Adult Bests / Adult
onset foveomacular vitelliform dystrophy
/Bests vitelliform macular dystrophy (AOFVD)
centre of the retina (the fovea) is affected,
leading to problems with central vision
This is a type of dry macular degeneration (ARMD), may be known as adult onset Bests disease. Alternatively, it is termed dry ARMD with drusenoid lenions...drusen or drussenoid retinal pigment epithelial detachment (Ophth13).
However, the retina does become thin in the
affected area, and the sight may get slightly worse with ag as the retina thins. About 1/50 patients (with the thicker lesions) develop atrophy every year Ophth13.
A few patients do eventually develop wet ARMD, or other problems. However, this is not part of the condition, and is really coincidental.
- Case 21 for photos and scans
- photo and another
- Case 22
Adult onset foveomacular vitelliform dystrophy / epiretinal membranes
- Adult onset foveomacular vitelliform dystrophy page alone
As with all types of ARMD, a healthy lifestyle may slow the condition down. It is likely smoking is extremely harmful: it is likely that smoking will promote significant progression and also the conversion into wet ARMD.
OCT..like a very large drusen under the fovea: ..vision 6/24, very little change in the last 2 years
...vision 6/18 2 years ago
blue circle outlines the drusen-like material under the fovea
symptoms for 3 years, 6/18 vision both eyes
Basal laminar drusen and glomerulonephritis, can have viteliform lesions NOTE this. Also had cme.
- Adult Bests may be associated with reticular drusen
- Retina 2010 OCT changes AJO 2011 Unilateral BJO 12 HTRA1
- Best vitelliform
- Egg yolk,brilliant autofluourescence
- oct space under rpe,
- 4 stages, possibly 5th..cnv
- Egg yolk breaks down to leave a space
- choroidal thickness Retina16
- some dursenoid PEDs, especially if unilateral, may represent old central serous retinopathy
- Retina 1
- Bests Bestrophin-1gene; gene vmd 2
- 100 mostly mistsence mutations
- accumulation of a2e, altered turnover of ros..rod out segment
- Vmd2: rRecessive
- may be multifocal best
- Autosomal recessive bestrophinopathy
- may be microcornea, narrow angles, microphthalmos