- Highest risk from maternal VZV infection at 13-20 weeks
The congenital varicella syndrome was first described by La Foret and Lynch, in 1947, and since then, approximately 130 cases have been reported. The mechanism of CVS is thought to be a consequence of the reactivation of the HZV in the uterus, akin to the mechanism of HZ development, rather than being due to the primary infection.
- Skin lesions in dermatomal distribution (73%)
- Neurological defects (62%): Cortical atrophy, microcephaly, seizures and mental retardation
- Ocular abnormalities (52%): : Chorioretinitis, cataract, Horner syndrome, and nystagmus
- Limb hypoplasia (46%)
- Low birth weight (23%)
It has a 30% mortality and miscarriage rate of 3-8%.