What are the other Names for this Condition? (Also known as/Synonyms)

• Fetal Effects of Varicella Zoster Virus
• Neonatal Varicella Infection
• Varicella-Zoster Infection in the Newborn

What is Congenital Varicella Syndrome? (Definition/Background Information)

• Congenital Varicella Syndrome is a condition that occurs in the fetus, because the mother had had an attack of chickenpox, in the early part of her pregnancy
• Chickenpox (or varicella) is an airborne, contagious viral infection caused by the varicella-zoster virus (VZV), a member of the herpes virus family
• The newborn babies suffer wide-ranging and noticeable abnormalities, which are apparent at birth. These include skin abnormalities in the arms and legs, defects affecting the eyes, brain, nervous system or any other body part, apart from low birth weight
• Severe Congenital Varicella Syndrome is fatal to the unborn fetus or to the newborn child; many are affected by serious complications, including retarded development, skin and bone abnormalities, and poor intellectual abilities
• A prompt diagnosis of the condition and appropriate immediate treatment, as soon as the child is born, may yield a better prognosis. 

Who gets Congenital Varicella Syndrome? (Age and Sex Distribution)

• The incidence of Congenital Varicella Syndrome is extremely rare
• Infants and newborns are affected by this condition, if maternal chickenpox occurred during the first 28 weeks of pregnancy (especially between weeks 8 and 28)
• Both boy babies and girl babies are affected
• No particular racial or ethnic group predilection is observed

What are the Risk Factors for Congenital Varicella Syndrome? (Predisposing Factors)

• A women affected by chickenpox for the first time during her pregnancy, has a certain risk (of almost 2%) of passing on the infection to the fetus, through the bloodstream

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Congenital Varicella Syndrome? (Etiology)

• If a pregnant women is affected by chickenpox, particularly during the first two trimesters of pregnancy, then there is a chance of Congenital Varicella Syndrome developing in the fetus
• The severity of the condition depends upon the period, when the pregnant woman is infected. This also dictates the type of defects and abnormalities that may be observed in the newborn child
• When an infection occurs, the fetus is unable to resist it since its immune system is underdeveloped. The VZV infection remains in blood and develops into Congenital Varicella Syndrome. The condition is also known as Fetal Varicella Zoster Syndrome

This syndrome disrupts the normal development of the baby and leads to many congenital defects and complications.

What are the Signs and Symptoms of Congenital Varicella Syndrome?

Many parts and organs of the body are affected by Congenital Varicella Syndrome. The signs and symptoms of this fetal syndrome may include:

• Skin-related:
  • Hard and thick skin with lesions and overgrown scars
  • Skin is inflamed and red in color
• Limbs-related:
  • Improper formation of the limbs, deficiencies in their structure
  • Underdeveloped limbs
• Nervous system related:
  • Outer portion of the brain is damaged; enlarged ventricle walls may be present
  • Psychomotor skills are affected
  • Learning and intellectual abilities are affected
• Growth:
  • Small baby size, low birth weight
  • Small head size in comparison to the head size of a normal or fully developed newborn baby
• Eyes:
  • Clouded eye lens that affects vision
  • Unusually small eyes
  • Eye movement is fast and involuntary
  • Infected choroid layer, that which lies behind the retina

How is Congenital Varicella Syndrome Diagnosed?

The diagnosis of Congenital Varicella Syndrome may involve the following methods:

• Physical examination and medical history evaluation of the mother
• Physical examination of the newborn child
• Fetal ultrasound will help diagnose Congenital Varicella Syndrome in the developing baby
• Other prenatal diagnostic tools, such as amniotic fluid analysis or chorionic villus sampling, are generally not useful

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Congenital Varicella Syndrome?

The complications associated with Congenital Varicella Syndrome may include:

• Abnormal and delayed growth of the fetus
• Underdeveloped fingers and toes
• Severe brain abnormalities, improperly functioning nervous system
• Psychomotor retardation: Defective coordination between one’s mental and physical activities
• Eye abnormalities such as cataracts and eye membrane scarring

A spontaneous abortion or death of the fetus at birth may also occur.

How is Congenital Varicella Syndrome Treated?

Congenital Varicella Syndrome treatment may involve:

• Varicella zoster immunoglobin is immediately administered after the birth of the child, who is affected by congenital varicella
• Other abnormalities and body part defects are treated symptomatically using a combination of medications, therapy, and surgery, as required

How can Congenital Varicella Syndrome be Prevented?

Congenital Varicella Syndrome preventive measures that may be adopted include:

• Avoid contact with any individual infected with chickenpox, especially if you are pregnant, or you are planning for a pregnancy
• It should be ensured that all individuals (family members) living with the pregnant woman are vaccinated against chicken pox
• Appropriate and early treatment of chickenpox in expectant mother, if it occurs
• During pregnancy, varicella vaccination should be avoided

What is the Prognosis of Congenital Varicella Syndrome? (Outcomes/Resolutions)

• An accurate diagnosis and medical intervention at the proper time can help save the child. This can also allow the baby lead a normal life, without any complications
• If Congenital Varicella Syndrome is severe, then many fetuses are spontaneously aborted or are stillborn. Also, the fatality during the first few years of life may be around 30%

Additional and Relevant Useful Information for Congenital Varicella Syndrome:

• Chickenpox and shingles test maybe performed to detect and diagnose a current or past chickenpox and/or shingles (herpes zoster) infection. The infections are caused by the varicella zoster virus (VZV)

The following article link will help you understand chickenpox and shingles test:

http://www.dovemed.com/common-procedures/procedures-laboratory/chickenpox-and-shingles-test/