Congenital Stromal Corneal Dystrophy (CSCD), cause treatments, genetics

👶 What is Congenital Stromal Corneal Dystrophy (CSCD)?

Congenital Stromal Corneal Dystrophy (CSCD) is a very rare genetic eye disorder
present from birth, characterized by snowflake or feather-like stromal opacities
appearing bilaterally in both the central and peripheral cornea.

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🔍 What happens in CSCD?

• Vision is often poor from early infancy
• It can interfere with normal visual development, leading to amblyopia (lazy eye)
• In some cases, corneal transplantation may be needed
• Unfortunately, the visual prognosis is generally poor

 

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🧬 What causes it?

The condition results from abnormal collagen fibrillogenesis,
which causes irregular collagen fibers to accumulate in the corneal stroma,
leading to band-shaped or lattice-like opacities.

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✅ In Summary

• A rare congenital inherited disorder
• Snowflake or feather-like corneal opacities
• Non-progressive, but vision is often poor from early childhood
• Limited treatment options, and the prognosis is poor