Eyelids & the Anterior Segment:
                       Case eighteen

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This 20 year-old man was seen in the eye clinic because his father had had a corneal graft. His vision was 6/9 in both eyes.

a. What is the diagnosis?

Granular dystrophy.

It is an autosomal dominant stromal dystrophy. The lesions appear as well-demarcated granular opacities and the cornea between the lesions are clear.

b. What is responsible for the corneal appearance?

It results from hyaline deposits which are non-collagenous protein containing tryptophan, arginine, tyrosine and sulfufr-containing amino acids. The hyaline deposits stain bright red with Masson trichrome stain.

c. What is the natural history of this condition?

The lesions appear early in life but usually remains asymptomatic for years. The dystrophy is slowly progressive and tends to enlarge and involve the deeper layer. The vision seldom drops below 6/60.
When the vision is significantly affected, lamellar or penetrating keratoplasty can be performed depending on the depth of the lesion.