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Ocular Pathology Case Presentation Jeffrey Healey, M.D. Leela Raju, M.D. March 2011 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 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 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 Ulcer H&E slides Epithelial Surface Acute and chronic lymphocytic corneal infiltration with stromal thinning Retrocorneal fibrosis and granualtion material H&E Corneal vascularization Epithelial Surface H&E slides Loss of corneal endothelial cells Polys Normal corneal endothelium H&E slides Corneal keratinization Normal corneal epithelium H&E slides Lymphocyte infiltration into corneal stroma Clinical diagnosis • PUK (Peripheral ulcerative keratitis) – Occurs most commonly in association with • • • • • • Rheumatoid arthritis Wegeners granulomatosis SLE Polyarteritis nodosa Ulcerative colitis Relapsing polychondritis 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 Management • Primary goal is to minimize corneal melting – Corneal wetting – Promote re-epithelialization – Suppressing systemic-mediated inflammation • Corticosteroid therapy • Cytotoxic agents (cyclophosphamide, methotrexate) Management • Surgical Intervention – Penetrating keratoplasty – Tectonic graft – Lamellar graft 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?
