Skip to Main Content

At a glance

A congenital, nongenetic disorder resulting from maternal transmission of varicella in the first and second trimesters of pregnancy manifesting with cutaneous, neurologic, and limb involvement.

Synonyms

Fetal Varicella Syndrome; Antenatal Varicella Virus Infection; Varicella Embryopathy; Varicella Fetopathy.

History

The first case report was published in 1947 by Eugene G. Laforet and Charles L. Lynch Jr.

Incidence

Most (>90%) women of child-bearing age have antibodies (IgG) against varicella zoster virus (VZV, also known as human herpes virus 3), hence infection (chickenpox) during pregnancy is rare. In industrialized countries, approximately 0.5-3:1,000 pregnancies are affected, but fortunately only about 2% of babies suffer from Congenital Varicella Syndrome (CVS) following maternal varicella infection in the first 20 weeks of pregnancy (with the greatest risk between weeks 8 and 20, but infection later in pregnancy resulting in CVS has rarely been described). However, a significantly higher rate is expected in developing countries. Approximately 130 cases have been described in the medical literature. Two-thirds of affected neonates are females.

Genetic inheritance

This is not a genetic disorder.

Pathophysiology

VZV is a highly contagious virus and transmitted either through direct contact with the vesicular fluid from skin lesions or by secretions from the respiratory tract. The incubation period is between 10 and 21 days. While the clinical course of chickenpox is generally mild and self-limiting, it may be more severe with a higher rate of complications in pregnant women (particularly during the third trimester) or in individuals older than 15 years or younger than 1 year of age. The route of fetal infection is most likely transplacental and leads to fetal viremia, although ascending infection from the cervix uteri seems also possible. Organ injuries in the fetus reflect the neurotropic nature of the varicella virus. It appears that due to the immaturity of the fetal immune system, the fetus is unable to generate a VZV-specific cell-mediated immune response, which at least in part explains the severe impact on the fetus. Maternal varicella infection closer to term carries a high risk for severe neonatal varicella infection with a mortality of around 30%. About 15% of children with CVS develop a herpes zoster infection (shingles) between the second and forty-first month of life. It has been recommended that children with zoster in that age range should be examined for signs of CVS. In extremely rare cases, severe/disseminated maternal herpes zoster infection (shingles) has resulted in fetal CVS.

Diagnosis

Based on the history of maternal chickenpox in the first or second trimester of pregnancy with clinical and serologic findings (IgM-specific antibodies in the neonate, persistence of IgG antibodies in the infant beyond the first 7 months of life) in a neonate with low birth weight and multiple, characteristic ...

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.