Congenital varicella syndrome

Varicela congénita

Infection with the varicella zoster virus (VZV) during pregnancy is encompassed within the spectrum of STORCH infections (acronym for syphilis, toxoplasmosis, others, rubella, cytomegalovirus and herpes virus), due to its teratogenic potential and impact on fetal well-being. Although only 2% of patients with VZV infection in pregnancy had fetuses with congenital varicella syndrome, its high morbidity and mortality makes it necessary to be aware of the proper use of prevention strategies. In our case, a 27-year-old mother gives birth to a newborn with multiple skeletal anomalies and typical lesions of the skin, associated with cardiac malformations and visceral calcifications diagnosed by imaging. Congenital varicella syndrome was diagnosed upon clinical and paraclinical confirmation of infection. The patient presented a torpid evolution from birth, with multidrug-resistant pneumonia, necrotizing enterocolitis and rapid deterioration of his respiratory pattern leading to death. Currently, vaccination and the use of post-exposure immunoglobulin are the main allies for the prevention of this syndrome in newborns. 

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