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Cystoid Macular Dystrophy

DCMD


Presentation

  • The clinical presentation and optical coherence tomography findings in a patient with maternally inherited diabetes and deafness (MIDD) are presented to highlight the presence of macular cystoid spaces in some patients with this disease.[experts.umich.edu]
  • […] diabetes and deafness (MIDD) are presented to highlight the presence of macular cystoid spaces in some patients with this disease.[octnews.org]
  • Download citation file: Ris (Zotero) EndNote BibTex Medlars ProCite RefWorks Reference Manager ARVO (1962-2015); The Authors (2016-present) Supplements Abstract Purpose: : The present study was conducted to describe the clinical characteristics in a large[iovs.arvojournals.org]
  • In this report we present the characterization of the C7orf9 gene locus encoding RFamide-related peptides (RFRPs) and its evaluation in dominant cystoid macular dystrophy (CYMD).[ncbi.nlm.nih.gov]
  • Hyperopia and strabismus are often present as well. Veils, strands, and white punctate deposits in the vitreous have been described. Wrinkling of the internal limiting membrane may be present.[disorders.eyes.arizona.edu]
Retinal Lesion
  • A retinal lesion resembling Sjögrens’s dystrophia reticularis lammae pigmentosae retinae. Perspectives in ophthalmology, vol. 2, ed. J.E. Winkelman & R.A. Crone, pp. 40–45, Excerpta Medica, Amsterdam (1970). Google Scholar Sorsby, A. & Crick, R.R.[link.springer.com]
  • Multiple retinal conditions may impact cataract surgery outcomes, including disorders such as: Diabetic macular edema (DME) Retinal vein occlusion (RVO) Uveitis Epiretinal membrane formation In addition, peripheral retinal lesions may also have bearing[reviewofoptometry.com]
Short Arm
  • Abstract In a family with autosomal dominant cystoid macular dystrophy (DCMD) linkage was detected with the dinucleotide marker D7S435 on the short arm of chromosome 7.[ncbi.nlm.nih.gov]
  • Biological context of CYMD RESULTS: The C7orf9 cDNA transcript consists of 1190 bp and is organized into 3 exons on the short arm of chromosome 7 within the critical region for CYMD [4]. Associations of CYMD with chemical compounds 1.[wikigenes.org]
Withdrawn
  • With Aflibercept in Patients With Diabetic Macular Edema Completed NCT02646670 Phase 4 Ranibizumab;Aflibercept;Ranibizumab and Aflibercept;Aflibercept and ranibizumab 27 Fluocinolone Acetonide Insert (ILUVIEN ) for Diabetic Macular Edema (FAD) Study Withdrawn[malacards.org]

Treatment

  • Until now, no efficient treatments for DCMD have been reported. The authors evaluated a somatostatin-analogue (octreotide acetate) as treatment for DCMD.[ncbi.nlm.nih.gov]
  • […] of Bevacizumab and Triamcinolone in Treatment or Macular Edema Secondary to CRVO Unknown status NCT00566761 Phase 4 bevacizumab and triamcinolone 2 Predictors of Treatment Response to Aflibercept and Aqueous Cytokine Levels in Patients With Persistent[malacards.org]
  • Treatment Treatment Options: No specific treatment is available for the macular disease but low vision aids are likely useful, at least early in the disease.[disorders.eyes.arizona.edu]
  • The cystoid changes responded to treatment with systemic immunosuppression and a topical carbonic anhydrase inhibitor (CAI), recurred when treated with topical CAI monotherapy, and finally resolved after an intravitreal triamcinolone acetonide injection[experts.umich.edu]

Prognosis

  • PURPOSE: Dominant cystoid macular degeneration (DCMD) is an autosomal dominant trait of cystoid macular edema with poor visual prognosis. Until now, no efficient treatments for DCMD have been reported.[ncbi.nlm.nih.gov]
  • Prognosis - there is no progression.[patient.info]
  • The prognosis in Best disease is relatively good. Visual acuity remains for the most part preserved over time.[aao.org]
  • Prognosis Prognosis for Best disease is mixed. Some carriers will never phenotypically express the disorder.[emedicine.medscape.com]
  • Treatment/Prognosis of Cystoid Macular Edema The prognosis for untreated CME after cataract surgery (Irvine-Gass syndrome) is very good. About half of the cases will regain normal vision in about 6 months.[mastereyeassociates.com]

Etiology

  • Based on the underlying etiology, the therapeutic approach can be either surgical or medical with anti-inflammatory drugs.[eprints.ucm.es]
  • Treatment Treatment of CME can be primarily addressed based on the inflammation etiology model: Topical steroids and NSAIDs work synergistically at the arachidonic acid cascade level to reduce inflammation.[healio.com]
  • While RP is a genetic disease, the etiology of progressive cell death, including that of associated cystoid macular edema (CME), is not completely understood.[clinicaltrials.gov]
  • The etiology for CME can be remembered with the mnemonic "DEPRIVEN" (Diabetes, Epinepherine, Pars planitis, Retinitis pigmentosa, Irvine-Gass Syndrome, Venous occlusion, E2-Prostaglandin analogues, Nicotinic acid/Niacin).[wikidoc.org]
  • It can be bilateral, depending on the etiology. The onset of symptoms is usually gradual; however, patients often only notice it suddenly, when they check one eye separately. Different causes of CME have different clinical presentations.[emedicine.medscape.com]

Epidemiology

  • Epidemiology of endogenous uveitis in north-eastern Italy. Analysis of 655 new cases. Acta Ophthalmol Scand. 79: 64-68, 2001. DOWNLOAD PDF Parolini B, Marcon G, Panozzo G. Central necrotic lamellar inflammation after LASIK.[barbaraparolini.it]
  • Epidemiology Frequency United States Best disease is rare. International Best disease is rare. Mortality/Morbidity Visual acuity is good in the previtelliform stage.[emedicine.medscape.com]
  • EPIDEMIOLOGY AND PATHOGENESIS CME is a common condition associated with intraocular inflammation, vitreoretinal traction, and vascular incompetence; CME following cataract surgery is known as the Irvine-Gass syndrome.[1] [2] Intracapsular cataract extraction[medtextfree.wordpress.com]
Sex distribution
Age distribution

Pathophysiology

  • Comprehensively updated to reflect everything you need to know regarding retinal diagnosis, treatment, development, structure, function, and pathophysiology, this monumental ophthalmology reference work equips you with expert answers to virtually any[books.google.com]
  • Pathophysiology of CME includes inflammation, surgical trauma of ocular tissue, retained lens fragments, vitreous traction, photic toxicity and possibly pharmacology (epinephrine, tamoxifen).[healio.com]
  • While environmental factors and an individual's genetic background likely influence the disease phenotype, more studies into the pathophysiology of BVMD are necessary.[aao.org]
  • Since RPE loss is implicated in the pathophysiology of both disorders, RPE replacement has been suggested as a therapeutic intervention for these conditions.[asociaciondoce.com]
  • Pathophysiology Lesions in Best disease are restricted to the eye. No systemic associations exist. Abnormalities in the eye result from a disorder in the retinal pigment epithelium (RPE).[emedicine.medscape.com]

Prevention

  • CONCLUSIONS: Somatostatin-analogues may reduce cystoid edema in DCMD and may thus prevent disease-related visual loss.[ncbi.nlm.nih.gov]
  • NCT00804206 Phase 4 bevacizumab;bevacizumab 12 Ozurdex Versus Ranibizumab Versus Combination for Central Retinal Vein Occlusion Unknown status NCT01827722 Phase 4 Ozurdex;Ranibizumab;Combination Ozurdex with Ranibizumab PRN 13 Intravitreal Ranibizumab for the Prevention[malacards.org]
  • Prevention Avoiding the risk factors for macular degeneration may help prevent it. This includes avoiding tobacco smoke and eating a diet low in saturated fat.[medical-dictionary.thefreedictionary.com]
  • Prevention Regular eye examinations can detect cystoid macular edema early. Contact Us Please contact us at (239)390-3339 or click here to use our contact form[retinaandmacula.com]
  • Compelling evidence suggests this potent antioxidant may be among the most important nutrients for the prevention of blindness.[articles.mercola.com]

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