Endothelial dystrophy such as

- Fuchs' endothelial dystrophy (the most frequently encountered form of corneal dystrophy).

Symptoms and diagnostic considerations: All patients suffer from a steadily increasing loss of visual acuity due to the generally gradual opacification of the cornea. This loss of visual acuity may progress to the point where a corneal transplant becomes necessary.

Macular dystrophy is the most rapidly debilitating form of the stromal dystrophies, resulting in a severe loss of visual acuity in the second decade of life. Epithelial and stromal corneal dystrophies are also often accompanied by painful and recurrent corneal erosion. Fuchs' endothelial dystrophy involves a gradual loss of endothelial cells that in time leads to bullous keratopathy (hydration of the cornea with stromal edema and epithelial bullae). The

Granular stromal corneal dystrophy.

Granular stromal corneal dystrophy.

Fig. 5.16 a Clinical findings include fragmented opacities surrounded by areas of clear cornea between the deposits.

b Histologic findings demonstrating hyaline deposits under Masson's trichrome stain.

patient typically will have poorer vision in the morning than in the evening, as corneal swelling is greater during the night with the eyelids closed.

Treatment: Depending on the severity of the loss of visual acuity (see above), a corneal transplant (penetrating keratoplasty; see p. 152) may be indicated. Because the cornea remains avascular in these disorders, the prognosis is good.

In Fuchs' endothelial dystrophy, a corneal transplant is the treatment of choice. Where the symptoms are not too far advanced, frequent application of hyperosmolar solutions can remove water from the cornea. However, this is generally only a temporary solution. The corneal transplant is performed in combination with a cataract extraction; patients with Fuchs' endothelial dys

150 5 Cornea Macular stromal corneal dystrophy.

150 5 Cornea Macular stromal corneal dystrophy.

Fig. 5.17 a Clinical findings include nodular opacities surrounded by areas of clear cornea between the deposits.

b Histologic findings demonstrating deposits of acidic mucopolysaccharides under AMP stain.

trophy that affects their vision are usually older and also have a cataract. The two procedures are combined because corneal decompensation often results from Fuchs' endothelial dystrophy following the surgical trauma of cataract extraction (see 5.2.5).

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