Corneal Dystrophy, Thiel-Behnke

Synonyms

Honeycomb corneal dystrophy; TBCD

Definition and Characteristics

Thiel-Behnke corneal dystrophy (TBCD) is an autosomal dominantly inherited disorder characterized by subepithelial alterations, affecting all but a peripheral 1-2 mm of the cornea. It presents in infancy with infrequent recurrent erosions and is associated with diffuse-cloudy opacities, which spread and become honeycomb-like with time. The honeycomb-like subepithelial opacities appear between the ages of 10-20 years and are to be regarded as a landmark of TBCD.

Prevalence

TBCD is a rare superficial corneal disorder described first in a family from Schleswig-Holstein, Northern Germany by Thiel and Behnke in 1967 [1]. Fifty-five family members in 11 generations were included in their study and 26 persons were found to be affected from TBCD. Weidle demonstrated that TBCD and Reis-Bücklers corneal dystrophy (RBCD) have been frequently confused in the literature [2]. A number