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[Schnyder corneal dystrophy and juvenile, systemic hypercholesteremia]
T Kohnen1, R W Pelton, D B Jones
1Cullen Eye Institute, Baylor College of Medicine, Department of Ophthalmology, Houston, Texas, USA.
Klinische Monatsblatter Fur Augenheilkunde
|August 1, 1997
Summary
Schnyder
Area of Science:
- Ophthalmology
- Genetics
- Dermatology
Background:
- Schnyder crystalline dystrophy involves cholesterol crystal deposition in the cornea.
- Typically autosomal dominant and non-progressive, rare sporadic and progressive forms exist.
- An association with Finnish ancestry and systemic hyperlipidemia is noted.
Observation:
- A 10-year-old male presented with photosensitivity and bilateral ring-shaped corneal opacities.
- Ocular examination revealed crystalline deposits in the anterior stroma.
- Elevated serum total cholesterol (201 mg/dl) and LDL-C (156 mg/dl) were detected.
Findings:
- This case represents a rare sporadic instance of Schnyder's corneal dystrophy.
- The patient exhibited ocular irritation and significantly elevated cholesterol levels.
- The elevated cholesterol levels suggest a potential link to systemic hyperlipidemia.
Implications:
- Early detection of Schnyder's corneal dystrophy is crucial for managing associated hypercholesterolemia.
- Screening for systemic hypercholesterolemia in patients and families with Schnyder's is recommended.
- Aggressive management of hypercholesterolemia may prevent cardiovascular complications.