ontracted through the bite of an infected mosquito or through sexual or other modes of transmission, Zika virus (ZIKV) infection can be prenatally passed from mother to fetus. 1 The virus was first identified in the region of the Americas in early 2015, when local transmission was reported in Brazil. 2 Six months later, a notable increase in the number of infants with congenital microcephaly was observed in northeast Brazil. 3,4 Clinical, epidemiologic, and laboratory evidence led investigators to conclude that intrauterine ZIKV infection was a cause of microcephaly and serious brain anomalies. [5][6][7] However, as with other newly recognized teratogens, these features likely represent a portion of a broader spectrum.A comprehensive review of the English literature, identified by searching Medline and EMBASE for Zika from inception through Sep-tember 30, 2016, was done to better characterize the spectrum of anomalies in fetuses and infants with presumed or laboratoryconfirmed ZIKV infection. A constellation of anomalies that is both consistent and unique, called congenital Zika syndrome (CZS), has emerged but specific components and presumed pathogenetic mechanisms previously have not been well-delineated. [8][9][10] Zika virus infection has spread to more than 45 countries in the Americas and 3 US territories, and, most recently, local transmission was confirmed in the continental United States in the state of Florida. 11 Mosquito-borne transmission of ZIKV in other areas of the United States is possible based on the estimated range of its vectors (Aedes aegypti and Aedes albopictus). 12 Recognition of the CZS phenotype by pediatric clinicians will help ensure appropriate and timely evaluation and follow-up of affected infants.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 w...
