FETAL VARICELLA SYNDROME

Abstract

A great deal of controversy has existed concerning the effects of maternal varicella infection on the fetus. The purpose of this study was to establish a firm association between congenital anomalies of infants and maternal varicella infection (VI) in pregnancy. To date, prospective studies have failed to establish this association due to the rarity of index cases. The infant population in the present study is derived from a retrospective analysis of cases reported in the English language literature, as well as a personal case. The criteria used for the fetal Varicella Syndrome (FVS) were: 1. evidence of maternal VI during pregnancy, 2. presence of congenital skin dermatome lesions characteristic of VI and 3. immunologic proof of in-utero VI of the infants. Eighteen children who fulfilled the above criteria constituted the patient population identified as having fetal varicella syndrome (FVS). These children had a characteristic spectrum of anomalies which included: maternal VI at ≤ 20 weeks' gestation (8-20), all had skin lesions corresponding to dermatomes, 81% were female, 50% small for dates, 47% preterms, 89% had neurologic anomalies, 78% had eye anomalies, 72% had skeletal hypoplasia, 28% had gastrointestinal anomalies and 28% had urologic anomalies. Thus, it is apparent that maternal and subsequent fetal varicella infection during early pregnancy can result in a characteristic spectrum of anomalies which can be referred to as the “fetal varicella syndrome.”