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The Congenital Varicella Syndrome


Maternal varicella during the first two trimesters of pregnancy may cause the congenital varicella syndrome (CVS). After infection in the first 20 weeks' gestation, the incidence is estimated to be about 2%. To date, nearly 100 infants born with signs of CVS have been reported in the literature, more than three quarters of them during the last two decades. The characteristic symptoms consist of skin lesions in dermatomal distribution (76%), neurologic defects (60%), eye diseases (51%), and skeletal anomalies (49%). About 30% of infants born with these lesions died in the first months of life. The diagnosis of CVS should be established by the appearance of maternal varicella, the presence of typical clinical symptoms as well as the laboratory evidence of in utero varicella-zoster virus (VZV) infection. In the reviewed infants, intrauterine VZV infection has been proved in about 60%. Passive immunization may reduce the risk of fetal infection but there is no evidence to prevent fetal viremia. Up to now, there are no controlled studies concerning antiviral chemotherapy in preventing CVS.

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Correspondence to Andreas Sauerbrei.

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Sauerbrei, A., Wutzler, P. The Congenital Varicella Syndrome. J Perinatol 20, 548–554 (2000).

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