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Ocular Findings in Infants With Congenital Zika Virus Infection
Sasapin G. Prakalapakorn, MD, MPH | May 7, 2018
| Contributor Information
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Image from Centers for Disease Control and Prevention
Ocular Findings in Infants With Congenital Zika Virus Infection
Sasapin G. Prakalapakorn, MD, MPH | May 7, 2018 | Contributor Information
Image from Moore CA, et al. JAMA Pediatr. 2017;171:288-295.[1] Republished with permission.
Ocular Findings in Infants With Congenital Zika Virus Infection
Sasapin G. Prakalapakorn, MD, MPH | May 7, 2018 | Contributor Information
Congenital Zika Virus Infection and Birth Defects
Zika virus is a flavivirus spread mostly by the bite of infected Aedes species mosquitos (A aegypti and A albopictus). The effects of the virus on the fetus have been identified only since the Zika virus outbreak began in the Americas in 2015.
The unique pattern of birth defects associated with Zika virus infection during pregnancy (often referred to as "congenital Zika syndrome") includes (1) severe microcephaly with partially collapsed skull; (2) thin cerebral cortices with subcortical calcifications; (3) macular scarring and focal pigmentary retinal mottling; (4) congenital contractures; and (5) marked early hypertonia and signs of extrapyramidal involvement.[1] The full range of birth defects caused by congenital Zika virus infection have yet to be determined.
Image from Dreamstime
Ocular Findings in Infants With Congenital Zika Virus Infection
Sasapin G. Prakalapakorn, MD, MPH | May 7, 2018 | Contributor Information
Ocular Findings in Infants With Congenital Zika Virus Infection
Here is a cross-sectional image of a normal eye. Light typically travels through the cornea and the lens in the anterior part of the eye, and is focused on the retina in the posterior part of the eye. The retina transmits signals through the optic nerve to the visual cortex of the brain.
Even before the Zika virus was recognized as a cause of birth defects, its effects on the eyes were suspected. Although most ocular findings have been reported in infants with microcephaly, ocular findings have also been reported in infants without microcephaly or other central nervous system findings. Abnormalities have been found both in the anterior and posterior parts of the eye.[2]
Image from Ventura CV, et al. Arq Bras Oftalmol. 2016;79:1-3. Republished with permission
Ocular Findings in Infants With Congenital Zika Virus Infection
Sasapin G. Prakalapakorn, MD, MPH | May 7, 2018 | Contributor Information
Most Commonly Reported Ocular Findings
Among infants with microcephaly and presumed congenital Zika virus infection, the most commonly reported structural ocular abnormalities involve the macula and the optic nerve.[2,3]
The macula is the area of the retina responsible for central vision. The most common macular findings associated with congenital Zika virus infection are focal pigmentary mottling (as seen here), loss of the foveal light reflex, and chorioretinal atrophy (seen on the next slide).
Image courtesy of Altino Ventura Foundation
Ocular Findings in Infants With Congenital Zika Virus Infection
Sasapin G. Prakalapakorn, MD, MPH | May 7, 2018 | Contributor Information
Chorioretinal Atrophy in the Macula
This image shows chorioretinal atrophy in the macula, another typical ocular finding in infants with congenital Zika infection.
Image from Ventura CV, et al. Arq Bras Oftalmol. 2016;79:1-3. Republished with permission
Ocular Findings in Infants With Congenital Zika Virus Infection
Sasapin G. Prakalapakorn, MD, MPH | May 7, 2018 | Contributor Information
Optic Nerve Findings
The optic nerve transmits signals from the retina to the brain, which interprets these signals as images. Optic nerve hypoplasia is a common finding in infants with congenital Zika virus infection.[3] The small, underdeveloped optic nerve is seen here with a double ring sign.
Image courtesy of Dr Camila Ventura
Ocular Findings in Infants With Congenital Zika Virus Infection
Sasapin G. Prakalapakorn, MD, MPH | May 7, 2018 | Contributor Information
Optic Nerve Cupping
Another optic nerve finding that is commonly seen with congenital Zika virus infection is optic nerve cupping,[3] an increase in the ratio between the size of the depression in the optic nerve and the size of the optic nerve. Along with optic nerve cupping, this image shows optic nerve pallor—a sign of insult or damage to the nerve.
Image courtesy of Altino Ventura Foundation
Ocular Findings in Infants With Congenital Zika Virus Infection
Sasapin G. Prakalapakorn, MD, MPH | May 7, 2018 | Contributor Information
Other Posterior Ocular Findings
Other posterior eye findings that have been reported in infants with microcephaly and presumed congenital Zika virus infection include pigmentary clumping and chorioretinal atrophy outside of the macula; subretinal hemorrhages; retinal coloboma; abnormal termination of the retinal vessels; focal areas of vascular dilation; and vascular tortuosity, straightening, and attenuation.[4,5] Along with optic nerve hypoplasia and a chorioretinal scar in the macula, this image shows vascular attenuation.
Image from Wikimedia Commons
Ocular Findings in Infants With Congenital Zika Virus Infection
Sasapin G. Prakalapakorn, MD, MPH | May 7, 2018 | Contributor Information
Anterior Ocular Findings
Anterior ocular findings that have been noted in infants with congenital Zika virus infection include iris colobomas[6,7] (shown here), which may be caused by the failure of some structures of the eye to close during gestational development.
Image courtesy of EyeRounds.org
Ocular Findings in Infants With Congenital Zika Virus Infection
Sasapin G. Prakalapakorn, MD, MPH | May 7, 2018 | Contributor Information
Lens Subluxation
Another anterior ocular finding associated with congenital Zika virus infection that has been noted is lens subluxation.[6] The lens is normally centered behind the iris. In this image, the lens is subluxed, or displaced, upward.
Image courtesy of EyeRounds.org
Ocular Findings in Infants With Congenital Zika Virus Infection
Sasapin G. Prakalapakorn, MD, MPH | May 7, 2018 | Contributor Information
Other Ocular Findings
Other ocular findings associated with congenital Zika virus infection that have been reported among infants with microcephaly include microphthalmia, cataract, intraocular calcifications, strabismus, and nystagmus.[8,9] Here is an image of a fetal ultrasound showing microphthalmia of one eye. Note that the eye on the left side (long arrow) of the image is smaller than the one on the right (short arrow).
Image courtesy of EyeRounds.org
Ocular Findings in Infants With Congenital Zika Virus Infection
Sasapin G. Prakalapakorn, MD, MPH | May 7, 2018 | Contributor Information
Congenital Glaucoma
Congenital glaucoma has also been reported in infants with presumed congenital Zika syndrome and microcephaly.[10] In glaucoma, increased intraocular pressure can damage the optic nerve, which can result in severe vision loss. Some common symptoms in congenital glaucoma include excessive tearing, light sensitivity, and an enlarged and cloudy cornea, as seen in this image.
Image from Centers for Disease Control and Prevention
Ocular Findings in Infants With Congenital Zika Virus Infection
Sasapin G. Prakalapakorn, MD, MPH | May 7, 2018 | Contributor Information
Ocular Abnormalities in Infants Without Microcephaly
Although most reported cases of infants with ocular findings have microcephaly, infants with possible Zika virus infection without microcephaly have also been identified with both anterior and posterior ocular changes, including microphthalmia, iris colobomas, optic nerve damage, and retinal abnormalities.[11,12] This image shows microphthalmia of the left eye.
Image from Centers for Disease Control and Prevention
Ocular Findings in Infants With Congenital Zika Virus Infection
Sasapin G. Prakalapakorn, MD, MPH | May 7, 2018 | Contributor Information
Overview: Eye Findings in Congenital Infections
Ocular manifestations are not unique to congenital Zika virus infection. This table compares the ocular findings reported in infants with congenital Zika infection with those in other common congenital infections.[2,3,13] There is some overlap across the diseases in ocular manifestations, such as the presence of chorioretinal atrophy, optic nerve pallor/atrophy, cataracts, and microphthalmia.
To date, no active inflammatory lesions have been reported in infants with congenital Zika infection, whereas they have been reported in other diseases. As with the other congenital infections, not only does the presence of ocular manifestations vary among infants, but so does the severity of these findings.
Image courtesy of American Printing House for the Blind
Ocular Findings in Infants With Congenital Zika Virus Infection
Sasapin G. Prakalapakorn, MD, MPH | May 7, 2018 | Contributor Information
Visual Impairment
Our knowledge of the effects of congenital Zika virus infection on the eye and on vision is still growing. Two studies have evaluated visual function among cohorts of infants with microcephaly due to suspected congenital Zika syndrome.[9,14] They found that although about 40% of these cohorts had ocular abnormalities, 100% of children tested had visual impairment. A reduction in visual response due to a neurologic problem that affects the visual part of the brain is known as "cortical visual impairment." Thus, cortical visual impairment might be the most common cause of blindness among children with congenital Zika syndrome. Children with cortical visual impairment usually have some vision and may show some improvement over time. Thus, it is important that children who experience visual impairment or visual loss be referred to a low vision specialist and receive early intervention to help them improve and maximize their abilities.
Image from National Eye Institute, National Institutes of Health
Ocular Findings in Infants With Congenital Zika Virus Infection
Sasapin G. Prakalapakorn, MD, MPH | May 7, 2018 | Contributor Information
Eye Screening Recommendations
Healthy-appearing infants with congenital Zika virus infection may have ocular findings and/or visual impairment, so screening is important. If an impairment in vision is identified, early referral for intervention is critical. The Centers for Disease Control and Prevention recommend a comprehensive ophthalmologic examination by 1 month of age for (1) infants who have abnormalities consistent with congenital Zika syndrome (and whose mothers had possible Zika virus exposure during pregnancy, regardless of maternal testing results), and (2) infants of mothers with laboratory evidence of Zika virus infection during pregnancy (including infants without abnormalities consistent with congenital Zika syndrome). The ophthalmologic examination should be conducted by an ophthalmologist experienced in the assessment and management of infants. Follow-up visits with an ophthalmologist after the initial eye examination should be based on the ophthalmologist's recommendations.[15]
The findings and conclusions in this presentation (except for slide 16) have not been formally disseminated by the Centers for Disease Control and Prevention and should not be construed to represent any agency determination or policy.
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Contributor Information
Sasapin G. Prakalapakorn, MD, MPH
Associate Professor of Ophthalmology and Pediatrics
Duke University
Durham, North Carolina;
Consultant
Centers for Disease Control and Prevention
Atlanta, Georgia
Disclosure: Sasapin G. Prakalapakorn, MD, MPH, has disclosed no relevant financial relationships.
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References
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Marquezan MC, Ventura CV, Sheffield JS, et al. Ocular effects of Zika virus—a review. Surv Ophthalmol. 2018;63:166-173.
Ventura CV, Maia M, Travassos SB, et al. Risk factors associated with the ophthalmoscopic findings identified in infants with presumed Zika virus congenital infection. JAMA Ophthalmol. 2016;134:912-918.
Miranda HA 2nd, Costa MC, Frazão MA, Simão N, Franchischini S, Moshfeghi DM. Expanded spectrum of congenital ocular findings in microcephaly with presumed Zika infection. Ophthalmology. 2016;123:1788-1794.
de Oliveira Dias JR, Ventura CV, Borba PD, et al. Infants with congenital Zika syndrome and ocular findings from São Paulo, Brazil: spread of infection. Retina Cases Brief Rep. 2017 Jan 2. [Epub ahead of print].
de Paula Freitas B, de Oliveira Dias JR, Prazeres J, et al. Ocular findings in infants with microcephaly associated with presumed Zika virus congenital infection in Salvador, Brazil. JAMA Ophthalmol. 2016 Feb 9. [Epub ahead of print].
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Zin AA, Tsui I, Rossetto J, et al. Screening criteria for ophthalmic manifestations of congenital Zika virus infection. JAMA Pediatr. 2017;171:847-854.
Ventura CV, Fernandez MP, Gonzalez IA, et al. First travel-associated congenital Zika syndrome in the US: ocular and neurological findings in the absence of microcephaly. Ophthalmic Surg Lasers Imaging Retina. 2016;47:952-955.
Mets MB, Chhabra MS. Eye manifestations of intrauterine infections and their impact on childhood blindness. Surv Ophthalmol. 2008;53:95-111.
Ventura LO, Ventura CV, Lawrence L, et al. Visual impairment in children with congenital Zika syndrome. J AAPOS 2017;21:295-299.
Adebanjo T, Godfred-Cato S, Viens L, et al. Update: interim guidance for the diagnosis, evaluation, and management of infants with possible congenital Zika virus infection—United States, October 2017. MMWR Morb Mortal Wkly Rep. 2017;66:1089-1099.
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