Congenital varicella is caused by the varicella zoster virus (VZV), which is acquired from a mother who contracts the virus for the first time during pregnancy. The time in pregnancy during which the mother contracts VZV has important implications.
Primary infection is rare, as 90% of pregnant women are immune.
Congenital varicella syndromes
| Syndrome | Timeline |
|---|---|
| Fetal varicella syndrome | Infection during 7 – 28 weeks of gestation. VZV affects neuromuscular development. This can be diagnosed clinically based on the mother’s history of VZV infection (VZV embryopathy). Babies are born stable since they acquire maternal antibodies, but insults in utero from the virus will manifest |
| Neonatal varicella syndrome | Infection within 2 weeks before delivery (highest risk at 5 days before or 2 days after delivery). Involves a progressive vesicular rash. Active viral infection occurs since insufficient immunity is passed to the fetus/neonate |
- Signs and symptoms of fetal varicella syndrome
- Limb hypoplasia (clavicle, scapula)
- Rib anomalies
- Scoliosis
- Umbilical hernia
- Microcephaly
- Cortical atrophy
- Seizures
- Chorioretinitis
- Mircropthalmia
- Hypoplasia of the optic disk
- Circatricial cutaneous scarring (in a dermatomal distribution)
- Signs and symptoms of neonatal varicella syndrome
- Progressive vesicular rash
- Ill-appearing
- Temperature instability
- Investigations
- VZV titres
- Positive results indicate exposure
- VZV titres
- Treatment of fetal varicella syndrome
- Supportive
- Since there is no active viral infection
- Supportive
- Treatment of neonatal varicella syndrome
- VZIG
- This is the best treatment
- Intravenous acyclovir
- VZIG
- Prevention
- Zoster immunoglobulin for:
- Susceptible pregnant women who had contact with suspected cases of varicella
- Infants whose mother develops varicella during the last 7 days of pregnancy or the first 14 days after delivery
- Zoster immunoglobulin for:
- Prognosis
- 30% die in the first month of life