Cornea White Eye Diseases

Describe PEE/SPK

Tiny epithelial defects, will stain with fluorescein or Lissamine Green/Rose Bengal. Early sign of corneal compromise and location can be indicative of cause 

Describe PEK and diseases associated with

Granular, opalescent swollen epithelial cell, will stain with Lissamine Green/Rose Bengal and have NEGATIVE fluorescein staining.

Seen with viral infection or Thygeson SPK 

Describe subepithelial infiltrates and diseases associated with

Non-staining inflammatory cells, white and ill-defined and will cause light scatter (blurry vision)

Adenoviral keratoconjunctivitis, HZO, marginal keratitis

Describe filaments and diseases associated with

Mucus strands attached to cornea where the epithelium is broken- will be surrounded by tear debris 

Seen with dry eye and SLK 

Describe epithelial edema

Swelling of cornea due to endothelial compromise. Present as small epithelial vesicles that can coalesce into a blister and rupture

Describe corneal neovascularization

BV growth into the cornea at the limbus d/t chronic ocular irritation or hypoxia such as tight CLs, tight lids, blepharitis- common

Describe pannus

Superficial neovascularization with subepithelial degeneration- tremendous corneal compromise

Describe deep infiltrate

Inflammatory cells/debris/tissue necrosis in anterior stroma. Appears white/gray with conjunctival hyperemia 

Describe corneal ulceration

Epithelial defect paired with deep infiltrate. Looks like a white ill-defined hazy mark

Describe corneal melting

Tissue disintegration with or without infiltrate. Perforation may occur. This is seen with severe disease

Describe folds in Descemet

Dark, deep-appearing, criss-cross lines in the posterior stroma, possibly with haziness d/t edema. Commonly seen after surgery

Describe breaks in Descemet

Tears due to corneal enlargement, birth trauma or keratoconus- look similar to folds in Descemet but more opaque and wider

Describe Descemetocele

Protrusion of Descemet membrane into the more anterior layers of the cornea, seen with severe ulceration where there is loss of epithelium and stroma

Describe corneal dystrophy

Group of SLOWLY progressive, usually BILATERAL corneal opacification

Starts out in the CENTER and moves PERIPHERALLY

NON-inflammatory = WHITE EYE

Genetic, mostly autosomal dominant

Occurs in YOUNGER patients

Who gets EBMD and when does it onset?

Onsets 10-20

NO clear inheritance pattern, may be d/t trauma, but most common corneal dystrophy

Females more than males

EBMD cause/pathophysiology

Abnormal basement membrane synthesis occurs when the adhesion between basement membrane and epithelium is faulty. Causes epithelial tissue to heap up which can lead to recurrent corneal erosions

EBMD presentation

Often asymptomatic, blurred vision worse in AM, diplopia, dry eye, FBS, maps/dots/fingerprints, negative-staining lesions

EBMD complications

Recurrent corneal erosions- this occurs 20-20s and causes severe pain esp upon waking and causes epithelial defects

What are the epithelial-subepithelial dystrophies?

EBMD and Meesmann

How are corneal dystrophies treated?

Asymptomatic: pt education, artificial tears

Mild: hypertonic salt solution, topical steroids, oral tetracycline/doxy

Moderate/Severe: debridement, phototherapeutic keratectomy, stromal puncture, bandage CL

What is Meesmann?

Rare epithelial-subepithelial corneal dystrophy with cysts

Who gets Meesmann/when does it onset?

Onsets first year of life, autosomal dominant

What is the presentation for Meesmann?

Intraepithelial cysts/vesicles look like round opacities with clear spaces in between.

Blurry vision/glare, photophobia, discomfort, extreme pain if blisters rupture

What are Reis-Buckler and Thiel Behnke?

Bowman layer corneal dystrophies whose variants are only differentiable by electron microscopy

Who gets Reis-Buckler/Thiel-Behnke and when does it onset?

Onsets 0-10, autosomal dominant

How do Reis-Buckler/Thiel-Behnke present?

Both have blurry vision, photophobia, and diplopia with gray-white geographic opacities

Reis-Buckler denses with age and has increased corneal thickness, reduced corneal sensitivity, and irregular astigmatism > more serious

Thiel-Behnke has less-defined opacities in a ring/honeycomb pattern and no corneal irregularities

What is Lattice?

Most common epithelial-stromal corneal dystrophy defined by amyloid deposits

Who gets corneal lattice and when does it onset?

Onsets 0-10, autosomal dominant, also linked to systemic amyloidosis

How does corneal lattice present?

Blurry vision, pain, lattice-patterned deposits, haze, reduced corneal sensitivity

What is Granular 1/Granular 2?

Epithelial-stromal corneal dystrophies characterized by hyaline deposition

Who gets granular dystrophy and when does it onset?

Granular 1 onsets 0-10, granular 2 onsets 11-20

Autosomal dominant

What is the presentation for granular dystrophy?

Blurry vision, photophobia, glare

1: crumblike hyaline deposits with space between, reduction in corneal sensitivity

2: is like gran 1 but with lattice. discrete gray-white opacities and lines which do NOT cross, thorn/ring/stellate shaped deposits

What is macular dystrophy?

Stromal corneal dystrophy characterized by mucopolysaccharide deposits

Who gets macular dystrophy/when does it onset?

Onsets 0-10, autosomal recessive, more common in Iceland and Saudi Arabia

What is the presentation for macular dystrophy?

Blurry vision with significant loss in teens/20s, ill-defined white-gray deposits that migrate to limbus, entire cornea is cloudy, edema and decreased sensitivity - RCE are UNCOMMON here

What is Schynder?

Stromal corneal dystrophy characterized by lipid deposits

Who gets Schnyder/when does it onset?

Onsets 0-10 but usually dx’d teens/20s

Autosomal dominant

What is the presentation for Schynder?

Vision loss (esp. in 50s), glare, ring-like opacities or central comma shaped crystals, central haze, prominent arcus senilis, reduced corneal sensitivity, crystalline deposits and increased blood lipid levels

What is Fuch’s dystrophy?

Endothelial corneal dystrophy characterized by endothelial cell loss and guttata

Who gets Fuch’s and when does it onset?

Onsets 20s OR 50s

Autosomal dominant or sporadic

Females more than males

What is the presentation of Fuch’s?

Blurry vision worse in morning, light sensitivity, pain as the disease progresses. Four stages:

1. Guttata start centrally and spread out, guttata may be pigmented, pt is asymptomatic

2. Guttata coalesce and endothelial cells start to change shape, pt experiences blurry vision d/t edema

3. Blister like lesions form causing extreme pain upon rupture, pt experiences FBS, corneal infections, and epithelial microcysts

4. Scarring and vascularization occur, pt experiences minimal pain as cornea is almost dead, visual acuity is significantly reduced

What is PPMD?

Posterior polymorphous dystrophy, an endothelial corneal dystrophy characterized by abnormal endothelial cell proliferation where the endothelial cells think they are epithelial cells

Who gets PPMD/when does it onset?

Onsets 0-10, autosomal dominant

What is the presentation for PPMD?

Can be asymptomatic or experience blurry vision d/t edema, geographic gray opacities, haze, whirl-like pattern, vesicular lesions (pathognomonic) which look like transparent cysts in lines or clusters, horizontal bands across the cornea (train tracks)

What are possible complications of PPMD?

Secondary glaucoma, iris abnormalities d/t endothelial cell migration

What are characteristics of corneal degenerations?

White eye (no inflammation), slowly progressive, unilateral and asymmetric, several layers affected, occur in the periphery, older patients, no genetic component

What are characteristics of corneal ectasias?

Non-inflammatory (white eye), bilateral asymmetric, progressive, corneal thinning and bulging

What is arcus senilis?

Most common peripheral corneal opacity; corneal degeneration caused by stromal lipid deposition

Who gets arcus senilis?

Affects all ages- in older pts usually due to aging, it younger pts usually has a systemic association

What is the presentation of arcus senilis?

Starts superior/inferior and progresses circumferentially, 1mm wide, clear zone b/n opacity and limbus

What is Vogt Limbal Girdle?

Common peripheral corneal opacity, corneal degeneration

Who gets Vogt limbal girdle/when does it onset?

Elderly individuals (60% of people over the age of 40 have it)

How does Vogt limbal girdle present?

Whitish crescent limbal bands at 3 and 9 with NO clear zone between lesion and limbus, chalky flecks, may have “swiss cheese” appearancce, more common nasally

What is Crocodile Shagreen?

Corneal degeneration that resembles crocodile skin

Who gets crocodile shagreen/when does it onset?

Onsets elderly individuals

How does crocodile shagreen present?

Greyish-white, polygonal stromal opacities resembling crocodile skin located in the peripheral anterior stroma; opacities are ill-defined and separated by clear spaces

What is band keratopathy?

Corneal degeneration caused by calcium salts in Bowman’s layer, basement membrane, and/or stroma

What causes band keratopathy?

Many causes- disease (mainly anterior uveitis and glaucoma), birthdays, metabolic disorders, phthiasis bulbi, chronic corneal edema, keratitis, end-stage renal failure, sarcoidosis, vitamin D toxicity, hyperparathyroidism

How does band keratopathy present?

Whitish lesions with transparent holes; advanced lesions are nodular/elevated which can lead to irriation; clear zone between deposits and limbus, gradual extension to the central cornea

How is band keratopathy treated?

Chelation to dissolve Ca

What is spheroidal degeneration?

Corneal degeneration where granules replace Descemet’s membrane due to chronic UV exposure

Who gets spheroidal degeneration?

Males more than females, people who spend hours outdoors daily

How does spheroidal degeneration present?

Amber-colored granules. In advanced cases there is blurry vision, central spread, and opacification

How is band keratopathy treated?

From low symptom severity to high severity: Sunglasses, phototherapeutic keratectomy, lamellar/penetrating keratoplasty

What is Salzmann Nodular Degeneration?

Corneal degeneration with stromal opacities and nodules with hyaline caused by chronic inflammation and irritation

Who gets Salzmann/when does it onset?

Onsets 59 years old, women more than men

What diseases can lead to Salzmann?

found in people with trachoma, chronic blepharitis, chronic allergic keratoconjunctivitis, and dry eye

How does Salzmann present?

Nodules are whitish/blue/gray and round or elongated; presents with dryness, FBS, and decreased vision

How is Salzmann treated?

Lubrication, superficial or therapeutic keratectomy, lamellar keratoplasty

What is pellucid marginal degeneration?

2nd most common corneal ectasia where the cornea bulges in shape and thins at the periphery

When does pellucid marginal degeneration onset?

Adulthood (later than keratoconus)

How does pellucid marginal degeneration present?

Blurry vision, ATR astigmatism, kissing bird/butterfly topography, crescentric 1-2mm band of inferior corneal thinning 4-8 o’clock, Fleischer ring, Vogt’s striae, hydrops (rare)

How is pellucid marginal degeneration treated?

Spectacles, soft/GP/scleral CLs, lamellar keratoplasty

What is keratoglobus?

Rare corneal ectasia where the cornea becomes globular in shape and thin

Who gets keratoglobus/when does it onset?

Onsets either birth or adulthood

• Birth: Ehlers-Danlos, Leber congenital amaurosis, blue sclera

• Adulthood: keratoconus, pellucid marginal degeneration

How does keratoglobus present?

Decreased VAs, myopia, irregular astigmatism, globular cornea with thinning, hydrops (rare), corneal rupture with mild trauma

How is keratoglobus treated?

Specs (eye protection), sclerals, lamellar keratoplasty

Who gets keratoconus/when does it onset/what is it associated with?

Autosomal dominant, onsets teens/20s

Systemic assoc: down syndrome, ehlers-danlos, marfan, osteogenesis imperfecta

Ocular assoc: vernal keratoconjunctivitis and eye rubbing

What is keratoconus?

Corneal ectasia where the cornea bulges and becomes conical, associated with blurred vision with multiple spec changes over short periods and difficulty refracting to 20/20

What visual symptoms are associated with keratoconus?

High myopia and irregular astigmatism, scissoring on retinoscopy, hard to refract to 20/20 and 20/30 is considered a success for these patients

What do keratometry and corneal topography look like for a keratoconic patient?

Keratometry: normal is 43-45 D

• Mild will be <48

• Moderate 48-54

• Severe >54

Corneal topography will show inferior steepening and distortion of Placido’s disc

What may you find on SLE on a keratoconic patient?

Conical corneal protrusion inferiorly, stromal thinning and scarring inferiorly with progression, Fleischer ring, Vogt’s striae, Munson sign (v shaped lower lid in downward gaze), charleaux sign (oil droplet on red reflex)

What is corneal hydrops?

Breaks in Descemet’s membrane causing aqueous to flow into the stroma and resulting in edema—results in pain, photophobia, epiphora, decreased vision, corneal haze, hyperemia and anterior chamber rxn—associated with keratoconus and rarely other corneal ectasias

How are hydrops treated?

Heals on its own in 6-10 weeks. Tx will depend on individual

• Topic cycloplegics = pain control

• Hypertonic salt ointment = pull water from cornea

• Topical antibiotics = prevent infection

• Topical NSAIDs/steroids = calm down inflammation

• Bandage CL = promote corneal healing

How is keratoconus treated?

Specs, rigid CLs, sclerals, intacts in the stroma to flatten the cornea (not as common anymore), corneal cross-linking, penetrating keratoplasty

What is corneal cross linking?

Tx for all keratoconic patients to halt its progression where cornea is exposed to Riboflavin (B12) and UVA to strengthen it—essentially giving the cornea “sun exposure” to turn it leathery while also protecting it from too much damage. Can be done with epi on or off, but more effective if epi is off

What is Wilson disease?

Metabolic disorder of the cornea with hepatolenticular degeneration, caused by a deficiency in copper-carrying blood protein and results in excess copper in certain tissues, especially liver, brain, and eyes

Who gets Wilson/when does it onset/what is it associated with?

Onsets teens to early 20s; associated with liver disease (jaundice, bodily edema, bruising/bleeding problems, fatigue), neurological symptoms, psychiatric symptoms

How does Wilson disease present in eye?

Brownish-yellow ring of Copper in Descemet membrane also called Kayser-Fleischer ring around limbus, may have sunflower cataract

How is Wilson disease treated?

Treated by PCP to lower copper levels but no ocular treatment

What is Fabry disease?

Metabolic disease of the cornea where there is abnormal accumulation of glycolipids in the tissues

Who gets Fabry disease and what is it associated with?

X-linked so males more common than females; associated with cardiomyopathy and renal disease

How does Fabry disease present?

White/golden-brown corneal opacities in a vortex pattern “Whorl/vortex keratopathy,” wedge/spoke shaped posterior cataract, conjunctival vascular tortuosity/aneurysms, retinal vascular tortuosity

How is Fabry disease treated?

No ocular tx, only systemic by PCP