Lattice Corneal Dystrophy Clinical Presentation

Updated: Jun 04, 2019
  • Author: Danielle Trief, MD; Chief Editor: Hampton Roy, Sr, MD  more...
  • Print
Presentation

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.

Next:

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.

Previous
Next:

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.

Previous
Next:

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]

Previous