IMPORTANCE: Zika virus infection can be prenatally passed from a pregnant woman 
to her fetus. There is sufficient evidence to conclude that intrauterine Zika 
virus infection is a cause of microcephaly and serious brain anomalies, but the 
full spectrum of anomalies has not been delineated. To inform pediatric 
clinicians who may be called on to evaluate and treat affected infants and 
children, we review the most recent evidence to better characterize congenital 
Zika syndrome.
OBSERVATIONS: We reviewed published reports of congenital anomalies occurring in 
fetuses or infants with presumed or laboratory-confirmed intrauterine Zika virus 
infection. We conducted a comprehensive search of the English literature using 
Medline and EMBASE for Zika from inception through September 30, 2016. 
Congenital anomalies were considered in the context of the presumed pathogenetic 
mechanism related to the neurotropic properties of the virus. We conclude that 
congenital Zika syndrome is a recognizable pattern of structural anomalies and 
functional disabilities secondary to central and, perhaps, peripheral nervous 
system damage. Although many of the components of this syndrome, such as 
cognitive, sensory, and motor disabilities, are shared by other congenital 
infections, there are 5 features that are rarely seen with other congenital 
infections or are unique to congenital Zika virus infection: (1) severe 
microcephaly with partially collapsed skull; (2) thin cerebral cortices with 
subcortical calcifications; (3) macular scarring and focal pigmentary retinal 
mottling; (4) congenital contractures; and (5) marked early hypertonia and 
symptoms of extrapyramidal involvement.
CONCLUSIONS AND RELEVANCE: Although the full spectrum of adverse reproductive 
outcomes caused by Zika virus infection is not yet determined, a distinctive 
phenotype-the congenital Zika syndrome-has emerged. Recognition of this 
phenotype by clinicians for infants and children can help ensure appropriate 
etiologic evaluation and comprehensive clinical investigation to define the 
range of anomalies in an affected infant as well as determine essential 
follow-up and ongoing care.