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Zika Embryopathy

29 May 2016


We present the first congenital case of Zika virus (ZIKV) infection in the United States, confirmed by high ZIKV IgM antibody titers in serum and cerebrospinal fluid (CSF). The phenotypic features of the patient fall within fetal brain disruption sequence, characterized by collapse of the calvarium occurring in the second half of pregnancy, suggesting impaired brain development. Brain imaging revealed an almost agyric brain with diffuse parenchymal calcifications in the cortex and basal ganglia, hydrocephalus ex vacuo, and cerebellar hypoplasia with cystic changes. Ophthalmologic examination revealed macular pigment stippling, optic nerve atrophy and fundal hypopigmentation. Other organ systems, including liver, heart and bone marrow, were not affected. The patient had progressive neurologic deterioration in the first month of life. There is reasonable concern that there is ongoing viral replication in the fetal and newborn brain, necessitating close follow up of these infants. The discovery of ZIKV infection in human fetal brain tissue and serologic confirmation of IgM antibody to ZIKV in serum and CSF of surviving neonates proves the vertical transmission of ZIKV. Therefore, ZIKV has joined the group of TORCH complex pathogens. The severity of ZIKV embryopathy, its apparent high incidence and the potential for rapid intercontinental spread constitute a major international public health emergency.

Fetal ultrasounds at 20 weeks gestation: (A) 3D reconstruction. Note the borderline microcephalic appearance, with normal appearing facies and normal appearing forehead. (B) Note the asymmetric lateral ventriculomegaly, scattered parenchymal echogenic foci with no acoustic shadowing, and normal extraaxial spaces.

Culjat M, Darling S, Nerurkar VR, Ching N, Kumar M, Min SK, Wong R, Grant L, Melish ME. Clinical and imaging findings in an infant with Zika embryopathy. Clinical Infectious Diseases 2016 May 18. pii: ciw324.