Transcription:
Vascular Malformations

Melanie Cole, MS: There's no handbook for your child's health, but we do have a podcast featuring world-class clinical and research physicians covering everything from your child's allergies to zinc levels. Welcome to Kids Health Cast by Weill Cornell Medicine. I'm Melanie Cole. And today, we're talking about vascular malformations with my panel, Dr. Angela Kadenhe-Chiweshe, she's an Assistant Professor of Surgery in the Division of Pediatric Surgery at Weill Cornell Medical College, Cornell University; and Dr. Bradley Pua, he's an Associate Professor of Radiology and Cardiothoracic Surgery at Weill Cornell Medical College Cornell University.

Doctors, I'm so glad to have you join us today. Thank you. So, Dr. Kadenhe-Chiweshe, can you please start and set the table for the listeners? What are vascular anomalies and how common are they?

Dr. Angela Kadenhe-Chiweshe: Thank you, Melanie, for having us. We really welcome the opportunity to share on this topic with our patients. So vascular anomalies is a group of congenital lesions that refer to a variety of conditions that are caused by the abnormal development of blood vessels in the body. The main blood vessels in the body include arteries and veins. And then there are others such as lymphatic vessels. And when these vessels form irregularly during development, you can have a vascular anomaly form. These can include birthmarks. They can include benign tumors or malformations.

Melanie Cole, MS: Well, thank you for that. So, Dr. Pua, what are the different types that you've seen?

Dr. Bradley Pua: Again, thank you for the invitation to do this. And they're really a myriad of different types and they're largely categorized in names secondary to what they're made up of as what Angela was referring to. Many being either solely arteries, veins or lymphatic channels or a combinations of such and whether or not they're a solid and more mass-like or just comprised of those abnormal vessels.

In general, they're actually separated in two categories. The general categories being benign vascular tumors and vascular malformations. The most common benign vascular tumors are named the congenital or infantile hemangiomas. While the vascular malformations may refer to entities like arterial venous malformations, venous malformations, lymphatic malformations, or venolymphatic malformations.

Melanie Cole, MS: Dr. Pua, sticking with you for a second, because we're going to talk about when these are typically found and symptoms, but can you tell us among these different types, the most common ones that you see, how do they affect the children and/or the families?

Dr. Bradley Pua: One thing about these anomalies is that without the proper information, it appears to be quite a big deal for both the family and the providers that are taking care of this particular entity, largely secondary to the fact that these present very early on, if not at birth, in very early childhood and predominantly are actually seen as these growth in various parts of the body. They are more commonly seen in the head and neck and, as you can imagine, that causes a lot of anxiety. So that's why, you know, forums like this is great for the general education. But the most common are really the hemangiomas. They probably comprise anywhere from 10% to 15%. It's much more common in kids that are born premature. But those in particular are interesting because the vast majority involute on their own. They go away by the age of seven. So, you know, I'm giving you one example, but depending upon what type it is, the natural history, it differs.

Melanie Cole, MS: And along those lines, then Dr. Dr. Chiweshe, as parents notice these or a provider pediatrician at a well visit, these are some symptoms of these, like the hemangioma. Then what is kind of the next step as far as if they are noticed or are they sometimes even noticed later in life as a child is growing into their teens?

Dr. Angela Kadenhe-Chiweshe: So that's actually a great question. And really one of the basis in which we really do push for patients and providers to seek experts in this field. Because a lot of what we know about these lesions is not necessarily always very common knowledge, it's not always very commonly taught certainly in schools and medical schools. And so experience really underscores the importance in caring for these patients.

Let's just stick for a second with the hemangioma. And along with what Brad was saying, the hemangioma is the most common vascular tumor. And a lot of children and babies will have a hemangioma, whether it's noticed at birth or whether it's noticed later. And we have learned a great deal about how these tumors do evolve over time and actually go away on their own. So in the wrong hands, you can imagine that a tumor like this would incite a lot of anxiety and worry in a family and potentially find this child undergoing a surgery or procedure to remove something that will ultimately go away on its own. So very important that families are directed towards providers who have experience in this setting.

If we shift a little bit towards malformations, these can be a little bit more challenging to take care of. Their natural history is that they will basically stay with the patient and they do not go away. They do not outgrow the patient, but they will grow with the child. And a lot of what we know about malformation and a lot of what we can offer in terms of management and treatment really depends on the type of the malformation and where it's located and what it's doing or affecting the child. So for instance, Brad mentioned that a lot of the malformations occur in the head and neck region. Well, we know that in the head and the neck region, we have a lot of important structures. We have our breathing pipe. We have our feeding pipe. We have vessels that feed the brain. If the malformation is intimately associated with these lesions, we know that then those children may be threatened by this malformation versus perhaps a malformation that's sitting on a child's shoulder or child's cheek, that may not necessarily pose a functional issue, but may potentially pose a cosmetic issue. Nonetheless, either way, with malformations, you have to assess the type that you're dealing with, the location, it's proximity to things that are important and then that will drive the intervention that maybe required.

Melanie Cole, MS: Dr. Pua, what about other family members? Is there any kind of genetic component? Do we know why these happen? Is it just random? And if one is identified in a baby and we're speaking about malformations, but the hemangiomas, do you then let the family know and other children are then checked? Or is this something if they have future babies that you look for? Tell us a little bit about that.

Dr. Bradley Pua: That's a great question and something that causes quite a bit of anxiety amongst family members when they bring certainly their newborn in. You know, the vast majority are considered congenital, meaning that you're born with it. It's an abnormal development. It's also something that occurs in that individual alone. That being said, there is a smaller group that are associated with other things, whether or not it be a syndrome where not that there would be malformations in family members, but the malformation is just the tip of the iceberg for other things occurring in that individual. So we would screen for that.

The other would be what you're referring to, which is the genetic. Are there additional syndromes or your specific other entities we should be looking for in first degree family members? One example of that would be patients with something called a Weber Osler Rendu syndrome or another name for it is hemorrhagic hereditary telangiectasias. These patients are prone to developing arteriovenous malformations. And they're also associated with other tumors in the brain and also other body parts. That is very commonly genetically acquired. And when we see stigmata of that in patients or patients with symptoms thought to be related to that, we would in fact screen all first degree family members with a very thorough history and imaging as necessary. And so this underscores what, you know, Angela was sort of really trying to hit home, which is very important when there's concern for any vascular anomaly that the entire family unit is being taken care of in a center that sees a very high volume of these and not only a high volume, but also a center that actually has multiple disciplines involved in the treatment and care.

Melanie Cole, MS: Dr. Chiweshe, you mentioned this a little bit earlier about surgery and that it necessarily is not something that always has to be done. So can you speak about the indications? Really, what treatments are available? What do you do for the child? And how do those treatments change as the child matures?

Dr. Angela Kadenhe-Chiweshe: So again, going along the lines of vascular tumors versus vascular malformation, in that tumor category where we talked about hemangiomas, as we mentioned before, Brad and I mentioned, a lot of those congenital infantile hemangiomas will resolve on their own. A cyst or lesion to the infantile hemangioma or congenital hemangioma, which may not necessarily go away on its own. But if there is a residual mass or tumor after a prerequisite amount of time, that may be amenable to removal if the parents should so wish to proceed with that.

Malformations, as I said, are different. Malformations do not go away on their own. The role of surgery has really evolved over time when it comes to malformations. Prior to the advent of a lot of the current modalities that are available and interventions that are available that Dr. Pua can speak to later on, we used to only have surgery as an option. You know, 20, 30 years ago, if a child was born with a malformation that was in that head and neck area, and we were concerned about the malformation potentially affecting the breathing pipe, and so affecting the child's breathing, the only option that we had at that point in time would be to do these big surgeries where we would go in and debulk the lesion. The problem with that one may ask is a lot of these malformations don't respect space, meaning that they tend to wrap themselves around these important structures. So one can imagine that doing a surgery where you have to debulk these lesions will lead to collateral damage to other structures. And so, really over time, there was a huge challenge for surgeons trying to weigh the benefits and the risk of these big interventions. But with the advent of interventional techniques which is Dr. Pua's specialty, we've really come a long way in terms of being able to maximize benefit and minimize risk for some of these patients.

Dr. Bradley Pua: So, as Angela was referring to, the interventional type therapies or to more minimally invasive type therapies that we've migrated to really involve needle punctures or utilizing advanced image guidance to actually deliver some form of either chemical or thermal therapy to the area to actually treat the lesion. The idea behind it is to really cause inflammation or cause scar tissue to form within malformations to prevent them from actually growing larger, functionally treating them. So a prime example would be percutaneous sclerotherapy. The procedure itself is actually very similar to how one would treat a varicose vein. Under ultrasound guidance, a small needle is actually placed into the target area and medications are injected to actually cause scarring and inflammation. I do want to add though that the goal of treatment for these lesions, especially in the malformation category is really not complete eradication. It's really tailored towards removing the presenting symptoms or removing what's ailing the patient and family because oftentimes you end up causing more harm than good if it's a very extensive malformation and you try to take everything out.

Dr. Angela Kadenhe-Chiweshe: Yes, interventional therapies have indeed now become first-line treatment. And I say treatment because as Brad was alluding to, it's very difficult to cure, meaning to get rid of these malformations completely. So we are geared towards symptom alleviation or alleviating threats or risk of having the lesion. And these interventional procedures have really come a long way and really allowed us again to maximize benefit and minimize risk.

However, we as surgeons haven't been completely been replaced by our colleagues in Intervention and actually, as time has gone on, we've actually found ways to collaborate well with each other. For instance, when we have patients who have venous malformations, so these are malformations that are comprised of abnormal veins. They put the patients at risk of developing clots within these venous malformations, and they can be a source of great pain and functional impact on the child. We have come to collaborate in some areas where we're able to have our interventional colleagues embolize the venous lesion. And then we can follow up with a surgery that allows us to then do a near complete resection. And so in that situation, we've partnered well. But sometimes there is a misconception that sclerotherapy or embolization has to be at the expense of surgery, but as we've come to learn over time, sometimes we can actually synergize and have great synergy with both approaches.

Melanie Cole, MS: Well, speaking of synergy and that leads beautifully into my last final thoughts for you both, I'd like to give you each a chance for a final thought. And Dr. Chiweshe, if you would speak a little bit about the multidisciplinary approach. When working with these kids, what providers are involved? But really working in the pediatric setting, it's such an important setting to have so many different providers because there is the family to help and support and any siblings that might be nervous about what's going on with their brother or sister and then, of course, the parents and then the child themselves. So can you speak about the importance of that multidisciplinary approach?

Dr. Angela Kadenhe-Chiweshe: Sure. I cannot emphasize enough the importance of the multidisciplinary approach when dealing with complex or potentially complex disorders. Fortunately, a lot of the vascular tumors and malformations that we manage or we see, you know, are relatively what I would say straightforward as long as the patient has been taken care of in the correct hands. But there is a certain complement of patients that we absolutely do see who are complex, whether it's due to the burden of disease, whether it's due to the fact that it's part of a syndrome as Brad alluded to. But certainly in these situations, more eyes, more different eyes, it's very important to bring that level of experience and collaboration to the table.

A vascular anomalies group or center is usually the general term that is used by centers. The International Society for the Study of Vascular Anomalies is our overarching international association, where we take a lot of direction as to how these multidisciplinary groups should be formed. We have a plethora of specialties. The main ones being surgery, interventional radiology, diagnostic radiology, some surgical subspecialties like orthopedics, urology, plastic surgery. Dermatology is very key to our care. Geneticists are very important. And then your general pediatricians and such who also carry an interest in this field. It is important that our group or these groups meet on a scheduled basis to review complex cases. And that's exactly what we do at Cornell with our group. We meet on a regular basis. We discuss our cases as a group. We review all imaging, pictures and, in some situations, you know, we may have to have the patient see multiple physicians, but we try to gather the information through one visit to alleviate any stress to the family and the patient. And then that information is then shared in a group setting. And then it's important for us to have consensus. It's important to come to an agreement as to what we think will be the best way to approach the condition not only for the patient, but for the family and also again to mitigate risk and to mitigate, you know, complications.

So the multidisciplinary approach is absolutely a first and foremost. We strongly encourage our colleagues in the community, our patients in the community, if they have any concerns or if they have any questions or if they're suspected that they're dealing with a vascular anomaly or vascular malformation, that they absolutely reach out to us.

Melanie Cole, MS: Dr Pua, last word to you, and Dr. Chiweshe just made some really important points. I'd like your final thoughts for parents listening. As we've said at the outset, this can be really scary for parents and scary for the child if they're old enough to know what's going on, speak to them and tell them what you would like them to know about the importance of that good provider care and followup. And give them some hope for the treatments that you can provide at Weill Cornell Medicine.

Dr. Bradley Pua: I think that while this entity can often be very scary, one wonderful thing about working with all the different providers that Dr. Chiweshe was speaking to in our multidisciplinary clinic and also understanding just how complex these situations are, I think that everyone needs to understand that this is not a one-time treatment. This is not you seek care, we treat it and, you know, we don't follow up with you. It's a journey together. It's a journey not only of treatment, but also of shared education.

You know, I think oftentimes, family members will actually bring new ideas to challenge the group as to innovations in care. It is also up to the group not only to work with various family members and answer questions and also grow with the patient. You know, one of the most gratifying things, frankly, is to see the child grow over time and watch them develop. So there's that relationship that is consistent.

You know, at the same time, as Dr. Chiweshe alluded to very early on, this is not something that's taught in medical school very commonly or really talked about in very generalized form. So it's to this group to also spend the time in not only educating the family, but also educating our colleagues, our pediatricians and those out in the community. It's really a shared team dynamic. And I've been at Cornell for more than a decade now, it's really amazing to me how well the team functions as a unit.

Melanie Cole, MS: What great information for parents and thank you. So many treatments available. Thank you both for joining us and sharing your expertise today.

And Weill Cornell Medicine continues to see our patients in person, as well as through video visits. And you can be confident of the safety of your appointments at Weill Cornell Medicine.

That concludes today's episode of Kids Health Cast. We'd like to invite our audience to download, subscribe, rate, and review Kids Health Cast on Apple Podcasts, Spotify and Google Podcast. For more health tips, go to weillcornell.org and search podcasts. And don't forget to check out our Back To Health. I'm Melanie Cole.

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