Dense peripheral corneal clouding in Scheie syndrome

C. G. Summers, Chester B Whitley, E. J. Holland, R. L. Purple, W. Krivit

Research output: Contribution to journalArticlepeer-review

12 Scopus citations


A 28-year-old woman with Scheie syndrome (MPS I-S) presented with the unusual feature of extremely dense peripheral corneal clouding, allowing maintenance of good central visual acuity. Characteristic systemic features, an abnormal electroretinogram result, and absent α-L-iduronidase activity confirmed the diagnosis despite the unusual corneal pattern of clouding.

Original languageEnglish (US)
Pages (from-to)277-279
Number of pages3
Issue number3
StatePublished - Jan 1 1994


  • Corneal clouding
  • Mucopolysaccharidosis
  • Retinal degeneration
  • Scheie syndrome


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