Ocular Findings in Infants With Microcephaly Associated With Presumed Zika Virus Congenital Infection in Salvador, Brazil

Abstract

Importance: The Zika virus (ZIKV) has rapidly reached epidemic proportions, especially in northeastern Brazil, and has rapidly spread to other parts of the Americas. A recent increase in the prevalence of microcephaly in newborn infants and vision-threatening findings in these infants is likely associated with the rapid spread of ZIKV.

Objective: To evaluate the ocular findings in infants with microcephaly associated with presumed intrauterine ZIKV infection in Salvador, Bahia, Brazil.

Design, setting, and participants: Case series at a tertiary hospital. Twenty-nine infants with microcephaly (defined by a cephalic circumference of ≤32 cm) with a presumed diagnosis of congenital ZIKV were recruited through an active search and referrals from other hospitals and health unities. The study was conducted between December 1 and December 21, 2015.

Interventions: All infants and mothers underwent systemic and ophthalmic examinations from December 1 through December 21, 2015, in the Roberto Santos General Hospital, Salvador, Brazil. Anterior segment and retinal, choroidal, and optic nerve abnormalities were documented using a wide-field digital imaging system. The differential diagnosis included toxoplasmosis, rubella, cytomegalovirus, herpes simplex virus, syphilis, and human immunodeficiency virus, which were ruled out through serologic and clinical examinations.

Main outcomes and measures: Ocular abnormalities associated with ZIKV.

Results: Twenty-three of 29 mothers (79.3%) reported suspected ZIKV infection signs and symptoms during pregnancy, 18 in the first trimester, 4 in the second trimester, and 1 in the third trimester. Of the 29 infants (58 eyes) examined (18 [62.1%] female), ocular abnormalities were present in 17 eyes (29.3%) of 10 children (34.5%). Bilateral findings were found in 7 of 10 patients presenting with ocular lesions, the most common of which were focal pigment mottling of the retina and chorioretinal atrophy in 11 of the 17 eyes with abnormalities (64.7%), followed by optic nerve abnormalities in 8 eyes (47.1%), bilateral iris coloboma in 1 patient (2 eyes [11.8%]), and lens subluxation in 1 eye (5.9%).

Conclusions and relevance: Congenital infection due to presumed ZIKV exposure is associated with vision-threatening findings, which include bilateral macular and perimacular lesions as well as optic nerve abnormalities in most cases.

Figure 1. Fundus Photographs of a 2-Month-Old Girl

The right eye has granular, pigmentary mottling in the macula (A), and the left eye has a chorioretinal lobulated atrophic lesion and slight pigmentary mottling (B).

Figure 2. Fundus Photographs of a 1-Month-Old Boy

The right (A) and left (B) eyes have paramacular superotemporal round chorioretinal atrophy surrounded by a hyperpigmented halo and hyperpigmented mottling.

Figure 3. Fundus Photographs of a 1-Month-Old Infant

The right eye has an enlarged cup-disc ratio and macular pigmentary mottling (A), and the left eye has a roundish macular chorioretinal atrophic lesion with a hyperpigmented halo and pigmentary mottling, as well as an enlarged cup-disc ratio of the optic nerve.

Figure 4. Fundus Photographs of a 1-Month-Old Infant

The right eye has a superotemporal perimacular chorioretinal scar with perilesional pigmentary mottling (A), and the left eye has similar findings (B).

Figure 5. Fundus Photographs of a 20-Day-Old Infant

The right eye has optic disc hypoplasia, peripapillary nasal atrophy, and an excavated nasal round lesion with a hyperpigmented halo, with a colobomatous-like aspect (A), and the left eye has optic disc hypoplasia, peripapillary nasal atrophy, and a retinal nasal lesion with a similar pattern (B).