What is congenital varicella syndrome?

Congenital varicella syndrome is caused by the same virus (varicella) as the chickenpox, a common childhood disease.

  • The risk of a mom passing the varicella virus onto her baby is extremely low. Only a primary varicella infection can cause the condition, and most adults and children have already had chickenpox or have been vaccinated against it.
  • Even if a mother does contract chickenpox while pregnant, there is only a 2 percent chance that the baby will develop congenital varicella syndrome.
  • Babies who are born with congenital varicella syndrome may have birth defects that affect various parts of their bodies.

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.

Symptoms & Causes

What causes congenital varicella syndrome?

A woman who gets varicella (chickenpox) 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 chickenpox, 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 chickenpox before.

What are the symptoms of congenital varicella syndrome?

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
  • 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

Diagnosis & Treatments

How do we diagnose congenital varicella?

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

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

How do we treat congenital varicella?

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. Specific symptoms of the disease can be treated accordingly.

Can I prevent congenital varicella?

If you are pregnant and have not had chickenpox before, the following steps can help prevent varicella syndrome:

  • Avoid contact with anybody who has chickenpox.
  • 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.

Pregnant women who contract varicella during pregnancy often have a more severe case of the disease than women who are not pregnant. Severe varicella may be treated with an antiviral medication given through an IV.

How we care for congenital varicella syndrome

The Boston Children's Division of Infectious Diseases treats congenital varicella syndrome in infants. In addition to treating children, we also are dedicated to researching better ways to diagnose, treat and prevent infectious diseases.

The Division of Newborn Medicine specializes in treating babies with a wide range of congenital and acquired conditions. Your baby will be seen by a specially trained team of physicians, nurses, therapists, and other health professionals.

At the Fetal-Neonatal Neurology Program, we identify problems as early as possible and intervene quickly.