1. Clinicopathologic Case
Ravi Patel MD MBA
Julia Kofler MD
Charleen Chu MD PhD

2. Brief History 69 year old African American Female
Patient had extensive history of idiopathic intraocular inflammation.
She progressed to where her vision was NLP from advanced glaucoma
Initially 180 degrees of cyclophotocoagulation was attempted without reduction of pressure
Eye then became painful and patient elected to proceed with enucleation (PHS )

3. Ciliary Body Depigmentation
- Likely due to previous cyclophotocoagulation
DISLOCATED IOL

4. Found in 27% of population, not believed to be clinically significant
Cobblestone Degeneration

5. Extensive Optic Nerve Cupping
Vascular Sheathing

6. Vascular Sheathing/Frosting
Macular Edema

7. Discussion- Macular Edema
Most commonly caused by retinal vascular disease from diabetes, hypertension and venous occlusive disease.
In this patient it is most likely from chronic intraocular inflammation as macular edema is a well known complication of uveitis
Other less common etiologies of macular edema include papilledema, choroidal neovascular membrane, macular degeneration, retinitis pigmentosa, toxic maculopathy, post-operative (Irwin-Gass Syndrome)

8. Treatment – Macular Edema
Widely accepted is focal/grid photocoagulation
Also accepted therapy is topical, periocular, intraocular or even systemic corticosteroids
There is emerging evidence to suggest efficacy of intravitreal anti-VEGF therapy (bevacizumab)
As a final resort, vitrectomy surgery is considered

9. Thickened Ciliary Body Epithelial Basement Membrane
Highlighted by PAS stain
Usually this membrane is barely visible.
Becomes more prominent in inflammatory conditions, and more commonly diabetes mellitus.

10. Neovascularization of Iris
Thin fibrovascular membrane on anterior iris surface

11. Discussion - Neovascularization
Usually a sequellae of retinal ischemia, now thought to be VEGF mediated
Commonly associated with diabetes mellitus, venous occlusive disease, carotid occlusive disease
Less commonly due to chronic intraocular inflammation, sickle cell disease, intraocular neoplasm, ROP, uveitis-glaucoma-hyphema syndrome
There is evidence to suggest treatment with anti-VEGF therapy and panretinal photocoagulation

12. Treatment-Neovascularization
Optimal therapy is to control the underlying disease
Ocular therapy revolves around improving oxygenation and reducing oxygen demand
Customarily panretinal photocoagulation is employed
Extrapolating from studies of macular degeneration patients with choroidal neovascularization, intravitreal anti-VEGF therapy has been helpful in promoting regression of neovascularization

13. Chronic Keratoconjunctivitis
Chronic lymphocytic infiltrate into conjunctival epithelium, usually nonkeratinized stratified squamous epithelium

14. Keratic Precipitates
Inflammatory aggregates on corneal endothelium

15. Chronic Iritis
Chronic lymphocytic infiltrate into iris stroma

16. Chronic Choroiditis
Mononuclear cells “small blue cells”

17. Retinal Vasculitis
Lymphoplasmacytic Perivascular Inflammation 17

18. Discussion - Uveitis
Any intraocular inflammation involving uveal tissue (iris, ciliary body, choroid) is termed uveitis. Disease is classified by which layers are affected, and chronicity.
This is a multi-factorial disease which can be:
Secondary to systemic infection
Noninfectious from systemic inflammatory disease
Idiopathic

19. Treatment - Uveitis
Infectious Uveitis involves treating the underlying infection along with supportive periocular therapy
Noninfectious Uveitis is usually managed using topical steroids and cycloplegics.
Recurrent episodes or posterior uveitis typically involves the use of periocular or systemic immunosuppression

20. Peripheral Anterior Synechiae
Segment represented by peripheral adhesion of iris tissue to cornea

21. Optic Nerve Cupping Paucity of Ganglion Cells Large Cup
Posterior Bowing of Lamina Cribosa

22. Discussion - Glaucoma Secondary Angle Closure Glaucoma
Due to neovascularization
Due to intraocular inflammation
Accepted theory is a fibrovascular membrane that “zips up” the angle thereby producing a resistance to aqueous outflow

23. Treatment - Glaucoma
Topical antiglaucoma therapy is often employed (caution is used to avoid miotics and prostaglandin analogues as they can exacerbate inflammation)
Second line therapy usually involve insertion of setons as trabeculectomies are subject to a high rate of failure.

24. Diagnosis:
SECONDARY GLAUCOMA WITH INFLAMMATORY AND NEOVASCULAR CONTRIBUTIONS Contributing factors: Idiopathic chronic panophthalmitis Hypertension