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What Is X-Linked Corneal Dystrophy?

X-linked endothelial corneal dystrophy (XECD) is a rare genetic disorder affecting the innermost layer of the cornea, leading to progressive clouding and vision loss.

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What Is X-Linked Corneal Dystrophy?

X-linked endothelial corneal dystrophy (XECD) is a rare genetic disorder affecting the innermost layer of the cornea, leading to progressive clouding and vision loss.

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Endothelial Dysfunction

The condition causes the endothelial cells to fail, leading to corneal edema. Over time, the cornea loses its transparency, resulting in a "ground glass" appearance.

Unique Inheritance

Unlike most corneal dystrophies which are autosomal dominant, XECD follows an X-linked pattern, meaning it is significantly more severe in males.

Clinical Presentation

Symptoms often appear in early childhood and may include light sensitivity and blurred vision that is worse in the morning due to overnight fluid buildup.

Surgical Solutions

In advanced stages where vision is significantly compromised, a partial-thickness corneal transplant (DSEK or DMEK) may be necessary to restore clarity.

Frequently Asked Questions About X-Linked Corneal Dystrophy

How is it different from Fuchs'?

Both affect the endothelium, but XECD appears much earlier in life and follows a different genetic inheritance pattern.

Does it affect both eyes?

Yes, corneal dystrophies are typically bilateral, meaning they affect both eyes relatively symmetrically.

Can eye drops cure it?

No, though saline drops can temporarily reduce swelling, they cannot fix the underlying genetic endothelial defect.