Congenital diffuse necrotizing herpetic retinitis.

Neonatal Herpes simplex infections are usually contracted from the birth canal, and the systemic lesions develop several days to weeks after delivery. We present the clinicopathologic findings in a newborn with a prenatal diagnosis of hydrocephalus who died at 1 day of age. Severe liquefaction necrosis and foci of calcification were present in the brain, adrenal glands, and retina. Cowdry type A intranuclear inclusions were present in the adrenal glands and retina. There was no clinical evidence of genital herpes in either parent. This is the first documented case of in utero transmission of Herpes simplex infection, confirmed by the polymerase chain reaction, and causing fulminant necrotizing retinitis and encephalitis.