Scott Webb (Host): Properly diagnosing and treating patients with vascular malformations requires a level of expertise and experience that very few people possess, but I'm honored today to be joined by one of them. Dr. Naiem Nassiri is here today to discuss vascular malformations. He's a vascular and endovascular surgeon and Director of the Vascular Anomalies and Malformations Program with the Vascular care Group.

Scott Webb (Host): Welcome to Answers in Vascular with the Vascular Care Group. I'm Scott Webb. Doctor, thanks so much for your time today. We're going to talk about vascular malformations and what that means exactly. So, let's start there. What exactly are vascular malformations and what separates them from other similar-sounding conditions, such as hemangiomas, maybe strawberry birthmarks, that kind of thing.

Dr. Naiem Nassiri: Vascular malformations are unique entities, and they have a broad clinical spectrum ranging from lesions that are cosmetically disfiguring to potentially life-threatening issues. And these are distinct in other ways as well, in that they represent errors in the development of the blood vessel and they are congenital, which means that the patient is born with these deformities and these errors in development. Unlike, for example, hemangiomas, which is a commonly used term and also a commonly misused term, vascular malformations are actually not tumors, right? Hemangiomas represent benign tumors, benign for the most part. There are rare aggressive hemangiomas as well that are not so benign. But for the most part, hemangiomas are benign tumors of the endothelial cells, which are the linings of the blood vessels, whereas vascular malformations are not tumors at all, but rather represent errors in the development of the blood vessel.

And so by virtue of that developmental error, the blood vessel forms in all kinds of shapes and sizes and leads to all different types of deformities and disfiguring problems. And obviously, we know that there are different types of vessels that are present in our body. These include arteries, veins, lymphatic channels, right? So depending on which type of vessel is affected, then you can have a different type of vascular malformation. And sometimes vascular malformations can happen in a combined fashion, so representing more than one type of blood vessel being affected. But the major distinction, and this is crucial to understand, is that vascular malformations are not tumors and hemangiomas are.

Host : Yeah. Not tumors. Why is proper nomenclature and diagnostic terminology so important in this particular field?

Dr. Naiem Nassiri: That's a very good question. This field has been fraught with errors in terminology. It's a very intimidating set of problems and diseases at first glance. So, it does require an expert eye, a multi-faceted eye and a very experienced eye to be able to decipher and distinguish and kind of work one's way through the chaotic landscape. And so historically, because these malformations affect various locations in the body, different body parts, there has been a whole slew of various physicians caring for these patients, various specialties. And so, there has been inconsistencies and lack of communication among specialties.

Furthermore, what appears to be resembling a hemangioma, right? Even though these are all blood vessel issues, right? So, if it looks like it's representing one thing or another, then the term hemangioma or derivatives thereof get thrown around. And our wards are very powerful as human beings. They carry a lot of weight. When we call something or designated as something and we say that this is what it is, well then that sets the expectation, the prognosis, the therapeutic options, the diagnostic modalities, that sets it in stone in some ways for the patient. So, if somebody may have a relatively inconspicuous, but dangerous, nevertheless, vascular malformation, when they go to someone and they say, "Well, this is a hemangioma. Don't worry about it, we're just going to watch it," that patient has certain expectations. And over time, when there are problems that arise as a result of the malformation that has been left untreated, then that patient is left essentially to their own device to figure out what's going on. And oftentimes, issues pertaining to the malformations are attributed to something else. And in fact, there have been papers that have been written about this of how crucial it is that we designate these vascular anomalies specifically and appropriately, and allow the patient to have the right pathway towards diagnosis and treatment and surveillance going forward. So, I am probably one of the biggest, if not the biggest, advocates for using proper terminology. Don't call something a venous malformation when it's an arteriovenous malformation. Don't call something a hemangioma when it's a lymphatic malformation, for example, because that is a completely different path that one takes. So, we call something, then we go down that rabbit hole, if you will, of diagnosis and treatment.

Host : Well, and as you said, doctor, hemangiomas are tumors and vascular malformations or not. So even for a lay person like myself, I can understand sort of misdiagnosing or misunderstanding or just classifying something as being something that it's not, you can see how the treatment path, the therapeutic path, all of that would be off or would be wrong, right?

Dr. Naiem Nassiri: Correct, 100%.

Host : Yeah. I'm just wondering, is there such a thing as an overarching roadmap for classification and categorization of vascular malformations that maybe could help simplify navigation of these lesions, you know, using the proper nomenclature?

Dr. Naiem Nassiri: Yes. This actually was accomplished thanks to the pioneering work of a renowned doctor named Dr. John Mulliken. He was a plastic surgeon in Boston. And he really was the first to be able to distinguish between hemangiomas and malformations and say that, well, the former are tumors, whereas the latter are errors in development of the blood vessel.

And so, based on that pioneering work, a group of people eventually got together and formed what's called ISSVA, that's I-S-S-V-A, and stands for the International Society for the Study of Vascular Anomalies. And I actually have sat on the ISSVA ad hoc committee for classification of malformations. And the ISSVA classification scheme is primarily focused on that initial broad distinction, separating tumors from vascular malformations and then breaking down vascular malformations based on their flow properties, right? The hemodynamics, as we like to call it, right? Because depending on what type of channel or blood vessel that's involved, the flow can be different.

So, there are, simply stated, low-flow vascular malformations, and then there are high-flow vascular malformations, which is fairly easy to remember. The low flows are then broken down into venous and lymphatic malformations or combinations thereof. This includes the classic port wine stain that one sees, and then the high flow malformations are broken down into arteriovenous malformations, and these can have various syndromes associated with it, but that's the major overarching breakdown using the ISSVA classification based on Dr. Mulliken's work.

Host : Sure. I'm going to ask you a question here and I'm sure that you are one of these people, maybe the top person in fact. But what does a typical thorough and adequate diagnostic workup of a vascular malformation look like and who's in the ideal position to order, interpret, and oversee those tests, besides yourself, of course?

Dr. Naiem Nassiri: Yeah, of course. I think, you know, this is truly a multidisciplinary effort, you know, one man or woman cannot do everything all at once. But that being said, whoever it is that's caring for these patients really needs to have dedicated their career to vascular malformations. In other words, a major chunk, if not all of their career or their practice, needs to be dedicated to these. Because without that level of commitment, just kind of dabbling in malformations really, it's too complex of a field and too complicated of a disease process and too diverse of a disease process affecting both pediatric and adult patients that just kind of episodically and leisurely looking at it once in a while, just will not be able to provide the patient the proper care. And I see this often, you have vascular experts or other surgical experts who are very good at what they do, but they really don't focus on malformations, and they'll treat a patient and it winds up just being kind of a temporary Band-Aid on one disease process that really needs a more comprehensive workup.

So, I would say, regardless of specialty, obviously, a very strong understanding of vascular biology and vascular interventions is required. And I would say that, in my own field of Vascular Surgery, there are not too many vascular surgeons who are at this level of expertise. I happen to be an anomaly in that. One's career really needs to be focused on it. I would say, broadly speaking, obviously, one would need to come in with a very broad understanding so that, you know, by merely questioning the patient, taking a history, and doing a physical examination, one should be able to narrow their diagnosis or their differential diagnosis to certain parameters. And then based on that, one can then go ahead and use proper diagnostic imaging modalities should it be indicated, and the mainstay of those is going to be duplex ultrasonography, and then oftentimes contrast-enhanced MRI. But very in-depth knowledge of these imaging modalities and the ability to be able to interpret those studies is also required.

And furthermore, because these are blood vessels and blood tends to flow through them, obviously, there can be hematologic derangements that are often missed, meaning that the body recognizes a wrong blood vessel or an erroneous blood vessel and it wants to shut it down. So, it utilizes all of its own clotting factors to shut down this unwanted blood vessel. Well, as a result, you can deplete yourself of all your important clotting factors and you can become prone to bleeding. This is a phenomenon called localized intravascular coagulopathy that one needs to screen for you. And then, we have enough medical expertise to be able to treat the patient and the treatment often relies on blood thinners. So, that's the sort of the workup.

Now, the therapeutic part of it obviously when we get into surgery and intervention and other treatments, obviously one would need to have both the medical and the surgical and the interventional expertise to be able to offer the patient a vast array of therapeutic options depending on what the patient needs idiosyncratically and not just being the old adage of, "I'm a hammer, everything is a nail, right?" Just because I'm a, for example, plastic surgeon, I'm not-- I'm a vascular surgeon, but let's just say I'm a plastic surgeon. I see a lesion, I'm going to go ahead and take it out. Whereas you go see a laser specialist and say, "Well, I'm going to laser it, right?" It shouldn't work that way. The proper most effective thing should be offered to the patient and not be limited by the doctor's expertise and capabilities.

Host : Well, as you say, you are an anomaly. And I mean that in the best possible way, of course, especially as we're talking about vascular malformations and you mentioned. And you mentioned mainstay of treatment. Just wondering, is there such a thing as a mainstay of treatment? And if so, what are they basically, or what is it? And if not, why isn't there?

Dr. Naiem Nassiri: Yeah. I would say, again, I told you, I am a surgeon. I'd like to make incisions and cut and excise. I enjoy that. But I also do all my own interventions. I have a very high level of expertise and skill in performing minimally invasive endovascular procedures and I also medically manage the patient. In other words, all these parameters I do myself. Obviously, if it comes down to, for example, a lesion that requires some cosmetically important sort of excision or reorientation, I would consult, for example, colleagues in plastic surgery or even Otolaryngology. If there is, for example, issues involving the heart, I may involve colleagues in Cardiac Surgery. If there are joints that are involved, I would get input from colleagues in Orthopedics. But as far as the actual malformations are concerned, I tend to tailor myself and my skill set and my expertise to what the patient needs.

I would say as a multifaceted doctor taking care of these patients, the mainstay of treatment for the vast majority of vascular malformations is going to be these minimally invasive catheter-based procedures or endovascular procedures that provide what we call embolization or embolotherapeutics, meaning we use a variety of different agents that are available to us and deliver them with a high level of skill set directly to the site where the malformation is. And we deliver and deploy these embolotherapeutic agents so as to provide closure or clotting of the unwanted blood vessels. And this may often require several sessions of treatment that you would plan out with the patient going forward. But I would say this is the mainstay.

Now some may disagree with me, who tend to be, for example, surgeons, right? They like to go in and take these things out. But as someone who does the surgeries himself and has seen patients undergo surgery, I know that surgical trauma in and of itself can be fertilizer for reformation of these malformations. They can lead to proliferation. So now, you have scar tissue from surgery, now the malformation has come back. I tend to reserve surgery for situations where I have provided adequate embolization. And then, I go ahead and excise the lesion to provide the best possible functionality and cosmesis for the patient. And that goes for many of the other therapeutic options that are available.

Now, this is not to say that one patient may not be an appropriate candidate for surgery. And if that's the case, certainly I would offer that. But I would say by and large the mainstay of treatment for vascular malformations is minimally invasive endovascular embolization.

Host : Okay. This has been so educational. I mentioned earlier that I'm just a lay person trying to hang on, you know, and trying to learn from you today. This has been really good stuff. I feel like we've just kind of scratched the surface. But as we finish up here today, doctor, anyway, what's new on the horizon therapeutically?

Dr. Naiem Nassiri: Yeah, there's some very exciting events that are happening. I am fortunate enough to sort of be on the forefront of this. I actually have a very good relationship with the United States Food and Drug Administration in terms of coming up with some-- if you remember earlier, I said, minimally invasively. Now, we're sort of going forward and we're talking about non-invasive extracorporeal means of treatment.

So, one very exciting clinical trial that I have coming up that we will be ready to roll out, this is the first in the world, in-human use of what we call therapeutic ultrasound. So, everybody knows about ultrasound, how it's used for imaging. Well, now we can use ultrasound beams and focus them in a very specific target area and use these ultrasound beams to actually, for lack of a better phrase, zap unwanted vascular structures. And so, this has tremendous promise in becoming really the first non-invasive modality for ablation or elimination of vascular malformations without anything entering the body. No needle, no catheter, no surgery, no blades, no sutures, nothing. Everything is just completely outside the body. And it really reduces the need for anesthetic as well as a relatively pain-free operation. So, that's going to be rolling out for us very soon over the next couple of months. This is in collaboration with the United States Food and Drug Administration. We're very excited about that.

And then, on a parallel front, because one needs to be multifaceted, I actually have founded a company based on a patented technique of delivering specific medications that we know actually counteract the very formation of the vascular malformation. So whatever the gene mutation was that led to the formation of the vascular malformation, well, it turns out that we have medications that can counteract that. But as opposed to giving this medication by mouth, we can actually deliver the medication via very sophisticated means directly into the malformation right where the disease is, and avoid exposing the other parts of the body to the malformation. In other words, we do away with the side effects of medication. So, this is just kind of a tip of the iceberg of some of the most exciting things that are happening in this field. And, again, I feel very fortunate to be at the forefront of this.

Host : It's really amazing. I've found myself shaking my head today, listening to you thinking, "Well, no, that can't be right. You can't be doing that," but you are in fact. And I really appreciate you dumbing it down for me and using the word zap.

Dr. Naiem Nassiri: Yeah, of course.

Host : Some of the words I've heard today that I wasn't familiar with, I am familiar with zap and I really understand. You know, even as a lay person can understand the benefits of minimally invasive, it's great. And that's kind of like the buzz thing in medicine right now, but non-invasive, zapping these vascular malformations, it's just really amazing. Appreciate your time, your expertise today. Thanks so much.

Dr. Naiem Nassiri: Of course. Thank you. Thanks for having me.

Host : That's Dr. Naiem Nassiri, vascular and endovascular surgeon and Director of the Vascular Anomalies and Malformations Program with the Vascular Care Group. And for more information, please visit our website at thevascularcaregroup.com. Thank you so much for listening to Answers in Vascular with the Vascular Care Group. I'm Scott Webb. Thanks for joining us.