Thiel-Behnke

• An unusual subepithelial dystrophy transmitted as an autosomal dominant trait, also called Honeycomb Dystrophy and classified as a variant of Reis-Bucklers'.

Clinical Features

• Presents in childhood.
• Symptoms: may present with recurrent painful erosions and decreased vision
• Signs
  • Often difficult to differentiate Honeycomb from Reis-Bucklers dystrophy, however in the former; the corneal surface is smooth, corneal sensation is normal
  • Typical honeycomb opacity in the corneal subepithelium region develops in the second decade of life

Treatment

• Superficial corneal debridement
• Photo therapeutic keratectomy maybe required in patient with severe recurrent erosions or reduced visual function.