Recurrence of Thiel-Behnke corneal dystrophy: an electron microscopic study.

The hereditary Thiel-Behnke corneal dystrophy, first described in 1967, is frequently confused with Reis-Bucklers dystrophy. Both conditions affect Bowman’s layer and can be difficult to differentiate both clinically and histopathologically. Previously, electron microscopic studies of corneal dystrophies affecting Bowman’s layer identified characteristic ‘‘curly fibres,’’ but it was unclear whether these were diagnostic of ThielBehnke or Reis-Bucklers. More recently, Kuchle et al have attempted to clarify the situation, identifying these corneal dystrophies as two separate disease entities, and proposing the less confusing nomenclature of corneal dystrophy of Bowman’s layer (CDB) type 1 (geographic or ‘‘true’’ Reis-Bucklers dystrophy) and CDB type II (honeycomb or Thiel-Behnke dystrophy). In this report we present a case of Thiel-Behnke dystrophy diagnosed by electron microscopy after recurrence in the corneal graft. This case illustrates the diagnostic difficulties surrounding corneal dystrophies of Bowman’s layer, and highlights the continuing importance of electron microscopy as a diagnostic tool.