Dense peripheral corneal clouding in Scheie syndrome.
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 alpha-L-iduronidase activity confirmed the diagnosis despite the unusual corneal pattern of clouding.
Summers
C G
CG
Department of Ophthalmology, University of Minnesota, Minneapolis.
Whitley
C B
CB
Holland
E J
EJ
Purple
R L
RL
Krivit
W
W
D000328:Adult; D003318:Corneal Opacity / Q000209:etiology* / Q000503:physiopathology; D004596:Electroretinography; D005260:Female; D006801:Humans; D008059:Mucopolysaccharidosis I / Q000150:complications*; D012162:Retinal Degeneration / Q000150:complications / Q000503:physiopathology; D014792:Visual Acuity