J Korean Ophthalmol Soc.  2019 Sep;60(9):887-891. 10.3341/jkos.2019.60.9.887.

Sjögren's Reticular Retinal Dystrophy

• Noh H
• Bae K
• Kang SW

• KMID: 2459134
• DOI: http://doi.org/10.3341/jkos.2019.60.9.887

Abstract

PURPOSE
To report a rare case of Sjögren's reticular retinal dystrophy.
CASE SUMMARY
A 54-year-old male presented with blurred vision and metamorphopsia in both eyes since a few years prior to his initial visit. There was a bilateral reticular network of yellow deposits throughout the posterior pole on fundus examination, which was hyperautofluorescent in fundus autofluorescence photographs. The pigment alterations were more visible with fluorescein angiography, which showed hypofluorescent lesions with hyperfluorescent borders. Spectral-domain optical coherence tomography showed elevations of the outer retina associated with the presence of subretinal hyperreflective material. Based on the conclusive correlation with clinical features, we diagnosed Sjögren's reticular retinal dystrophy.
CONCLUSIONS
Sjögren's reticular retinal dystrophy is characterized by its specific pigment changes at the level of clinical manifestations and the retinal pigment epithelium. In cases of Sjögren's reticular retinal dystrophy, close monitoring is required because it has a lifetime risk of choroidal neovascularization.

Keyword

Macular dystrophy; Pattern dystrophy

MeSH Terms

Choroidal Neovascularization
Fluorescein Angiography
Humans
Macular Degeneration
Male
Middle Aged
Retina
Retinal Dystrophies*
Retinal Pigment Epithelium
Retinaldehyde*
Tomography, Optical Coherence
Vision Disorders
Retinaldehyde

Figure

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  Figure 1 Fundus photograph and fundus autofluorescence photograph of the patient. (A) Fundus photograph presents a reticular network of yellow deposits throughout the posterior pole. (B) Fundus autofluorescence photograph shows the hyperautofluorescent lesions with reticular pattern.

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  Figure 2 Fluorescein angiography of the patient. Multiple hypofluorescent lesions with hyperfluorescent borders were scattered at the posterior pole and peripheral retina in both eyes.

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  Figure 3 Spectral-domain optical coherence tomography (SD-OCT) of the patient. SD-OCT of the right (A) and left (B) eyes shows subretinal hyperreflective materials, ellipsoid zone and external limiting membrane elevations with multiple focal defects of cone outer segment tips. The elevations of the outer retina are associated with the presence of subretinal hyperreflective material. After 8 years, SD-OCT of the (C) right eye and (D) left eye looks stationary and has no evidence of choroidal neovascularization.

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  Figure 4 Electroretinography of the patient. (A) The bright flash and (B) photopic standard electroretinography showed normal amplitude and implicit time in both eyes.

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