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Incidence and Severity of Congenital and Neonatal Varicella in Australia, Schemes and Mind Maps of Literature

Data on the incidence and severity of congenital and neonatal varicella in australia from 1995 to 1997. It includes case studies of affected infants and their mothers, as well as statistics on the annual incidence and gender, age, and severity of the cases. The document also discusses the potential benefits of vaccinating non-immune women prior to pregnancy.

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2021/2022

Uploaded on 09/27/2022

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Congenital and Neonatal Varicella
Background
Varicella in pregnancy may cause spontaneous abortion or premature
delivery. If the infection occurs in the first 20 weeks of gestation, the fetus
may be affected. The literature suggests the risk of embryopathy is
approximately 0.4% when maternal infection occurs before 13 weeks and 2%
when it occurs between 13 and 20 weeks gestation. Fetuses exposed to
varicella also have between 0.8 and 1.7% risk of developing herpes zoster in
the first two years of life. When maternal varicella occurs in the four weeks
before delivery, fetal infection rate is high and infants may develop varicella
after birth. Postnatal exposure may also cause neonatal varicella, which has
been considered to carry a high mortality, although no information on its
effects had been collected in Australia prior to this study.
Objectives
To estimate the incidence and severity of congenital and neonatal varicella in Australia
To estimate the potential benefits of vaccinating non-immune women prior to pregnancy
Case definition
Congenital varicella (including varicella embryopathy)
Any stillborn, newborn infant or child up to the age of 2 years who, in the opinion of the
notifying paediatrician, has definite or suspected congenital varicella, with or without
defects, based on history, clinical and laboratory findings.
Neonatal varicella
Any infant with clinical or laboratory-confirmed varicella, with onset in the first month of life,
and without features of varicella embryopathy. The infection may result from peripartum
maternal infection or postnatal exposure.
Results - 1997
In 1997 there were three confirmed cases of congenital varicella and 13 confirmed cases of
neonatal infection. Cases came from New South Wales (6), Queensland (5), Western
Australia (3) and Victoria (2). Maternal ages ranged from 21 to 34 years and seven cases
were first children.
Congenital varicella
In Western Australia, one pregnancy was terminated at 21 weeks because of hydrocephalus
following maternal varicella at 11 weeks gestation. Skin scars were found at autopsy.
Another mother had varicella at 16 weeks gestation in her second pregnancy. The child was
severely affected with neurological damage (encephalopathy, bulbar palsy, neurogenic
bladder), colonic atresia and skin scars, but had no eye defects. Varicella virus was isolated
from the infant, who also developed shingles at six weeks of age. In NSW, a mother had
varicella at eight weeks gestation. The infant was severely affected with limb, skeletal, heart
and nervous system defects, but had normal eyes. The child died at 12 days of age and at
autopsy varicella virus was found in the brain by polymerase chain reaction.
Neonatal varicella
Details of the 13 neonatal cases are shown in Table 12. Five mothers had varicella before
delivery, six had varicella after delivery and one had a varicella contact at term, but had no
illness. In one infant, the source of infection was unknown. Use of zoster immune globulin
(ZIG) in 10 children was associated with mild illness. Two of these children also received
acyclovir. One infant who did not receive ZIG and whose mother had contact with varicella at
term, but no illness, was the only case with a moderately severe illness. He was treated with
acyclovir.
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Congenital and Neonatal Varicella

Background

Varicella in pregnancy may cause spontaneous abortion or premature

delivery. If the infection occurs in the first 20 weeks of gestation, the fetus

may be affected. The literature suggests the risk of embryopathy is

approximately 0.4% when maternal infection occurs before 13 weeks and 2%

when it occurs between 13 and 20 weeks gestation. Fetuses exposed to

varicella also have between 0.8 and 1.7% risk of developing herpes zoster in

the first two years of life. When maternal varicella occurs in the four weeks

before delivery, fetal infection rate is high and infants may develop varicella

after birth. Postnatal exposure may also cause neonatal varicella, which has

been considered to carry a high mortality, although no information on its

effects had been collected in Australia prior to this study.

Objectives

  • To estimate the incidence and severity of congenital and neonatal varicella in Australia
  • To estimate the potential benefits of vaccinating non-immune women prior to pregnancy Case definition Congenital varicella (including varicella embryopathy) Any stillborn, newborn infant or child up to the age of 2 years who, in the opinion of the notifying paediatrician, has definite or suspected congenital varicella, with or without defects , based on history, clinical and laboratory findings. Neonatal varicella Any infant with clinical or laboratory-confirmed varicella, with onset in the first month of life, and without features of varicella embryopathy. The infection may result from peripartum maternal infection or postnatal exposure. Results - 1997 In 1997 there were three confirmed cases of congenital varicella and 13 confirmed cases of neonatal infection. Cases came from New South Wales (6), Queensland (5), Western Australia (3) and Victoria (2). Maternal ages ranged from 21 to 34 years and seven cases were first children. Congenital varicella In Western Australia, one pregnancy was terminated at 21 weeks because of hydrocephalus following maternal varicella at 11 weeks gestation. Skin scars were found at autopsy. Another mother had varicella at 16 weeks gestation in her second pregnancy. The child was severely affected with neurological damage (encephalopathy, bulbar palsy, neurogenic bladder), colonic atresia and skin scars, but had no eye defects. Varicella virus was isolated from the infant, who also developed shingles at six weeks of age. In NSW, a mother had varicella at eight weeks gestation. The infant was severely affected with limb, skeletal, heart and nervous system defects, but had normal eyes. The child died at 12 days of age and at autopsy varicella virus was found in the brain by polymerase chain reaction. Neonatal varicella Details of the 13 neonatal cases are shown in Table 12. Five mothers had varicella before delivery, six had varicella after delivery and one had a varicella contact at term, but had no illness. In one infant, the source of infection was unknown. Use of zoster immune globulin (ZIG) in 10 children was associated with mild illness. Two of these children also received acyclovir. One infant who did not receive ZIG and whose mother had contact with varicella at term, but no illness, was the only case with a moderately severe illness. He was treated with acyclovir.

Table 12 Neonatal varicella in Australia, 1997 Time of mother’s varicella contact Gender Rash onset (days of age) Disease severity Age baby given ZIG Time mother given ZIG Acyclovir for baby Before delivery* M F F F F

at birth 13 1

at birth day 1 at birth

day 2 at infection at contact At term M 21 ++ – Yes After delivery (^) F F F F M M

day 1 day 2 day 5 day 3 day 11 day 2 Yes Yes No known source of infection (^) NA 21 + – Severity of disease: + mild ++ moderate ZIG zoster immune globulin NA not available Overview 1995- Over this three year period, 75 notifications were received and 71 (95%) questionnaires were returned. From these, 51 confirmed cases of congenital and neonatal varicella infection, including one termination, were identified. The annual incidence, shown in Table 13, is expressed per 100,000 live births and excludes the termination of pregnancy. Of the 50 incident cases, 20 were males, 26 females and the sex of four remains unknown. Maternal ages ranged from 18 to 39 years. No cases were reported from Tasmania. Table 13 Annual incidence of congenital and neonatal varicella, 1995- Congenital varicella Incidence (95% CI) Neonatal varicella Incidence (95% CI) 1995* 3 1.2 (0.3-3.7) 15 5.9 (3.4- 9.9) (^1996) 1 0.4 (0.02-2.6) 16 6.3 (3.7-10.5) (^1997) 2 #^ 0.8 (0.3-1.8) 13 5.2 (2.9- 9.1) Total 6 0.8 (0.3-1.8) 44 5.8 (4.3- 7.8)

  • national incidence per 100,000 live births per annum

(^) the termination of pregnancy was excluded

Congenital varicella The incidence of congenital varicella did not vary over the study period (Table 13). Clinical severity of cases varied (Table 14). Severe malformations were seen both following maternal infection in the first 13 weeks as well as later in the pregnancy. Timing of maternal infection ranged from eight to 26 weeks gestation. There were too few cases to determine whether the risk of congenital malformation was significantly lower after maternal infection in the first 13 weeks than between 13 and 20 weeks gestation, as has been shown in larger series. In the literature, there are few reported cases of congenital varicella following maternal infection after 20 weeks’ gestation. It is therefore interesting that the infant exposed to maternal infection at 26 weeks gestation was severely affected. Conversely, two infants whose mothers had varicella at 13 and 18 weeks were born without defects. They developed zoster at five and four months of age respectively, with complete recovery. One child with defects, whose mother was infected at 12 weeks gestation, also developed herpes zoster at the age of 10 weeks.