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Biomedical subjects

J Sarks

Publications and source records attributed to J Sarks.

6 recordsLinked to original sources

Development of atrophy of the retinal pigment epithelium around disciform scars.

BACKGROUND/AIMS: Eyes with burnt out disciform scars secondary to age related macular degeneration (AMD) are regarded as visually stable. The aim of this study is to report the subsequent development of atrophy of the retinal pigment epithelium (RPE) around the scars and discuss the possible basis. METHODS: 20 eyes from 18 patients were observed to develop atrophy around choroidal neovascularisation (CNV). A method of measuring expansion of the atrophy over time is described using the Topcon Imagenet 2000 system. An additional 10 clinicopathological examples were reviewed. RESULTS: Clinically CNV became surrounded initially by a ring of pallor that progressed to an expanding band of atrophy of the RPE. It developed most rapidly in the first 3 years after CNV became quiescent but then continued to expand slowly to more than three times the size of the scar. Histopathological specimens showed large choroidal vessels entering the scars directly and a reduced number of small choroidal vessels beneath and around the scar CONCLUSIONS: Disciform scars may become surrounded by an expanding band of atrophy of the RPE, postulated to result from remodelling of the choroidal circulation. The ongoing enlargement of the resulting scotoma may need to be considered when planning management and assessing treatment outcomes.

Aged↗

Traumatic retinopathy resembling acute macular neuroretinopathy.

BACKGROUND: Acute macular neuroretinopathy (AMNR) is an uncommon disease of uncertain aetiology. We report on four males who developed unilateral changes following trauma which closely resembled acute macular retinopathy. METHODS: Retrospective review of selected cases. RESULTS: Four male patients developed a central or paracentral scotoma immediately or soon after an accident in which they suffered moderately severe bodily injury without direct ocular trauma. Vision was reduced in three patients. All four patients had characteristic well-circumscribed, dark macular lesions best seen using red-free light. The lesions cleared slowly in the two cases who returned for review. CONCLUSIONS: A picture that closely resembles AMNR may be seen after trauma. The mechanism may involve a sudden rise in ocular venous pressure. This may be an unrecognized cause of visual loss after non-ocular trauma which may be regarded as functional. The subtle retinal lesions may be overlooked unless the fundi are examined in red-free light and the macular dark areas are compared.

Acute Disease↗

[Retinal angiomatosis in association with Stargardt's disease. A case report].

We present what we believe to be the first reported association between retinal angiomatosis and Stardgardt's disease (Gass type 3). We have had the opportunity of following photographically a patient for the last eleven years. The earliest photos showed the macular problem whilst the latter shots showed the presence of a capillary angioma involving the temporal part of the optic disc and the adjacent retina of the left eye. We believe that this tumour arose in previously normal tissue. Examination and investigation of the patient revealed a peripapillary choroidal rupture at the site of the angioma, thalassaemia minor, retinitis pigmentosa, mildly dilated lateral ventricles, hepatic angiomata and a negative family history apart from a younger sister who was mildly retarded and showed an identical fundal picture of Stargardt's disease. Later examination revealed a small peripheral angioma inferiorly in the same eye. The diagnostic and management problems will be discussed.

Angiomatosis↗

Ocular manifestations of adult Niemann-Pick disease: a case report.

Niemann-Pick Disease Type B in a 33-year-old man is described. No family, racial or hereditary factors were noted in this case as in other reports in the literature. The ocular abnormalities in our patient were mild myopia, a mild generalized colour abnormality, and fundi showing a lipid ring-form opacity about the foveolas resulting in minimal visual impairment. Sphingomyelinase deficiency was found with the resulting deposition of phospholipid occurring most probably in the Müller cell. No neurological features were present and the patient was otherwise well.

Adult↗

Retinal changes in myotonic dystrophy: a clinicomorphological study.

This report appears to be the first ultrastructural study of the maculopathy and peripheral pigmentary retinopathy in myotonic dystrophy. Nine eyes from five patients observed during life are described. The findings were similar in all eyes, the retinal pigment epithelium in the macular region containing an accumulation of lipofuscin in large hyperpigmented cells. Pigment-laden profiles found in the subpigment epithelial space or subretinal space were interpreted as an attempt to discharge the pigment. Stress fibres of actin microfilaments were thrown into prominence by the irregularity of the pigment epithelium. In the periphery migration of retinal pigment cells into the retina occasionally resulted in the formation of bone corpuscles around occluded vessels, as occurs in retinitis pigmentosa; but more often the clumps were coarser and surrounded basement membrane material. Central and peripheral epiretinal membranes were also observed.

Cataract↗