The most common epithelial dystrophy seen in the fellowship exam.
INHERITANCE PATTERN
AD
Visual Acuity
Symptoms begin in the first or second decade with painful, recurrent epithelial erosions. Vision is affected by both anterior scarring with surface irregularity and anterior stromal edema.
CORNEA
On Slit Lamp Examination, Multiple subepithelial ring-shaped opacities (honey-comb appearance) in the center of the cornea.
The corneal surface is irregular with ferritin deposition.
Fluorescein staining for corneal erosions
FELLOW EYE
Similar Changes
Corneal graft, which may be penetrating or lamellar.
Recurrences are common so that the graft may have signs of recurrence.
Question:
What are the features of Reis-Buckler's syndrome?
Autosomal dominant
It appears in the first few years of life and mainly affects Bowman's layer.
Keratoplasty (lamellar or penetrating) for impaired VA. Recurrence is common.
What are the pathological changes seen in Reis Buckler's Dystrophy?
Histopathologically, there is disruption and absence of Bowman's layer, with replacement by fibrocellular tissue that corresponds clinically to areas of subepithelial opacification.
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