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A look at vascular anomalies

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AngelaAngela Younger has been around hospitals and doctors her whole life. Born with blue rubber bleb nevus syndrome (BRBNS), the 23-year-old has endured multiple surgeries and interventions since she was born. Now, with the help of the Vascular Anomalies Center (VAC) at Children's Hospital Boston, her life is greatly improved.

BRBNS is a rare congenital venous malformation characterized by abnormally formed, dilated veins. Most often, the rubbery, bluish venous lesions occur on the skin and within internal organs, particularly the gastrointestinal tract. Although they are considered benign, the lesions tend to get larger and more numerous over time, may be painful, and can cause chronic clotting, bleeding and anemia.

The precise cause of BRBNS is unknown and like many vascular anomalies, the condition may be difficult to diagnose. In fact, it is estimated that up to 60 percent of vascular anomalies are misdiagnosed.

At Children's, management of rare and complex conditions like BRBNS is the primary focus of the VAC. Founded in 1978, it is considered the most advanced program in the country and draws patients from around the world for expert diagnosis and treatment. Because these conditions often affect many organ systems, 24 physicians from 19 disciplines of medicine collaborate in the evaluation and management of VAC patients.

In Angela's case, interventional radiology plays a vital role in her treatment. To date, she has undergone more than 20 rounds of sclerotherapy to treat the venous malformations in her left leg. In this procedure, an interventional radiologist manipulates needles under imaging guidance and carefully injects abnormal veins with an agent designed to destroy the endothelial lining of the malformations and eventually shrink the veins.

"Careful planning for this procedure is essential," says interventional radiologist and VAC team member Ahmad Alomari, MD. "They're often challenging, and require all your skills and knowledge to make sure you get a positive outcome without any complications." Side-effects include bruising and swelling in the affected area. "There's some pain. It feels like a really, really bad bruise," says Angela. "But they give me steroid medication to reduce the swelling. And at home I wear an ace bandage or compression garment on my leg and am on crutches for a few days."

There are many advantages of interventional radiology: it's easier for patients; the procedures are minimally invasive and involve no large incisions, less risk and pain, and shorter recovery times.

However, like most vascular anomaly patients, Angela, requires long-term follow-up, including regular visits, usually once a year, for additional treatments. According to Dr. Alomari, this is why having a full multi-disciplinary clinical service like VAC is so important. "The Vascular Anomalies Center draws children from all over the world, and they are often the most complex cases that cannot be taken care of with just one treatment," he says. "It is a long-term process that requires a staff that is experienced, skilled and dedicated."

VAC specialists are available to consult with physicians seeking information or who wish to discuss a specific patient. The staff works collaboratively with referring physicians, involving them in decisions about their patient's care, maintaining regular communication, and providing referrals to specialists close to the patient's home whenever possible.

As for Angela, she's back on her feet and happy with the care she continues to receive at Children's. "I'm not going to lie and say my life is perfect, and I'm out running the Boston Marathon," she says. "But thanks to the doctors at Children's, I am a normal young adult."

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