Venous Malformation Symptoms & Causes

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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

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?
     
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