Congenital varicella syndrome

Congenital varicella syndrome (CVS), also known as fetal varicella syndrome or congenital varicella embryopathy, is a rare condition that occurs when a pregnant woman becomes infected with the varicella-zoster virus (VZV, the virus that causes chickenpox) during the first 20 weeks of pregnancy. The virus crosses the placenta and damages the developing fetus, leading to a characteristic pattern of birth defects. CVS is not an inherited genetic disorder but rather an infectious embryopathy caused by in utero viral exposure. The syndrome affects multiple body systems. Key clinical features include skin scarring in a dermatomal distribution (cicatricial skin lesions), limb hypoplasia (underdeveloped arms or legs), neurological abnormalities such as microcephaly, cortical atrophy, intellectual disability, and seizures, as well as eye abnormalities including chorioretinitis, cataracts, and microphthalmia. Musculoskeletal defects, autonomic nervous system dysfunction (such as neurogenic bladder), and low birth weight are also commonly observed. Some affected infants may develop early-onset herpes zoster (shingles) in infancy or childhood due to reactivation of latent VZV. There is no specific curative treatment for congenital varicella syndrome. Management is supportive and multidisciplinary, addressing the individual manifestations such as orthopedic interventions for limb abnormalities, ophthalmologic care, neurological rehabilitation, and developmental support. Prevention is the most effective strategy: vaccination of women of childbearing age before pregnancy and administration of varicella-zoster immune globulin (VZIG) to susceptible pregnant women exposed to VZV can significantly reduce the risk. The risk of CVS following maternal varicella infection in the first 20 weeks of pregnancy is estimated at approximately 1–2%.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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