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Original Investigation
December 2016

Optical Coherence Tomography of Retinal Lesions in Infants With Congenital Zika Syndrome

Author Affiliations
  • 1Department of Ophthalmology, Altino Ventura Foundation, Recife, Brazil
  • 2Department of Ophthalmology, Pernambuco Eye Hospital, Recife, Brazil
  • 3Department of Ophthalmology and Visual Sciences, Paulista School of Medicine, Federal University of São Paulo, São Paulo, Brazil
  • 4Bascom Palmer Eye Institute, Miami, Florida
  • 5Department of Pediatric Neurology, Barão de Lucena Hospital, Recife, Brazil
JAMA Ophthalmol. 2016;134(12):1420-1427. doi:10.1001/jamaophthalmol.2016.4283
Key Points

Question  What are the optical coherence tomographic (OCT) findings in infants with congenital Zika syndrome who present with chorioretinal scars?

Findings  In this case series of 8 infants with congenital Zika syndrome and associated retinal abnormalities who were born in Pernambuco, Brazil, OCT imaging performed in 9 of the 16 eyes, of which 8 were affected, showed substantial neurosensory retinal thinning with discontinuation of the ellipsoid zone, choroidal thinning, and hyperreflectivity underlying areas of presumed atrophic retinal pigment epithelium.

Meaning  Zika virus can cause substantial damage to the retina, including the internal and external layers, and the choroid.


Importance  Zika virus (ZIKV) can cause severe changes in the retina and choroid that may result in marked visual impairment in infants with congenital Zika syndrome (CZS), the term created for a variety of anomalies associated with intrauterine ZIKV infection.

Objective  To evaluate the affected retinal layers in infants with CZS and associated retinal abnormalities using optical coherence tomography (OCT).

Design, Setting, and Participants  This cross-sectional, consecutive case series included 8 infants (age range, 3.0-5.1 months) with CZS. Optical coherence tomographic images were obtained in the affected eyes of 7 infants with CZS who had undergone previous ophthalmologic examinations on March 17, 2016, and in 1 infant on January 1, 2016. An IgM antibody-capture enzyme-linked immunosorbent assay for ZIKV was performed on the cerebrospinal fluid samples of 7 of the 8 infants (88%), and other congenital infections were ruled out.

Main Outcomes and Measures  Observation of retinal and choroidal findings in the OCT images.

Results  Among the 8 infants included in the study (3 male; 5 female; mean [SD] age at examination, 4.1 [0.7] months), 7 who underwent cerebrospinal fluid analysis for ZIKV had positive findings for IgM antibodies. Eleven of the 16 eyes (69%) of the 8 infants had retinal alterations and OCT imaging was performed in 9 (82%) of them. Optical coherence tomography was also performed in 1 unaffected eye. The main OCT findings in the affected eyes included discontinuation of the ellipsoid zone and hyperreflectivity underlying the retinal pigment epithelium in 9 eyes (100%), retinal thinning in 8 eyes (89%), choroidal thinning in 7 eyes (78%), and colobomatouslike excavation involving the neurosensory retina, retinal pigment epithelium, and choroid in 4 eyes (44%).

Conclusions and Relevance  Zika virus can cause severe damage to the retina, including the internal and external layers, and the choroid. The colobomatouslike finding seen in the OCT images relate to the excavated chorioretinal scar observed clinically.