Show simple item record

dc.contributor.authorAleman, Tomas S.
dc.contributor.authorVentura, Camila V. [UNIFESP]
dc.contributor.authorCavalcanti, Milena M.
dc.contributor.authorSerrano, Leona W.
dc.contributor.authorTraband, Anastasia
dc.contributor.authorNti, Akosua A.
dc.contributor.authorGois, Adriana L.
dc.contributor.authorBravo-Filho, Vasco
dc.contributor.authorMartins, Thayze T.
dc.contributor.authorNichols, Charles W.
dc.contributor.authorMaia, Mauricio [UNIFESP]
dc.contributor.authorBelfort, Rubens [UNIFESP]
dc.identifier.citationJama Ophthalmology. Chicago, v. 135, n. 10, p. 1069-1076, 2017.
dc.description.abstractIMPORTANCE 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 boysen
dc.description.abstractage range, 3-5 months) and 8 patients with cblC deficiency (3 girls and 5 boysen
dc.description.abstractage 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.en
dc.description.sponsorshipNational Institutes of Health
dc.description.sponsorshipHope for Vision
dc.description.sponsorshipFoundation Fighting Blindness
dc.description.sponsorshipPennsylvania Lions Sight Conservation
dc.description.sponsorshipNational Council for Scientific and Technological Development of Brazil
dc.publisherAmer Medical Assoc
dc.relation.ispartofJama Ophthalmology
dc.rightsAcesso restrito
dc.titleQuantitative Assessment of Microstructural Changes of the Retina in Infants With Congenital Zika Syndromeen
dc.description.affiliationUniv Penn, Scheie Eye Inst, Dept Ophthalmol, Perelman Ctr Adv Med,Perelman Sch Med, Philadelphia, PA les W. 19104 USA
dc.description.affiliationAltino Ventura Fdn, Dept Ophthalmol, Recife, PE, Brazil
dc.description.affiliationHOPE Eye Hosp, Dept Ophthalmol, Recife, PE, Brazil
dc.description.affiliationUniv Fed Sao Paulo, Dept Ophthalmol, Paulista Med Sch, Sao Paulo, Brazil
dc.description.affiliationAltino Ventura Fdn, Rehabil Ctr Menina Olhos, Recife, PE, Brazil
dc.description.affiliationUnifespUniv Fed Sao Paulo, Dept Ophthalmol, Paulista Med Sch, Sao Paulo, Brazil
dc.description.sourceWeb of Science

Files in this item


There are no files associated with this item.

This item appears in the following Collection(s)

Show simple item record