Sorsby's fundus dystrophy: a clinical study

PJ Polkinghorne, MRC Capon, T Berninger, AL Lyness… - Ophthalmology, 1989 - Elsevier
PJ Polkinghorne, MRC Capon, T Berninger, AL Lyness, K Sehmi, AC Bird
Ophthalmology, 1989Elsevier
A survey was undertaken of a family known to have Sorsby's fundus dystrophy. Fifty
members were reviewed, and 14 were found to be affected. Many of Sorsby's original
conclusions were confirmed, including the pattern of inheritance and age of visual loss.
Yellow material was present at the level of Bruch's membrane early in the course of the
disease. However, the earliest phenotypic marker was delayed filling of the choriocapillaris.
Abnormalities of choroidal perfusion became more profound and extended centrifugally with …
Abstract
A survey was undertaken of a family known to have Sorsby's fundus dystrophy. Fifty members were reviewed, and 14 were found to be affected. Many of Sorsby's original conclusions were confirmed, including the pattern of inheritance and age of visual loss. Yellow material was present at the level of Bruch's membrane early in the course of the disease. However, the earliest phenotypic marker was delayed filling of the choriocapillaris. Abnormalities of choroidal perfusion became more profound and extended centrifugally with time. The loss of central vision was commonly due to atrophy of the outer retina and choroid. Subretinal neovascularization was a rare occurrence. The homology between this dystrophy and age-related macular disease underlines the importance of the clinical findings in this family.
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