Macular Corneal Dystrophy, symptom, prognosis genetics treatment

👁 What is Macular Corneal Dystrophy?

Macular Corneal Dystrophy (MCD) is a rare inherited disorder
characterized by grayish, ill-defined stromal opacities that progressively involve the entire cornea, including the limbus.

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🔍 Key Features

• Onset typically before age 10
• Begins in the superficial central stroma and spreads to the deep layers and periphery
• Appears as gray-white nodular lesions with diffuse haze between deposits
• Eventually leads to severe vision loss, especially after age 30

 

 

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🧬 Pathology

• Caused by accumulation of abnormal glycosaminoglycans (GAGs)
  resembling keratan sulfate
• Deposits found in stroma, Descemet's membrane, and endothelial cells
• Stains positive with Masson trichrome stain

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🧬 Genetics & Prognosis

• Autosomal recessive inheritance
• Much rarer and more severe than granular or lattice corneal dystrophies
• Most patients experience significant vision loss by their 30s or 40s

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💊 Treatment

• Penetrating keratoplasty (corneal transplant) is often required
• However, the disease may recur in the graft postoperatively

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✅ Summary

• Rarest and most severe of the stromal dystrophies
• Progressive, diffuse corneal opacities with early onset
• Corneal transplantation needed in most cases
• Recurrence in graft is possible