Compatible clinical presentation:
- A newborn or stillborn1 infant who has cicatricial skin lesions in a dermatomal distribution and/ or pox-like skin scars and / or limb hypoplasia, with or without other abnormalities2, and with or without history of maternal varicella exposure or varicella during pregnancy OR
- An infant who develops herpes zoster in the first year of life OR
- A spontaneous abortion or termination following varicella infection during pregnancy with ultrasonographic findings3 or pathological findings compatible with congenital varicella syndrome.
AND with laboratory confirmation by:
- Isolation or direct antigen detection of varicella-zoster virus (VZV) in skin lesions or autopsy tissue or from another appropriate clinical specimen (amniotic fluid, fetal blood) OR
- Detection of VZV nucleic acid (e.g., PCR) in an appropriate clinical specimen OR
- Detection of varicella-specific IgM antibodies in cord blood or in serum specimen taken in the first 3 months of life (only 25% of cases are positive) OR
- Persistence of varicella specific IgG antibody in a child aged beyond 6 months of age
1Stillbirths are defined as births with a birth weight ≥500 g or a gestational age at delivery of ≥20 weeks that show no evidence of life at birth.
2Other abnormalities may include:
- microcephaly, hydrocephalus, cerebellar hypoplasia, motor or sensory deficits, sphincter dysfunction and peripheral nervous system defects
- microphthalmia, cataracts, Horner’s Syndrome, chorioretinitis, nystagmus, retinal scars, optic atrophy
- gastrointestinal abnormalities including colonic atresia, hepatitis, liver failure
- genitourinary abnormalities
- cardiovascular abnormalities
- intrauterine growth retardation
3Prenatal diagnosis through ultrasonography may detect limb deformity, microcephaly, hydrocephalus, polyhydramnios, soft tissue calcification and intrauterine growth retardation.
