KidsMD Health Topics

Venous Malformation

  • Overview

    We know it can be hard to hear that your child has a venous malformation (VM). But at Children’s Hospital Boston, we view the diagnosis as a starting point: Now we’re able to begin the process of treating your child — with all the means at our disposal — so that we may effectively manage the condition and allow your child to have a healthy life.

    Here’s what you need to know about venous malformations:

    • They’re caused by widened, abnormally shaped veins that can enlarge as your child grows older.
    • Your child may have a wide range of symptoms based on where the malformation occurs and how large it is.
    • They don’t go away on their own and often recur after treatment.

    How Boston Children's Hospital approaches venous malformations

    The Vascular Anomalies Center (VAC) at Children's is a team of 25 clinicians, representing 16 medical and surgical specialties, who have developed sub-sub specialization in the field of vascular anomalies.

    VAC physicians have extensive experience treating children and adults with venous malformations and other vascular anomalies from the U.S. and abroad. As such, the VAC is often regarded as the premier center for the treatment of individuals with vascular anomalies.

    We specialize in innovative, family-centered care. From your first visit, you’ll work with a team of professionals who are committed to supporting all of your family’s physical and psychosocial needs.

    Venous malformation: Reviewed by Arin Greene, MD, MMSc.
    © Children’s Hospital Boston, 2010



    Boston Children's Hospital
    300 Longwood Avenue
    Fegan Building, 3rd Floor
    Boston MA 02115

     617-355-5226
  • In-Depth

    It can be hard to find accurate information about venous malformations (VMs) because they’re relatively rare. Here at Children’s Hospital Boston, we understand VMs — and we know when and how to treat them. Read on for information about VMs, and when you meet with our experts, we can explain your child’s condition and treatment options in detail.

    What’s a VM?

    A VM is a bluish lesion that’s the result of improperly formed veins. The walls of these veins are unusually thin because of a relative lack of smooth muscle cells — and that’s why they can stretch and cause problems.

    How common are they?

    They’re pretty rare, although they are the most common type of vascular malformation we treat at our Vascular Anomalies Center.

    When does a VM appear?

    VMs can appear anytime during childhood, adolescence or adulthood. Most are present at birth, though they may not be diagnosed until later— especially if your child’s lesion is small or not in an obvious location.

    Where do VMs occur?

    They’re most common in the skin, but can be present in other tissues and organs as well.

    What are the most common complications of VMs?

    Some possible complications include:

    • They can suddenly expand or cause pain if a clot forms within the VM.
    • They can get bigger as the result of injury.
    • They can get bigger during puberty.
    • A VM in your child’s head or neck may grow and obstruct vital functions like breathing or vision.
    • A VM in your child’s limb can cause a difference in size between the affected and non-affected limb. It can also lead to fractures of the affected bone and arthritis.
    • A large VM that affects your child’s deep venous system can lead to a pulmonary embolism if a blood clot blocks one of the vessels that lead to the lungs.
    • Gastrointestinal VMs can cause chronic bleeding and anemia.

    Are there other kinds of VMs?

    Yes. There are also rare sub-types of vascular malformations, which make up approximately 10 percent of all VMs. Some of these rare VMs include:

    • Glomuvenous malformation (GVM) — Glomus cells are abnormally formed smooth muscle cells believed to function in the normal regulation of blood flow.
    • These lesions usually occur in multiple numbers and are:
      • small
      • often painful
      • blue-purple in color
    • Cerebral-cavernous malformation (CCM) — This is a familial disorder characterized by the formation of multiple VMs in the brain. These lesions often bleed and expand. About 10 percent of kids with this disorder develop skin VMs.
    • Blue rubber bleb nevus syndrome (BRBNS)  — This syndrome typically results in your child having multiple VMs of the skin and internal organs.
       
    • The lesions tend to get larger and more numerous over time and may be painful.
       
    • The intestinal lesions can cause chronic bleeding, intermittent abdominal pain or sudden bowel blockage.
       
    • Some kids with BRBNS need chronic blood transfusions.
    • Maffucci syndrome — This is a condition involving multiple benign bone tumors (enchondromas) and VM-like lesions of the skin.
    • The enchondromas develop in childhood and cause deformities due to abnormal bone development.
    • They can also result in fractures and can sometimes become cancerous bone tumors (although when this happens, the cancerous tumor is typically low grade and can be surgically removed).

    Causes

    What causes VMs?

    VMs are caused by genetic mutations during the embryonic stage of life. No known food, medication or activity during pregnancy can cause a VM.

    Signs and symptoms

    Often, venous malformations can be misdiagnosed by your child’s primary physician, simply because they’re not that common.

    What are the common signs of all VMs?

    VMs have three characteristic signs. They are typically:

    • blue
    • soft
    • compressible

    A venous malformation can range from a very small lesion in one spot on your child’s body to a widespread lesion that also affects the underlying tissue, muscles and bones.

    What symptoms will my child have? 

    VMs affect kids differently depending on how large the malformation is and where it’s located. Here are some of the more common symptoms of VMs:

    • pain
    • swelling
    • psychological/social issues related to the appearance of the lesion

    Long-term outlook

    What is the long-term outlook for my child?

    Because there’s no cure for a venous malformation, we focus on managing your child’s symptoms.

    The long-term outlook for your child depends on a few factors:

    • the extent of the disease
    • the abnormal blood vessels’ response to therapy
    • the overall health of your child
    • your child's tolerance of specific medications, procedures, or therapies
    • new developments in treatment

    Every child is unique and your care team will work with you to develop a treatment plan that works for your family.

    Will my child’s VM get better?

    No. A VM is a chronic condition, which means that it will stay with your child as he grows. That said, many kids — especially those with mild VMs — live normal, healthy lives.

    What do we do after treatment is over?

    Depending on the severity of your child’s condition, your doctor may recommend a series of follow-up visits to check for complications and make sure that we’re managing your child’s VM effectively. A typical follow-up visit may include some or all of the following:

    • a physical exam
    • laboratory testing
    • imaging scans

    FAQ

    Q: What’s a venous malformation?
    A:
    A VM is a bluish lesion that’s the result of improperly formed veins.

    Q: How common are they?
    A:
    They’re pretty rare, although they are the most common type of vascular malformation we treat at our Vascular Anomalies Center.

    Q: What are the common signs of VMs?|
    A:
    VMs affect children differently, but often have three characteristic signs. Typically, they’re:

    • blue
    • soft
    • compressible

    You or your child may also notice:

    • pain
    • swelling
    • psychological/social issues related to the appearance of the lesion

    Q: What are the most common complications of VMs?
    A:
    Some possible complications include the following:

    • They can suddenly expand or cause pain if a clot forms within the VM.
    • They can get bigger as the result of injury.
    • They can get bigger during puberty.
    • A VM in your child’s head or neck may grow and obstruct vital functions like breathing or vision.
    • A VM in your child’s limb can cause a difference in size between the affected and non-affected limb. It can also lead to fractures of the affected bone and arthritis.
    • A large VM that affects your child’s deep venous system can lead to a pulmonary embolism if a blood clot blocks one of the vessels that lead to the lungs.
    • Gastrointestinal VMs can cause chronic bleeding and anemia.

    Q: How is a VM diagnosed?
    A:
    In many cases, a simple physical exam gives your doctor enough information to diagnose a VM. If there’s any doubt, your doctor may recommend an MRI, ultrasound or a CT scan. For more information, see the Diagnosis section.

    Q: How is a VM treated?
    A:
    In some kids, a VM doesn’t result in any health problems. We only recommend treating your child’s VM if the malformation is doing any of the following:

    • causing pain
    • creating a deformity
    • obstructing a vital structure
    • causing gastrointestinal bleeding

    For more information, see the Treatment & Care section.

    Q: What is the long-term outlook for my child?
    A:
    Because there’s no cure for a venous malformation, we focus on managing your child’s symptoms. Every child is unique and your care team will work with you to develop a treatment plan that works for your family.

    Q: Will my child’s VM get better?
    A:
    No. A VM is a chronic condition, which means that it will stay with your child as he grows. That said, many kids — especially those with mild VMs — live normal, healthy lives.

    Q: What makes Children’s different?
    A:
    We’re home to the first and largest vascular anomalies center in the world.

    When doctors in other states or other countries need help diagnosing or treating children with vascular anomalies such as venous malformations, they often come to us.

    Questions to ask your child’s doctor

    After your child is diagnosed with VMs, you may feel overwhelmed with information. It can be easy to lose track of the questions that occur to you.

    Lots of parents find it helpful to jot down questions as they arise – that way, when you talk to your child’s doctors, you can be sure that all of your concerns are addressed.

    If your child is old enough, you may want to suggest that she write down what she wants to ask her health care provider, too. Some of the questions you may want to ask include:

    • How will my child’s VMs be treated?
    • What side effects or complications might my child experience due to treatment?
    • What kind of follow-up care should my child receive? How often will she need to come in?
    • How much experience does this center and my child’s doctor have in treating children with VMs?
       
    Child Life specialists
    "Don't forget to tell them the rules," 7-year-old Lia DiFronzo says to Amber Soulvie, her Child Life specialist. Read more about how our Child Life specialists improve the hospital experience for children
  • Tests

    The first step in treating your child is forming an accurate and complete diagnosis. Diagnosing a venous malformation (VM) can be difficult for many physicians simply because it’s not a common condition.

    The specialists at the Vascular Anomalies Center at Children’s Hospital Boston can easily identify a VM — and begin treatment to help your child get better.

    Should we talk to a vascular anomalies specialist?

    In certain cases, yes. You should definitely get in touch with a specialist if any of the following criteria apply to your child's VM:

    • It’s in a critical location, such as around her eye, face or neck.
    • It involves her hands, feet or an internal organ, such as the gastrointestinal tract, liver or brain.

    How is a VM diagnosed?

    We’ll start with a complete medical history and a thorough physical exam. In many cases, that initial exam gives us enough information to diagnose a VM. If there’s any doubt, your doctor may recommend one or more of the following imaging tests, which can help determine the correct diagnosis and the appropriate next steps in treatment:

    • ultrasound (also called ultrasonography) — An ultrasound helps your doctor see your child’s venous system to examine abnormalities.
       
    • magnetic resonance imaging (MRI) — This high-resolution scan can confirm the diagnosis, and allow your doctor to determine the size of the malformation and plan the next steps in treatment.
       
    • computerized tomography scan (also called a CT or CAT scan) – Your doctor may recommend a CT scan to examine a VM that’s affecting your child’s bones.

    After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. Then we will meet with you and your family to discuss the results and outline the best treatment options.

    CarePages
    Keep family and friends up to date during your child’s treatment by creating a free Children’s CarePage.
  • Undoubtedly, you were distressed when you learned that your child has a venous malformation (VM). That's understandable — there is no known cure for VMs and they can sometimes be complicated to treat. However, you're in the right place.

    The specialists at the Vascular Anomalies Center at Boston Children's Hospital have treated many kids with VMs, so we understand more about how these malformations respond to therapy — which means that your child will be cared for by some of the world's most experienced physicians.

    In some kids, a VM doesn't result in any health problems, so we only recommend treating your child's VM if the malformation is doing any of the following:

    • causing pain
    • creating a deformity
    • obstructing a vital structure
    • affecting your child's vision
    • causing gastrointestinal bleeding

    What kind of treatments are there?

    Treatments fall into several categories, which we've outlined below.

    Supportive care

    compression garments — If your child has a VM on his arm or leg, your doctor may recommend that he wear these tight-fitting pieces of clothing on the affected limb to reduce pain and swelling.

    anticoagulant therapy — One of the potential risks of a large VM is that a blood clot formed in the abnormal blood vessels will cause pain. Your doctor may recommend that your child take a daily dose of an anticoagulant (blood-thinning) medication to help prevent clots from forming.

    Sclerotherapy

    sclerotherapy  — Sclerotherapy is our first choice of therapy. It's safe and effective in 75 to 90 percent of kids with VMs.

    • The procedure uses the injection of an irritating solution to help shrink and eliminate your child's abnormal veins in the affected area.
    • One of our experienced interventional radiologists can perform this procedure while your child is under general anesthesia.

    Although sclerotherapy reduces the size of the lesion and improves symptoms, your child's VM will not go away—and, in fact, the lesion can re-expand after sclerotherapy, which means that your child may have to have additional treatments.

    It's also possible that your child will have a visible deformity after treatment —if that's the case, an operation may improve the deformity.

    Surgical care

    resection — This is a procedure where a surgeon removes part or all of your child's lesion. It's rarely our first choice of treatment for several reasons:

    • It's usually difficult to remove the entire lesion.
    • The lesion often comes back because we can't see all the affected blood vessels.
    • There's a risk of blood loss because of the size and number of affected blood vessels.

    Your doctor may consider resection if:

    • Your child has a small lesion that can easily be removed completely.
    • Your child has GVMs, which are small and typically don't respond well to sclerotherapy.
    • Your child has gastrointestinal VMs.
    • Your child has a deformity after the completion of sclerotherapy treatments.

    And keep in mind that — just as with sclerotherapy — VMs can re-expand after surgery, which might require your child to undergo additional operations.

    What kind of support will we get?

    At Children's we consider you and your child integral parts of the care team and not simply recipients of care. Your care team will be with you every step of the way to ensure that you and your child are getting the support you need.

    We want to maximize the safety and effectiveness of whatever therapy you and your child's doctor decide upon. We do everything we can to prevent and treat clotting, pain and side effects of treatment to keep your child as comfortable as possible while we're working to manage the VM.

    Your child's physician and other members of your care team will also work with you to set up a schedule of follow-up visits.

    Rehabilitation

    Children with VMs sometimes need multidisciplinary care. Some specialists your doctor may recommend for your child include the following:

    • dermatologist
    • plastic surgeon
    • general surgeon
    • interventional radiologist
    • orthopedist
    • hematologist

    Depending on your child's symptoms and the progression of the disease, we may also bring in the following specialists.

    • physical therapists — VMs can affect your child's arm or leg, making it hard or painful for your child to move it. A physical therapist can help ease the pain and increase your child's range of movement.
    • occupational therapists — Children with VMs sometimes get behind on developing motor skills because of physical problems related to the disease. An occupational therapist can get your child back on track.
    • counselors — One characteristic of VMs is the bluish lesion on your child's skin. As your child gets older, he or she may be uncomfortable with that skin lesion. One of our counselors can help your child deal with the psychological and social issues related to having a birthmark.

    Coping and support

    We understand that you may have a lot of questions when your child is diagnosed with a venous malformation. How will it affect my child long term? What do we do next? We've tried to provide some answers to those questions in the following pages, but there are also a number of other resources to help you and your family through this difficult time.

    Patient education: From the first office visit, our nurses will be on hand to walk you through your child's treatment and help answer any questions you may have — What kinds of treatment are available? What do we do about the pain? They will also reach out to you by phone, continuing the care and support you received while at Children's.

    Parent to parent: Want to talk with someone whose child has been treated for a VM? We may be able to put you in touch with other families who can share their experience.

    Faith-based support: If you are in need of spiritual support, we will help connect you with the Children's chaplaincy. Our program includes nearly a dozen clergy representing Episcopal, Jewish, Lutheran, Muslim, Roman Catholic, Unitarian and United Church of Christ traditions who will listen to you, pray with you and help you observe your own faith practices during your hospital experience.

    Social work and mental health professionals: Our social workers and mental health clinicians have helped many other families in your situation. We can offer counseling and assistance with issues such as coping with your child's diagnosis, stresses relating to coping with illness and dealing with financial difficulties.

    On our For Patients and Families site, you can read all you need to know about:

    • getting to Children's
    • accommodations
    • navigating the hospital experience
    • resources that are available for your family

    There's help available online, too:

    • Our Vascular Anomalies Center offers links to organizations that offer support and education for parents who have a child with a vascular anomaly. 
    Integrative therapies
    Our patient-centered approach means that we want your child to not only get better, but also feel good along the way. Throughout the hospital, you¹ll find clinicians trained in therapies that can make your child feel more comfortable, learn to shift focus away from pain and enjoy some peaceful moments during what may be an anxious time. Read more about how acupuncture, guided meditation, guided imagery, massage, Reiki and therapeutic touch could help your child.
  • Research & Innovation

    Boston Children's Hospital is a world leader in opening new avenues of  "translational research," bringing laboratory advances to the bedside and doctor's office as quickly as possible. Our Vascular Anomalies Center (VAC) conducts research that may lead to the development of new, more effective therapies and perhaps ultimately result in ways to prevent these anomalies. Learn more.

    Battling venous malformations at their source

    A VM is caused by errors that occur when blood vessels are forming, and our investigators are currently probing the genes and molecules that regulate the formation and growth of blood vessels. Understanding the genes that control these molecular events will hopefully result in new therapies for vascular malformations.

    New treatment for venous malformations?

    Venous malformations can sometimes grow, requiring aggressive treatment to protect your child's health. Research at Children's has shown that urine testing can help monitor VMs and predict those about to become a serious threat.

    These findings suggest that angiogenesis (the formation of new blood vessels) plays a role in the progression of vascular malformations, raising the possibility of curbing these difficult-to-treat anomalies with anti-angiogenic drugs.

    ''Prior to this study, we had thought it was not possible to treat vascular malformations with drugs, since congenital anomalies generally do not respond to drugs,'' says Steven Fishman, MD, a surgeon on Children's Vascular Anomalies team. ''This study gives us hope that with further research we'll be able to develop drug treatments.''

    We’re currently studying why VMs enlarge or return after treatment. Currently no medication is available to treat venous malformations , but our goal is to develop drug treatments for kids with these lesions. Each time we operate on a VM, we investigate a portion of the lesion to learn how the blood vessels enlarge.

    “We believe hormones and blood vessel stem cells cause VMs to worsen,” says surgeon Arin Greene, MD, of the Vascular Anomalies Center. “These findings may lead to improved treatment.”

    Clinical and Translational Study Unit

    Read about a day in the life of the Clinical and Translational Study Unit at Children’s.

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