1. Ocular Pathology Case Presentation Jeffrey Healey, M.D. Leela Raju, M.D. March 2011

2. Case Presentation 49 y.o male history of severe atopic disease presents with corneal pannus and thinning inferiorly OU Initial treatment aimed to preserve native corneal tissue: –doxycycline PO –Vitamin C –E-mycin ointment –Patanol –Preservative free artificial tears –Punctal occlusion

3. Clinical History Despite medical management progressive corneal thinning present Patient underwent corneal gluing OS and tectonic graft inferiorly OD Lab work-up: –PPD negative –VDRL/HSV/HIV negative –RPR positive –LP: high glucose and high protein in CSF –ANA/WBC/CRP/C-ANCA normal –RF positive –ESR slightly elevated (17) –FTAbs positive –CXR and Chest CT normal

4. H&E slides SPECIMEN: CORNEA, RIGHT EYE, PENETRATING TRANSPLANT 1. MIXED ACUTE AND CHRONIC KERATITIS WITH ULCERATION (see comment) 2. EXTENSIVE STROMAL SCARRING AND VASCULARIZATION 3. KERATINIZATION OF THE CORNEAL EPITHELIUM 4. PAUCITY OF CORNEAL ENDOTHELIAL CELLS 5. RETROCORNEAL FIBROSIS/GRANULATION TISSUE WITH ABUNDANT PIGMENT CELLS

5. H&E slides Acute and chronic lymphocytic corneal infiltration with stromal thinning Retrocorneal fibrosis and granualtion material Epithelial Surface Ulcer

6. H&E Corneal vascularization Epithelial Surface

7. H&E slides Loss of corneal endothelial cells Normal corneal endothelium Polys

8. H&E slides Corneal keratinization Normal corneal epithelium

9. H&E slides Lymphocyte infiltration into corneal stroma

10. Clinical diagnosis PUK (Peripheral ulcerative keratitis) –Occurs most commonly in association with Rheumatoid arthritis Wegeners granulomatosis SLE Polyarteritis nodosa Ulcerative colitis Relapsing polychondritis

11. Clinical Diagnosis Conjunctival biopsy typically demonstrates evidence of vaso-occlusive vasculitis Presentation: –May be bilateral but usually presents unilaterally –Usually limited to one sector of the peripheral cornea –Limbic vaso-occlusion present –+/- infiltrate present

12. Management Primary goal is to minimize corneal melting –Corneal wetting –Promote re-epithelialization –Suppressing systemic-mediated inflammation Corticosteroid therapy Cytotoxic agents (cyclophosphamide, methotrexate)

13. Management Surgical Intervention –Penetrating keratoplasty –Tectonic graft –Lamellar graft

14. Discussion Questions 1) What other corneal disease states will give you endothelial cell loss similar to that seen in this case? 2) What features seen in the pathology of this cornea are risk factors for graft failure?