- Thanks Bob, appreciate it. Let's start with what I think is an illustrative example. This is a 14 year old boy who had a symptomatic vascular malformation treated in the typical fashion, direct puncture, ethanol injection, who had substantial on-table decompensation. Rapid pulmonary angiography demonstrates this obvious,
extensive pulmonary embolization. So how does this happen? This shouldn't happen. Well, post hoc evaluation of the venographic images show that there are large communications between the superficial venous malformation and the deep venous system,
and in fact, if you look closely not only can you see the abnormal communication you can see clot in transit. This is based on review after the fact, so it's actually occurring during the procedure. Whenever treating superficial venous malformations, we have to understand that there are extensive normal communications
between the superficial and deep venous structures. They are all over the leg and in fact, these can be conduits for anabolic material to enter the deep venous system causing acute or ever chronic complications. So what are these? Well they're simple, they're perforators.
Those of us who are involved in the treatment of superficial venous insufficiency are familiar with them. We know that they're distributed broadly throughout the leg. And their normal pattern of flow is from superficial to deep, the exact opposite direction we would want if we're treating a superficial venous malformation.
So, when treating a malformation like this, and doing your initial venogram, if you identify a large communication with the deep venous system, I think it's prudent, when technically feasible, which is usually, to embolize these communications and then go on and treat the malformation.
Now, we see communications with the deep venous system routinely, and not all of these need to be treated, many of them are quite small, like in this example, and can probably be left untreated and the malformation safely embolized in standard fashion. That said, when large, it seems imprudent to embolize
this malformation, leaving this potentially disastrous conduit to the deep venous system pedant. So after the communicating vein is embolized, the comfort level with treating the remaining malformation is much greater. And in fact when you stumble across these, in some cases
I don't even understand how the malformation could be treated without embolizing the associated perforator vein. It's important to remember that the perforators communicate the deep and the superficial system, and the deep system is a mechanical pumped system which is much higher pressure than the superficial veins, or the
superficial venous malformation. So if you leave these perforators untreated, when this person ambulates, they're literally pumping high pressure deep venous blood into the superficial system or into the superficial malformation, exacerbating symptoms significantly.
So how do we diagnose these? Well it's actually harder than one would think. In an ideal setting we would identify these all pre-intervention and make a plan in advance. The problem is our pre-procedural imaging is supine. We get MR exams in most of these patients, and the
perforator system is postural, it's a gravity dependant distensible system, so even though, if we look closely, we can see these perforating veins on MR, they look, for the most part, irrelevant. They're small, they tend to be tortuous and they're not well seen in a single plane
And even in retrospect, when you see a large perforator and you go back to your malformation, sometimes they're surprisingly challenging to identify. Now the ideal way to visualize these would be with a standing MR, which of course we don't routinely do, but we do routinely do standing ultrasound exams.
Certainly anyone who treats superficial venous insufficiency is familiar with a standing ultrasound exam. It's a little bit technically challenging for your sonographer, but once they get used to it, it's relatively replicable. And you can identify and map the abnormal
perforators along the length of the vein. That said, much of the time we do make the diagnosis at the time of therapy, and when I'm doing my initial venogram to treat a superficial venous malformation, and I see a large communicating vein, which I think we can say is a perforator in almost all cases,
I generally think that the prudent thing to do is to embolize this. And when you see this post embolization, post disconnection with the deep venous system appearance of the venous malformation and you compare it to the pre-perforator embolization appearance,
my comfort level with embolizing what you guys see on screen right would be very high. My comfort level with embolizing what I see screen left is certainly lower. And so, I disconnect the system in essentially all cases when I identify these malformations.
Sometimes, it's unclear in a large malformation with a relatively small communicating vein whether it's clinically relevant, and I don't know. If I can readily compress which was demonstrated in the previous talk, I won't necessarily embolize each and every one of these.
Sometimes it's technically challenging to embolize them and in those cases I'll leave them alone. But if possible I'll embolize most of these and I think it gives me and probably anyone else who does these a different comfort level with using these liquid embolics. As I've become more interested in these communicating
veins and their relationship to vascular malformation complications, I've stumbled on a small number of patients who appear to have a pure perforator based vascular malformation. These patients have typical vascular stigmata at birth. They're often carrying the diagnosis of KT Syndrome
or sort of a juvenile onset form of severe superficial venous insufficiency, and when we do standing ultrasounds maps of these patients, we generate these kind of ridiculous hand drawn images where we'll see 20, 30, or even more individual abnormal perforators. And in this situation treating the superficial component
is certainly important for symptom resolution, but to cure these lesions you have to include these perforators, and sometimes you have to include all of them, which may be dozens. So this is the typical approach that I take in a patient like this.
I identify one or two, or maybe a few specific perforators that I want to treat. I access the vessel, and then I use ultrasound over the accessed vessel and compare it with the documented standing ultrasound abnormal perforator to make sure that I'm actually in the correct vessel,
because there is dozens and dozens of perforators, not all of which may be abnormal. Once I'm in that location, the appropriate location, I occlude it as per normal, and then treat the residual malformation, so I'm doing two things. Number one, I'm increasing the safety of my
superficial embolization by preventing deep system flow, plus I'm also treating what I believe is potentially a nidal component to the malformation. So, when you stumble across these, if you haven't diagnosed them in advance, sometimes you're unsure whether they should be treated or not, but when you look at
malformations such as this and you see the degree of malformation filling without the perforators being occluded, and then you re-evaluate after the perforators have been occluded, you see a dramatic difference. And I think in disconnecting the system, not only do we make it safer, we increase the delivery of the
liquid agent to the malformation, and I think treat the lesion better. So, essentially the method here is, the embolic target, the vascular malformation, should be separated from the complication target, which is the deep venous system, by occluding the complication conduit,
which is the perforator vein. It's important to remember, many of these, most of these are young, healthy patients, these are elective procedures, and the acceptable threshold for complications is actually quite low in many cases. Thank you.
- [Audience Member] I have a question. Do you have this deep venous system more in the lower extremity, or you have it also in the upper extremity? What's the percentage? - [Scott] So the deep venous system is certainly present in the upper and lower extremity.
We do the standing ultrasound exams in the lower extremity, whereas those are normally done in the supine position. In the upper extremity, these exams are normally done with the patient sitting, and so it's quite easy to map the malformations, but if you look, upper extremity or lower extremity, there's
perforator veins throughout on both. They're clinically less relevant in the upper extremity system because superficial venous insufficiency is so rare in that setting. - [Audience Member] Yeah but we had one patient with venous malformation of the elbow, with a large
draining vein and she actually did a pulmonary embolism but that was 15 years ago, so I was wondering if this risk of pulmonary embolism is more common in the lower extremity or the upper extremity. - [Scott] I think it's more common in the lower extremity, primarily related to the fact that the calf muscle
acts as a peripheral heart, or a venous heart, and any clot that does find it's way into the deep veins is rapidly pumped centrally, and so the common thing that we hear, is not so much the case that I described of the patient that decompensates on table, but often when the patient gets off the table,
when they first move, that's when everything hits and that's because they activate their calf muscle pump. - [Audience Member] Thank you. - [Host] Are there any other questions from the audience? I would have another question. So I'm not sure with which embolic agent you're working,
but in case you use coil occlusion to reduce the outflow to the deep venous system, would that effect also the amount of the agent you inject in total? - [Scott] So almost exclusively ethanol, yeah I think that embolizing the deep system communication does concentrate or saturate the
malformation to a greater degree, you're not having this leakage of ethanol through the perforator. And so I think you do use less alcohol and also I think you get a better treatment, and so you're treating in the same manner, but you could probably get the lesion treated with less overall
separate embolization sessions. - [Host] Thank you. - [Scott] Thank you.
- (speaks French) liver surgeon I perform hepatobiliary surgery and liver transplantation. Maybe I don't belong here, I so probably more rested than anybody in the room here. But today I will present about liver surgery and hepatectomy. I work at The Royal Free where I have the honor and pleasure to have seen Krassi. We are in the
little island in the North Sea. There is many things going wrong there including Brexit but, the guys uh, we have a major advantage. The NHS favors centralization. Centralization look there: London is bigger than New York Uh, eight million, 50 million greater London
and we drain about six millions of people with our HPB center. In the center we perform about 2,000 operations, of major surgery. In five years, half of them are liver surgery. And most of them have uh, benign, malignant tumor. A very small percentage have benign tumor.
I count here for complications uh, and mortality look there, 3.1% of only the malignant because the benign are young people and we perform a different strategy, they have no mortality. Today Hepatic Hemangioma, look there it is uh, 1898 is a key year. Not only the first description
of the lady that died after bleeding out in an autopsy but also, Hermann Pfannenstiel uh, Professor Pfannenstiel. I will introduce you to him. He described the first operation. Now, we're talking of congenital malformations, they uh, lesions occur in the liver and they may grow,
but only 20% they grow. They have a chaotic network of vessels and they have fibrotic, fibrotic development within it. I introduce you Hermann Pfannenstiel, he was a gynecologist, famous, famous, important incision that we still use today.
Remember him, we'll talk to him later. Microscopically, the microscopic is our well-circumscribed lesion, they're compressible. Important you see down there that they compress the liver that is normal close to it. This has an implication because if you operate,
you fill find a blood duct or a vessel and it will bleed or leak by. Microscopically, they are ectatic blood vessels and they are fed by arteries. This is also an important point, for therapy. Separated by fibrous septa, this is also important
because they become harder and they become bigger. And they have distorted blood vessels. They're more frequent uh, benign tumor. Prevalence up to 7%, they have non-neoplastic this must be clear, they are non-cancer. The proliferation of endothelial cells, women
have more and particularly pregnant women, more pregnancy or contraceptive. We divide them in cavernous and capillary and we'll have a word on that. Symptomatic being half of the cases, multiple in 10%, they rarely bleed and they rarely rupture.
Capillary Hemangiomas cells small, I show you an MRI here. The differential with HCC liver cancer is most important. They both are theorized but they continue to appear on late face. They are asymptomatic please, do not touch them, they do no harm.
And so we will not speak of them. We speak only of the cavernous hemangioma. And here, the cavernous hemangioma bleeds Oh my God, no, it's not true. There are 83 reports of bleeding since the report of Hermann Pfannenstiel. Uh, 97 cases, adenomas bleed more frequently.
Frequently, in the past they were confused. Hemangioma and adenoma, adenoma does bleed. There are only true cases, 46 in the literature. Size is not important and they are very rare in elderly people.
This is what we see when they are giant cavernous hemangiomas, they're serious, they are rather easy to diagnose. Diagnostic criteria, uh, look up typical for uh, cavernous hemangioma. How do you point here? Yep, you stop. If you then see that you have
an atypical hemangioma, you jump over to an MRI. MRI is too nowadays, diagnostic and uh, the important thing is you stop. Once you have the diagnosis with MRI, you stop, do nothing yet, do not follow, bye-bye. Treatment modalities surgery: Selective TAE, Radiotherapy, Medication: two classes,
Propranolol, to decrease the hyper circulation. Bevacizumab as a class of drugs of inhibitors of inferior growths and endories, eventually are cold. This is seminal paper, about 35 years ago "Do not treat asymptomatic patients." This is a key: do not bother with hemangioma.
If you do have the algorithm, you look at complaints that can present incidentally when they have complained, not complained, no treatment of abdominal pain. Unrelated to no treatment, we have to eventually make sure that the pain is not related to the cavernous hemangioma. If there is other futures
like compression giant, you can do surgery. If you have a doubt in diagnosis, today rare with MRI, then you can perform a biopsy. The surgical indication then remain progress, severe, disabling symptoms. Diagnostic uncertainty nowadays not the case, with MRI.
Consumptive coagulopathy or Kasabach-Merritt syndrome is a serious, we will see when you perform human transplants. Spontaneous rupture with bleeding as an emergency. Rapid growth in 25%. This is a paper that shows that the size of the cavernous hemangioma is here,
and you can see that operation has been performed for larger size, however, look that even in non-symptomatic or partially asymptomatic patients, you can reach sizes up to 15 centimeters. And this a review of the literature from a Chinese group where they revised a thousand to a hundred cases,
no mortality in the series and enucleation versus the anatomic resection is better. Less complications, less blood less, less time of surgery, and less hospital stay. So please, in this case of surgery, we do enucleation. I was asked by my society the HPBA to speak
about transplantation for liver tumor. You can that an indication is unresectable disease, severe symptoms and mass occupying effects. Pre-cancerous behavior is not for hemangioma only for adenoma differential diagnosis with HCC. And you have to be attentive that you avoid
liver insufficiency during your resection. So, in conclusion, for benign lesions, hemangioma technically is the only indication. And now the systematic review that shows around several emothing United States UNOS and the ELTR Several, several benign tumors but if you break down
for type of tumors you see that most of them are Polycystic disease or partly cavernous hemangioma are very low. 77 in Europe, out of 97,000 operation of transplantation. So, let's get an old paper. The pioneer of transplantation again, extremely low,
one out of 3,200. An extremely low percentage. It's my personal experience I was working at Essen, Germany. Almost a thousand transplants we performed. Unfortunately most of them I did and we never transplanted one hemangioma, my experience for transplantation is zero because it should not be done.
So, my advice for hemangioma. Biopsy not advised, see a liver surgeon in a serious center, diagnosis is done my MRI, observe doubt symptoms and observe. Let the patient beg you for surgery, if significant increase in size and symptoms, we can do surgery. Embolization is possible.
Sometimes it's harmful. The role of the surgeon is to confirm the diagnosis, differentiate it from cancer, exclude causes of other symptoms and avoid unnecessary surgery that's the main thing. Surgery for severe symptoms of Kasabach-Merritt. Only for complicated symptomatic lesions, or where the
diagnosis is uncertain. Ladies and gentleman, I will conclude with a couple of questions. If you have a daughter or son with a liver tumor, would you go to a center or a competent surgeon or to a gynecologist. Professor Pfannenstiel for instance or another doctor. If your car has a problem,
would you go to a good mechanic once for all, or to a small shop for 20-40 times. It is a matter of experience and a matter of costs. And with this, I am ready for your questions. - [Audience Member #1] When have you personally operated on these lesions?
- [Speaker] I am. And the experience that I have in the past I seemed young but I practiced for many years. When I started 25-30 years ago, we were operating many of these because we were not so certain. Then MRI came, and MRI basically made the diagnosis so easy and straight-forward and we started observing
patients. We still do operate today, but they are very large tumors and when I do personally, I avoid the androbolization before because you have more skylotec reaction, just (grainy sound effect) to peel it away from the normal parenchymal.
This is our experience. - [Audience] Thank you. - [Speaker] Thank you very much, yes? - [Audience Member #2] Yes, one question. When you operate, and with all of the experience you have, what are the complications of
(mumbles) - [Speaker] The main, so first of all, there has been also an evolution in the type of operation we don't do anymore the resections where you have some bi-leaks. If you operate correctly, it's bleeding and one infection not one born. If you have to watch bi-leak is the one
that you have to watch and that's because the tissue is pushed away and you may miss something during the enucleation.
- Okay, liver hemangioma, let's continue on. It's a misnomer in the literature, it's not a hemangioma at all, a hemangioma is a tumor of infancy that makes itself manifest within the first month of life as a proliferative phase, and then it goes away. These are present throughout life, so they cannot be that, you see them in adults.
It's most often, when ultrasound came about is when we really found what's going on because everybody gets an ultrasound, they always look at the liver, and they're in so many people we don't even care. It's the most prominent, common benign tumor of the liver
and the vast majority, there's nothing to do. So in the rare lesion that is large, or one that is large, but not real big, but it still can produce significant pain symptoms in the periphery by stretching the capsule. So it's largely by liver capsule stretching
that the pain fibers are done, and these patients have their problems. It's a rare problem, but it's still there. Again, liver hemangioma is a venous malformation of the liver. Rectal hemangioma is another misnomer,
that's venous malformation of the rectal wall. Vertebral body hemangioma is another misnomer. This is intraosseous venous malformation of vertebral body mixed into the pedicle and transverse processes. It can even have epidural venous extension
with cord compression or nerve root compression. All are venous malformations. Now this again, we just had the talk on that. With my patients, we had one with acute hemorrhage with NY in significant pain as it enlarged by the hemorrhage into it.
These are big venous sinusoids, is all they are. And, we had one patient with close to the diaphragm, had intractable hiccups, but all of them present with abdominal pain and that's what brings them in, by and large. And we have about 28 patients with this distribution,
we've done this many procedures. We had one patient get an infection, put on antibiotics, and that's when I got the ... and one patient during the angiogram, very skinny gal, she pressed on her sciatic nerve from her fanny, (laughs) from her ischial tuberosity,
had a transient (laughs) foot drop (laughs) from a liver angio. In these, we've always seen shrinkage of the mass, pain symptoms completely resolved by decreased stretching of the liver capsule, hiccups resolved, foot drop resolved obviously.
Here, give you some examples. Here's a 39 year old male with abdominal pain in the right upper quadrant, especially with exercise. He even quit his exercise. You can see here. You can see he had two of them,
one immediately adjacent to the capsule. Another view. On arteriography, you can see it's a post-capillary venous malformation. So that's why on dynamic CTs you see the peripheral enhancement going central
'cause it's post capillary. I've never seen a direct shunt in one. We have a problem because people have done embolizations of these transarterially. And that affects nothing. All it does is affect the portal triad
and give you biliary strictures. 'Cause it does not get to the malformation, and it's stopped at the capillary level. So, it's a failure of thinking to think you're going to treat any of these by an arterial embolization.
It's by direct puncture, like every venous malformation, you're post-capillary, and you're in it. Portal triad is spared, and you're not going to get any biliary strictures or injury. So again, later phase, showing these lesions, that one on the bottom there is not a lesion.
It's a gallbladder from the cystic artery showing opacification in the most inferior rim-like thing. Here's what they all look like. They're not cavernous. They may be large, but every one of these is microvenular.
Doesn't matter the size of the malformation. Every one is microv... I've never seen one, other than this, I've never seen a cystic lesion, anything. They are all this microvenular, just kind of like (blowing) like a cloud expanding.
Here's after thrombosis. Here's the other lesion, you can see thrombosis in both compartments. Here's the next day on CT, proving yes, we were in fact within that lesion. I quit doing these CTs anymore
once I was convinced what we were doing. Here we are again, showing how they shrink and the pain goes away. The big peripheral one. It's showing the little scar down without any opacification, and it forms scar
just like any healing process, and they shrink and the pain goes away. Everybody can tolerate a 21-gauge needle into the liver. 49 year old female. Again, large lesion, inferior area, direct puncture into it, again they're all identical.
They all look like this, microvenular. Thrombosis. There it is shrinking, the pain goes away. 41 year old female, worsened by ambulation, preventing deep breathing. Here's again (mumbles) you see
the peripheral enhancement, again post-capillary venous malformation, here's another view, arteriogram showing it, draping of vessels, slow flow into the vein malformation post-capillary, they all look like that.
Direct puncture, microvenular, thrombosis, then they shrink, and the pain goes away. 42, another one, abdominal pain, stopped her exerc... This is one of my physicians in the ICU,
a pulmonologist, she's a real skinny gal and just a mountain climber, she had to stop everything. It hurt her to run or even do her stationary bike. So it was lifestyle limiting, the pain for her. And here it is, in this one I told her I was a little worried.
Usually these are self-contained within the liver, this was exophytic. If she ever had an injury, I'd worry about a rupture and peritoneal bleed. So I told her there's a couple of reasons. One is your symptoms obviously, but I worry (cell phone ringing) with all your high action
(cell phone ringing) and impact activities (cell phone ringing) you could fall, (cell phone ringing) have an injury, (cell phone ringing) something like that. (cell phone ringing) So again, you can see it (cell phone ringing) draped inferiorly.
(cell phone ringing) And here again, (cell phone ringing) they all look alike, (cell phone ringing) microvenular lesions. (cell phone ringing) And then they ... That me? (audience laughs softly) How the (mumbles)
and so, here we are, treating it goes from there to shrinkage, and you can see the bright signals, the lack of fluid in there. Just scarring down, she went back to her activities. 36 year old female, right sided
abdominal pain, again, this is abutting the capsule, again, they all look alike, microvenular lesions, there it is abutting the capsule, all three views, and there it is causing here, to shrink to that,
this view to shrink to that. So they all shrink, they all form scar, and the pain goes away with the stretching. This is a kind of an interesting case. We've had several of these
that are massive involvement and pain. And again, you see the peripheral enhancement. Again, the arteriogram showing that, no shunts. Direct puncture, again these are all microvenular lesions no matter the size, all identical. And it goes from this, causing gross expansion,
to shrinkage. From this, to that. She went from just sitting in bed having trouble breathing 'cause of the pain sitting up. She'd rather lie flat.
When I first saw her, it was tough walking, she couldn't hold a job now, she went to running marathons at this stage. So her life changed, she was able to take care of her kids. She was very happy.
So our conclusions are, it's effective in treating just like venous malformations of any other tissue. It's tolerated well with a 21-gauge needle, tough to hurt somebody in the liver with that. I use Ancef and Flagyl after I had
that one patient that had an infection, because I got to thinking, what's coming into the liver? Portal system. Where does the portal system come from? The bowel.
What does the bowel have? Bugs. So we cover gram-positives and gram-negatives, haven't had an infection, abscess, nothing since. So I recommend that being done. Imaging studies, again, document the thrombosis,
the scarring and shrinkage, and malformation. Thank you. - Any questions, wise or otherwise? Yes. (man speaks off microphone) Sure.
Why not? But I'm in the camp not either / or, I'm in the camp of both / and. I don't want to keep a patient on a pill for life. I think we get 'em fixed. And if we can make 'em less symptomatic,
or even better, make that smaller so there's less to treat, wonderful! Thank you. - Professor. - Oh, yes. - So, the last case you showed
of the giant hemangioma, that was the only one I saw that where you had multiple accesses. The other one's a five or eight centimeter symptomatic hemangioma, are you treating it single session with one needle, are you able to ... - In these large ones, you're not going to do a single session.
It's going to be a few treatments. - And what kind of volume, on those images you showed, what kind of volume of alcohol? - In the beginning, I used to use and not go beyond about 20 cc. Sometimes I ask them how are you tolerant,
'cause I'm going to cause an acute thrombosis and expansion, and then it goes down, and we cover 'em with steroids. And that brings it down quickly, than without. But then I saw patients handling things, like that giant one,
I give that lady 40 to 50 cc of alcohol every treatment. And then she comes back the next day saying, "Can you do it again?" (laughs) She's a tough Laotian girl. (laughs) Yes. - In one of those you treated,
there was hepatic vein filling and renal filling. - Yep. - Do you see that routinely, and do you worry about (voice trails off) - Oh, of course. But what you do is, you do the angio
and you see what the volume was to fill til you get to the point you see that. So you have two options at that point. One, reposition. Or two, inject to that point of volumes and it just stops, 'cause there's really almost
no flow in these things. So you inject to that point where you see, it takes like six cc, then you see the vein. I go five cc of alcohol at the same rate of injection to stop. Wait 10 minutes, and do the angio
to see what's thrombosed or you need a little more. Yes. - When you puncture it, are you direct puncturing it or are you going through some healthy liver tissue? - Liver tissue, especially the deep ones. If it's peripheral, you're not going to hit much liver.
- So you're avoiding to puncture through healthy liver? - No, no, no, I do not. No, it's 21 gauge needle. You're not going to hurt anything with that. Our CT body images put 18 gauges all day long and bigger, and what complications
they have? Almost nil. 21? Doesn't exist. Yeah. - Thank you. I just would like if you would be willing to make a publication on the classification of these so-called
hepatic hemangioma of the liver. Because I think this would be very important for the people are not experienced in this lesion because we see many adults coming with so-called hepatic hemangioma that are actually venous malformation
that has been treated for years on propanolol and of course there's no response because it's not an infantile hemangioma. So it would be really great to have a better classification and I saw beautiful management charts of all the different lesion
but it will be great if there would be the correct name on these and what to do for infantile hemangioma that actually respond often quite well to propanolol and even to vincristine and do not need to be operated
in contrast to venous malformation, where you need to do a more aggressive treatment. So thank you.
- Alright, thank you for asking me to speak. It's always worrisome when the interventional radiologist is talking about hematologic things, and I see some of the faces there that know probably a lot more about this me. There's not much literature written on this subject, with respect to vascular malformations.
Basically, the venous malformations particularly, originally thought to be a relatively benign phenomenon. But, Enjorlras and Mazoyer, I can't really see over there, did the first papers on this,
and they first noticed an association with purely venous malformations in a distinct hematologic syndrome that was different from Kasabach-Merritt syndrome. Now vascular malformations basically deal with Virchow's Triad,
and a venous malformation has a local environment that's very conducive to thrombosis. Basically you have altered blood flow with abnormal valveless vessels, relatively slow flow, and that leads to clotting. Similarly, I have endothelial injury,
which again leads to clotting. And this sets up a hypercoagulable viscous circle where you have increasing thrombosis and you can get localized intravascular coagulation. So in these original two papers back in '97 and 2002, they found that basically these patients had
episodes where they had a lot of bleeding during surgical procedures, and it led to lower levels of fibrinogen and increased degradation complex and they said a low platelet count, it was low-ish, but not abnormal.
And also leading to phlebolith formation and other bleeding complications peri-surgically. So what is LIC? Well we all know our coagulation pathway, the end of the cascade leading from fibrinogen to fibrin and what they've found is that with patients
who have LIC within a venous malformation, you can have elevated D-dimer levels. I won't get into the actual numbers. Suffice it to say, whatever your lab is, they're elevated. Fibrin degradation products are elevated. And then you can sometimes have low fibrinogen levels,
but it's important to realize that there's a normal PT, normal PTT, and normal platelet count in these patients. So how prevalent is this within this population? Well, in venous malformation in their original 1997 paper, it was very prevalent. 88% of patients had elevated D-dimers and
some had decreased fibrinogen and low-ish platelet counts. Again for the paper in 2002, you shouldn't expect to read that, but graphically I've got these, those little red lines increase how many more times normal the D-dimer levels were
and some of them were off the charts literally, and the other little set of little red dots over here are how many times less the fibrinogen levels were than what would be considered normal. So when it's present, it's really present. Now the thing is,
these are all the papers that actually talk about its prevalence that have ever been published. And each one of them, I'm just going to show you these sets of figures here, the important point to notice is that, sorry I'm going to go back, I apologize.
One second here. Is if you go through all of these, you'll see that incidence of localized intravascular coagulation is around 40 or 50 or even 60%, all things being considered equal. Now, what is the relationship between the incidence
of LIC and lesion characteristics? Well, this is a great paper from 2015 where they looked at 70 patients, and this is a great little diagram I like, is that if you look at just, they divvied up lesions from less than 250 CCs,
250 to 500, and then greater than 500, and looked at the incidence of LIC in their population. In the smaller lesion, there was hardly any, and if you look in the larger lesions, the patients who had LIC greatly outnumbered the ones that didn't.
So they also found that spongy lesions, and ones with phlebolith, as well as ones that were non-superficial, were the most likely to cause it. So why is LIC clinically relevant? Well, it causes pain, and over time, you can get lumps,
phleboliths within the lesion, and you can also have other thromboembolic complications as a result of it. But most important, it's relevant because LIC can proceed onto DIC, which is obviously a much more serious condition,
and the things that can stimulate that are trauma to the lesion, fracture, surgery, prolonged immobilization, menstruation, and pregnancy, and of course, sclerotherapy. So if you have a patient who suffers from LIC, messing with the lesion if they're vulnerable
can lead to DIC. So it's very important. And remember that DIC has all the components of LIC, but you have reduced platelet count and elevated PT and all other sorts of abnormalities, so it's the whole shebang.
Similarly too, when I was preparing for this talk, reading the hematologic literature, in addition to overt DIC, there's something between LIC and DIC, which they call non-overt DIC, so there's a spectrum. And there's all these different international criteria.
You don't want to get bogged down. But the meat and potatoes in the last three minutes of the talk here are when do we intervene clinically to address these hematologic issues, what parameters and what clinical setting are important,
and how do we stratify? Well, all patients with venous malformations, you're going to treat conservatively. Encourage activity, avoidance of activities that cause symptoms, and have compression garments. These can reduce the volume of the lesion
and make everybody feel better. They can decrease incidence of LIC symptoms and pain. We all know our heparin pathways, but the issue of heparin and anti-Xa therapy, looking at low molecular weight heparin, it's been found that when you give
low molecular weight heparin, in painful lesions the D-dimer levels drop precipitously. That's proven beyond a doubt. So what does that mean? Well, when should we use low molecular weight heparin? We assess risk.
All patients who have large sized, multi-focal lesions, venous ectasia or an overgrowth syndrome, or any kind of combined lesion should have a hematologic work up looking at the D-dimer, PT, PTT, fibrinogen, CBC. That's your first step.
If they don't have that, just conservative therapy, but if they do, and you're considering intervention, if you look at all of those risk factors, if they're negative for those risk factors, again conservative therapy, no low molecular weight heparin, compression garments.
But if they're positive, before you treat the venous malformation, either surgery or sclerotherapy, you give them half a milligram per kilogram of subcutaneous low molecular weight heparin, for one week before, look at the labs,
and give it another week. So basically, all literature says 10 to 14 days before you do something, give the goods. After therapy you give low molecular weight heparin too, and you pick the longest of two things. You either go for the same dose again
for two weeks after therapy, or until they're ambulatory, whatever is longest. So that's the most important slide. Chronic therapy, all venous malformations if you have these risk factors, if you have an elevated D-dimer
or if it's negative, go conservative. If it's positive, look at the fibrinogen level. If it's not elevated, go conservative. But if it is decreased, give low molecular weight heparin. Similarly, if your D-dimer level is elevated and you have pain,
give low molecular weight heparin. If not, conservative. A word on DOACs, direct oral anticoagulants. There's some early promise that if you look at this graph, this patient here received a DOAC instead of low molecular weight heparin,
and their fibrinogen levels bounced back up. We're radiologists here, pictures say everything. Here's a patient who was on low molecular weight heparin and their anti-Xa activity and all of their fibrinogen levels stayed the same. They were transitioned to dabigatran.
Everything stayed the same. So it does work. Final slide. Aspirin therapy, anecdotal evidence only. Vitamin K therapy's only anecdotal as well. But you certainly don't want to give aspirin
in the pediatric population, and we don't know if there's more trouble and complications and again, vitamin K may be a good thing, may be not. That's sort of the tour of hematologic issues in venous malformations.
Not that much literature. Bottom line is follow the rules for low molecular weight heparin. I think these talks are online afterwards so you can get all of the little data on there. Thank you.
Any questions? - [Audience Member] (mumbling). I was finding after treatment, they started getting bruises and things (mumbling). A lot of these patients are positive D-dimers, low fibrinogens, and low platelets.
It's not unusual for us to treat (mumbling) foot malformation over several days. You can send them (mumbling). So anybody that's got a large malformation (mumbling) all these things to know ahead of time (mumbling) to improve the situation (mumbling).
They were there, did an angiogram (mumbling). Four inch hole. We almost lost this guy. 35 years old. This is a real event. How in the hell, killed by a four inch catheter.
He would have been DIC if we pulled (mumbling). - [Gerald] I think I've been whistling past the graveyard for years until recently, but you always hear these horror stories. (audience member mumbling) Yeah, exactly.
- [Audience Member] Gerald, what's the relationship between pain and the administration of low molecular weight heparin in some of these larger lesions? - [Gerald] It does decrease it. The literature shows they do get better.
It's the only thing proven in the several studies to make a difference. - [Audience Member] And in your experience, and that of others, has there been a dramatic decrease in pain, elimination of pain? Let's say you have a large painful lesion,
they haven't responded to conventional NSAIDs or conventional therapy, do you see any dramatic, or has anybody seem dramatic benefits in terms of pain reduction with low molecular weight heparin administration? (audience member mumbling)
Yeah, I understand. - [Gerald] But with oral (mumbling), the early literature coming up, but I bet you're going to see that's fertile ground to randomize between two groups and look at their visual analogs.
- [Audience Member] Because remember, the pain is the biggest issue. - [Gerald] Absolutely, it's not the lesion. - [Audience Member] But it depend of the cause of the pain. If the pain is really due to local thrombosis, it's very acute pain that will go away with
the low molecular weight heparin. If the pain is due to functional limitation, due to the extension of the VM, it will not help. - [Audience Member] Yeah but we also have patients who just have a painful lesion. It's not necessarily functional.
The thing hurts like hell and the question is, in those patients, obviously we treat them, but in those patients administration of low molecular weight heparin, can that reduce the pain? - [Audience Member] It depend on the biology. If they have very high D-dimer with
normal or normal low fibrinogen, I would think it would help, but if the coagulation is normal, it would not. - [Gerald] Yeah I think (mumbling) good for preventing acute episodes of pain and definitely better for painful episodes.
I agree with you, yes. - [Audience Member] Thank you. - [Audience Member] You've got to ask the patient is it a burning pain or is it a sharp pain? Burning pain is reflexive (mumbling). So you really have to ask (mumbling).
- [Audience Member] Maybe one last comment. We've been using at the beginning, 10 days before like you just said, but we realized that if the fibrinogen is normal, you can start just the day before. It's enough.
If the fibrinogen is low, then we usually send it at least one month before to the hematologist and usually give it until the fibrinogen is normalized before the sclerotherapy which take one to two months. - [Audience Member] Alright.
- [Audience Member] Thank you. Very important topic. - [Audience Member] Yes, very interesting topic and I think we learned a lot.
- I just like the title 'cuz I think we're in chaos anyway. Chaos management theory. Alright, unfortunately I have nothing to disclose, it really upsets me. I wish I had a laundry list to give you. Gettin' checks from everybody, it would be great. Let's start off with this chaos, what has been published.
Again "Ul Haq et al" is a paper from Hopkins. Bleomycin foam treatment of malformations, a promising agent. And they had 20 patients, 21 Bleomycin procedures. (mumbles) sclerosants in a few other patients, 40% complication rate, 30% minor, 10% major.
On a per procedure basis it was a 29% with about 7% major. All patients had decrease in symptoms. But to say "I use Bleomycin" or "I use X" because a complication (mumbles) is nonsense, you're mentally masturbating. It ain't going to be that way, you're going to have complications.
Alright, the use of Bleomycin should be reserved for locations where post-procedure swelling would be dangerous. Well they used it, and one patient required intubation for four days and another patient 15 days. So, it can happen with any agent.
So I don't know why that statement was made. "Hassan et al", noninvasive management of hemangiomas and vascular malformations using Bleomycin again, this handles the plastic surgery a few years ago. 71% effectiveness rate, 29% failure rate,
14% complication rate, 5 major ulcerations. Ulcerations happen with any agent. You're not going to escape that by saying, "Oh, well I'm not going to use alcohol because (mumbles)." No you're going to get it anyway. You all in the literature.
"Sainsbury", intra-lesional Bleomycin injection for vascular birthmarks five year experience again, 2011. 82% effectiveness, 17.3 for failure. Compli- severe blistering, ulcers, swelling, infections, recurrences. Okay, everybody's reporting it.
"Bai et al" sclerotherapy for lymphatic, oral and facial region, 2009. 43% effectiveness, but they found if they used it with surgery they had a higher effectiveness rate. Good. But again that's their effectiveness.
"Young et al", Bleomycin A5 cervico-facial vascular surgery, 2011. 81% effectiveness rate 19% failure for macrocystic. 37% failure from microcystic disease. Complications: ulcerations, hematoma, bleeding, fevers, soft tissue atrophy.
"Zhang et al." Now this is a study. They're goin' head-to-head alcohol versus Bleo. Oh, isn't that a nice thing to do. Huh, funny how that can happen sometimes. There's another paper out of Canada
that doesn't matter, there's 17 pages and there's no statistical significance for that. 138 patients, you got a lot of statistics. "Zhang et al", 138 children. 71 of 75 patients, which is 95% of that serie, were either cured,
markedly effective, or effective, with alcohol. In the Bleo group 41 of 63, that is 65% of the patients, had effective treatment. That means no cures, no markedly effective, just effective. That's their head-to-head comparison. Difference between Ethanol and
the Bleo group again was statistically significant. Ethanol at 75 patients of 14 cases skin necrosis. Bleo group at 63 patients of 5 cases skin necrosis. And in that group they stated it is statistically superior to Bleo. 95 versus 60, that's a big deal.
Again, cured, disappearance post-treatment without recurrence. Markedly effective, meant that greater than 80% was ablated. Effective means about less that 80% reduction but improved. Ineffective, no change. That was their criterion on that paper.
Again, 30 cases, superficial VMs effective rate was 95% in the Ethanol group and the deep group 94%. Okay. What was in the Bleo group? 68% superficial, 56% of deep group. So that's a statistical significance
of failure, between the two agents, comparing head-to-head in anatomic areas. Ethanol VM papers, let's go on to that, we're goin' to do other stuff. "Lee et al", advanced management, 2003, midterm results. 399 procedures in 87 patients,
95% significant or complete ablation, 12.4% complication. "Johnson et al", Kansas. University of Kansas med center, 2002. 100% success rate in tongues. One patient had a massive tongue and had breathing difficulties prior to treatment
remained intubated 5 days and then uneventfully discharged, that was their only complication. "Su et al", ethanol sclerotherapy, face and neck. Again, these are complex anatomies with complex issues of cranial nerves as well as airway control. 2010, 56 of 60 procedures, 90%, four minimal residual,
no skin necrosis, no nerve injuries. "Orlando", outpatient percutaneous treatment, low doses under local anesthesia. This is a very interesting paper out of Brazil. They did 'em under IV sedation, just a little bit by little bit.
They said they had trouble gettin' general so they had to figure another way. Smart, I like people thinkin' things out. Who here doesn't have a problem with anesthesia? Gettin' 'em not to quit before two o'clock? (laughs)
Alright, used local only 39 patients extremity VMs, main symptoms of pain. Cure or significant improvement in 94%. One ulcer, 3 transient paresthesias. "Lee et al", sclerotherapy craniofacial again, 2009. 87 patients, 75% were reductions.
71 of 87 excellent outcomes. One patient transient, tongue decreased sensation. One transient facial nerve palsy, no skin injuries. "Vogelzang" is a very important paper of a single center. Is that author- anybody here? Again, they did VMs and AVMs in this series
and then a per patient complication rate is 13.3, in AMVs 9.7 per patient, but I think what also is important is to do things with regards to procedures. And they listed both. So we'll just, it's about time to quit. This is our embolization series.
And neck, upper extremity, all the anatomies. And we're about a 10 to three ratio with regards to VM/LMs to AVMs in numbers. I think everybody's pretty much like that, a third of their practice. Again, our minor complications are that.
Major complications are these. Summary, what we found in the literature is that Ethanol publications state its efficacy rate routinely at 90 to 100%. And all other second tier sclerosants are 60 to 80%. So I think that's the take home message.
- So I have nothing to disclose. Let me see here, I'm just going to go back here, just so you know what I'm talking about. Low flow venous malformation management, again, sort of a catch all talk, it's going to touch on things that you guys have already probably talked about.
In terms of the clinical work up of these things, we haven't really talked about this, I'm speaking to radiologists, it's obviously very straight forward for vascular surgeons but office consultation, where you assess the patient, be able to describe risks and benefits,
look at their labs, get medical photography, anesthesia, consultation. They're all things that we need to do in advance seeing the patient. And part of that clinical work up is that it's got to be multi-disciplinary.
You've got to get your colleagues involved. Look at the imaging together, and talk to the vascular surgeons, plastic surgeons. We're big on the team of orthopedic oncologists. There are mass ologists at our hospital. And also, of course, medicine.
And do biopsies if necessary. I'm going to just fly through this. Always be worry in older patients who have an atypical history. If it doesn't smell right. Do you get that ultrasound and then go on to more sophisticated imaging.
Have a low threshold to biopsy. I've got no problem biopsying these things. Similarly, if the history is classic, be prepared to find something not classic on MR, and go on to biopsy. And even when you're doing a phlebogram
prior to sclerotherapy, if it don't spell right, get a biopsy. You had two angio sarcomas in the last 20 years, and it just looked like a VM in every way, shape, or form. Yeah, I was blown away. One of them actually had a biopsy before and it was incorrect in another institution,
but angio sarc. So, my general rules of thumb is if it's not classic all the way down, anything, then if it has been presence in childhood, if you're older, at least a few years, get a biopsy. And if it's good, go onto sclero, if not,
well, think about something else. Again, sclerotherapy, this is from some guy who may be in the audience. Basically, you have to kill... well he didn't use that word, but you got to destroy the cell that starts the whole process.
This is from BB Lee, Indications for Sclerotherapy One absolute indication or two relative indications that's what's proposed. I kind of just take the Freudian approach. How does it make you feel? And that's what necessitates us going on.
Scleratheraputic technique is from Dr. DuBois, using looking for cavitary, spongy, or dysmorphic anatomy. That helps direct how we do the sclerotherapy. I like the Phlebographic patterns of types 1, 2, 3, 4. It's a mental exercise where one is no venous drainage that you can really discern. Those are the hemangiomas
sorry for using the term, in the liver kind of almost look like type 1s, whereas 2, 3, and 4 is increasing proclivity to drain rapidly. These are examples of that. Morphology matters because the cavitary, spongy ones are easier to treat. Type 1 or 2 or the lower MR grade,
you get better results. The choice of sclerosant. OK, This is where speaking right after Dr. Yakes. I do have a little variability in mind, but there's no question alcohol works very well. But you got to look at the lesion morphology.
Always you have to think of relative toxicity, viscosity, your previous experience, and proximity to vital structures. And you may consider sometimes more mild agents. This is straight from Dr. Yakes. Ethanol is quite strong, but you shouldn't give more than .1ml per kg,
per 10 minute period. No more than 1ml per kg in a case. The most I've ever given is 1/2 a ml per case per kg. Tromboject, that's what it looks like in Canada. I think was it Trombovar, is what it's called in the states. Or sotradecol, sorry, but it was 3% solution. It's hard to find literature with the maximum is,
It's from KT Tan and Tronal, but sort of .5ml per kg. Bleomycin, we've already kind of talked about it. The long story short is, don't give more than 15 units per case. Don't give more than 90 a lifetime of patient even though the literature, there's maybe 1 or 2 cases
now of pulmonary fibrousus as a result of this. This is our cheat sheet from our hospital basically showing different concoctions the pharmacists preparing for a 15mg dose. You can see the bottom one there. We actually give albumin to make it a little more viscous.
Other proprietary options. We've used Varithena and also basically VenaSeal for closed venous outflow. Nothing in the literature on those things. But just a little talk about Varithena. It's nice because
the foam is actually made out of O2 and CO2. It's not using room air so it's completely sucked up. It has anesthetic properties and it decreases our reliance on anesthesiologists. It's only 1% though. You have to stack a bunch of cases to make it cost effective
The canisters can be used for multiple sessions. The disadvantages though. It's only 1%. Sometimes I get the impression based on the literature that 2, 3, and 4% would work better. Also, the silicon free syringes that you have to use with it, they're kind of rickety. They kind of sputter a bit.
So, they don't have that responsiveness. So, bottom line is I don't have a default sclerosant. I'll use them synergistically. I try to start a little bit conservative sometimes and then ramp up to stronger agents. I know other people may differ.
That's kind of how I do it, and if it's in certain critical locations, sometimes I'll avoid alcohol. So, multi-session therapy. The pressure is often directed to the most appropriate area. The first will direct the sclerosant and then redirect it
as the vessel channels shut down. Often many sites I like to use 23 gauge needles. Different foam concoctions. The important thing to realize is that if you want to be successful, you want to have the highest concentration possible
and for it to stay there as long as possible. So, this is us making our foam with Lopiodol. There's different add mixtures. For a 10cc concoction I'll usually use about 3ccs of the 3% Sodium Tetriacle Sulfate. 1/2 a cc of Lipiodol and the rest air taking it up to 10ccs.
Seems to work for us and it seems pretty stable. Ultrasound is great when you're using these. This is before and after within the lesions. You can see the markedly increased echogenicity. The technique I like to use, if I'm using foam is you take a mask at the beginning and the illucent foam
pushes out the contrast. And then you can also assess the venous drainage and use the displacement technique. Here is an example of that. You can see it's displacing. So, we fill the lesion up, everybody's happy.
And then we follow with the administration of sclerosum, which will turn white, pushing away the darker contrast. So, at least you get that nice confirmation of what's going on. You can use that without foam too right. You can use a neat like ethanol and push it out.
This is using Polidocanol for this particular lesion. Going in under ultrasound, popping into a vascular space, and then administering the sclerosant using the displacement technique. You can see it filling that area and you can see on an ultrasound it's becoming
increasingly echogenic in that area. Sometimes we use a little Lipiodol. I like to consider it like a tracer bullet and tells me where I'm going. You can see here on this lesion here, you can see, this is sped up.
It looks like an AVM but it's not. But you can see the little bit of Lipiodol mixed in. That's with Ethanol actually, that one. Here's also another Lipiodol Ethanol add mixture. An interocitous venous malformation in the sacrum again sped up. It looks like an AVM but it's
a veinous malformation and that's what it looked like afterwards. And the Lipiodol eventually goes away. And use gravity. If you're getting into a lesion, get into the bottom of it if you're using a foam. Cause foam rises.
If you get superficial you won't be able to see anything. So, off it goes, and I think that is it. Followup. Just conservative analgesia. I use Ketorolac if I have an anesthetic case compression. Low Molecular Weight Heparin. I like to do sclerotherapy once a month.
And groups of three and then an MR greater than 6 months. And I think we'll stop there cause those are the things that have already been talked about. Thank you very much. - Beautiful. - Questions? Just one quick question.
Gerald, this keeps going around and around, we talking about this for years. Tell us why for example, you would not use Ethanol in the lesions you showed and why you think the profile of the agent you did chose is superior. - OK. That's a really good question.
One of the things is that I've got a lot of patients and a lot of patients I have, some of the lesions are not that huge and they're just barely into the threshold. So, I'll use a more mild agent just because I think that in the lower doses
the complication profile is lower. That being said, some of my worst complications have been with sodium tetradycal sulfate. We've talked about this before. I don't know. I'm kind of influid on this still.
I, no pun intended. Hi Dillan. I vascilate. I have Ethanol periods and I have foam periods. - Great. Let's move on. - I have one quick question if you allow me.
I mean missing the diagnosis of sarcoma is a crucial issue and what would you recommend in case there's a lesion and you have a negative biopsy start treatment. Would you repeat biopsy during treatment? - Usually for mytotically active lesions, the biopsies are all right.
I just had that one case where a biopsy at a small hospital is not as used to those kind of things. It was negative, but a biopsy, a good biopsy, like a core. None of these little fine needle things. Yeah, 18 gauge core, and you know what, They've got these beautiful sets,
like these hunter sets where you can put a little collagen pledge if you want to do it. The other services too, with their asked to do the biopsy they get squeamish with a venous malformation. I always say to them, what do you think I'm going to do to the lesion?
Right, so, yeah. - I think the most important thing to some of these if there's a change that's noticed. 19 year old boy came to me at this body venous malformation. Treated for years, came in when you came.
I never saw him before. I said let me see your pictures. They were different. - Right. - Half the body of angio sarcoma died within 20 years. They had changed. I had a patient with an ankle venous malformation. I noticed a change. Biopsy.
Angio sarcoma. I have one other case of MR. I think veinous malformation, whatever. If something's funny, get an ultrasound. Solve it. You know fibroma. AVM in the forearm. Angio, looks like AVM to me. MR.
There's something funny. Biopsy. Malignant fibrocystic sarcoma. So anytime, like you said, _____ put a needle in it. An 18 gauge doesn't hurt anybody. - I think that's very important. - Couple times too,
I've been ready to do a therapy a patient's asleep I might, you know what. The flow is too high on this thing. It doesn't look like an AVM. Somethings right, do a biopsy and say guess what this is a biopsy under anesthesia case today. So be it.
- Thank you very much.
- Alright, we'll talk about some crazy stuff and see, again, nothing to disclose really upsets me. Wish I had a laundry list. All kinds of checks coming in. Alright, let's talk about venous and lymphatic malformations in some complex areas, and see what's possible. Again, occur anywhere, connected to veins, discontinuous.
We talked a lot about this. MR, we had great talks on that, with Dr Reznik. I like what Dr Reid stated back in the archives of surgery in 1925. "In view of the common development on each side of the vascular tree,
and in view of the enormous constructive and destructive changes necessary before the final pattern of the vascular tree is reached, it is a marvel not that abnormal congenital communications occasionally, or even rarely, occur. But that they don't occur more often.
So it means it's a pretty damn good mechanism, but nothing's a 100%. Go figure, isn't that life? Okay, endothelial cell theory. I think we've gone through this before, but I think that's the,
there's been some papers and statements now in the New England Journal, supporting my thesis on this. That it's endothelial cell that's the culprit. Whenever it sees thrombosis, there's no blood flow, it has an ischemic state, 'cause there's no oxygen transfer now,
and it wants to fix the situation. It does it by then sending out angiogenesis factor, then cause neovascular stimulation, and chemotactic cellular factor that causes macrophage infiltration to carry out debris, and that's called recanalization.
So, that's the two mechanisms by endothelial cell mediated by them, and here they are there. Action of ethanol, well it destroys those endothelial cells, we had a great doctor out of Dallas that did a bunch of kidneys with alcohol before they resected, and he did hundreds of histologic evaluations,
and that's what they showed. Destruction of the endothelial cell, denuding of the vascular wall, and fractures to the level of the internal elastic lamina, and then platelet aggregation on that denuded wall until thrombosis.
And hundreds and hundreds of cases of that were pulled out, Brian Ellman of Dallas, Texas had all those papers back late 70's early 80's. So it was known for a long time the action of ethanol on a vascular structure. So yet again we went through this.
Head and neck malformations again is a complex anatomy and you also have issues with regards to the airway. Frequently in the head and neck they can be focal, or they can be diffusely involving, it can involve both sides of the face, palate, tongue, pharyngeal area, submandibular area,
anterior neck, they can go anywhere. Parotid gland, you name it. Here's a 19 year old male with testicular involvement, and he was having bleeding and infections, as the skin breaks down you can see the verrucous lesions there,
and after seven treatments no bleeding occurred and the skin is normalized, because it wants to heal. It's the malformation itself that's preventing healing. Here's a lady, a 20 year old with vulval, labial, and vaginal venous malformations. Her OB/GYN physician said never have a kid.
An episiotomy or a tear at birth and you could exsanguinate. She had several children and here's the lesion, after eight treatments completely gone, and she's a mother of kids now. No problem. Now here's another crazy area, bone.
Intraosseous venous malformation, look at the multiple cystic spaces seen on MR. You have expansion of the bone, and you have thinning of the cortex, this child was crawling and even did not want to do that. This is a three-year-old not
walking yet because of the pain, it went down to the epiphysis and stopped, but the micro-fractures it was causing, and by the periosteum around it was very sensitive, and many nerves, and it was painful. So they had a crawling kid at aged three.
You can see the expansion of bone on multiple cystic levels of the venous malformation, all the way down to the epiphysis, Here was the direct puncture just like any other cystic venous malformation is it not? And here it is three years later,
that's aged six now, and you have complete marrow throughout that area, the cortex is thickened and the bone is normalized, it's not expansile anymore it wants to heal, but the malformation prevents it from doing that. Walking and running with the kids at school now,
so that was real good. Okay, cystic and microcystic forms of lymphatic malformation here's one on the tongue. You can see the median raphe pushed over to the left, expansile lesions you see the clear and bloody lesions not infrequently venous and lymphatic
malformations co-exist together. Here you can see in the tongue the bright stuff, it's full of that stuff, large large lesion. Here's what they look like on arteriography on the lymphatic forms, they have the lymphatic involvement. They have this spiculated pattern very commonly,
and also on the diagnostic venous study, here is again that spiculated arterial pr-oo-ng tree kind of look. Here's a direct puncture again that's cystic spaces but these microvenular spaces. This is what they look like
and after tree-thrombose. Before. After... Thrombose. Then you go from this after treatment. To this, it's not completely done yet. There's still some mass effect.
To normalcy of the mucosa. It wants to heal, but it just cannot with the malformation. And here's what the (mumbles) I'm sorry, but the MR shows a complete wipe out. Here's a lymphatic malformation involving bowel. The child was loosing weight.
You can see there's not much fat going on in this kid. And he was, every time he ate he had intestinal angina and it was very painful. And you can see all that bright stuff was wrapped around the mesentery, all the way up. Here's a AP view.
Deep to the transverse colon. Here's another view, showing it wrapped around the aorta. And another view showing that and... And here's what it looks like with the microcystic spaces by direct puncture. And it goes from this after treatments,
to this. From this, to that. This, to this. The kids playing with the kids, gaining weight and doing well. Lest we forget, we're going to have complications with these crazy things.
It's not just a bunch, a pool of stuff and nothing can happen. Here's a 4 month old that presented to us. Was with us for about two and a half years from Kuwait, and she had everywhere in the head and neck. You name it, it was there.
This is not at all anything you can resect. There's the kid, that ear pushed up. Trache in place since birth. Massive (mumbles) head and neck, pharyngeal, tongue, submandibular, you name it, it's there.
Look at that tongue and submandibular area, it's everywhere. It's a nightmare. So, after some treatments, this is about, 17 months of age now. And the patient had a fever and a tense, left side of the face.
Did an MR. We got an abscess. Abscess. Drainage. Sucked it all out, that much and then we irrigate till clear. It goes from this, abscess. No fever, afebrile, and of course antibiotics on top of it.
But it went right down... Without a surgical debridement. It was just a needle and sucking it out, and irrigating it to a clear. And here we go here. A massive thing. To that after the abscess.
To this after further treatment. From this. To that. This. To that. This. To that. Then there's a little bit left
around the base of the carotid. So we went ahead and you can see that on the axil there with the carotid behind it. We treated that and it went away. And there she goes, is at four months. 30 months.
Four and a half years. This is endovascular lone. You got to keep and eye out for this patient. Here it is into her orbital. You're facing the globe. Here's what it looks like with the
microvenular venous spaces on this patient. They could be saccular, they could be either. Here it is after treatment. Eyes back in the head. You've got a little bit of a redundant optic nerve. Had no visual changes, I was surprised when I evaluated him.
We hadn't (mumbles) it was normal vision, even with that proptotic eye. So we had to know we had to preserve vision. And it goes from this. To that. Again, this is a quick one. This is a case where the wheelchair bound, 21 year old,
University of Texas of Austin student. Her father was an ER doc searching around. How do we fix this. How do we fix my daughter. She had massive pulmonary hypertension at aged 21, who was wheelchair bound, couldn't take three steps. She has congenital absence of her
left subclavian and axillary vein. And this massive chest thing that was causing thrombi formation and embolization into the rudimentary innominate stump. The only way out. And was having PE for 21 years,
'till she finally fried her lungs and was at end stage. When I suggested a treatment plan, when we did a study, her pulmonary artery pressures were 126. I mean that's systemic. She's near death.
Just sitting in her chair. So we went ahead and proceeded and the plan was first, to reconstruct and give her a subclavian vein system. Therefore it excludes it from the malformation, and prevents further emboli. Then treatment of that lesion,
and then doing pulmonary embolectomy. Now, the lesions been treated. It can move forward with that to treat the malformation. And then treat her lungs with the pulmonary embolectomy. So that was the plan. Here's that rudimentary stump of an innominate
that all the emboli were going into. So we had to go with a three man team. Two interventionalists and vascular surgeon. We worked like dogs, and a lot of the (mumbles) wasn't made for this. But we had to dig down deep,
and push and stuff into the groin and get it up higher. He had a fun time down there. And here we are going across. Snaring, getting the measurement wire. Getting across, putting our first stent Viabahn. I had to talk with cross-ee,
and I talked with material science people. 'Cos I don't have the PHD in that. 'Cos this 21 year old had to keep this for 60, 70 years. Don't you think? What materials might last that long? So that's what we were thinking.
Cross is a master of putting in endographs and all of that so he was important to talk to on this. We decided on the Viabahn covered stent, versus bare stents or other types. And we overlapped them, so when you do AFB. You've got large to small.
We had to think reverse, small to big. To the innominate. So we started putting the stents in. Bigger and bigger overlapping. Then we put the ku-ger-cloo-ter in at the end. That was 20 mm at the innominate and 16 at the stent. Then we reconstructed the whole thing.
Then treated the malformation, we sent the patient to Cleveland Clinic. There's only a couple of places that do pulmonary embolectomy in the US and that's San Diego and Cleveland Clinic and they went there.
Had it done. Here you can see the stent and the CT. Now her pressures are 22 in her lungs. She's back to living a normal life. And she's about four years out with this. I had my surgeon also put in a fistula,
to increase flow through this. 'Cos we all know stuff through a vein thrombosis goes away. But if we put a fistula to create a high-flow state, it may reendothelize and stay open. And it has. So, she's gone back to her normal life and... There it is. Alright. Thank you
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