The uveal effusion syndrome and trans-scleral flow.

Journal Article (Journal Article)

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.

Full Text

Duke Authors

Cited Authors

  • Forrester, JV; Lee, WR; Kerr, PR; Dua, HS

Published Date

  • 1990

Published In

Volume / Issue

  • 4 ( Pt 2) /

Start / End Page

  • 354 - 365

PubMed ID

  • 2379646

Pubmed Central ID

  • 2379646

International Standard Serial Number (ISSN)

  • 0950-222X

Digital Object Identifier (DOI)

  • 10.1038/eye.1990.48

Language

  • eng

Conference Location

  • England