1. Ocular Pathology Case Presentation Kristin Rarey, M.D. February 2010

2. Case Presentation 32 y/o M s/p OD PKP Clinical history unknown, preliminary diagnosis unspecified H&E slides: – Mild bullous keratopathy – Centrally there is duplication of Bowman’s layer with healthy-appearing stroma intervening –Peripherally there is some epithelial down growth and irregularity of the stroma –Endothelium intact without evidence of guttae

3. Case # PHS10-1980 Arrows show double bowman’s membrane

5. Magnified view showing double bowman’s membrane

6. Clinical History Patient presented to UPMC Eye Center in 2005 for a second opinion regarding his high and irregular astigmatism Per the patient, he had surgery bilaterally for “masses” on his corneas at age 6 years –Clinically, evidence of previous bilateral epikeratophakia Patient using rigid gas permeable contact lenses at presentation

7. Myopic Degeneration OU OD - The disc is surrounded by peripapillary atrophy. There are central pigment changes. The fundus is tigroid in appearance.

8. Pentacam study demonstrates highly irregular corneal profiles bilaterally after LK. There is temporal thickening resulting in a nearly 30D difference in height between the temporal and nasal parts of the cornea in each eye.

9. Visante Anterior OCT Showing the irregular corneal curvature of the right eye

10. Surgical History 1983 –Bilateral Lamellar Keratoplasties (epikeratophakia) 10/29/2008 –Astigmatic Keratotomy OD: incisions made at the steep axis of astigmatism to induce corneal flattening 1/19/2010 –Penetrating Keratoplasty OD

11. PKP Histology Corneal button removed entirely and replaced with a button of donor cornea Variable findings depending on the indication for transplant –Bullous keratopathy –Corneal scarring –Keratinization –Corneal thinning

12. Epikeratophakia Histology Epithelium removed from patient and replaced with epithelium-Bowman’s- stroma from a donor (epikeratophakia) 2 Bowman’s layers observed: deeper Bowman’s is patient’s native tissue, more superficial Bowmans is donor tissue (along with epithelium and stroma)

13. Final Diagnosis Pathology slides PHS10-1980 are consistent with previous epikeratophakia (transplantation of donor epithelium/Bowman’s/stroma). Keratoconus is the most common indication for epikeratophakia –This is an attractive procedure because, unlike conventional, full- thickness transplants, it is 1)reversible and 2) associated with a low incidence of graft rejection as the endothelium is not transplanted –Alternative procedures include full thickness PKP, Deep Anterior Lamellar Keratoplasty (DALK), use of rigid gas permeable contact lenses in patients who can tolerate them, or placement of INTACS within the corneal stroma