Lattice Corneal Dystrophy Differential Diagnoses

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

Diagnostic Considerations

Lattice corneal dystrophy is differentiated among the three major stromal corneal dystrophies (granular, macular, and lattice) based on history and examination.

Family history may distinguish macular dystrophy (autosomal-recessive inheritance) from granular and lattice (autosomal-dominant inheritance). Clinically, all three may present in the first decade of life; however, lattice and granular corneal dystrophy typically do not result in reduced visual acuity until later in life.

On examination, each has a distinct appearance. In granular dystrophy, the corneal opacities are discrete globular opacities with clear intervening stroma sparing the limbus. In macular dystrophy, opacities have indistinct margins, the intervening stroma is hazy, and lesions may extend to the limbus. In lattice dystrophy, lesions are linear and refractile, and, while there may be diffuse central haze, the limbal zone is clear except in extreme cases. The table below, adapted from Cornea (1996), presents characteristics of the three major corneal dystrophies. [13]

Table 1. Characteristics of the Three Major Stromal Corneal Dystrophies (Open Table in a new window)

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

Recurrent erosions in lattice dystrophy may follow the branching pattern of the underlying lattice network and can therefore appear similar to the dendritic lesions characteristic of epithelial herpetic keratitis. Epithelial lesions associated with lattice dystrophy, however, lack terminal bulbs and elevated borders. Owing to the implications in treatment planning, distinguishing recurrent erosions in lattice corneal dystrophy from herpetic keratitis is essential to the diagnostic process. [15]

Differential Diagnoses