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The uveal effusion syndrome and trans-scleral flow.

Publication ,  Journal Article
Forrester, JV; Lee, WR; Kerr, PR; Dua, HS
Published in: Eye (Lond)
1990

Recent studies of the uveal effusion syndrome, a rare condition characterised by idiopathic spontaneous serous detachment of the retina and peripheral choroid, have suggested a primary scleral abnormality as the underlying cause. In particular, abnormal deposition of glycosaminoglycans within the sclera may impair normal trans-scleral flow of fluid and contribute to increased scleral thickness. In four cases of uveal effusion syndrome, we have confirmed the accumulation of glycosaminoglycans in the sclera. Histochemical studies show that most of this material is proteodermatan sulphate with a smaller contribution from proteochondroitin sulphate, while electron microscopy showed an increase in collagen fibril thickness. Secondary changes within the retinal pigment epithelium were also observed, particularly foci of proliferation which corresponded to the characteristic 'leopard-spot' fundal appearances of this disorder. We therefore suggest that the uveal effusion syndrome is due to a primary defect in proteodermatan synthesis and/or degradation by scleral fibroblasts and may represent a form of ocular mucopolysaccharidosis.

Duke Scholars

Published In

Eye (Lond)

DOI

ISSN

0950-222X

Publication Date

1990

Volume

4 ( Pt 2)

Start / End Page

354 / 365

Location

England

Related Subject Headings

  • Uveal Diseases
  • Syndrome
  • Sclera
  • Retinal Detachment
  • Ophthalmology & Optometry
  • Middle Aged
  • Male
  • Humans
  • Glycosaminoglycans
  • Female
 

Citation

APA
Chicago
ICMJE
MLA
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Forrester, J. V., Lee, W. R., Kerr, P. R., & Dua, H. S. (1990). The uveal effusion syndrome and trans-scleral flow. Eye (Lond), 4 ( Pt 2), 354–365. https://doi.org/10.1038/eye.1990.48
Forrester, J. V., W. R. Lee, P. R. Kerr, and H. S. Dua. “The uveal effusion syndrome and trans-scleral flow.Eye (Lond) 4 ( Pt 2) (1990): 354–65. https://doi.org/10.1038/eye.1990.48.
Forrester JV, Lee WR, Kerr PR, Dua HS. The uveal effusion syndrome and trans-scleral flow. Eye (Lond). 1990;4 ( Pt 2):354–65.
Forrester, J. V., et al. “The uveal effusion syndrome and trans-scleral flow.Eye (Lond), vol. 4 ( Pt 2), 1990, pp. 354–65. Pubmed, doi:10.1038/eye.1990.48.
Forrester JV, Lee WR, Kerr PR, Dua HS. The uveal effusion syndrome and trans-scleral flow. Eye (Lond). 1990;4 ( Pt 2):354–365.

Published In

Eye (Lond)

DOI

ISSN

0950-222X

Publication Date

1990

Volume

4 ( Pt 2)

Start / End Page

354 / 365

Location

England

Related Subject Headings

  • Uveal Diseases
  • Syndrome
  • Sclera
  • Retinal Detachment
  • Ophthalmology & Optometry
  • Middle Aged
  • Male
  • Humans
  • Glycosaminoglycans
  • Female