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June 1984

Picture of the Month

Author Affiliations

Contributed from the Department of Pediatrics, Hialeah (Fla) Hospital.

Am J Dis Child. 1984;138(6):603-604. doi:10.1001/archpedi.1984.02140440087024

Denouement and Discussion 

Congenital Varicella Syndrome 

Manifestations  Major manifestations involve the limbs, eyes, skin, and brain. The limb defects vary but consist mainly of hypoplasia and paralysis with muscular atrophy. Hand abnormalities include hypoplastic or missing fingers. Horner's syndrome is a common finding, along with chorioretinitis, cataracts, and optic atrophy. There are cutaneous scars or cicatricial skin that are the residuum of earlier infected bullous skin lesions. Central nervous system involvement includes seizures, encephalitis, cortical atrophy, and mental retardation.

Origin  The syndrome occurs in infants whose mothers have been infected with the varicella-zoster virus usually between the eighth and 19th week of gestation, but the infection can occur later. The neurotropic properties of the virus are responsible for the birth defects. This occurs when the virus invades the brachial or lumbar plexus (limb defects), cervical sympathetic nerves (Horner's syndrome), and brain (encephalitis). Varicella-zoster virus infection can be confirmed by the

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