ETIOLOGY:
•Autosomal dominant disorder.
•Mutation in the TGFBI (Transforming growth factor beta-induced) gene on chromosome 5q31.
•Begins in Early childhood (2 years) and has a very slow progression.
SYMPTOMS:
•Glare and photophobia.
•Visual acuity is affected in late stages when the opacities become more confluent.
•Painful recurrent erosions are rare.
SIGNS:
•Well-defined white discrete irregular granular opacities (snow-flake appearance) with clear intervening stroma within the central anterior stroma.
•Periphery is spared.
PATHOLOGY:
•Deposition of HYALINE in the corneal stroma that stains bright red with Masson trichome.
TREATMENT:
•Asymptomatic patients: no treatment required.
•Mild ocular irritation: Preservative-free lubricating eye drops.
•Acute corneal erosions: Topical broad-spectrum antibiotic eye drops and preservative-free lubricating eye drops. Overnight patching with broad-spectrum antibiotic ointment and cycloplegic.
High oxygen transmissible BCL.
•Recurrent corneal erosions: Epithelial debridement, Excimer laser PTK, DALK or PK in advanced cases.
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Image from Rajan Eye Care Hospital