Congenital Varicella Symptoms & Causes

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In-Depth

At Children’s Hospital Boston, we understand that you may have a lot of questions when your child is diagnosed with congenital varicella syndrome, such as:

•           What exactly is it?

•           How did he get it?

•           What are potential complications in my child’s case?

•           What are the treatments?

•           Are there any possible side effects from treatment?

•           How will it affect my child long term?

We’ve tried to provide some answers to those questions here, and when you meet with our experts, we can explain your child’s condition and treatment options fully.

Background
A baby may contract a varicella infection in the uterus when the mother catches chicken pox and carries it through her bloodstream to the baby.

  • The developing fetus is especially vulnerable to illness because its immune system is not yet strong enough to permanently fight off infection.

Since a baby in utero cannot completely get rid of an infection, the varicella virus remains in the body, and can lead to congenital varicella syndrome, which may prevent the child's vulnerable organs from developing correctly.

Causes

A woman who gets varicella (chicken pox) for the first time during her pregnancy can pass it on to her unborn child, causing the syndrome.

  • If you’re pregnant and haven’t ever had chicken pox, be very careful because varicella is highly contagious — there is a 90 percent chance that an infected person will spread the disease to a household member who has not had chicken pox before.

How is chicken pox spread?
For adults and children, varicella is spread through direct skin contact with the chicken pox rash or through the droplets in the air.

  • Varicella can also be spread from a pregnant mother to her fetus through the bloodstream.

What is the likelihood that my baby will get congenital varicella syndrome?
Most adults and children have already had chicken pox or been vaccinated against it, so the risk of a mother passing the varicella virus on to her baby is very low.

  • Even if a mother does contract chicken pox while pregnant, there is only a 2 percent chance that the baby will develop congenital varicella syndrome.

Symptoms
Babies born with congenital varicella syndrome may have may have some or all of the following symptoms:

  • skin(mostly on arms and legs)
    • thickened, overgrown scar tissue
    • hardened, red and inflamed skin
       
  • limbs
    • limb atrophy — limb deficiencies, malformations and underdevelopment
       
  • autonomic nervous system — controls involuntary functions
     
  • brain
    • ventriculomegaly — enlarged ventricles of the brain
    • cortical atrophy — degeneration of outer portion of brain
       
  • growth
    • low birth weight
    • microcephaly — abnormally small head
       
  • eyes
    • cataracts — clouding over the lens of the eye
    • abnormally small eye(s)
    • rapid, involuntary eye movement
    • chorioretinitis — inflammation of the choroids layer behind the retin
       
  • psychomotor skills — motor movements caused by mental process
     
  • learning disabilities
     
  • intellectual disabilities

Long-term outlook

It’s important to identify early if your child is at risk for developing congenital varicella syndrome. Pregnant women who contract chicken pox will be monitored by ultrasound to see if the virus is affecting the fetus.

If your baby is born with congenital varicella syndrome, specific symptoms of the disease can be treated accordingly. For more information, see the Treatment and Care tab.

Questions to ask your doctor

If you’re pregnant and at risk for contracting chicken pox, you may have lots of questions about congenital varicella syndrome and how it can affect your baby.

You may find it helpful to jot down questions as they arise—that way, when you talk to your doctor, you can be sure that all of your concerns are addressed.

Here are some questions to get you started:

  • I’ve never had chicken pox; should I get vaccinated?
  • How can I avoid exposure to chicken pox?
  • What can I do to prevent infection if I’m exposed?
  • I’ve got chicken pox. Is there any way to prevent passing it on to my baby?
  • What steps can we take if my baby does get infected?
  • What’s the long-term outlook for a baby born with congenital varicella syndrome?

Prevention
You don’t have to worry about congenital varicella syndrome at all if you have already had chicken pox or been vaccinated against it.

However, if you are pregnant and have not had chicken pox before, the following steps can help prevent congenital varicella syndrome:

  • Avoid contact with anybody who has chicken pox.
  • Susceptible people who are living with a pregnant woman should get the varicella vaccine.
  • If you are already pregnant, DO NOT get the varicella vaccine, as it contains a live version of the virus. Get vaccinated at least a month before your pregnancy or after giving birth.

FAQ

Q: What is congenital varicella syndrome?

A: A baby can be born with congenital varicella syndrome if a mother infected with chicken pox passes the varicella virus to her fetus.  

Q: Why is congenital varicella syndrome a problem?
A:
Varicella can be spread from a pregnant mother to her fetus through the bloodstream.

  • Babies born with congenital varicella syndromemay have birth defects.

Q: Is there any way to prevent infection?

A: You don’t have to worry about congenital varicella syndrome at all if you have already had chicken pox or been vaccinated against it.

However, if you are pregnant and have not had chicken pox before, the following steps can help prevent congenital varicella syndrome:

  • Avoid contact with anybody who has chicken pox.
  • Susceptible people who are living with a pregnant woman should get the varicella vaccine.
  • If you are already pregnant, DO NOT get the varicella vaccine, as it contains a live version of the virus. Get vaccinated at least a month before your pregnancy or after giving birth.

Q: How is congenital varicella syndrome diagnosed?

A: If you contract chicken pox during your pregnancy, fetal ultrasounds can monitor your baby to determine if varicella affects its development.

Q: What symptoms might my baby have?

A: Babies born with congenital varicella syndromemay have birth defects that affect the following parts of their bodies:

  • skin(mostly on arms and legs)
    • thickened, overgrown scar tissue
    • hardened, red and inflamed skin
       
  • limbs
    • limb atrophy — limb deficiencies, malformations and underdevelopmen
       
  • autonomic nervous system — controls involuntary functions
     
  • brain
    • ventriculomegaly — enlarged ventricles of the brain
    • cortical atrophy — degeneration of outer portion of brai
       
  • growth
  • eyes
    • cataracts — clouding over the lens of the eye
    • abnormally small eye(s)
    • rapid, involuntary eye movement
    • chorioretinitis — inflammation of the choroids layer behind the retin
       
  • psychomotor skills — motor movements caused by mental process
     
  • learning disabilities
     
  • intellectual disabilities

Q: What are our treatment options?

A: Our Division of Infectious Diseases treats congenital varicella syndrome in infants.

If your baby is born with congenital varicella syndrome, we’ll administer Varicella-zoster immune globin (VZIG) immediately after birth, in order to lessen the severity of the disease.

For more information, see the Treatment and Care [LINK] tab.

Q: What is my child’s long-term outlook?

A:It’s important to identify early if your child is at risk for developing congenital varicella syndrome. Pregnant women who contract chicken pox will be monitored by ultrasound to see if the virus is affecting the fetus.

If your baby is born with congenital varicella syndrome, specific symptoms of the disease can be treated accordingly. For more information, see the Treatment and Care tab.

Q: What makes Children’s different?

A: Our physicians are expert, compassionate and committed to focusing on the whole child, not just his condition—that’s one reason we’re frequently ranked as a top pediatric hospital in the United States.

Physicians and researchers in our Division of Infectious Diseases are constantly learning more about how diseases develop and spread as well as how the body uses its defenses to fight back. 

And at Children’s, we consider you and your child integral parts of the care team and not simply recipients of care. You and your care team will work together to customize a plan of care for your child.

Fetal-Neonatal Neurology Program
Learn more about our program for babies with congenital neurological conditions.
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- Sandra L. Fenwick, President and CEO

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