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An Illinois mother with no history of varicella vaccination or documented disease delivered a premature infant with congenital varicella infection -- reportedly only the third U.S. case of congenital varicella syndrome since 1995.

The infant was born at 24 weeks gestation following induction of labor after ultrasound showed fetal abnormalities. The baby died shortly after birth, reported Jessica Leung, MPH, of the CDC, and colleagues.

"This case reaffirms current Advisory Committee on Immunization Practices recommendations for preventing varicella that all adults be assessed for varicella immunity, and that those who do not have evidence of immunity should receive 2 doses of varicella vaccine, with special emphasis for adult groups at high risk, including nonpregnant women of childbearing age," the authors wrote in the

Varicella-zoster immunoglobulin should be administered within 10 days of exposure to any woman exposed to varicella virus, they added.

In addition to assessing pregnant women for evidence of immunity, and recommending post-partum vaccination for susceptible women, "it is important to assess and assure documentation of evidence of immunity with each pregnancy, in advance of future pregnancies," the authors wrote.

Leung's group noted that 0.4%-2.0% of newborns born to women with varicella develop congenital varicella syndrome, and prior to the introduction of varicella vaccine, there were 44 cases of this disease per year.

The authors detailed one such case last year.

In January 2021, a 27-year-old woman 12 weeks pregnant was diagnosed with varicella by her primary care provider after developing a rash all over her body. She had a positive varicella virus (VZV) IgM result and received treatment for her symptoms. The rash had resolved by the time she visited her ob/gyn the following month.

The woman previously was VZV IgG equivocal at her first prenatal visit, at about 6 weeks gestation. Born outside the U.S., she was healthy, with no evidence of varicella vaccination or disease. Her 2-year-old son had received one dose of vaccination against varicella in 2019, and he had no evidence of a rash when his mother developed varicella. Her source of exposure was unknown, though she worked at a large retail store, the authors noted.

By 22 weeks' gestation, the ultrasound revealed fetal abnormalities, including abnormal profile, abnormal "flexion of arms and legs with no movement," complex cardiac defect, and echogenic bowel. Amniotic fluid tested positive for VZV via PCR, but negative for other congenital abnormalities, including cytomegalovirus, toxoplasmosis, and parvovirus.

Labor was induced in April 2021. The newborn weighed 526 g at birth and had a heart rate of 60 beats/minute and an Apgar score of 1. The baby died 15 minutes after delivery. Antibody screening was negative for direct immunoglobulin IgG, according to Leung's team.

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