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An autosomal dominantly inherited cystoid macular edema manifesting with macular atrophy, strabismus and, sometimes, pericentral retinitis pigmentosa. It is associated with a poor visual prognosis.
Features include: Strabismus, Pericentral retinitis pigmentosa, Hypermetropia, and Edema and 2 more.
Organ System | Phenotype Count | Example Features |
|---|---|---|
Eyes | 3 | Strabismus, Cystoid macular edema, Macular dystrophy |
Biomarker and diagnostic research for cystoid macular edema has been reported in the published literature.
Estimated prevalence: <1 in 1,000,000 (VERY_RARE).
4 clinical trials registered, 3 recruiting. Interventions under study include drug therapy and other interventions. Pipeline includes 1 PHASE4, 1 PHASE2, 1 PHASE1. Research is primarily sponsored by academic and government institutions.
256 publications have been identified in PubMed for cystoid macular edema. Kisho has analyzed 161 by research type. Research spans Epidemiology / Natural History (27%), Review / Meta-Analysis (24%), and Case Report / Case Series (19%).
Research Type | Count | % of Total |
|---|---|---|
Disease patterns and progression | 44 |
Data assembled from 6 of 12 sources · Last updated Sep 19, 2026, 4:30 PM UTC
Online Mendelian Inheritance in Man
European rare disease database
Genetic and Rare Diseases Info Center
27%
Research summaries | 38 | 24% |
Patient case studies | 31 | 19% |
Clinical study results | 25 | 16% |
Testing and diagnosis research | 9 | 6% |
Laboratory research | 7 | 4% |
New treatment approaches | 4 | 2% |
Other research | 3 | 2% |
Mohammed TK (2026). [PMID: 42181665](https://pubmed.ncbi.nlm.nih.gov/42181665/). *Ophthalmol Sci*. [Basic Science / Preclinical]
Al-Moujahed A (2026). [PMID: 42147783](https://pubmed.ncbi.nlm.nih.gov/42147783/). *J Vitreoretin Dis*. [Epidemiology / Natural History]
Hosny R (2026). [PMID: 42159842](https://pubmed.ncbi.nlm.nih.gov/42159842/). *Int Ophthalmol*. [Epidemiology / Natural History]
Campos A (2026). [PMID: 41197879](https://pubmed.ncbi.nlm.nih.gov/41197879/). *Surv Ophthalmol*. [Review / Meta-Analysis]
Rohowetz LJ (2026). [PMID: 39903917](https://pubmed.ncbi.nlm.nih.gov/39903917/). *Retin Cases Brief Rep*. [Case Report / Case Series]
Moro-Muniz M (2026). [PMID: 41786135](https://pubmed.ncbi.nlm.nih.gov/41786135/). *Arch Soc Esp Oftalmol (Engl Ed)*. [Case Report / Case Series]
Valmaggia P (2026). [PMID: 41730283](https://pubmed.ncbi.nlm.nih.gov/41730283/). *Klin Monbl Augenheilkd*. [Case Report / Case Series]
Aychoua N (2026). [PMID: 41296346](https://pubmed.ncbi.nlm.nih.gov/41296346/). *JAMA Ophthalmol*. [Basic Science / Preclinical]
Muayad J (2026). [PMID: 41485710](https://pubmed.ncbi.nlm.nih.gov/41485710/). *Ophthalmol Glaucoma*. [Epidemiology / Natural History]
Yamamoto M (2026). [PMID: 40982423](https://pubmed.ncbi.nlm.nih.gov/40982423/). *Retina*. [Epidemiology / Natural History]
AI-curated news mentioning cystoid macular edema
Updated May 27, 2026
A new report discusses the challenges in managing macular edema following acute retinal necrosis. This condition complicates treatment strategies and highlights the need for further research in effective management approaches.
A case report identifies recurrent bilateral macular edema associated with NOTCH2NLC GGC repeat expansion. This discovery may enhance understanding of the genetic factors contributing to this ocular condition.