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Dense peripheral corneal clouding in Scheie syndrome
C G Summers1, C B Whitley, E J Holland
1Department of Ophthalmology, University of Minnesota, Minneapolis.
Cornea
|May 1, 1994
Summary
Scheie syndrome (MPS I-S) can present with unusual peripheral corneal clouding, preserving central vision. Diagnosis was confirmed by systemic features, electroretinogram, and absent alpha-L-iduronidase activity.
Area of Science:
- Ophthalmology
- Genetics
- Metabolic Disorders
Background:
- Scheie syndrome, a subtype of mucopolysaccharidosis type I (MPS I-S), is a rare genetic disorder.
- Corneal clouding is a common feature in MPS disorders, but the pattern can vary.
- Maintaining good central visual acuity despite peripheral corneal opacity is atypical.