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Internal Medicine

Congenital varicella syndrome (CVS)

Spectrum of fetal anomalies associated with maternal varicella zoster virus (VZV) infection during the first trimester of pregnancy.

Spectrum of fetal anomalies associated with maternal varicella zoster virus (VZV) infection during the first trimester of pregnancy.

  • Highest risk from maternal VZV infection at 13-20 weeks

History:

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.


Clinical features

  • 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%.

Female neonate with hemangiomatous skin lesions compromising the right scalp and forehead, hemifacial microsomia, homolateral microphthalmia, microtia, and commissural labial deviation | Villota, V. A., Delgado, J., & Pachajoa, H. (2014). Congenital varicella syndrome in a monochorionic diamniotic twin pregnancy. Journal of research in medical sciences : the official journal of Isfahan University of Medical Sciences, 19(5), 474–476.

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