History
Patients with lattice corneal dystrophy may have decreased vision, photosensitivity, ocular discomfort, diminished corneal sensation, and/or eye pain (from recurrent corneal erosions). Recurrent corneal erosions may precede corneal opacities and recognizable stromal disease. [1] As the pattern of inheritance for this dystrophy is autosomal dominant, one of the parents of the patient likely also has lattice corneal dystrophy.
Physical
Lattice corneal dystrophy is characterized by branching, refractile lattice lines, which are observed best in retroillumination. Both corneas are usually symmetrically involved. In the most common form, lattice corneal dystrophy type I, the refractile lines are more prominent centrally than peripherally. Later in life, stromal haze can develop. Eventually, significant stromal scarring and subepithelial fibrosis may occur.
Causes
The genetic defect of lattice corneal dystrophy type I has been mapped to the TGFBI (BIGH3) gene of chromosome 5q. Several other corneal dystrophies also have genetic defects of the TGFBI (BIGH3) gene, including granular dystrophy, Avellino dystrophy, and Reis-Bückler dystrophy. Lattice corneal dystrophy type II results from a mutation in the GSN gene.
Physical Examination
Lattice corneal dystrophy is diagnosed based on family history and slit-lamp examination.
Under slit-lamp examination, early manifestations of lattice dystrophy include subtle anterior stromal opacities or small refractile linear lesions. Later manifestations include thicker, radially oriented branching lines that form a latticelike pattern. Patients may also develop a central anterior stromal haze.
Later in the course of disease, lattice lines extend to the corneal periphery and progress to deep stroma. Retroillumination techniques may facilitate visualization of lattice changes. [13]
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Lattice corneal dystrophy. Image courtesy of James J. Reidy, MD, FACS, Associate Professor of Ophthalmology, State University of New York, School of Medicine & Biomedical Sciences, Buffalo, New York.
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Slit lamp image of lattice corneal dystrophy. Image courtesy of James J. Reidy, MD, FACS, Associate Professor of Ophthalmology, State University of New York, School of Medicine & Biomedical Sciences, Buffalo, New York.
Tables
Feature |
Granular Dystrophy |
Macular Dystrophy |
Lattice Dystrophy |
|---|---|---|---|
Age of onset |
First decade of life |
First decade of life |
First decade of life |
Heredity |
Autosomal dominant |
Autosomal recessive |
Autosomal dominant |
Reduced vision |
Fourth or fifth decade of life |
First or second decade of life |
Second or third decade of life |
Erosions |
Uncommon |
Common |
Frequent |
Opacities |
Discrete, intervening stroma Clear, not to limbus |
Indistinct margins, intervening Stroma hazy, extends to limbus |
Refractile lines and dots Usually not to limbus |
Material |
Hyaline |
Glycosaminoglycans |
Amyloid |
