Case Study: January 2007

A 25-year-old male presents to the ophthalmologists with a bilateral corneal problem. A history of bilateral recurrent corneal erosions is elicited.

He undergoes a left penetrating keratoplasty.

1-macro image of left corneal disc.

2-macro higher power of figure 1

3-macro cut surface of cornea. The arrows point to the pathology.

4 and 5-H & E of the corneal pathology at two magnifications.

6-Transmission electron micrograph of the corneal pathology.

Special histological stains-no significant findings.


1. What do figures 1 and 2 show ?

2. What does figure 3 show ?

3. What do figures 4 and 5 show ?

4. What is the light microscopic differential diagnosis

5. What does figure 6 show?

6. What is the diagnosis?

Click to show answers

1. Honeycombed subepithelial opacities and iron lines.

2. An undulating sub-epithelial band.

3. Eosinophilic , subepithelial undulating fibrous band with effacement of Bowman’s. Irregular epithelial morphology.

4. Reiss-Buckler and Thiel-Behnke corneal dystrophy.

5. “Curly” collagen fibers (with a diameter of 9-15 nm)

6. Thiel-Behnke corneal dystrophy. The curly collagen fibres are highly distinctive for Thiel-Behnke (see last reference).

Note that many cases of Thiel-Behnke are clinically, incorrectly called Reiss-Buckler corneal dystrophy.

Key references

Kobayashi A, Sugiyama K. In vivo laser confocal microscopy findings for Bowman’s layer dystrophies (Thiel-Behnke and Reis-Bucklers corneal dystrophies).
Ophthalmology. 2007 Jan;114(1):69-75.

El-Ashry MF, Abd El-Aziz MM, Hardcastle AJ, Bhattacharya SS, Ebenezer ND. A clinical and molecular genetic study of autosomal-dominant stromal corneal dystrophy in British population.
Ophthalmic Res. 2005 Nov-Dec;37(6):310-7.

Gear HC, Ramaesh K, Roberts F. Recurrence of Thiel-Behnke corneal dystrophy: an electron microscopic study.
Br J Ophthalmol. 2005 Apr;89(4):518.

Munier FL, Frueh BE, Othenin-Girard P, Uffer S, Cousin P, Wang MX, Heon E, Black GC, Blasi MA, Balestrazzi E, Lorenz B, Escoto R, Barraquer R, Hoeltzenbein M, Gloor B, Fossarello M, Singh AD, Arsenijevic Y, Zografos L, Schorderet DF. BIGH3 mutation spectrum in corneal dystrophies.
Invest Ophthalmol Vis Sci. 2002 Apr;43(4):949-54.

Kuchle M, Green WR, Volcker HE, Barraquer J. Reevaluation of corneal dystrophies of Bowman’s layer and the anterior stroma (Reis-Bucklers and Thiel-Behnke types): a light and electron microscopic study of eight corneas and a review of the literature.
Cornea. 1995 Jul;14(4):333-54.