Vitelliform dystrophy

Common Name(s)

Vitelliform dystrophy

Best vitelliform macular dystrophy is an early-onset autosomal dominant disorder characterized by large deposits of lipofuscin-like material in the subretinal space, which creates characteristic macular lesions resembling the yolk of an egg ('vitelliform'). Later, the affected area becomes deeply and irregularly pigmented and a process called 'scrambling the egg' occurs. The disorder is progressive and loss of vision may occur ({5:Braley, 1966}; {46:White et al., 2000}). Although some morphologic similarities exist between adult vitelliform macular dystrophy and the juvenile form of vitelliform macular dystrophy, they are generally considered distinct entities ({7:Brecher and Bird, 1990}; {13:Felbor et al., 1997}; {23:Kramer et al., 2000}). In multifocal vitelliform macular dystrophy, lesions are typically manifested as sharply demarcated yellowish cysts in the macula, near the retinal vascular arcades, and surrounding the optic disc (summary by {4:Boon et al., 2007}).
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Vitelliform dystrophy" for support, advocacy or research.

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Vitelliform dystrophy" returned 47 free, full-text research articles on human participants. First 3 results:

Quantitative fundus autofluorescence and optical coherence tomography in best vitelliform macular dystrophy.
 

Author(s): Tobias Duncker, Jonathan P Greenberg, Rithambara Ramachandran, Donald C Hood, R Theodore Smith, Tatsuo Hirose, Russell L Woods, Stephen H Tsang, Fran├žois C Delori, Janet R Sparrow

Journal:

 

Quantitative fundus autofluorescence (qAF), spectral domain optical coherence tomography (SD-OCT) segmentation, and multimodal imaging were performed to elucidate the pathogenesis of Best vitelliform macular dystrophy (BVMD) and to identify abnormalities in lesion versus nonlesion fundus areas.

Last Updated: 14 Mar 2014

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Outer retinal structure in best vitelliform macular dystrophy.
 

Author(s): David B Kay, Megan E Land, Robert F Cooper, Adam M Dubis, Pooja Godara, Alfredo Dubra, Joseph Carroll, Kimberly E Stepien

Journal: JAMA Ophthalmol. 2013 Sep;131(9):1207-15.

 

Demonstrating the utility of adaptive optics scanning light ophthalmoscopy (AOSLO) to assess outer retinal structure in Best vitelliform macular dystrophy (BVMD).

Last Updated: 13 Sep 2013

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Intravitreal ranibizumab for type 3 choroidal neovascularization complicating adult onset foveomacular vitelliform dystrophy.
 

Author(s): G Querques, L Querques, N Leveziel, F Bandello, E H Souied

Journal: J Fr Ophtalmol. 2013 Jan;36(1):e1-4.

 

To describe the results obtained with intravitreal ranibizumab injections in a patient with adult onset foveomacular vitelliform dystrophy (AOFVD) complicated by Type 3 choroidal neovascularization (CNV).

Last Updated: 12 Feb 2013

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Reviews from the PubMed Database

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The terms "Vitelliform dystrophy" returned 0 free, full-text review articles on human participants.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Stem Cell Models of Best Disease and Other Retinal Degenerative Diseases.
 

Status: Recruiting

Condition Summary: Retinal Disease; Bestrophinopathy; Best Vitelliform Macular Dystrophy; Adult Onset Vitelliform Macular Dystrophy; Autosomal Dominant Vitreoretinalchoroidopathy

 

Last Updated: 11 Jun 2014

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Cell Collection to Study Eye Diseases
 

Status: Recruiting

Condition Summary: Retinal Disease; AMD; Retinal Degeneration

 

Last Updated: 17 Sep 2014

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Intravitreal Aflibercept Injections In The Treatment Of Pattern Dystrophy
 

Status: Recruiting

Condition Summary: Pattern Dystrophy of Macula

 

Last Updated: 17 Oct 2013

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