BALTIMORE, Md. (Ivanhoe Newswire) - An AVM is a malformation in the face that happens when there's an abnormal connection between veins and arteries. They are disfiguring, dangerous and difficult to treat. Now, some patients are rolling the dice on a risky procedure.
Susan Adamns had an Arteriovenous malformation – or AVM– growing on her face. The slightest bump could cause massive bleeding.
"It would rupture where it would actually project three or four feet away from you," Susan told Ivanhoe.
She worried a bad bleed could kill her. After six surgeries failed to remove the AVM, Susan lost hope.
"When doctors tell you there's nothing they can do for you, it does get difficult," Susan said.
Then, surgeons at Johns Hopkins tried a risky but potentially life-saving procedure.
"To be honest, I was quite scared when I saw it," Amir Dorafshar, M.D., a plastic and reconstructive surgeon at Johns Hopkins University, explained.
Along with Doctor Monica Pearl, a needle was used to puncture Susan's lip under ultrasound guidance. Doctor Pearl then cut off the AVM's blood supply and glued it shut.
"But preserve her normal tissue, so that she could heal from her surgery," Monica S. Pearl, M.D. an assistant professor at division of interventional neuroradiology at Johns Hopkins University said.
Doctor Amir Dorafshar then carefully cut out the AVM and reconstructed Susan's face.
"Not only did they save my life, but they changed my quality of life, so I'm happy, very thankful," Susan said.
AVMs affect about 250,000 people in the United States. The standard way to treat them is through a catheter inserted in the groin. Doctors originally told Susan her AVM was untreatable because of previous surgeries that failed to fix the problem. If Susan's AVM was not removed, it could have led to fatal blood loss.
RESEARCH SUMMARY
BACKGROUND: Arteriovenous malformations (AVMs) are defects of the circulatory system that are generally believed to arise during embryonic or fetal development or soon after birth. Although AVMs can develop in many different sites, those located in the brain or spinal cord can have especially widespread effects on the body. Most people with neurological AVMs experience few, if any, significant symptoms. The malformations tend to be discovered only incidentally, usually either at autopsy or during treatment for an unrelated disorder. But for about 12 percent of the affected population (about 36,000 of the estimated 300,000 Americans with AVMs), these abnormalities cause symptoms that vary greatly in severity. For a small fraction of the individuals within this group, such symptoms are severe enough to become debilitating or even life-threatening. Each year about one percent of those with AVMs will die as a direct result of the AVM. (Source: NINDS)
TYPES/SIGNS: Seizures and headaches are the most generalized symptoms of AVMs, but no particular type of seizure or headache pattern has been identified. Seizures can be partial or total, involving a loss of control over movement, convulsions, or a change in a person's level of consciousness. Headaches can vary greatly in frequency, duration, and intensity, sometimes becoming as severe as migraines. Sometimes a headache consistently affecting one side of the head may be closely linked to the site of an AVM. More frequently, however, the location of the pain is not specific to the lesion and may encompass most of the head. (Source: MedicineNet)
TREATMENT: Medication can often alleviate general symptoms such as headache, back pain, and seizures caused by AVMs and other vascular lesions. However, the definitive treatment for AVMs is either surgery or focused irradiation therapy. The decision to perform surgery on any individual with an AVM requires a careful consideration of possible benefits versus risks. (Source: NINDS)
NEW TECHNOLOGY: A new minimally invasive procedure was designed to achieve separation between vessels, thereby decreasing the blood flow to the AVM clot. Guided by an angiogram, interventional neuroradiologists Philippe Gailloud and Monica Pearl injected glue into the AVM to decrease the blood flow to the clot.
INTERVIEW
Monica Pearl, M.D., an Interventional Neuroradiologist at Johns Hopkins, talks about AVMs and the risky procedure that could be life-saving for some patients
What is AVM?
Dr. Monica Pearl: An AVM is a vascular malformation. When thinking about the types of vascular malformations there are low flow vascular malformations or high flow vascular malformations. An AVM is in the high flow vascular malformation category because it has an arterial component. That being said it's a fast flow lesion.
Do we know how these are formed?
Dr. Monica Pearl: The majority of these lesions are congenital in nature.
How rare are they?
Dr. Monica Pearl:
Extracranial or superficial AVMs are rare and the exact incidence is unknown. These lesions are different from intracranial AVMs, i.e. AVMs inside the brain.
What is a traditional way to remove AVMs?
Dr. Monica Pearl: Traditional approaches for treating arterial venous malformations are via a transarterial approach, meaning from a transfemoral approach. On the inside one goes through the femoral arteries, travels through the arteries inside the brain, and then glues or closes off the AVM from the inside.
From the groin?
Dr. Monica Pearl: Correct.
What did you do in Susan's case that was difference?
Dr. Monica Pearl: Susan's case is different because she had multiple previous surgeries and embolization type procedures, but the major paths to her AVM had been closed. Those routes or normal accesses to her AVM were not available, so in order for Dr. Dorafshar, the plastic and reconstructive surgeon with whom I work, to have a safe surgery and resect and reconstruct her lip, he needed me to devascularize her AVM. Since the paths from the inside were no longer available we went from the outside; a direct percutaneous puncture to her lip from the outside in. We use real-time ultrasound guidance. I put an ultrasound on her lip, that way we can identify where the AVM is. Then I put a small butterfly needle and puncture her lip using the ultrasound to guide me, and since it's a high flow arterial venous malformation one gets prompt return of arterial blood throughout the tubing. Once that's in place we inject some contrast through the tubing and watch under live fluoroscopy to ensure that you're in the AVM. When you're in the AVM and not in normal tissues then it's safe to glue it. We would then proceed with what's called the glue embolization.
So you had to be right in the exact spot with that needle?
Dr. Monica Pearl: Yes, that's the whole purpose of the treatment. It's to glue the AVM only, but preserve her normal tissue so that she could heal from her surgery.
Has this ever been done before?
Dr. Monica Pearl: It has been done before, here at Hopkins; but the approach is very uncommon because the normal treatment paths are from the inside.
What's this approach called?
Dr. Monica Pearl: Direct percutaneous embolization.
Did they call it the outside, inside approach?
Dr. Monica Pearl: You can consider that because treatments for AVMs are usually from the inside of the arteries. You go from the femoral artery, travel through the arteries inside the body, and then you attack the AVM from the inside. However, if those pathways are not available then the only other way to get to the AVM is from the outside; so a direct puncture from the outside in.
Was it a successful surgery?
Dr. Monica Pearl: Yes, it was successful and we're very happy with the procedure. On the angiogram you can see that she has a big AVM, but there was no big arterial feeder that could allow us to access her AVM. So in order for me to devascularize her lesion, you could not do it from the inside. Instead, we went from the outside in and we have a very nice glue distribution throughout her entire AVM, which could not be achieved from the inside.
Your job in this was to go in and just stop the blood flow?
Dr. Monica Pearl:. My job for Susan's treatment was to devascularize her AVM; remove the blood components of her vascular malformation while preserving her normal tissues. This was so that Dr. Dorafshar could safely remove and resect her AVM the following day but still have normal blood supply to normal surrounding tissues so that she would heal properly.
Is it complex?
Dr. Monica Pearl: Yes.
If you didn't remove this what would happen to Susan?"
Dr. Monica Pearl: Her AVM would continue to grow with her and she would be fraught with continued nose bleeds, she could rupture her AVM and it could lead to severe blood loss. The natural history of AVMs is they continue to grow with you throughout life. She had so many previous surgeries in the past that she didn't want to deal with it anymore because of all the complications and the many procedures, but it was becoming so disrupting and disabling with the more frequent nosebleeds, that she had to have something done.
Is she now out of the woods or is there a chance it could come back?
Dr. Monica Pearl: There is a chance that it could come back, however, her embolization and resection couldn't have been better. That's really the key for treating these complex vascular malformations, a multidisciplinary approach. As an Interventional Neuroradiologist, I'm able to safely glue Susan's AVM and prepare her for surgical resection and rescontruction by Dr. Dorafshar.
Personally how rewarding was this to be able to help her?
Dr. Monica Pearl: It's wonderful, we changed her life. She's a beautiful woman and she can walk around freely without having to be afraid or embarrassed by anything. It's gratifying. Now she doesn't have this big mound of inflamed red tissue in the middle of her face. Instead she smiles proudly and doesn't have to worry about nosebleeds or ridicule.
FOR MORE INFORMATION, PLEASE CONTACT:
David March, MHA
Senior Media Relations Representative/Assistant Director
Johns Hopkins Medicine
(410) 955-1534
dmarch1@jhmi.edu
