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A rare congenital nongenetic disorder resulting from maternal transmission of varicella in the first and second trimesters of pregnancy manifesting with cutaneous, neurologic, and limb involvement.

Fetal Varicella Syndrome; Varicella Virus Antenatal Infection; Varicella Embryopathy; Varicella Fetopathy.

Because most women of child-bearing age (>90%) have antibodies (IgG) against the varicella-zoster virus, infection during pregnancy is rare. Approximately 2% of babies suffer from the syndrome following maternal varicella infection in the first 20 weeks of pregnancy (greatest risk between weeks 8 to 20). In industrialized countries, approximately 0.5-3:1000 pregnancies are affected. However, a significantly higher rate is expected in developing countries. Approximately 100 cases have been described in the medical literature. Two thirds of affected neonates are females.

Not a genetic disorder.

The route of infection most likely is transplacental and leads to fetal viremia, although ascending infection from the cervix uteri is possible. Organ injuries reflect the neurotropic nature of the varicella virus.

Based on the history of maternal chickenpox in the first or second trimester of pregnancy and clinical and serologic (IgM-specific antibodies, persistence of IgG antibodies beyond the first 6 months of life) findings in the neonate with low birth weight and multiple, characteristic abnormalities (see Clinical Aspects).

At any stage of pregnancy, severe maternal chickenpox may result in fetal death. The features of the disorder may involve the skin (cicatricial lesions associated with underlying tissue hypoplasia; the lesions appear depressed and hyperpigmented and often have an irregular border; certain skin areas [mainly arms and legs in dermatomal distribution] may exhibit thickened and hypertrophic scars with induration, redness, and inflammation of the surrounding skin), the eyes (microphthalmia, microcornea, anisocoria, chorioretinitis, optic nerve atrophy and hypoplastic optic disc, cataract, corneal opacities, enophthalmia, strabismus, and nystagmus), the central nervous system (cortical and spinal cord atrophy, cerebellar aplasia, seizures, encephalitis, deafness, generalized hypotonia, hyperreflexia, intermittent myoclonic seizures, Horner syndrome, developmental delay, limb paresis), the musculoskeletal system (signs of hypoplasia and/or reduction deformities of the limbs including fingers and toes, hypoplastic mandible, clavicle, scapula, ribs, muscles), the gastrointestinal tract (hepatic calcifications, gastroesophageal reflux, duodenal stenosis, jejunal dilatation, chronic constipation, microcolon, colonic atresia, anal sphincter dysfunction), the urogenital tract (vesicoureteral reflux, neurogenic bladder), and the cardiovascular system. Intrauterine growth retardation is common. Almost one third of these patients die in the first months of life.

The patient is infectious, so avoid contact with medical personnel who is at risk for contracting varicella or whose immune status is unknown. Isolation from other patients is required because varicella is highly contagious. Evaluate the patient for less common manifestations, such as cardiovascular abnormalities (in 8% of patients) and maintain a low threshold for echocardiography. Obtain a full medical history and evaluate neurologic development, seizure control, and extent of paresis. Laboratory investigations should include a complete blood count, serum concentrations of electrolytes, creatinine, and urea (because vesicoureteral reflux may affect kidney ...

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