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 slidesSPECIMEN: 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

H&E slides

Acute and chronic lymphocytic corneal infiltration with stromal thinning

Retrocorneal fibrosis and granualtion material

Epithelial Surface

Ulcer

H&E

Corneal vascularization

Epithelial Surface

H&E slides

Loss of corneal endothelial cells

Normal corneal endothelium

Polys

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?