Congenital herpes simplex

The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.

Orpha Number: 293

Definition

Congenital herpes virus infection is a group of anomalies that an infant may present as a result of maternal infection and subsequent foetal infection with herpes virus. This virus causes recurrent cutaneous infections in adults, often involving the lips or the genitalia. Herpes infections in other organs, such as the liver or central nervous system, are less frequent.

Clinical description

Skin lesions and scars, chorioretinitis, microcephaly, hydrencephaly, and microphthalmia have been described in infants with in utero primary herpes virus infections, but a causal relationship between these manifestations and the infection could not be established. Pregnancy complications including preterm delivery, intrauterine growth retardation, and neonatal infection have been attributed to the herpes virus.

Etiology

There are two types of virus (HSV-1 and HSV-2) and both appear to have the same activity. However, type 1 infections more commonly involve the oral cavity, whereas type 2 infections more commonly involve the genital region. It has been suggested that latent HSV-2 infections occur in as many as 20 to 40% of women. Exposure of the foetus to herpes virus at the time of delivery carries a serious risk of infection for the newborn. This risk may be high during the initial infection of the mother (33 to 50%), but probably does not exceed 3% in cases of reinfection because of the protective effect of maternal antibodies.

Differential diagnosis

It remains difficult to distinguish between cases with a primary infection and those with a previously undetected recurrent infection that has become symptomatic during pregnancy.

Management and treatment

In spite of described cases of serious foetal illness, primary infection with herpes virus during pregnancy is not an indication for therapeutic abortion, and the use of antivirals in the treatment of primary herpes infections during gestation has been recommended. Some authors recommend caesarean delivery for women with first-episode genital herpes lesions at the time of delivery.

Visit the Orphanet disease page for more resources.