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Related Experiment Videos

Progressive Schnyder's corneal dystrophy

H J Ingraham1, H D Perry, E D Donnenfeld

  • 1Department of Ophthalmology, Geisinger Medical Center, Danville, Pennsylvania 17822-2120.

Ophthalmology
|December 1, 1993
PubMed
Summary

Schnyder's crystalline dystrophy, a corneal condition, may progress significantly over time. This challenges the traditional view of this cholesterol deposition disorder as nonprogressive after childhood.

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Area of Science:

  • Ophthalmology
  • Genetics
  • Corneal Diseases

Background:

  • Schnyder's crystalline dystrophy involves bilateral central anterior corneal deposits of cholesterol and lipids.
  • Traditionally considered nonprogressive after childhood, recent studies question this long-held belief.

Purpose of the Study:

  • To investigate the long-term progression of Schnyder's crystalline dystrophy.
  • To challenge the established understanding of the condition's nonprogressive nature.

Main Methods:

  • A family pedigree was analyzed, including a patient first reported in 1954 and followed for 25 years.
  • Corneal biopsies underwent light and electron microscopy for pathological examination.
  • Visual acuity was monitored over the follow-up period.

Main Results:

  • The study patient exhibited dramatic progression of corneal crystal deposition in a dense, discoid pattern.
  • Best-corrected visual acuity decreased from 20/30 to 20/80 in both eyes.
  • Ocular pathology confirmed cholesterol deposition consistent with Schnyder's crystalline dystrophy.

Conclusions:

  • Schnyder's crystalline dystrophy can exhibit significant progression over a patient's lifetime.
  • The classification of Schnyder's crystalline dystrophy as a stationary condition requires reevaluation.

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