title: Expanded Spectrum of Congenital Ocular Findings in Microcephaly with Presumed Zika Infection.
abstract: To describe the ocular findings of 3 cases of suspected congenital Zika viral infection with microcephaly and maculopathy.
  
  
  
  Retrospective, consecutive case series.
  
  
  
  Three male infants born in northern Brazil whose mothers demonstrated a viral syndrome during the first trimester and who subsequently were born with microcephaly.
  
  
  
  Observational report of macular findings.
  
  
  
  Continued observation.
  
  
  
  Three male infants were born with microcephaly to mothers who had a viral syndrome during the first trimester of gestation in an area that subsequently has demonstrated epidemic Zika infection, a flavivirus related to Dengue. Ocular examination was performed. All 6 eyes demonstrated a pigmentary maculopathy ranging from mild to pronounced. In 4 eyes, well-delineated macular chorioretinal atrophy with a hyperpigmented ring developed. Three eyes demonstrated vascular tortuosity and 2 eyes demonstrated a pronounced early termination of the retinal vasculature on photographic evaluation. Two eyes demonstrated a washed out peripheral retina with a hypolucent spot. One eye had scattered subretinal hemorrhages external to the macula. Finally, 1 eye demonstrated peripheral pigmentary changes and clustered atrophic lesions resembling grouped congenital albinotic spots (polar bear tracks).
  
  
  
  Zika virus has been linked to microcephaly in children of mothers with a viral syndrome during the first trimester of pregnancy. Ocular findings previously described a pigmentary retinopathy and atrophy that now can be expanded to include torpedo maculopathy, vascular changes, and hemorrhagic retinopathy. Ophthalmologic screening guidelines need to be defined to determine which children would benefit from newborn screening in affected regions.
authors.affiliation: Ophthalmic Telemedicine Santa Casa of São Paulo, São Paulo, Brazil.
authors.forename: Homero Augusto de
authors.initials: HA
authors.lastname: Miranda
authors.affiliation: Ophthalmic Telemedicine Santa Casa of São Paulo, São Paulo, Brazil.
authors.forename: Marcelo Cavalcante
authors.initials: MC
authors.lastname: Costa
authors.affiliation: Ophthalmic Telemedicine Santa Casa of São Paulo, São Paulo, Brazil.
authors.forename: Maria Auxiliadora Monteiro
authors.initials: MAM
authors.lastname: Frazão
authors.affiliation: Department of Ophthalmology, University of São Paulo, São Paulo, Brazil.
authors.forename: Natália
authors.initials: N
authors.lastname: Simão
authors.affiliation: Department of Ophthalmology, University of Santo Amaro, São Paulo, Brazil.
authors.forename: Sandra
authors.initials: S
authors.lastname: Franchischini
authors.affiliation: Department of Ophthalmology, Horngren Family Vitreoretinal Center, Byers Eye Institute, Stanford University School of Medicine, Palo Alto, California. Electronic address: dariusm@stanford.edu.
authors.forename: Darius M
authors.initials: DM
authors.lastname: Moshfeghi
mesh_terms: D001938:Brazil; D003317:Corneal Dystrophies, Hereditary / Q000175:diagnosis; D015828:Eye Infections, Viral / Q000175:diagnosis* / Q000821:virology; D005260:Female; D005865:Gestational Age; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D008831:Microcephaly / Q000175:diagnosis* / Q000821:virology; D011247:Pregnancy; D011251:Pregnancy Complications, Infectious / Q000175:diagnosis* / Q000821:virology; D012164:Retinal Diseases / Q000175:diagnosis* / Q000821:virology; D012166:Retinal Hemorrhage / Q000175:diagnosis; D012171:Retinal Vessels / Q000473:pathology*; D012189:Retrospective Studies; D000071243:Zika Virus Infection / Q000175:diagnosis* / Q000821:virology