Melanie Cole, MS: Welcome to UAB MedCast. I'm Melanie Cole. And joining me today is Dr. Jesse Jones. He's an Assistant Professor and an interventional neuroradiologist at UAB Medicine and Dr. Anthony Morlandt. He's a head and neck surgeon in Head and Neck Surgical Oncology, Oral and Maxillofacial Surgery, and he's an Associate Professor at UAB Medicine. And they're here to highlight vascular malformations of the head and neck.

Doctors, thank you so much for being with us today. And Dr. Morlandt, I'd like to start with you. If you'd start by giving us the general classifications used to describe vascular malformations of the head and neck, what are we talking about here today?

Dr. Anthony Morlandt: Well, that's great. Thank you, Melanie, for having us. This is an important topic. It's an often confused topic. I think the classification has changed quite a bit over the years, and at least when most of us were in training, we talked about two general classifications. We talked about hemangiomas, which had sort of increased cell turnover and increased numbers of cells; and then malformations, which had abnormal cell growth, but the rates of the cell turnover was not increased, so normal cell turnover. That led to quite a bit of confusion because the treatment really didn't depend on the rate of cell turnover. That's something that really we would apply to a neoplasm like a cancer.

But in this case, the more common phraseology really divides these into two basic groups, vascular tumors and vascular malformations. And under the general classification of vascular tumors, you might find some of these congenital hemangiomas that occur in children. But really, what we're here to talk about today are vascular anomalies that can be classified primarily as either high-flow or low-flow. And that's really important, because clinically a high-flow lesion may have more of an arterial component. It may have an arteriovenous fistula and rapid expansion and, in the head and neck, and especially in the face, these have significant cosmetic and functional issues. So, it takes a multidisciplinary team. It takes a head and neck surgery unit. And an endovascular neurosurgeon, like Dr. Jones, to treat these. It really takes two different sides from both a chemotherapeutic and embolization perspective and a surgical perspective to take care of these complex problems.

Melanie Cole, MS: Thank you so much, Dr. Morlandt. And so Dr. Jones, Dr. Morlandt just mentioned a bit, but why are these such a challenge to head and neck physicians and the complexity in diagnosing and then therefore managing these. Speak a little bit about some of the challenges.

Jesse Jones: Well, I think part of it comes down to the characteristic of the lesions, they're variable. I think one of the problems is just the sheer variety in these lesions. As Dr. Morelandt mentioned, there's various classifications, but they can come on all sorts of colors and shapes and sizes with high-flow lesions, slow-flow lesions, lesions that have a primarily venous component versus those that have an arterial component, and the treatments really differ markedly. And I think there's quite a lot of complexity in there and it really takes a multidisciplinary team to kind of get to the bottom of what's going on and to propose a treatment plan that's most appropriate.

Melanie Cole, MS: And we're going to get into that treatment plan, but Dr. Jones, sticking with you for a second. Tell us about some of the advances in radiologic imaging that have really augmented your diagnostic and therapeutic capabilities for these. Are there any that have changed the landscape of treatment for you?

Jesse Jones: Well, I think MRI has made a huge difference in diagnosing the kind of malformation and the extent of the malformation. Some of these lesions can be quite trans-spatial, meaning they cover multiple different spaces of the head or neck. And those can be difficult to appreciate on just physical exam or even ultrasound. And MRI does a beautiful job of delineating the borders of these lesions and also within the lesion itself, depicting areas of high-flow, low-flow and lesions that have both, showing where those components may be within a larger complex lesion. So we like to get an MRI at UAB whenever possible, when we're first evaluating these patients to get a good sense of the complexity and the full extent of their lesions.

Melanie Cole, MS: Dr. Morlandt, speak about special considerations for treatment of these lesions that must be made. And due to the sensitivity of the area, as you mentioned at the beginning and the intricate nature of this type of situation, speak about the treatment options and what you're doing at UAB.

Dr. Anthony Morlandt: I think when we evaluate a patient with an anteriovenous malformation or an anomaly of the face, vascular anomaly of the face, it's helpful to divide the face of into thirds. The upper third, which primarily is the forehead; the midface, from the lower portion of the orbits, the infraorbital rim to include the upper jaw and the upper lip, the nasal complex, the malar complexes, including the cheeks; and then the lower third of the face, which is from the philtrum or the subnasal region, down to the bottom of the mentum or the chin.

And so within each of those areas, we have sort of a special set of organs. We have the eyes in the upper third, we have the nose in the middle third, and we have the mouth in the lower third. So when evaluating these, it helps to have both a soft tissue appreciation. And in the soft tissue category, we would include placing incisions along natural skin creases, along resting skin tension lines to minimize their appearance postoperatively. We want to make sure that we always hide the incisions in a way that's the most cosmetically optimal. so we have the soft tissue, we have the underlying bone, and then we have these sort of special areas of eyes, nose, and mouth. And so we treat a lot of these that are around the orbit, for example, where we have to maintain vision. And so we're trying to preserve the upper and lower eyelid function. We're trying to work with Dr. Jones's team to make sure that he, in treating this with embolization, doesn't sacrifice or cause damage to the vision. And then surgically, for example, in an orbital or peri-orbital lesion, we're trying to minimize the risk of disrupting that entire apparatus, all of that orbital septum and tarsal system that supports the eye and supports the eyelids. The mouth is important too. We're trying to make sure that the tongue and the teeth and the lips are not adversely affected when we're removing these.

I think if we step back from an even higher view though, we're also very concerned when the skin is involved. There's a Schobinger classification of high-flow arteriovenous malformations. And Jesse, correct me if I'm wrong, but you know, if we have a lesion that involves the skin with ulceration, we're really thinking of a totally different treatment plan than if we have one that doesn't yet involve the skin, and we're trying to avoid that. So we don't want embolization to cause skin necrosis. We don't want surgery to leave an external defect. So some of these more advanced lesions, we're really considering similar to how we treat a head and neck cancer or a cosmetic surgery patient. We follow all the same principles employed in facelifts and fillers and facial suspension and everything that we employ in our head and neck cancer population to give them the best outcome possible.

Melanie Cole, MS: Dr. Jones, would you like to chime in here and add anything to what Dr. Morelandt just said?

Jesse Jones: Yeah. Speaking to the purpose of preserving these various functions and the head and neck is a highly functional region for multiple reasons. It has a very important psychosocial dimension. It's what we see when we look at each other in the face. And we breathe, we eat, all these things occur through the head and neck. And so when we're trying to preserve these various functions, it does take an interdisciplinary approach. So for instance, if Dr. Morelandt is planning on resecting a high-flow lesion around the mouth or the nose, that's where my team would come in, to prepare the patient for his surgery. And by preparing, that would be a procedure called embolization, where we would go in there and slow down the blood flow to these various structures. So when it's time for surgery, there's less blood loss during surgery, there's better visibility during surgery and the overall result is superior.

Melanie Cole, MS: And Dr. Morlandt, as we've spoken about and mentioned a few times, the multidisciplinary and interdisciplinary approach, how do facial surgeons and endovascular neurosurgeons work together to really optimize patient care and outcomes? Now, you two represent two specialties that are focused on treating these kinds of malformations. So I'd like you to tell us about your combined clinic, why it's relevant and what you're finding are the largest benefits.

Dr. Anthony Morlandt: I think one of the most important things that having an endovascular approach does for the surgeon is by going in prior and using agents to control bleeding and minimize blood loss during surgery, we can do a much better job preserving vital structures. And so it's pretty common in the face to have a vascular anomaly that's supplied, at least in part, by the facial artery as an example. And the facial artery, if you palpate your own facial artery, which is at the antegonial notch of your mandible, right in front of the angle of your mandible, by sort of the back of your jaw, then you could feel how that artery courses along the face and that's intimately associated with the branches of the facial nerve.

So there are several cases we've treated together where Dr. Jones has gone in prior, embolized the feeder vessels for a high-flow lesion in this area. And then, when I take this patient to surgery and make a facelift incision and use a parotidectomy approach, it's a much drier field. I can identify and preserve the branches of the facial nerve. And we can have a much better outcome that both is important from a pathology standpoint, not oncologic perspective because these aren't cancers, but it's important from a tumor control and vascular malformation clearance perspective, because we really have to clear all of the components of these lesions. So they don't recur. So it's important, not only on that end, but also from a cosmetic and a functional outcome as well. So having a nice dry field is key, it's paramount, and it helps us do a really good job. And it also helps us be able to know what reaction the skin will have in surgery, keeping in mind that many times the arteriovenous malformation, since we're speaking of those here, they may provide some of the blood supply to the underlying skin. And so in resecting an underlying deeper vascular lesion, we may compromise the blood supply to the skin surgically. And that's a huge problem for overlying skin on the face.

So by having embolization upfront, it allows me to really predict whether that skin will necrose, and have a external scarring. Maybe I need to go ahead and excise some ischemic or necrotic skin and rotate local flaps into position to provide the best result. So it really is a nice and sophisticated way to do it. And it's what would be considered the standard of care in the Western world at least.

Melanie Cole, MS: Dr. Jones, do you have any final thoughts for other providers on when you feel it's important that they refer to the specialists at UAB Medicine?

Jesse Jones: Well, I think when these lesions are first discovered, there's kind of a decision tree at that point. a lot of these are purely asymptomatic or incidental and for those lesions, we do not propose or suggest treatment. It's when these lesions become bothersome to the patient. And that can be for a number of reasons. It could be something functional, like it's interfering with their ability to swallow or to breathe, especially at night with things like sleep apnea, or it may be a psychosocial component. The lesion is disfiguring to them or their child. And when those situations arise, I think treatment is warranted and that's probably the time you want to reach out to us at UAB, so we can do those things like we talked about earlier, do the MRI get together in a multidisciplinary fashion, start talking about treatment options.

Melanie Cole, MS: This is such an interesting topic and you're such a great guest, Dr. Morlandt. Can you please have the last word here, as we're talking about these types of malformations, and we've mentioned briefly the effects that they can have. Dr. Jones mentioned the effects on appearance and function of the patients, and they can be pretty disabling and socially isolating, really have a significant impact on quality of life. So I'd like you to end with your best information for other providers about that particular aspect of this and how really, because of the intricate, sensitive nature of this, that the experts at UAB Medicine are uniquely qualified to deal with these malformations.

Dr. Anthony Morlandt: Well, I think the best way to explain it is by outlining a case. And there's a particularly memorable case I have of a young woman who was pregnant and in the course of her pregnancy with all of the vasodilatation, the growth factors, the increased blood volume, had quite a massive bleeding, arteriovenous malformation involving her orbit, her upper cheek, her nose and her lip. And not only was she dealing with bleeding and blood loss and admitted to the high-risk maternal-fetal medicine unit. She had other kids at home who were terrified by this massive facial tumor. And this is a patient who doesn't have cancer, who's not going to get radiation or chemotherapy. This is a patient who has a non-metastasizing, clinically benign, but certainly not safe or unimportant tumor. And so, we have many aspects of the patient's safety, their health and wellbeing to be concerned with, especially in patients who have high risk syndromes, like HHT. And UAB is now working to become an HHT Center of Excellence for patients and to be a resource for physicians all across the Southeast for this condition. HHT stands for hereditary hemorrhagic telangiectasia, which is a congenital syndrome associated with some of these high-risk arteriovenous malformations.

So we have the risk of bleeding, obviously is paramount. We have facial disfigurement. We have the fact that it's not a cancer, but really in a sense, these patients are going through some of the exact same treatments that a cancer patient would. And then we have the permanent components the permanent scarring and sometimes even disfigurement when these are treated without the support of a large center. And so it's important, I think, that we respect these patients concerns. We respect that there's tremendous amounts of embarrassment from walking around with one of these lesions.

The last thing I'd mention is that these aren't static. For many patients, they are positional in nature. They lean over to tie their shoes or pick something up off the ground, and it fills up with blood and you have a large purple pulsating lump on your cheek or on your face. I had a young woman who had one on her left forehead, and every time she leaned over to tie her shoes, it popped up like a grape. And it would happen without notice, so people in the store or at work or at a restaurant would become quite alarmed because it seemed to grow in front of their eyes. People thought it was an emergency.

And so there's all of this stigma that goes along with these. And so I do want to remind doctors that were not dealing with a cancer, but the effects of this are quite involved for patients and families. And so we treat all these patients, everyone who comes to us, with a special ounce of compassion to deal with these lesions.

Melanie Cole, MS: Wow. Thank you so much for sharing your outcomes and patient stories as well. And such an interesting topic. And your passion comes right through. Dr. Morlandt, Dr. Jones, thank you so much both of you for joining us today. You are both excellent guests. And a physician can refer a patient to UAB Medicine by calling the MIST line at 1-800-UAB-MIST or by visiting our website at uabmedicine.org/physician. That concludes this episode of UAB MedCast. I'm Melanie Cole. Thanks so much for joining us today.