Fuchs dystrophy

Fuchs (endothelial) dystrophy has a component of guttata, but the involvement is such that corneal physiology is affected adversely. Fuchs dystrophy occurs bilaterally, has been reported to be transmitted dominantly (with incomplete penetrance), and females are three times more likely to develop the condition. Prominent guttata initially occur centrally and then become extensive enough to involve the peripheral cornea. In Fuchs dystrophy the... 

Treatment options are primarily palliative, with the goal of improving patient comfort and function. The use of topical ophthalmic hypertonic agents may reduce epithelial edema related to Fuchs dystrophy however, these agents do not reduce stromal edema. The use of topical 5% sodium chloride drops six to eight times daily, along with 5% sodium chloride ointment instilled into the conjunctival sac at bedtime, may be instituted to determine... 

To help relieve patient discomfort due to the rupture of epithelial bullae, a therapeutic soft contact lens may be tried. Effective restoration of patient comfort and visual function for well-established Fuchs dystrophy, however, may be best achieved through penetrating keratoplasty (Figure 26-7). Fuchs dystrophy is the primary condition... 

Figure 26-6 Stromal haze of Fuchs dystrophy with bullae arrows). (Courtesy of Pat Caroline.)...

Patients with bullous keratopathy should have their lOP measured (even though corneal edema results in underestimated lOP) because angle-closure glaucoma can cause similar corneal edema. In addition, patients with Fuchs dystrophy have an increased risk of developing open-angle glaucoma in addition to the bullous keratopathy. Topical carbonic anhydrase inhibitors should be avoided in these patients because of the potential of worsening the corneal decompensation. 

Yuen HK, Rassier CE, Jardeleza MS, et al. A morphologic study of Fuchs dystrophy and bullous keratopathy. Cornea 2005 24 319-327. 

Regarding OVD application with intraoperative complications, tissue stability and movement must be differentiated from selective tissue isolation (Fig. 53). Healon 5, Healon GV, Microvisc (Plus), Morcher Oil (Plus), Viscorneal (Plus) (identical to Allervisc (Plus) in content), and other viscoelastic substances are considered suitable to counter a flattened anterior chamber, widen a small pupil, simplify a complicated capsulorhexis, resolve synechiae, or counter iridal and vitreous prolapse (Fig. 54). Endothelial dystrophy (Fuchs Dystrophy, advanced cornea guttata), torn posterior capsule or vitreous prolapse are particularly well suited for Viscoat application (followed by AMO Vitrax , HPMC Ophta H, Visco Shield , and other HPMC products). These viscoelastics are preferred substances in case of zonular dialysis with vitreous body prolapse or sinking nucleus (Fig. 55). 

French DD, Margo CE. Postmarketing surveillance of corneal edema, Fuchs dystrophy, and amantadine use in the Veterans Health Administration. Cornea 2007 26 1087-9. 

In humans, mutations in the a2(VIII) gene result in corneal endothelial dystrophies, Fuchs endothelial dystrophy (FECD), and posterior polymorphous comeal dystrophy (PPCD). Recently, it was reported that the mutation L450W in COL8A2 causes an early-onset subtype of Fuchs corneal dystrophy. ... 

Lederfein, D., Levy, Z., Augier, N., Mornet, D., Morris, G., Fuchs, O., Yaffe, D., and Nudel, U., 1992, A 71-kilodalton protein is a major product of the Duchenne muscular dystrophy gene in brain and other nonmuscle tissues, Proc Natl Acad Sci USA, 89, pp 5346-5350. 

Topical application of glycerin in concentrations from 50% to 100% results in a significant reduction of corneal edema within 1 to 2 minutes. Because application to the eye is painful, a topical anesthetic must be instilled before use. It is useful in ophthalmoscopic and gonioscopic examination of the eye in acute angle-closure glaucoma, bullous keratopathy, and Fuchs endothelial dystrophy. 

Figure 26-8 Diagram of the deep lamellar endothelial keratoplasty procedure for patients with Fuchs corneal dystrophy and pseudophakic bullous keratopathy. (Diagram courtesy of Dr. Mark Terry of the Devers Eye Institute in Portland, Oregon.)...

Although cataract surgery is a potential precursor to bullous keratopathy, there are many other causes. Fuchs endothelial dystrophy, infection, trauma, retained foreign body, posterior polymorphous dystrophy, chronic uveitis, chronically elevated intraocular pressure (lOP), and vitreous touch are all known causes of bullous keratopathy. Other less common causes of bullous keratopathy include corneal thermal injury secondary to carbon dioxide laser skin resurfacing, air bag trauma, the use of topical dorzolamide hydrochloride in glaucoma patients with endothelial compromise, and use of mitomycin C during trabeculectomy surgery. 

Approximately 16% to 46% of RCEs are associated with ABMD. Other corneal dystrophies associated with RCE include Fuchs , Reis-Biicklers, lattice, and granular. Dystrophic RCEs are typically bilateral and less severe. 

Serious Side Effects. Corneal decompensation in patients with preexisting endothelial compromise (e.g., Fuchs endothelial dystrophy) and hypotony have been reported with topical CAIs. Common adverse reactions to oral CAIs are summarized in Box 34-6. 

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