Fuchs endothelial dystrophy

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. ... 

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). 

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. 

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... 

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. 

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. 

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.)...

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