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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

Overview

Historical Perspective

  • In 1935, the first case resembling neonatal HSV, was described with the presence of intranuclear inclusion bodies in a premature infant in the liver and the adrenals.[1]

Pathophysiology

Pathogenesis

The risk for transmission to the neonate from an infected mother is high (30%–50%) among women who acquire genital herpes near the time of delivery and low (<1%) among women with prenatal histories of recurrent herpes or who acquire genital HSV during the first half of pregnancy

Transmission of infection

  • Exposure to the fetus from active genital herpes lesions during delivery, accounts for majority of neonatal herpes cases.
  • Intrauterine infection accounts for 5% of cases with neonatal herpes simplex.
  • Postnatal trasmission by contact with HSV shed from infected patients. It accounts for 10% of the cases.

Epidemiology and Demographics

  • The annual incidence of neonatal herpes is estimated to be 10 cases per 100,000 livebirths.

Causes

  • 85% of cases are caused by HSV type I
  • 1%% of cases are caused by HSV type II

Differentiating Congenital Varicella Syndrome From Other Diseases

Natural History, Prognosis and Complications

Natural History

Complications

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

Treatment

Medical Therapy

Surgical Therapy

Prevention

Primary Prevention

  • Women without known genital herpes should be counseled to abstain from vaginal intercourse during the third trimester with partners known or suspected of having genital herpes.
  • Pregnant women without known orolabial herpes should be advised to abstain from receptive oral sex during the third trimester with partners known or suspected to have orolabial herpes.
  • Type-specific serologic tests may be useful for identifying pregnant women at risk for HSV infection and guiding counseling regarding the risk for acquiring genital herpes during pregnancy.
  • All pregnant women should be asked whether they have a history of genital herpes. At the onset of labor, all women should be questioned carefully about symptoms of genital herpes, including prodromal symptoms, and all women should be examined carefully for herpetic lesions. Women without symptoms or signs of genital herpes or its prodrome can deliver vaginally. Although cesarean delivery does not completely eliminate the risk for HSV transmission to the neonate, women with recurrent genital herpetic lesions at the onset of labor should deliver by cesarean delivery to reduce the risk for neonatal HSV infection.

Secondary Prevention

References

  1. Hass GM (1935). "Hepato-Adrenal Necrosis with Intranuclear Inclusion Bodies: Report of a Case". Am J Pathol. 11 (1): 127–142.5. PMC 1910753. PMID 19970188.