Expanded Spectrum of Congenital Ocular Findings in Microcephaly with Presumed Zika Infection
Homero Augusto de Miranda1, Marcelo Cavalcante Costa1, Maria Auxiliadora Monteiro Frazão1
1Ophthalmic Telemedicine Santa Casa of São Paulo, São Paulo, Brazil.
Purpose:
To describe the ocular findings of 3 cases of suspected congenital Zika viral infection with microcephaly and maculopathy.
Design:
Retrospective, consecutive case series.
Participants:
Three male infants born in northern Brazil whose mothers demonstrated a viral syndrome during the first trimester and who subsequently were born with microcephaly.
Methods:
Observational report of macular findings.
Main Outcome Measures:
Continued observation.
Results:
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).
Conclusions:
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.
Insights
Congenital Zika infection in infants can cause severe ocular abnormalities, including pigmentary maculopathy and chorioretinal atrophy. Early ophthalmologic screening is crucial for affected newborns.
Area of Science:
- Ophthalmology
- Virology
- Pediatrics
Background:
- Congenital Zika virus infection is linked to microcephaly in infants born to mothers with first-trimester viral syndromes.
- Zika virus, a flavivirus, is associated with Dengue and has demonstrated epidemic spread.
Observation:
- This study reports on 3 male infants born with microcephaly in Brazil.
- Mothers experienced a viral syndrome during the first trimester of pregnancy.
Findings:
- All 6 eyes exhibited pigmentary maculopathy, with 4 showing macular chorioretinal atrophy and hyperpigmented rings.
- Vascular abnormalities, including tortuosity and premature retinal vasculature termination, were observed in multiple eyes.
- Additional findings included peripheral retinal changes, subretinal hemorrhages, and lesions resembling grouped congenital albinotic spots.
Implications:
- Ocular findings in congenital Zika infection extend beyond pigmentary retinopathy to include torpedo maculopathy, vascular changes, and hemorrhagic retinopathy.
- Ophthalmologic screening guidelines are needed for newborns in affected regions to identify those who would benefit from screening.
- This research highlights the significant ocular impact of congenital Zika virus infection, necessitating further investigation and clinical management strategies.
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