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Original Investigation
April 13, 2017

Ophthalmic Manifestations of Congenital Zika Syndrome in Colombia and Venezuela

Author Affiliations
  • 1Department of Vitreoretinal Surgery, Clínica de Ojos, Maracaibo, Venezuela
  • 2Vitreoretinal Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia
  • 3Department of Glaucoma, Clínica de Ojos, Maracaibo, Venezuela
  • 4Clínica Oftalmológica Peñaranda, Cúcuta, Colombia
  • 5Department of Pediatric Ophthalmology, Clínica de Ojos, Maracaibo, Venezuela
  • 6Division of Neurosciences, Department of Biomedical Sciences, University of Texas Rio Grande Valley School of Medicine, Brownsville
  • 7Retina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland
  • 8Johns Hopkins Zika Center, Johns Hopkins University School of Medicine, Baltimore, Maryland
JAMA Ophthalmol. Published online April 13, 2017. doi:10.1001/jamaophthalmol.2017.0561
Key Points

Question  What are the ophthalmic manifestations in infants with microcephaly associated with clinically diagnosed Zika virus in Colombia and Venezuela?

Findings  In this case series of 43 patients, substantial anterior and posterior segment abnormalities, including congenital glaucoma, were found in 5 (12%) and macular or optic nerve abnormalities in 38 (88%).

Meaning  These findings suggest that all infants with microcephaly associated with clinically diagnosed Zika virus should undergo evaluation for ophthalmic findings.


Importance  The ocular manifestations and sequelae of Zika virus infection are not well known. Recently, the World Health Organization changed the declaration of Zika as a public health emergency and designated the viral outbreak and related microcephaly clusters as a long-term program of work. This change indicates the urgent need to evaluate and document ophthalmic manifestations in patients for timely management of this disease. In addition, confirmation whether the public health problem in Brazil extends to other regions in South America is needed.

Objective  To report the ocular manifestations of congenital Zika syndrome with microcephaly in Colombia and Venezuela.

Design, Setting, and Participants  This prospective case series included 43 patients from 2 ophthalmic centers in Colombia and Venezuela who underwent evaluation from October 1, 2015, through June 30, 2016, and were clinically diagnosed with congenital Zika syndrome. Twenty patients were Hispanic; 13, African; 8, white; and 2, Native American.

Interventions  Ophthalmic and systemic evaluations and serologic testing were performed on all infants. Patients underwent external ocular examination and dilated ophthalmoscopy. Serologic testing ruled out toxoplasmosis, rubella, cytomegalovirus, syphilis, and human immunodeficiency virus.

Main Outcomes and Measures  Ophthalmic manifestations of congenital Zika syndrome.

Results  Of the 43 patients included in this series (28 female and 15 male), the mean (SD) age at examination was 2.1 (1.5) months. The mothers of all the children had no ophthalmic findings and did not report ocular symptoms during pregnancy. All patients had bilateral ophthalmic manifestations. Optic nerve findings included hypoplasia with the double-ring sign, pallor, and increased cup-disc ratio in 5 patients (11.6%). Macular abnormalities included mild to severe pigment mottling in 27 patients (63%) and lacunar maculopathy in 3 (6.9%). Chorioretinal scarring was present in 3 patients (7%). Eleven patients (26%) had a combination of lesions in the posterior pole. Five patients (12%) were diagnosed with congenital glaucoma, characterized by the clinical triad of epiphora, photophobia, and blepharospasm; increased intraocular pressure; corneal clouding at birth; and buphthalmos. These data reveal that 12% (95% CI, 5%-24%) of cases of congenital Zika with microcephaly had anterior segment abnormalities and 88% (95% CI, 76%-94%) had important macular and optic nerve abnormalities. The visual sequelae of these ophthalmic manifestations remain unknown.

Conclusions and Relevance  Congenital Zika syndrome in the current study had severe ocular abnormalities, and all patients had bilateral involvement. Ocular findings were focal macular pigment mottling, chorioretinal atrophy with a predilection for the macular area, congenital glaucoma and optical nerve hypoplasia, and optic disc abnormalities. Ophthalmic examination is recommended in patients with congenital Zika syndrome.