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Parkes Weber Syndrome

  • Overview

    At Children’s Hospital Boston, we know how difficult a diagnosis of Parkes Weber syndrome (PWS) can be, both for your child and for your whole family. It’s a rare condition that’s not widely understood.

    Here’s what you need to know:

    • PWS is a rare congenital (present at birth) condition that results in your child having a large number of abnormal blood vessels.
    • It’s very similar to the more common Klippel-Trenaunay syndrome and is treated in much the same way.
    • In contrast to children who have Klippel-Trenaunay syndrome, kids with PWS have arteriovenous malformations that can cause heart failure.
    • Possible complications of PWS include:
      • swelling and overgrowth of one of your child’s limbs (usually a leg)
      • bleeding from the capillary malformation (sometimes called a “port-wine stain”), which is a flat, warm, pinkish stain that appears at birth and typically covers a large area
      • the large amount of blood flowing to the affected limb can wear out your child’s heart.

    How Boston Children's Hospital approaches Parkes Weber syndrome

    Here at Children’s Vascular Anomalies Center, we have the world’s largest database of children whose vascular tumors have caused Parkes Weber syndrome. When doctors anywhere in the world have questions about whether a child’s birthmark may be a vascular tumor that could lead to Parkes Weber syndrome, they often call us. Our physicians have treated more children with Parkes Weber syndrome than any other hospital in the world—which means that your child will get expert care from experienced physicians.

    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.

    Surgeons at Children's are leading the charge in removing the extra tissue that grows as a result of PWS. Children with PWS sometimes have legs that are so large as to be debilitating, forcing them to use a wheelchair. In a debulking procedure, our surgeons remove a great deal of the extra tissue, which can allow your child to regain function of her leg. Read more about our treatment here.

    A young woman shares her story

    Read and watch a video of one patient’s inspiring story about her struggle with a condition similar to PWS, called Klippel-Trenaunay syndrome.

    Parkes Weber syndrome: Reviewed by Arin Greene, MD, MMSc.
    © Children’s Hospital Boston, 2010

  • In-Depth

    It can be hard to find accurate information about Parkes Weber syndrome (PWS) because it’s such a rare condition. Here at Children’s Hospital Boston, we understand the condition — and we know when and how to treat it.

    What causes PWS?

    No one knows the precise cause. Some doctors believe it’s caused by a mutation — in the embryonic stage of life — in the cells that form the vascular system and other tissues. No known food, medication or activity during pregnancy can cause PWS.

    How common is it?

    It’s exceptionally rare.

    When does it appear?

    Doctors can sometimes see evidence of PWS in a prenatal ultrasound and the capillary malformation (“port-wine stain”) is almost always apparent when your child is born.

    Who has PWS?

    It’s equally common in boys and girls.

    Signs and symptoms

    Often, PWS can be misdiagnosed by your child’s primary physician, simply because the condition is so rare. It often has three characteristic signs:

    • a flat, pink, warm birthmark caused by a capillary malformation on your child’s skin
    • hypertrophy — or excessive growth — of a limb (usually a leg)
    • arteriovenous malformations (AVM) — an abnormal connection between the arteries and veins in the affected limb; AVMs can result in much higher blood flow than normal, and can lead to heart failure

    What symptoms will my child have?

    PWS affects different kids in different ways. One child may have a mild form of the syndrome with a small capillary malformation and one hand or foot slightly larger than the other. Another child may have a capillary malformation that runs from her chest down her leg, lots of swelling, heart problems and one limb that is much larger than the other.

    Here are some of the more common symptoms of PWS:

    • cellulitis (infections in the skin) — your child may be more prone to infection because he is more likely to bleed easily as a result of the abnormal capillaries near the surface of his skin
    • anemia — due to blood loss because of easy bleeding
    • pain and heaviness of the affected limb — the pain can often be severe
    • difference in size between the affected and non-affected limb
      • If there's a big difference in the length of your child's legs, your doctor may recommend an operation.

    It’s also possible that the increased blood flow through the AVMs in your child’s limb could strain his heart and lead to something called high-output cardiac failure.

    Long-term outlook

    What is the long-term outlook for my child?

    Because there’s no cure for PWS, 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 amount of hypertrophy in your child’s limb or limbs
    • the condition of your child’s heart
    • 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.

    Does PWS get better?

    No. PWS is a progressive condition, which means that it will grow as your child grows. That said, we have many effective ways to manage your child’s symptoms. For more information, see the Treatment & Care section.

    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 the PWS effectively. A typical follow-up visit may include some or all of the following:

    • a physical exam
    • laboratory testing
    • imaging scans

    FAQ

    Q: What are the signs that my child has PWS?

    A: PWS often has three characteristic signs:

    • a flat, pink, warm stain on your child’s skin
    • hypertrophy — or excessive growth — of a limb (usually a leg)
    • arteriovenous malformations — an abnormal connection between the arteries and veins in the affected limb; AVMs can result in much higher blood flow than normal, and can lead to heart failure

    Q: What symptoms will my child have?

    A: Here are some of the more common symptoms of PWS:

    • cellulitis (infections in the skin) — your child may be more prone to infection because he is more likely to bleed easily as a result of the abnormal capillaries near the surface of his skin
    • anemia — due to blood loss because of easy bleeding
    • pain and heaviness of the affected limb — the pain can often be severe
    • difference in size between the affected and non-affected limb
      • If there's a big difference in the length of your child's legs, your doctor may recommend an operation.

    It’s also possible that the increased blood flow through the AVMs in your child’s limb could strain his heart and lead to something called high-output cardiac failure.

    Q: How is PWS diagnosed?

    A: Doctors can sometimes see evidence of PWS in a prenatal ultrasound and the capillary malformation (sometimes called a “port-wine stain”) is almost always apparent when your child is born. If your child is older and you believe he has PWS, 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 PWS. If there’s any doubt, your doctor may recommend an MRI, ultrasound, CT scan, angiogram or echocardiogram, which can help determine the correct diagnosis and the appropriate next steps in treatment. Read more about diagnosing PWS.

    Q: How is PWS treated?

    A: It’s a complicated condition, and it affects different kids in different ways. The first step is to have your child evaluated by members of an experienced interdisciplinary vascular anomalies team. No single specialist can manage PWS and its associated problems, as different interventional techniques and surgical procedures are often needed.

    For more information, see the Treatment & Care section.

    Q: What kind of support will we get from Children’s?

    A: 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.

    Q: What is the long-term outlook for my child?

    A: Because there’s no cure for PWS, 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 amount of hypertrophy in your child’s limb or limbs
    • the condition of your child’s heart
    • 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.

    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 rare vascular anomalies such as PWS, they often come to us.

    Questions to ask your child’s doctor

    After your child is diagnosed with PWS, 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 PWS 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 with treating PWS?
    Kid-centered care

    Treatment for PWS can be intimidating for young children, so we’ve built our treatments around concepts that work for kids. Our entire staff is highly trained and experienced. And more importantly, we’re all dedicated to working with families — to make sure that your child is as comfortable as possible during treatment.

    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 help children like Lia.

  • Tests

    Diagnosing Parkes Weber syndrome (PWS) can be difficult for many physicians simply because it’s such a rare condition. The specialists at the Vascular Anomalies Center at Children’s Hospital Boston can easily identify PWS — and begin treatment to help manage your child’s symptoms.

    How is PWS diagnosed?

    Doctors can sometimes see evidence of PWS in a prenatal ultrasound and the capillary malformation (sometimes called a “port-wine stain”) is almost always apparent when your child is born.

    If your child is older and you believe he has PWS, 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 PWS. 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:

    • magnetic resonance imaging (MRI) — This high-resolution scan shows the extent of the hypertrophy of tissue and gives your doctor information about what problems it may be causing.
    • ultrasound (also called ultrasonography) — An ultrasound helps your doctor see your child’s vascular system to examine abnormalities and determine how much blood is flowing through the arteriovenous malformations.
    • computerized tomography scan (also called a CT or CAT scan) A CT scan shows detailed images of the area affected by PWS and is especially helpful for evaluating the bones in the affected limb.
    • angiogram — In this study, an interventional radiologist injects a dye into your child, giving doctors a detailed view of the blood vessels in the affected limb.
    • echocardiogramAn echocardiogram allows your child’s doctor to check the condition of her heart.

    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.

    Your appointment
    Questions about your visit? Read about directions, contact information and other important information.
    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 Parkes Weber syndrome (PWS). That's understandable — there is no known cure for PWS and it can be complicated to treat.

    However, you're in the right place. The specialists at the Vascular Anomalies Center at Boston Children's Hospital have seen and treated kids with PWS, so we understand more about this rare condition — which means that your child will get the best care from the most experienced physicians.

    And at Children's, 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.

    How is Parkes Weber syndrome treated?

    PWS is a complicated condition, and it affects different kids in different ways.

    • The first step is to have your child evaluated by members of an experienced interdisciplinary vascular anomalies team.
      • No single specialist can manage PWS and its associated problems, as different interventional techniques and surgical procedures are often needed.

    Because there is no cure for PWS — and it's a progressive condition — we believe that treating your child's symptoms is the most effective way to manage the disease.

    So what kind of treatments are there?

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

    Supportive care

    • compression garments — Your doctor may recommend that your child wear these tight-fitting pieces of clothing on the affected limb to reduce pain and swelling. They can also help protect your child's limb from bumps and scrapes, which can cause bleeding.
    • heel inserts— If your child's legs are slightly different lengths (less than one inch different), a heel insert can help your child walk normally.
    • other medications — Your doctor may also recommend various pain medications and antibiotic medications for your child.

    Surgical care

    • orthopedic surgery — PWS can cause your child's foot or leg to become excessively large; in some cases your doctor may recommend an operation.
      • An orthopedic surgeon can reshape your child's foot to help him fit it into a shoe.
      • An orthopedic surgeon can perform a procedure called epiphyseodesis, which interrupts the growth plate and stops the leg from growing when it's reached a certain length.
        • We've got great orthopedic surgeons who can estimate how long your child's leg will be when it's fully grown. Then, when the affected leg reaches that length, they perform this procedure to stop it from growing longer.
        • This helps keep your child's legs approximately the same length.
        • We only perform epiphyseodesis if the expected difference between the length of your child's legs is greater than an inch — and typically the operation is done when your child is between 10 and 14 years old.
    • Other surgeries — Your doctor may recommend one of these other surgical procedures if your child's symptoms call for it.
      • A surgeon can also help reduce the size of your child's affected limb with something called a debulking procedure. 

    Other treatments

    • embolization — To help shrink your child's abnormal vessels and reduce bleeding and the risk of heart failure, your doctor may propose the injection of a substance that eliminates the abnormal connections between your child's arteries and veins. One of our experienced interventional radiologists can perform this procedure.
    • laser therapy— Laser treatments can help lighten your child's capillary malformation (“port-wine stain”). Laser treatment can also speed healing if your child's lesion begins to bleed.

    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.

    Supportive care

    We want to maximize the safety and effectiveness of whatever therapy you and your child's doctor decide upon.

    • Supportive care involves preventing and treating infections and side effects of treatment to keep your child as comfortable as possible while we're working to manage the symptoms of PWS.

    Continual follow-up care

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

    Rehabilitation

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

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

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

    • physical therapists — PWS 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 PWS 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 PWS is the typically large capillary malformation (“port-wine stain”) 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 PWS. 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 PWS? It's a very rare condition, but 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

    Our Vascular Anomalies Center (VAC) offers links to organizations that offer support and education for parents who have a child with a vascular anomaly.
     

    Surgery explained

    Watch a short video of one of our surgeons describing the various surgical procedures used to treat kids with PWS and Klippel-Trenaunay syndrome. (Requires RealPlayer)

    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

    Children’s Hospital Boston is home to the world’s largest vascular anomalies center. When doctors in other states or other countries need help diagnosing or treating vascular anomalies like PWS, they come to us. Here are some reasons why:

    Innovative treatment

    Traditionally, debulking — a surgical procedure where many of the abnormal vessels and some of the overgrown tissue is removed — has not been a standard treatment for kids with PWS. However, over the past 15 years, surgeons at Children’s Hospital Boston have been performing large-scale debulking procedures to help kids whose overgrown legs have left them wheelchair bound.

    We’re hoping that this practice will achieve widespread acceptance because it makes a big difference in the lives of the kids who’ve had it performed here at Children’s.

    Watch a video of one of our doctors talking about this procedure, and one in which he talks about recovery from the procedure. (Requires RealPlayer)
     

    Battling PWS at its source

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

    The VAC conducts research that may lead to the development of new, more effective therapies and perhaps ultimately result in ways to prevent these anomalies. Read more about our research. 

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

    Phone Number

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

    617-355-5226

     

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