Congenital Varicella syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Aravind Kuchkuntla, M.B.B.S[2]

Synonyms and keywords: Fetal varicella syndrome, Congenital varicella-zoster syndrome, Varicella embryo-fetopathy, Varicella embryopathy, Varicella fetopathy, Fetal varicella-zoster syndrome


Congenital varicella syndrome is a rare disease resulting from Varicella Zoster virus(VZV) infection during the period of gestation. Viremia during the primary infection can result in transplacental transmission of the infection to the developing fetus. An estimated 25% of fetuses get infected with varicella infection when mother has a varicella infection during thepregnancy but the risk of developing congenital varicella syndrome is around 2%, therefore majority of the outcomes are normal newborns. Patients with primary infection before 20 weeks of gestation are at a higher risk of developing the severe form of infection, affecting the eyes, limbs, skin and the central nervous system. Diagnosis requires a documented history of primary infection in the mother and serial ultrasound demonstrating features suggestive of congenital varicella syndrome. There is no definitive treatment, termination of pregnancy in fetuses with severe features is recommended. Vaccination to prevent maternal varicella infection and proper counseling to avoid contact with infected people are important for the management options to reduce the incidence of congenital varicella syndrome.

Historical Perspective


There is no classification for congenital varicella syndrome.



Epidemiology and Demographics

Congenital varicella syndrome is a rare disease with over a 100 cases reported in literature.[11]


Congenital varicella syndrome is caused by Varicella zoster virus (VZV), a human alpha herpes virus.

Differentiating Congenital Varicella Syndrome From Other Diseases

The most important congenital infections, which can be transmitted vertically from mother to fetus are the TORCH infections. These infections have overlapping features and hence, must be differentiated from congenital varicella syndrome:[12][13]

Congenital Infection Cardiac Findings Skin Findings Ocular Findings Hepatosplenomegaly Hydrocephalus Microcephaly Intracranial calcifications Hearing deficits
Congenital Varicella syndrome -
  • Cicatrical Skin Lesions
  • Skin edema
Toxoplasmosis Diffuse intracranial calcifications
Congenital Syphillis
Cytomegalovirus (CMV) Periventricular calcifications
Herpes simplex virus (HSV)
Parvovirus B19

Natural History, Prognosis and Complications

Natural History

VZV infection during pregnancy results in a normal newborn birth in majority of the patients, however, in a few patients it can result in congenital varicella syndrome or neonatal varicella or clinical zoster during infancy, the outcomes are dependent on the gestational age of fetus at the time of infection. Early gestational period infection via the transplacental route can result in congenital varicella syndrome resulting in a misscarriage, abortion or a newborn with features affecting the limbs, eyes, central nervous system, autonomic nervous system and present with features such as low birth weight, cutaneous scarring, limb hypoplasia, microcephaly, cortical atrophy, chorioretinitis and cataracts.[14][15]


Severe infection of the fetus can result in an abortion. Infants born with signs of congenital varicella syndrome have poor prognosis and die during the first few months of life.[16][14] Infants with milder symptoms can have a normal development and good prognosis.[17][18]


Congenital varicella infection can result in the following complications:[19]


History and Symptoms

Symptoms of primary infection in Mother :

Symptoms in the Neonate

Physical Examination

Physical examination findings suggestive of congenital varicella syndrome include:[24][25]

Physical examination findings in congenital varicella syndrome
  • Cicatricial lesions( Zig-Zag scarring in dermatomal distribution)[26]
  • Hypopigmentation
Central Nervous System
Musculoskeletal system
  • Limb hypoplasia
  • Muscle hypoplasia
Systemic Manifestations

Table adopted from varicella in fetus and newborn[30]

Laboratory Findings

The diagnosis of congenital varicella syndrome is based on a documented history of varicella infection during the pregnancy and the presence of fetal manifestations on ultrasound.[31]

Key findings for diagnosis of congenital varicella syndrome
Fetus / Neonatal Findings
  • Presence of characteristic cicatrical skin lesions, eye lesions, neurological deficits, limb abnormalities
Proof of Intrauterine Varicella infection

Table adopted from Herpes simplex and varicella-zoster virus infections during pregnancy: current concepts of prevention, diagnosis and therapy. Part 2: Varicella-zoster virus infections[32]

Diagnosis of primary infection in the mother : In pregnant women diagnosis of a primary infection requires a combination of clinical manifestations and series of diagnostic tests. The tests are performed on the samples from the vesicular skin lesions and include the following:

  • Culture for VZV, but takes 10 to 12 days to obtain the results.
  • Direct fluroscent antigen staining with monoclonal antibodies detects the VZV glycoproteins in the cells.
  • PCR for VZV DNA
  • Serological tests are not useful for the dectection of primary infection in the mother as it takes time for the IgG antibodies to be produced aganist VZV.

Prenatal Diagnosis

Imaging Studies



Prenatal MRI is a useful investigation to assess the extent of CNS involvement and to confirm the findings of ultrasound.[39]

Postnatal Diagnosis


Medical Therapy

  • In patients with established infection early in the period of gestation, regular follow up and ultrasound examination is recommended.[32]
  • Termination of pregnancy is indicated in cases with the presence of definitive signs of congenital varicella infection.
  • There is insufficient evidence regarding the prevention of transmission and treatment of congenital varicella syndrome with IgG immunoglobulins and acyclovir.[6]
  • Varicella infection doesnot progress postnatally, so treatment with acyclovir is not indicated.[41]
  • Isolation is recommended in patients with active skin lesions.

Surgical Therapy

There are no surgical therapies for treatment of congenital varicella syndrome.


Primary Prevention

Secondary Prevention


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