A 7-year-old girl with rubella retinopathy, sensorineural hearing loss and cardiac defects presented to our center with a decrease in vision in the right eye (RE) for 8 months. On examination, visual acuity in OD was 1/60 and in OS was 6/6. On fundus examination, the RE revealed a grey lesion at macula with pigmentary changes suggestive of a choroidal neovascular membrane along with salt and pepper retinopathy [Fig. 1a and b]. Fundus autofluorescence (FAF) [Fig. 1c] was suggestive of RPE atrophy, and SS-OCT of the macula revealed a spindle-shaped thickened hyperreflective area above the retinal pigment epithelium adjacent to fovea along with thinning and loss of outer retinal layers [Fig. 1d], suggestive of a long-standing membrane with resultant foveal atrophy. The left eye (LE) showed salt and pepper retinopathy with a dull foveal reflex.
Figure 1
Right eye (OD). (a) Ultra-wide field pseudo-color fundus photograph shows salt and pepper pigmentary changes. (b) Clinical photograph of the macula showing a choroidal neovascular membrane with surrounding pigmentary changes and areas of hemorrhage. (c) Ultra-wide field FAF image showing hypoautofluorescent area indicative of RPE atrophy with a surrounding rim of hyper autofluorescence inferiorly indicating viable pigment epithelial cells. (d) SSOCT showing foveal atrophy with type 2 CNV complex (predominantly inactive). The Cone outer segment tips and Ellipsoid zone are also disrupted
Right eye (OD). (a) Ultra-wide field pseudo-color fundus photograph shows salt and pepper pigmentary changes. (b) Clinical photograph of the macula showing a choroidal neovascular membrane with surrounding pigmentary changes and areas of hemorrhage. (c) Ultra-wide field FAF image showing hypoautofluorescent area indicative of RPE atrophy with a surrounding rim of hyper autofluorescence inferiorly indicating viable pigment epithelial cells. (d) SSOCT showing foveal atrophy with type 2 CNV complex (predominantly inactive). The Cone outer segment tips and Ellipsoid zone are also disruptedCases of congenital rubella commonly present to the ophthalmology department with cataract, microphthalmos, glaucoma, or pigmentary retinopathy.[1] Vision loss is commonly due to cataract or coexistent microphthalmos and nystagmus.[2] Pigmentary retinopathy per se does not usually lead to visual loss. Choroidal neovascularization associated with pigmentary retinopathy due to chronic atrophy of the retinal layers at the macula leads to vision loss.[3] Kiziltoprak et al. has injected intravitreal aflibercept in a case of CNV associated with rubella retinopathy and found significant improvement in visual acuity with a single injection.[4] In our case, foveal atrophy had already set in and the CNV had regressed to form a scar by course of time hence guarded prognosis was explained to the parents and they were counseled regarding not going ahead with any treatment for the same.
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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Authors: Hasan Kiziltoprak; Esat Yetkin; Kemal Tekin; Mustafa Koc; İrfan Akalin; Mehmet Yasin Teke Journal: Int Ophthalmol Date: 2018-02-06 Impact factor: 2.031
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