Parkes Weber Syndrome | Diagnosis & Treatments

How is Parkes Weber syndrome diagnosed?

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

If a child is older and you believe he has PWS, the first step to making a diagnosis is a complete medical history and a thorough physical exam. In many cases, that initial exam gives us enough information to diagnose PWS.

What tests may my child need?

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 (overgrowth) 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 AVM.
  • 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's blood vessels, giving doctors a detailed view of the blood vessels in the affected limb.
  • Echocardiogram: An echocardiogram allows your child’s doctor to check the condition of her heart.

After we complete all necessary tests, our team of vascular anomalies specialists will 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 for your child.

How is Parkes Weber syndrome treated?

Children with Parkes Weber syndrome (PWS) often need multidisciplinary care. Some specialists your doctor may recommend for your child include:

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

While there is no cure for PWS, the symptoms of the condition can be managed.

Supportive care for Parkes Weber syndrome
  • Compression garments: Your doctor may recommend that your child wear these tight-fitting items 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 difference), a heel insert can help your child walk normally.
  • Other medications: Your doctor may recommend various pain medications and antibiotic medications for your child.
Surgical care for Parkes Weber syndrome
  • Surgery: Surgeons can perform what's called a debulking procedure, during which abnormal vessels and some of the overgrown tissue are removed.
  • Orthopedic surgery: If PWS affects the foot or leg, sometimes the limb can become excessively large. Should that be the case, your doctor may recommend an operation. For instance, an orthopedic surgeon can reshape your child's foot if it cannot fit into a shoe.
  • Epiphysiodesis: Sometimes in PWS one leg grows longer than the other. Orthopedic surgeons can estimate how long a child's leg will be when she is fully-grown. If it appears the difference will be more than an inch they may recommend a procedure called epiphysiodesis. This procedure, which is usually done when a child is between 10 and 14 years old, interrupts the leg's growth plate and stops the leg from growing when it’s reached a certain length.

Other treatments for Parkes Weber syndrome
  • Embolization: A specialist called an interventional radiologist can inject a substance that eliminates the abnormal connections between your child’s arteries and veins.
  • Laser therapy: Laser treatments can help lighten your child’s capillary malformation, and speed healing if your child’s lesion begins to bleed.

Other specialists that care for patients with Parkes Weber syndrome

Depending on your child’s symptoms and the progression of the disease, we may also bring in:

  • 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 — As your child gets older, he or she may be uncomfortable with the capillary malformation on his or her skin. One of our counselors can help your child deal with the psychological and social issues related to having a birthmark.

Long-term outlook for children with Parkes Weber syndrome

PWS is a progressive condition, which means that it will grow as your child grows. The long-term outlook depends on a few factors:

  • the extent of the disease
  • the amount of hypertrophy (overgrowth) 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

Depending on the severity of your child’s PWS, your doctor may recommend a series of follow-up visits to check for complications and make sure that we’re managing the PWS effectively.