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Anterior Corneal Dystrophies


Key Concepts

  • The epithelial corneal dystrophies each have distinguishing morphologic features that allow for accurate clinical diagnosis.

  • Epithelial basement membrane corneal dystrophy (EBMD; map-dot-fingerprint dystrophy) is the most common anterior dystrophy. While some cases are familial, EBMD is considered to be a degeneration or secondary to trauma in the majority of cases.

  • The primary symptoms of epithelial basement membrane corneal dystrophy are blurred vision and recurrent epithelial erosion.

  • Debridement of the centrally located epithelial basement membrane followed by diamond burr polishing is recommended and is cost effective when the patient is symptomatic with blurred vision and/or recurrent corneal erosion.

  • Reis-Bücklers and Thiel-Behnke corneal dystrophies are characterized by deposits in the region of Bowman membrane and the superficial cornea stroma. They have very similar presentations, disease course, and treatment.

The anterior corneal dystrophies include disorders that involve the corneal epithelium only, the epithelium and epithelial basement membrane, and Bowman membrane. The dystrophies involving Bowman membrane usually exhibit pathologic changes in the adjacent superficial stroma as well as the epithelial basement membrane and epithelium. Given the location of pathology, the anterior corneal dystrophies are typically symptomatic with recurrent corneal erosions (RCEs) and/or decreased vision secondary to either irregular astigmatism or anterior corneal opacity.

The most recent revision of corneal dystrophy nomenclature by the International Committee for Classification of Corneal Dystrophies (IC3D) is based on current phenotypic description, genetic analysis, and pathologic examination. This chapter describes Meesmann corneal dystrophy (MECD, C1), Lisch epithelial corneal dystrophy (LECD, C2), gelatinous drop-like corneal dystrophy (GDLD, C1), epithelial basement membrane corneal dystrophy (EBMD, majority degenerative, rarely C1), Reis-Bücklers corneal dystrophy (RBCD, C1), and Thiel-Behnke corneal dystrophy (TBCD, C1).

Epithelial Corneal Dystrophies

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