Can you cure a vascular anomaly?
We have certain patients where their malformation involves large amounts of their body, and you can only hope to get things under control. I think that’s probably one of the things that we try to emphasize to patients is our goal is not necessarily a cure. This is part of you; you were born with a congenital abnormality of your blood vessels, so short of removing it with a surgery (where you get recurrences 15-20% of the time) you’re not going to get cured in the sense that you’re never going to get rid of this thing. So then your goal is controlling the symptoms and getting long-term control of the symptoms. Once you’ve put it in that perspective for patients, then they have a better realization of how to frame things in their mind. The thing is that some of our patients go to other institutions that say, “We’ll take care of it, we’ll get rid of it.” My philosophy is, from knowing the inherent nature of these malformations, the pathological, the microscopic nature of these malformations—you never really get rid of them. You just have to hope to get the best long-term control of the swelling, the pain, the flow from a high-flow lesion.
Horacio Padua, MD
What are different types of treatments you recommend?
Many different treatments for vascular anomalies exist. Because an operation is the most invasive form of treatment and is associated with the most risks, we typically try to manage lesions with drugs, sclerotherapy, embolization, or laser when possible.
Arin Greene, MD, MMSc
For a growing infantile hemangioma, we want to try to minimize the size and destruction. The standard therapy is systemic or intralesional corticosteroid. Propranolol has become a new agent and everybody’s using it like water. We’re trying to document the complications, which seem to be small but they are potentially dangerous. Other tumors require other chemotherapeutic agents. For example, the kaposiform hemangioendothelioma that causes the bleeding disorder, “Kasabach–Merritt phenomenon”, can be managed with vincristine or rapamycin.
There’s always a role for surgical removal of infantile hemagioma during its life cycle. I’ve written extensively on how and whether to remove it during infancy, early childhood or late childhood. For the malformations, you generally don’t have any drugs, although we wish we did. The major intervention for the malformations is sclerotherapy for the slow-flow lesions and embolization for fast-flow anomalies. There’s also a role for a surgeon, depending on the malformation. There’s the possibility that drugs will someday be used to treat malformations, particularly when they cannot be controlled by sclerotherapy, embolization, or surgical intervention.
John Mulliken, MD
How do you decide what sort of treatment to use?
The venous malformations can be treated with sclerotherapy. The high-flow lesions are treated with a combination of both the sclerotherapy procedures that we do as well as plugging the arteries, also known as embolization. We use catheters to get to the abnormal arteries and actually plug those arteries with material so they don’t have flow in them anymore, and that’s our goal; to isolate the abnormal network of vessels from both sides, from the vein side and from the artery side.
Typically the lesion and the malformation decide the treatment, for the minimally invasive. Venous malformations and lymphatic malformations are treated with sclerotherapy. Arteriovenous malformations are treated with embolization and sclerotherapy on top of it. Also we tend to see more surgical attempts with arteriovenous malformations because they respond variably to interventional treatment.
Horacio Padua, MD
Often the location and the functional impairment help decide what type of treatment to use. If there is functional impairment, we’re going to be more aggressive about it. If it looks like it’s amenable to a procedure like excision then we would do that.
Marilyn G. Liang, MD
The simple answer is the severity. Sometimes people come, and they’re perfectly functional, they have no pain. They just have a vascular stain on their limb and maybe it’s a little bigger and then you can just reassure them that nothing needs to be done. Some people come and they’re in agonizing pain and they’re living bed-ridden, and then our goal is really how to treat their pain. So I would say it really depends on the severity of the problem and then what the particular problem is… We very much tailor the treatment and the approach to what kind of problem we have.
Samantha Spencer, MD
How do you decide between surgery and interventional radiology?
We really think about the modality that’s best for the patient. It’s very, very common that I’ll be in the clinic and a patient will be referred to me for what they think is going to be a surgical procedure, and I’ll say to them, “You know what? I’m a surgeon. I make a living operating on kids, particularly with vascular malformations, but if this were me or my child I would not let anybody cut because there’s a less invasive, safer, less painful procedure that these guys can do, and if it doesn’t work, then we can talk about surgery.” I think that provides tremendous reassurance to the patient or the family. Probably at least 90% of the time that I see a vascular malformation patient for the first time, I’ll have that conversation because most of the time IR is a better option. Similarly, I think our IR group has matured to the point where they recognize the limitations of their procedures, and if they think they’re going to do 10 procedures and then we end up cutting it off anyway, they’re now becoming much more comfortable at the beginning saying, “You know what? We can try this once or twice but if it doesn’t make a big difference, you should get it cut off.” So we all trust each other in that regard and I think that one of the big advantages of our multidisciplinary program is that patients see that. They see that people recommend the other guy’s approach.
Steven Fishman, MD
When deciding on surgery over sclerotherapy, we look at the three-dimensional extent of the malformation. We consider where it is, how isolated it is, how diffuse it may be, what the vascular channels look like. For instance, if the channels are small (which we frequently see in lymphatic malformations) sclerotherapy doesn’t always work very well, so we may operate on those. If they’re very large channels, sclerotherapy is very effective. A good example is in the neck or up in the armpit where the brachial plexus is. These are large, saccular pockets in lymphatic malformations. They respond very well to sclerotherapy, so that would be our first choice of treatment.
With the malformations, particularly in the extremities, we usually go to interventional radiology for sclerotherapy. There are certain regions where surgery in the extremities is better. Those regions are in critical areas like the palm along the digits where you have nerves, arteries, gliding tendons that can be injured during sclerotherapy and which are very accessible surgically. Those areas we may favor surgery over sclerotherapy. For other areas in the arm, for example malformations inside the muscle, sclerotherapy is the favored treatment for those. Sometimes when they involve nerves and there’s a tremendous amount of pain then surgery’s better. Initially, however, our first treatment recommendation is usually some type of interventional radiology.
Joseph Upton, MD
Are families ever resistant to treatment recommendations?
I would say by the time they come here, they’re seeking our advice… Some cases fall into a grey zone where there are lots of different possible ways to go about it, and they may actually hear contradictory advice from different places, and then it’s really up to the parents to follow the advice that they feel most comfortable with. A lot of my time is spent talking to families about treatment options and strategies and risks and benefits. I feel like in most cases, they end up choosing to stay here. There have been a few cases where they’ve gone elsewhere, but I don’t think that’s an illogical decision. You may just feel more comfortable with someone else’s advice; that’s different than not wanting to treat your child at all.
Darren Orbach, MD, PhD
By the time they’ve found us, many patients and families are pretty motivated. Generally speaking, our approach is to set expectation, understand the disease from the patient’s perspective and then we want to treat what’s bothering them. It’s not up to us whether something bothers a patient. Because we take that approach, it’s relatively rare that families are resistant to treatment. Sometimes the treatments are so drastic or foreign, that families may need processing time before deciding about a therapy. So long as we’ve explained why we recommend a particular treatment and any dangers of not treating, we respect patients’ decisions about treatment.
Cameron Trenor, MD
Recommendations for treatment are typically symptom-based. Patients may struggle with pursuing treatment if they are currently not having symptoms but know their lesion(s) may progress to cause problems over time. Also patients that are in pain may get discouraged if told a series of treatments may be needed and after the first few do not have the desired relief. We offer support by providing education on all of the options based on our team’s experiences with treating similar patients.
Erin Spera, MS, CPNP