a simple lesion, but illustrative of what we're trying to do here. A foot AVM with a single out-flow vein, this is cured by a combination of direct puncture right at the vein. And you know I would say that the beauty of
venous approach is actually something which it isn't widely acknowledged, which is the safety element. Let's say you're wrong, let's say you're treating an AVM and you think okay I'm going to attack
from the vein side, well, if you're not successful from the vein side, you've lost nothing. The risk in all of these folks is, if you're in the artery and you don't understand that the artery is feeding significant tissue,
these are where all the catastrophic, disastrous complications you've heard so much about have occurred. It's because the individuals do not understand that they're in a nutrient artery. So, when in doubt direct puncture
and stay on the venous side. You can't hurt yourself with ethanol and that's why ethanol is as safe as it is when it's used properly. So, three B finally is multiple in-flow arteries/arterioles shunting into an aneurysmal vein
this is multiple out-flow veins. So direct puncture, coils into multiple veins multiple sessions. So, here's an example of that. This is with alcohol this is a gentleman I saw with a bad ulcer,
and this looks impossible correct? But look at the left hand arteriogram, you can see the filling of veins. Look at the right hand in a slight oblique. The answer here is to puncture that vein. Where do we have our coil.
The answer is to puncture here, and this is thin tissue, but we're injecting there. See we're right at the vein, right here and this is a combination arteriogram. Artery first, injection into the vein.
Now we're at the (mumbles), alcohol is repeatedly placed into this, and you can see that we're actually filling the nidus here. See here. There's sclerosis beginning destruction of the vein
with allowing the alcohol to go into the nidus and we see progressive healing and ultimately resolution of the ulcer. So, a very complex lesion which seemingly looks impossible is cured by alcohol in an out-flow vein.
So the Yakes classification of AVMs is the only one in which architecture inform treatment and produces consistent cures. And venous predominant lesions, as I've shown you here, are now curable in a high percentage of cases
when the underlying anatomy is understood and the proper techniques are chosen. Thanks very much.
- This is a little bit more detailed explanation of the pathophysiology behind Type IV AVM's. Medical disclosures are none. And this is the Yakes classification and this is Type IV lesion we are going to talk about now. So, this angioarchitecture has not been described before, and was first described in the Yakes classification.
What is so unique? It has multiple arteries, arterioles, but these arterioles form innumerable fistulas that are of a microsize, and they infiltrate the affected tissue. So, this is, this can affect every kind of tissue,
skin involvement and muscle involvement, and other than brain AVM, bleeding occurs if mucosa involvement is present or if an ulcer is present. So, we have to think about the definition of an AVM, which is an artery to vein connection
without an intervening capillary bed. But, what applies in Type IV? As you can see here, very nice example of this infiltrating type is that the tissue where the AVM is located is also viable, so the assumption is that
normal capillary beds are interspersed into these innumerable AVMs existing next to the malformed AVM fistulas, and this is a new definition of AVM. So, how to access this lesion? Of course, transarterial is possible
with a catheter or micro catheter. If anatomy doesn't allow transarterial approach, direct puncture is an option. Also, as you can see, in the direct puncture in the lower video, you can see the venus drainage of these fistulas,
and direct puncture of the vein compressed to reflux ethanol into the fistulas is also an approach. But, what is the challenge here? If you want to treat this lesion, you have to keep in mind
that you don't want to occlude the capillaries that are supplying the tissue. So, to find the right treatment approach, the physiologic concept is often important to understand that the arteriovenous fistulas drain into multiple veins and arterialize these veins
so we have a high pressure on this venus outflow site. The normal capillaries have a normal outflow too but this is of lower pressure, and this comes to competition between the arterialized veins and the normal venus outflow, which is, which is inferior to the normal capillary outflow.
So, what follows is a restriction of normal tissue flow with back-up to the capillaries, and backing up into the arterial inflow. So, we have the situation that the arterial venus fistulas have a lower pressure, lower resistance, and an increased arterial flow
compared to the normal capillaries, and this has to be taken into advantage for treatment. How can this be achieved? Thicken the fluid and dilute the ethanol by creating a mixture of 50/50 contrast and ethanol. So, this mixture will follow the preferential flow
into the arteriovenous fistulas in transarterial injections bearing the normal capillaries. So, if it's possible to puncture into the fistulas, pure ethanol can be used, but especially in transarterial access where normal nutrient vessels can be filled,
50:50 mixture contrast is the key to treat a Type IV AVM, Type IV Yates AVM, and here, you can see, using this approach, how this AVM can partly be treated in many several treatment sessions. And here you can see the clinical result. So, this huge ulcer, after seven treatments, healed
because of the less venus hypertension in the lesion. So the additional benefit of 50/50% ethanol contrast mixture is that your injection is visible on flouroscopy so you can see if which vessels you are including. You can react and adjust the pressure you're injecting. So, it also has to be considered
that the more you give diluted, the more total ethanol can be needed, but it's not efficient in larger vessels. This is also the advantage that you just treat the microfistulas. It's of importance that you use non-ionic contrast
as ionic contrast precipitates in the mixture. So here, you can see again, see the Type IV AVM of the arm and hand, which I already showed in my first talk, and here, you see the cured result after multiple sessions showing good arterial drum without fistulas remaining.
So, the conclusion is that Yakes Type IV is a new entity. It's crucial to understand the hemodynamics and the concept of 50/50 contrast ethanol mixture to treat this lesion with also a curative approach. Thank you very much.
- Thank you. Ethanol embolisation for the cure of complex AVM. Case study of 100 consecutive AVM cases from the Yakes Vascular Malformation Center. So the purpose of this study is to describe the efficacy of the Yakes Classification and the application. To describe angiographic outcome, cure rate and
clinical correlation in symptom resolution for the patients with complex AVMs. And also treatment strategies and efficacy of that. So Retrospective Single Center analysis, of course, we talk about Yakes malformation center. So we're talking about One Operator experience and
what we included in this study was analysis of all AVM patients with a minimum of one treatment in a in a four year period. All consecutive treatments were included and it was based on the Yakes classification system using coils and ethanol.
Excluded were Venous Malformations and head and neck AVMs and Venous malformations. So Demographic Patient data center extended of AVM all was included and analyzed Extensive Localized data dijon extent was analyzed and of course the type of AVM in the following groups as seen on the screen.
Angiographic result and follow up as well as the treatment period was documented and the number of Treatment Sessions. Clinical results, Complications and follow up was gotten through questionnaires and Angiographic results were defined by expert consensus and these were the categories
we based the results on. Cured was in this series 100% salvage and cure after AVM. So Angiographic no AVM visible. Markedly improved, improved no change and failure accuration was also documented.
Clinical results were defined into four categories from Complete resolution of symptoms, Considerable reduction, Unchanged or Worse. So the Total Cohort was as mentioned, 100 patients 46 male patients and the locations you can see was distributed all over the left arm
and of course also trunk. So we had 77 Extensive cases which was defined as areas that extended a joint region. So our skin involvement and muscle involved with so involvement of severed tissues .
If this was applicable in the patients, it was an extensive lesion and 77 of them were extensive. 56 had previously failed treatments in other institutions and were referred too instead. Here we see the results of the Total Cohort. We had cured patients in we had a cured Lesion in 49
patients. And no symptoms in 34 patients reduce symptoms and 47 patients. So the cure rate was almost 50%. And you see that this group of patients with Markedly Improved or also Improved
outcomes were still considered under treatment, so they were not at end of the treatment. To complications, in the Total Cohort were six and 33 Minor complications including wounds, skin breakdown. No complications in 61 patients. This is an interesting slide showing
the group of cured patients. You see that Failed previous treatments was present in 23 of this group, and they were cured during the treatment in the Vascular Malformation Center. No previous treatment was present in 26 and
the total number of cured as mentioned 49. This is the Clinical Outcome of the Cured Patient group with No Symptoms and 30 Reduce Symptoms and 16 and just three were Unchanged but there were reasons that could also explain an unchanged state in one patient there was an additional Venous Malformation and
other also a DVT in the treated area. To complication and the cured patient group was one forefoot amputation and in one infected coil pack in the pelvis and rectum area. Minor complications also included No complications and 34 patients.
This is the Treatment Plan and this comes now to the follow up of the group of cured patients. There was a large group of 28 who already had cured in long term follow up and as we can see here that also we had few patients that were Angiographically Lost to Follow Up.
The reasons was that some of them had no symptoms or interest issues was also reason for that. Here eight patients were treated in one session but this is the number of treatment sessions for all cured patients with mean of 11.7 treatments, the length of the treatment period was 50 months.
Let's go back because this is important. Mean Follow Up after cure, means regular annually Angiogram and was with a huge range from one to 120 months. So also long term Follow Up follow up was caught in this analysis.
Here you see a comparison of the number of treatment sessions compared between the cured Yakes type one, two, three and the cured Yakes type fuor. And you see that the type for patients have a significantly higher number of treatments. And this explains or could give a conclusion that is a very
difficult lesion to treat. Here you see the number of treatment months also the length of the treatment period and you see again, that type four complex AVMs have also a significant longer, longer duration of treatment, which was 62 months significantly more.
So the conclusion is the Yakes Classification is a highly effective tool to achieve cure with low complication rates. The classification system is useful and guides how to use ethanol and coils. And its appears to be more complex AVMs if it comes to type four AVMs instead of Venous Predominant lesions.
Yeah, thank you. (audience clapping) - Okay , comments, questions? Well. - AVMs can be cured? The answer is very simple,
the Yakes classification cures AVM. And that's all you need to say because the data is unequivocal, and it's very, very convincing in my view and those classification usage know that we can cure these now. - But one thing is a treatment and
the other thing is a classification. - So the classification should guide our treatment. I think we we have to separate that and it's totally convincing and I think to distinguish between the predominantly Venous and it's subtypes and
the more small vessel thing and it's a different treatment and direct puncture and retrograde, the transvenous and I think it's very helpful for all of us and very worthwhile to correctly classify and to correctly treat. But I think it's two different things.
One is the classification and the other is treatment. The treatment follows out of the classification. So thanks a lot.
Disclaimer: Content and materials on Medlantis are provided for educational purposes only, and are intended for use by medical professionals, not to be used self-diagnosis or self-treatment. It is not intended as, nor should it be, a substitute for independent professional medical care. Medical practitioners must make their own independent assessment before suggesting a diagnosis or recommending or instituting a course of treatment. The content and materials on Medlantis should not in any way be seen as a replacement for consultation with colleagues or other sources, or as a substitute for conventional training and study.