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[Progression of Bietti's crystalline dystrophy]
1Istituto di Clinica Oculistica dell'Università di Palermo, Italia.
Journal Francais D'Ophtalmologie
|January 1, 1991
Summary
Bietti's crystalline dystrophy, a rare tapetoretinal degeneration, progresses slowly with late-onset visual impairment. This case highlights late symptom development and advanced choriocapillary atrophy.
Area of Science:
- Ophthalmology
- Medical Genetics
Background:
- Bietti's crystalline dystrophy is a rare tapetoretinal degeneration.
- Characterized by glittering deposits in the retina and cornea.
- Often presents with progressive vision loss.
Observation:
- A case study of a patient with Bietti's crystalline dystrophy over 5 years.
- Initial examination at age 27.
- Progression noted with impaired night vision but stable visual acuity.
Findings:
- Reduced retinal crystals and increased pigmented atrophy at the posterior pole.
- Development of choriocapillary atrophy.
- Electroretinogram amplitude reduction and paracentral visual field defects.
Implications:
- Confirms late onset and slow progression of Bietti's dystrophy.
- Highlights choriocapillary and choroidal atrophy in advanced stages.
- Advanced Bietti's dystrophy can mimic severe retinitis pigmentosa.