Quantitative Assessment of Microstructural Changes of the Retina in Infants With Congenital Zika Syndrome

Quantitative Assessment of Microstructural Changes of the Retina in Infants With Congenital Zika Syndrome

Author Aleman, Tomas S. Google Scholar
Ventura, Camila V. Autor UNIFESP Google Scholar
Cavalcanti, Milena M. Google Scholar
Serrano, Leona W. Google Scholar
Traband, Anastasia Google Scholar
Nti, Akosua A. Google Scholar
Gois, Adriana L. Google Scholar
Bravo-Filho, Vasco Google Scholar
Martins, Thayze T. Google Scholar
Nichols, Charles W. Google Scholar
Maia, Mauricio Autor UNIFESP Google Scholar
Belfort, Rubens Autor UNIFESP Google Scholar
Abstract IMPORTANCE A better pathophysiologic understanding of the neurodevelopmental abnormalities observed in neonates exposed in utero to Zika virus (ZIKV) is needed to develop treatments. The retina as an extension of the diencephalon accessible to in vivo microcopy with spectral-domain optical coherence tomography (SD-OCT) can provide an insight into the pathophysiology of congenital Zika syndrome (CZS). OBJECTIVE To quantify the microstructural changes of the retina in CZS and compare these changes with those of cobalamin C (cblC) deficiency, a disease with potential retinal maldevelopment. DESIGN, SETTING, AND PARTICIPANTS This case series included 8 infants with CZS and 8 individuals with cblC deficiency. All patients underwent ophthalmologic evaluation at 2 university teaching hospitals and SD-OCT imaging in at least 1 eye. Patients with cblC deficiency were homozygous or compound heterozygotes for mutations in the methylmalonic aciduria and homocystinuria type C (MMACHC) gene. Data were collected from January 1 to March 17, 2016, for patients with CZS and from May 4, 2015, to April 23, 2016, for patients with cblC deficiency. MAIN OUTCOMES AND MEASURES The SD-OCT cross-sections were segmented using automatic segmentation algorithms embedded in the SD-OCT systems. Each retinal layer thickness was measured at critical eccentricities using the position of the signal peaks and troughs on longitudinal reflectivity profiles. RESULTS Eight infants with CZS (5 girls and 3 boys

age range, 3-5 months) and 8 patients with cblC deficiency (3 girls and 5 boys

age range, 4 months to 15 years) were included in the analysis. All 8 patients with CZS had foveal abnormalities in the analyzed eyes (8 eyes), including discontinuities of the ellipsoid zone, thinning of the central retina with increased backscatter, and severe structural disorganization, with 3 eyes showing macular pseudocolobomas. Pericentral retina with normal lamination showed a thinned (< 30% of normal thickness) ganglion cell layer (GCL) that colocalized in 7 of 8 eyes with a normal photoreceptor layer. The inner nuclear layer was normal or had borderline thinning. The central retinal degeneration was similar to that of cblC deficiency. CONCLUSIONS AND RELEVANCE Congenital Zika syndrome showed a central retinal degeneration with severe GCL loss, borderline inner nuclear layer thinning, and less prominent photoreceptor loss. The findings provide the first, to date, in vivo evidence in humans for possible retinal maldevelopment with a predilection for retinal GCL loss in CZS, consistent with a murine model of the disease and suggestive of in utero depletion of this neuronal population as a consequence of Zika virus infection.
xmlui.dri2xhtml.METS-1.0.item-coverage Chicago
Language English
Sponsor National Institutes of Health
Hope for Vision
Foundation Fighting Blindness
Pennsylvania Lions Sight Conservation
National Council for Scientific and Technological Development of Brazil
Date 2017
Published in Jama Ophthalmology. Chicago, v. 135, n. 10, p. 1069-1076, 2017.
ISSN 2168-6165 (Sherpa/Romeo, impact factor)
Publisher Amer Medical Assoc
Extent 1069-1076
Origin http://dx.doi.org/10.1001/jamaophthalmol.2017.3292
Access rights Closed access
Type Article
Web of Science ID WOS:000412869200016
URI https://repositorio.unifesp.br/handle/11600/57288

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