Clinicopathologic Case

Ravi Patel MD MBAJulia Kofler MD

Charleen Chu MD PhD

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 (PHS09-28620)

DISLOCATED IOL

Ciliary Body Depigmentation- Likely due to previous cyclophotocoagulation

Cobblestone Degeneration

Found in 27% of population, not believed to be clinically significant

Vascular Sheathing

Extensive Optic Nerve Cupping

Macular Edema

Vascular Sheathing/Frosting

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)

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

Thickened Ciliary Body Epithelial Basement Membrane

Highlighted by PAS stainUsually this membrane is barely visible.

Becomes more prominent in inflammatory conditions, and more commonly diabetes mellitus.

Neovascularization of Iris

Thin fibrovascular membrane on anterior iris surface

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

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

Chronic Keratoconjunctivitis

Chronic lymphocytic infiltrate into conjunctival epithelium, usually nonkeratinized stratified squamous epithelium

Keratic Precipitates

Inflammatory aggregates on corneal endothelium

Chronic Iritis

Chronic lymphocytic infiltrate into iris stroma

Chronic Choroiditis

Mononuclear cells “small blue cells”

Retinal Vasculitis

Lymphoplasmacytic Perivascular Inflammation

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

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

Peripheral Anterior Synechiae

Segment represented by peripheral adhesion of iris tissue to cornea

Optic Nerve Cupping

Posterior Bowing of Lamina Cribosa

Large Cup

Paucity of Ganglion Cells

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

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.

SECONDARY GLAUCOMA WITH INFLAMMATORY AND NEOVASCULAR CONTRIBUTIONS

Contributing factors:Idiopathic chronic panophthalmitis

Hypertension

Diagnosis: