Thiel-Behnke

 Thiel-Behnke Dystrophy

  • 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.
  • Clinical features:
    • 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.