Varicella embryopathy.
Varicella embryopathy is a rare entity afflicting infants born to mothers who have contracted varicella during the first 20 weeks of pregnancy. The teratogenicity of varicella has not been established from epidemiologic studies, but isolated case reports describe characteristic malformations following early maternal infection. We describe a male neonate delivered at 40 weeks' gestation to a 26-year-old grava 2, para 2 mother who developed varicella during the first trimester. The infant lived 7 days and died of bronchopneumonia. At postmortem examination there was growth retardation, multiple cicatricial skin lesions, flexion contractures of all major joints, hypoplastic right diaphragm, bilateral hydroureters and mucosal fibrosis of the trachea, as well as intestinal fibrosis and colonic stricture. The brain contained areas of cystic necrosis involving the frontal, parietal, temporal, and occipital lobes, with generalized ventriculomegaly. The midbrain, pons, and medulla were hypoplastic. There was denervation atrophy of muscles of the lower limbs and loss of dorsal root ganglia as well as of neurons of the anterior horn of the spinal cord. The cerebral white matter was degenerated, with proliferation of reactive astrocytes. Chorioretinitis was not observed. Immunocytochemical stains using two commercially available antivaricella antibodies were negative in all tissues examined. The sporadic nature and pathogenesis of the varicella embryopathy, which may have been caused by focal defects in the fetal T-cell immune response, are discussed.