Multiple mechanisms of visual impairment in congenital zika syndrome

Purpose:To describe and analyze the involved mechanisms of visual impairment in a patient with microcephaly and presumed Congenital Zika Syndrome. Settings and study design: This is a clinical case presentation and was carried out at CAVIVER, a non-governmental organization clinic for children with visual impairment in the city of Fortaleza CE, located in the Northeast region of Brazil. The ophthalmological evaluation included retinoscopy, slitlamp examination, binocular indirect ophthalmoscopy, eye fundus digital imaging, visual acuity testing with Teller acuity cards and strabismus assessment. Casereport: A 13 months-old girl was referred to the ophthalmological clinic presenting microcephaly. Head circumference at birth was 26cm. Ophthalmologic abnormalities included: macular circumscribed chorioretinal atrophy, focal mottled retinal pigment epithelium, optic nerve hypoplasia with double ring sign, early onset strabismus (exotropia), nystagmus and low visual acuity. Macular atrophic scar and optic nerve hypoplasia with a double ring sign were present in both eyes. Refractive error of -14.00 sph -2.50 cyl (180º) in both eyes was detected. Vision function and visual developmental milestones analysis were abnormal. Conclusion: Multiple mechanisms as anatomical, neurological, optical and functional abnormalities lead to severe visual impairment in this patient with microcephaly due to presumed Congenital Zika Syndrome.