Varicella syndrome, congenital P35.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Congenital varicella syndrome; Congenital Varicella Syndrome; Fetal varicella syndrome

Definition
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Congenital varicella syndrome is either a multisystemic malformation or the infestation of only a single organ in varicella infection of the mother during early pregnancy (usually between the 13th and 20th week of pregnancy). In very rare cases, zoster can also be the cause of this complicated malformation syndrome (Ahn KH et al. 2016).

Pathogen
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Occurrence/Epidemiology
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The risk of falling ill is highest in the 1st trimester (4.9%) and is 0.8/100,000 live births.

Etiopathogenesis
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Diaplacental infection with varicella zoster virus.

Clinical features
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Embryonic malformations, especially hypoplasia of the limbs, cerebral dysfunctions and zosteriform scars In 60% of cases the eyes (microphthalmia, cataract, chorioretinitis) and in 30% the CNS (microcephalus, cortical atrophy, encephalitis) are affected. There are also developmental delays and urogenital, gastrointestinal and cardiovascular abnormalities. The mortality rate in the first months of life is 30%.

Internal therapy
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Aciclovir (e.g. Zovirax) i.v.

Note(s)
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In the case of a varicella infection that occurs 5-7 days before birth until 2 days after birth, the newborn is most at risk(neonatal varicella), as no protective specific antibodies are formed at this time.

Literature
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  1. Ahn KH et al (2016) Congenital varicella syndrome: A systematic review. J Obstet Gynaecol 36:563-566.
  2. Andrews EB et al (1988) Acyclovir in Pregnancy Registry. An observational epidemiological approach. On J Med 85: 123-128
  3. Brunell PA (1993) Varicella in pregnancy, the fetus and the newborn: problems in management. J Infect Dis 166: 42-47
  4. Joffe A (1992) Risk of acyclovir therapy in pregnant adolescent. J Pediatr 120: 627-633
  5. Mazzella M et al (2003) Severe hydrocephalus associated with congenital varicella syndrome. CMAJ 168: 561-563
  6. Müllegger RR et al (2010) Skin infections during pregnancy. dermatologist 61: 2066-2069
  7. Sauerbrei A et al (2003) Intracerebral varicella-zoster virus reactivation in congenital varicella syndrome. Dev Med Child Neurol 45: 837-840
  8. Smego RA et al (1991) Use of acyclovir for varicella pneumonia during pregnancy. Obstetric Gynecol 78: 1112-1116

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Last updated on: 29.10.2020