Phacoantigenic Response to Ruptured Lens Capsule: Clinicopathologic Case

Emily Deschler, MD

Charleen Chu, MD, PhD

March 2011

Brief History and Course

• 58 year-old woman

• Blind left eye since teens – unknown etiology

• No history of trauma

• Referred to glaucoma service when left eye became painful

Initial Physical Exam

• Vision:– Right eye: 20/20– Left eye: NLP

• IOP:– Right eye: 11– Left eye: 16

• Pupil:– Right eye: 4-3– Left eye: Irregular

• Anterior segment– Right eye: Shallow anterior

chamber with patent PI– Left eye: injected

conjunctiva, cornea with PEE, shallow anterior chamber with patent PI x2, iris with florid rubeosis, posterior synechiae, mature cataract

• Fundus:– Right eye: Normal, c/d 0.6

with healthy neuroretinal rim

– Left eye: no view

Brief History and Course

• Symptoms initially attributed to ocular surface disease and treated with artificial tears, topical steroids

• 1 year later, pain continued and patient elected to proceed with enucleation (PHS10-35123)

Calcific Band Keratopathy

• Gross photo of the cornea – entire cornea is

affected – calcification

spans from limbus to limbus

Calcific Band Keratopathy

• Seen clinically as calcific plaques in the interpalpebral zone – deposition of calcium in the epithelial basement

membrane, Bowman’s layer, and anterior stroma.– usually an intervening region of cornea between the

limbus and the calcification that is unaffected• 6 main causes

– chronic ocular disease (usually inflammatory), hypercalcemia, hereditary transmission, elevated serum phosphorus with normal calcium, exposure to mercury, silicone oil in ahakic eye

• Tx: remove overlying epithelium and then apply EDTA

Calcific Band Keratopathy

• H&E– 1. stippled

basophilia of Bowman’s layer, the calcium deposits merge to form a linear array along Bowman’s layer

– 2. keratocyte nuclei

2

1

Neovascularization of the Iris & Hyphema

1. Neovascularization of the iris – at this power cannot see individual vessels, but flattened anterior iris is a clue

2. Hyphema

1

2

Disorders Predisposing to Neovascularization of Iris and Angle

• Systemic vasular disease– Carotid disease– Giant cell arteritis

• Ocular vascular disease– Diabetes– CRVO– Coats– Eales– ROP– Persistent fetal vasculature– Anterior segment ischemia

• Ocular disease– Cronic uveitis– Chronic retinal detachment– Endophthalmitis– Stickler syndrome– Retinoschisis

• Intraocular tumors– Uveal melanoma– Metastatic disease– Retinoblastoma

• Ocular therapy– Radition therapy

Peripheral Anterior Synechia

• Iris stroma obliterates the angle recess

• Mechanisms of PAS formation:

– Contraction of an inflammatory, hemorrhagic or vascular membrane, band or exudate in the angle

– Forward displacement of the iris-lens diaphragm leading to a prolonged shallow/flat anterior chamber

• Extent of PAS may correlate with visual field damage, larger vertical cup-to-disc ratio, and higher untreated intraocular pressure

• Poor prognosis • Treat underlying cause

(ischemic retina with PRP) anti-VEGF, filtering surgery

1. Chronic Iritis 2. Neovascularization of the Iris

• Lymphocytes – small cells with

round, hyperchromatic nuclei and scant cytoplasm

– found in chronic inflammatory process

1

2

Chronic Plasmacytic Cycloiritis

• B lymphocytes may differentiate into plasma cells and produce immunoglobulin

• Eccentric “cartwheel” or “clockface” nuclei

Chronic Choroiditis

• Small blue cells

Phacoantigenic Response to Ruptured Lens Capsule

• Granulomatous reaction

• Macrophages filled with degenerated lens cortical material

Phacoantigenic Response to Ruptured Lens Capsule - PAS

Phacoantigenic vs PhacoanalyticGlaucoma

Phacoantigenic– Sensitized to own lens protein

following surgery or penetrating trauma

– Granulomatous reaction• Clinically

– Moderate anterior chamber reaction with KP on endothelium and lens capsule

– Low grade vitritis– Synechial formation– Not commonly associated with

glaucomatous optic neuropathy

• Treatment– Corticosteroids, aqueous

suppressants, and if unsuccessful, remove residual lens material

Phacolytic– Leakage of lens proteins

through the capsule of a mature or hypermature cataract

– Proteins, phagocytizing, inflammatory debris obstruct the uveal meshwork

• Clinically – Prominent cell and flare with

possible psuedohypopyon in the anterior chamber

– No KP– Morgagnian cataract with

wrinkled anterior lens capsule– Open anterior chamber angle

• Treatment– IOP lowering medications,

ultimately cataract extraction

Discussion: Phacoantigenic response to ruptured lens capsule

• Occurs following surgery or penetrating trauma causing sensitization to one’s own lens protein– Granulomatous reaction– Variable clinical presentation: moderate AC reaction

with KP on corneal endothelium and anterior lens surface (unlike phacolytic) with moderate AC reaction. Low grade vitritis, synechial formation, residual lens material may be in AC

– Not commonly associated with glaucomatous optic neuropathy.

• Treatment– Corticosteroids, aqueous suppressants, and if

unsuccessful, remove residual lens material

Discussion: Phacolytic response to mature cataract

• Proteins, phagocytizing macrophages, and other inflammatory debris obstruct the trabecular meshwork

• Generally an elderly person with a history of poor vision presents with sudden onset pain, conjunctival injection, elevated IOP, corneal edema, prominent cell and flair without KP and open anterior chamber angle

• Cellular debris may be present in the anterior chamber presenting as pseudohypopyon. The anterior capsule may appear wrinkled representing loss of lens material

• Treatment: – IOP lowering medications, ultimately cataract extraction

• Diagnosis:– Phacoantigenic immune response to

ruptured lens capsule

• Additional diagnoses:– Idiopathic chronic panophthalmitis– Neovascularization of the iris– Peripheral anterior synechiae– Calcific band keratopathy

Sources

• Basic Clinical Science Course Section 10: Glaucoma. American Academy of Ophthalmology 2009-2010

• Yanoff, Myron and Fine, Ben. Ocular Pathology. Mosby. 2002