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Premature cataracts associated with generalized lentigo.
Transactions of the American Ophthalmological Society
|January 1, 1979
Summary
Generalized lentigo, also known as Leopard syndrome, is an autosomal dominant disorder. Ocular evaluations reveal distinct lens opacities appearing in the third decade, impacting vision over time.
Area of Science:
- Ophthalmology
- Genetics
- Dermatology
Background:
- Generalized lentigo (Leopard syndrome) is an autosomal dominant disorder.
- It presents with a constellation of characteristic features including lentigines, sensorineural deafness, growth retardation, and cardiac anomalies.
Observation:
- Ocular examinations of patients with generalized lentigo identified unique opacities.
- These opacities manifest as small, white, punctate, and comma-shaped structures within the lens cortex and nuclei.
Findings:
- Corneal opacities were observed to first appear in patients during their third decade of life.
- While potentially extensive, these lens opacities do not significantly impair visual function for approximately 20 years after their initial appearance.
Implications:
- Early ophthalmological screening is crucial for individuals with generalized lentigo.
- Understanding the progression of lens opacities can aid in managing visual impairment associated with Leopard syndrome.