lattice dystrophy

Etiology: amyloidosis Epidemiology: 1) can occur at any time in life 2) usually arises in children age 2-7 Pathology: 1) accumulation of amyloid deposits throughout the middle & anterior stroma 2) amyloid can accumulate under the corneal epithelium 3) erosion of the epithelium (recurrent epithelial erosion) a) alteration of the cornea's normal curvature, resulting in temporary vision problems b) expose the nerves that line the cornea, causing severe pain c) by about age 40, some patients will have scarring under the epithelium, resulting in a haze on the cornea that can greatly obscure vision Clinical manifestations: 1) funduscopy: a) deposits in the stroma as clear, comma-shaped overlapping dots & branching filaments, creating a lattice effect b) over time, the lattice lines will grow opaque & involve more of the stroma 2) clouding of the cornea may reduce vision 3) blinking can be painful Management: 1) eye drops & ointments to reduce the friction on the eroded cornea & to reduce eye pain 2) eye patch may be used to immobilize the eyelids 3) erosions usually heal within 3 days, although occasional sensations of pain may occur for the next 6-8 weeks 4) corneal transplantation a) disease may also arise in the donor cornea within 3 years after transplantation b) 50% recurrence within 2-26 years c) of these, 15% required a second corneal transplantation d) early lattice and recurrent lattice arising in the donor cornea responds well to treatment with the excimer laser

General

corneal disease (keratopathy) amyloidosis

References

Lattice dystrophy http://www.nei.nih.gov/health/cornealdisease/index.asp#i