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Case of the Day

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BIETTI CRYSTALLINE DYSTROPHY

Anju Bansal and Virendra Agrawal

This 42YO male has a long history of night blindness, difficulty adapting to bright light, and poor vision. He had been advised elsewhere to have cataract surgery. Ocular family history was negative. Vision was counting fingers OU, and slit lamp examinations were normal.

Fundus imaging shows extensive pigmentary degeneration with subretinal crystalline deposits involving both posterior poles. OCT shows severe central and outer retinal atrophy, with more eccentric outer retinal atrophy. Fundus autofluorescence (FAF) shows diffuse hypo-FAF throughout each posterior pole with a patchier pattern of hyper- and hypo-FAF more peripherally.

Learning Points:
Bietti crystalline dystrophy is an autosomal recessive ocular disorder that affects both the anterior and posterior segments. Clinical features include crystalline deposits in the retina, cornea, and rarely the crystalline lens, as well as retinal pigment epithelial clumping and atrophy (Saatci et al, Clinical Ophthalmology 2023;17:953-967). Mutations in CYP4V2 causing dysfunctional lipid metabolism have been implicated in its pathogenesis. Although no definitive treatments exist, anti-VEGF therapy can be used for secondary macular neovascularization.

 

Article of the Day

Oral therapeutic interventions for central serous chorioretinopathy: A systematic review

Hamzah F, Azrina A, Supanji S, Adriono GA, Shinojima A.

Graefes Arch Clin Exp Ophthalmol. 2026 Sep;264(9):2589-2599. doi: 10.1007/s00417-026-07253-w.

Summary

ICSC oral therapeutics: Mineralocorticoid receptor antagonists may reduce SRF, although VA benefits are uncertain. Propranolol limited evidence for benefits — Systematic Review

Abstract

Purpose: Central serous chorioretinopathy (CSCR) is characterized by serous neurosensory retinal detachment that predominantly affects working-age men. Many acute cases resolve spontaneously, but chronic or recurrent forms can lead to permanent visual impairment. Oral pharmacological therapies have emerged as a noninvasive alternative to photodynamic therapy (PDT), particularly in the context of verteporfin shortage. This systematic review aims to evaluate the efficacy of oral agents for CSCR, focusing on subretinal fluid (SRF), best-corrected visual acuity (BCVA), choroidal thickness, and recurrence.

Methods: Following PRISMA 2020 guidelines, we searched MEDLINE, the Cochrane Library, and Web of Science (January 1990 to May 2025) for randomized controlled trials (RCTs) assessing oral therapies for CSCR. Risk of bias was assessed using the Cochrane RoB 2.

Results: Our search returned 14 trials that met our inclusion criteria. We found that mineralocorticoid receptor antagonists appeared to reduce SRF in most studies (6 of 8 trials), while their effects on BCVA and choroidal thickness were inconsistent. Helicobacter pylori eradication therapy showed no significant SRF reduction or effects on BCVA compared to placebos or control group. Propranolol improved both complete SRF resolution and BCVA, while metipranolol demonstrated no significant effects. Due to high heterogeneity between studies, we did not perform a meta-analysis.

Conclusion: Mineralocorticoid receptor antagonists may reduce SRF in CSCR, although visual outcomes remain uncertain. Propranolol may offer potential benefit based on limited evidence. Other oral therapies lack supportive evidence. Given study heterogeneity and absence of meta-analysis, findings should be interpreted with caution. Further large-scale RCTs are needed.

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