Corneal Deposits and Pigmentations

What are the symptoms of band keratopathy?

• asymptomatic

• may cause decreased VA, (+) FBS

• white spot on cornea that look like "Swiss cheese"

• subepithelial or anterior stromal deposit

• calcium phosphate salt crystals

Band keratopathy

How do we treat band keratopathy?

• chelation therapy with EDTA

• PTK

• DALK or PKP

What condition is this?

Band keratopathy

What condition is this?

Band keratopathy

What are the etiologies of band keratopathy?

• Ocular: uveitis (JRA — Still's Triad), phthisis bulbi, silicone oil, chronic corneal edema or keratitis

• Age-related

• Metabolic: elevated serum calcium, phosphorus, hyperuricemia, chronic renal failure

• Hereditary: familial cases, ichthyosis

What drugs can cause vortex keratopathy?

• amiodarone (HTN med)

• chloroquine/hydroxychloroquine (anti-malarial med)

• indomethacin (pain reliever / arthritis med)

• Rhopressa (netarsudil 0.02% once/day glaucoma medication)

  • present in —21% of patients at one month visit of 4 clinical trials

What drugs can cause corneal precipitates?

ciprofloxacin (topical fluoroquinolone)

• Usually epithelial in nature

• Causes: drug-related, Fabry disease

Vortex keratopathy

• X-linked lysosomal storage disorder

• Alpha-galactosidase-A deficiency

• Accumulation of glycosphingolipids

• Poor prognosis

Fabry disease

What condition is this?

Vortex keratopathy

What condition is this?

Vortex keratopathy

• white corneal deposit

• associated with topical ciprofloxacin therapy

  • 3rd generation fluoroquinolone

  • Tx: bacterial conjunctivitis, microbial keratitis

• precipitates occur in area of epithelial defect

• higher risk in older patients

• spontaneous resolution after d/c therapy

Corneal precipitates

What condition is this?

Corneal precipitates

Systemic diseases with associated corneal deposits

• Cystinosis

• Mucopolysaccharidosis

• Wilson disease

  • *Kayser-Fleischer ring

• Norum disease

• Immunoprotein deposits

• Fabry disease

  • *vortex keratopathy

• Tyrosinemia Type Il

• rare (3.5/1 M births)

• defect in amino acid metabolism

• accumulation of cysteine crystals in lysosomes

• affects cornea, conjunctiva, iris, lens, retina

  • evert lids; can see in TM with gonioscopy

• categorized based on age: infantile (often fatal), intermediate (juvenile), or non-nephropathic (adult) forms

Cystinosis

What is the inheritance pattern of cystinosis?

Autosomal Recessive

What condition is this?

Cystinosis

What condition is this?

Cystinosis

What are the symptoms of cystinosis?

Photophobia, eye pain, FBS, corneal abrasion

How do we treat cystinosis?

Topical cysteamine solution (oral is ineffective)

• Cystaran 0.44%

• Cystadrops 0.37%

• inherited deficiencies

• unable to hydrolyze mucopolysaccharides

• GAGs accumulate in tissues and organs

• corneal clouding/opacification - most common reason for ophthalmic consultation

  • diffuse punctate stromal opacities

  • corneal edema

Mucopolysaccharidoses

What is mucopolysaccharidoses associated with?

Pigmentary retinopathy, glaucoma, chronic papilledema, optic atrophy

What is the inheritance pattern of Mucopolysaccharidoses?

Autosomal recessive

What condition is this?

Mucopolysaccharidoses

Milder adult variant of mucopolysaccharidoses similar to arcus in appearance

MPS I (Hurler syndrome)

How do we treat Mucopolysaccharidoses?

Enzyme replacement therapy, bone marrow transplantation, supportive therapies

What condition is this?

Mucopolysaccharidoses

What is the inheritance pattern of Wilson Disease?

Autosomal recessive

• Decreased serum ceruloplasmin levels

• Widespread copper deposition

• Muscle rigidity, tremor, dementia

• “Sunflower” cataract

Wilson Disease

What condition is this?

Kayser-Fleischer ring

Copper deposition in Descemet's membrane

• golden-brown, ruby-red, or green ring

• can visualize with gonioscopy

• starts superiorly, then spreads to meet inferior deposits

• not manifested by all patients

Kayser-Fleischer ring

How do you treat a Keyser-Fleischer ring?

Refer to internist!

• order testing

  • serum copper and ceruloplasmin levels

  • urine copper level

• start chelating agents

• refer to neurologist

What is the inheritance pattern of Norum Disease/Familial LCAT Deficiency?

Autosomal recessive

• complete LCAT deficiency

• cholesterol accumulates in plasma and tissue

  • associated corneal opacification

  • lipid deposits (a "lipid keratopathy")

Norum Disease/Familial LCAT Deficiency

What are the three things that categorize Norum Disease/Familial LCAT Deficiency?

• corneal opacities

• hemolytic anemia

• renal failure

How do we treat Norum Disease/Familial LCAT Deficiency?

PKP

What is the inheritance pattern of Fish-Eye Disease?

Autosomal recessive

• partial LCAT deficiency

• corneal opacification

  • lipid deposits (a "lipid keratopathy")

  • concentrated peripherally - arcus-like configuration

• reduced VA (20/40 to HM)

Fish-Eye Disease

What condition is this?

Fish-Eye Disease

Bilateral bands of punctate, flake-like, crystalline deposits in the cornea

Immunoprotein deposits

What condition is this?

Immunoprotein deposits

What may be the presenting sign for a serious immunoglobulin disorder?

Immunoprotein deposits

Excess synthesis of immunoglobulins by plasma cells

• multiple myeloma (polychromatic)

• Waldenstrom macroglobulinemia (amorphous)

• monoclonal gammopathies (iridescent)

• rheumatoid arthritis

Immunoprotein deposits

How do we treat immunoprotein deposits?

Systemic therapy; DALK if vision affected

• Lysosomal storage disorder

• a-galactosidase-A deficiency

• accumulation of glycosphingolipids

  • Vortex keratopathy

  • wedge-shaped cataract

  • conjunctival + retinal vascular changes

  • CN Ill palsy

  • nystagmus

Fabry Disease

What is the inheritance pattern of Fabry disease?

X-linked

How do you treat Fabry Disease?

Enzyme replacement therapy

What condition is this?

Fabry Disease

What is the inheritance pattern of Tyrosinemia Type 2?

Autosomal recessive

• deficiency in amino acid metabolism

  • tyrosine aminotransferase

  • high plasma tyrosine levels

• recalcitrant bilateral pseudo-dendritic keratitis

  • inferotemporal

  • normal corneal sensitivity

  • no end bulbs, limited NaFl staining

Tyrosinemia Type 2

What are the symptoms of Tyrosinemia Type 2?

Photophobia, tearing, eye pain, conjunctival injection

What condition is this?

Tyrosinemia Type 2

What systemic conditions are associated with Tyrosinemia Type 2?

• Painful palmar and plantar hyperkeratotic lesions

• Cognitive impairment

How do we treat Tyrosinemia Type 2?

Dietary restriction

• small corneal opacity in white ring-like shape

• indicates the presence of a previous FB

• deposit contains iron and calcium

• superficial corneal stroma

Coats white ring

How do we treat coats white ring?

No treatment indicated

What condition is this?

Coats white ring

What is the histology of epithelial iron deposits?

Iron is deposited intracellularly as a ferritin-like material

Where is the source of iron in epithelial iron deposits?

The tear film

What is the etiology of corneal iron lines?

Altered tear flow secondary to distorted corneal shape

How do we treat corneal iron lines?

No treatment necessary

• epithelial deposition of iron due to irregular tear pooling at the base of cone

• found in 86% of KCN patients

• may be earliest sign of KCN

• enhanced visualization with cobalt blue filter

Fleisher ring

What condition is this?

Fleisher ring

Corneal iron line in the inferior-central cornea

• at the junction of middle and lower thirds of the cornea

• 0.5mm wide and 1-2mm long

• central part of the line curves downward (due to epithelial migration)

• age-related

Hudson-Stahli line

Corneal iron line at the leading edge of a pterygium

Stocker line

Corneal iron line at the edge of a glaucoma filtering bleb

Ferry line

Where can corneal iron lines be found (- the big 3)?

• within the margin of corneal grafts

• between radial keratotomy (RK) scars

• following laser in situ keratomileusis (LASIK)

• following intrastromal corneal ring placement

• during orthokeratology lens wear

• adjacent to other corneal elevations, e.g. Salzmann nodule

What condition is this?

Hudson-Stahli

What condition is this?

Stocker line

What condition is this?

Ferry line

• increased melanin in the basal epithelium (conjunctival or limbal) without elevated mass

• can extend onto cornea with streaks/whorls

• more common in African American patients (95%)

• note in EHR in case changes occur (photodocument)

• bilateral

• benign

Benign melanosis/melanosis oculi

How do we treat benign melanosis/melanosis oculi?

No treatment necessary

What condition is this?

Benign melanosis/melanosis oculi

• traumatic penetration of the globe with iron-containing foreign body

• corneal stroma can exhibit a rust-colored hue

Ocular siderosis

What additional testing should you do for ocular siderosis?

• DFE

• CT scan

• B-scan

What condition is this?

Ocular siderosis

• Deposits on the endothelium due to hyphema (especially 8 ball)

• Clears slowly from the periphery

Corneal blood staining

What condition is this?

Corneal blood staining

• fine dusting of pigment on endothelium

• will be same color as iris

• Age related or patholigic

  • secondary to

    • inflammation (uveitis)

    • glaucoma/PDS (Krukenberg spindle)

  • associated with

    • guttata

      • can be an early sign of Fuchs dystrophy

Endothelial pigment dusting

• stippled brown vertical line on endothelium

  • central cornea

  • shape d/t aqueous humor dynamics

Krukenberg spindle

Where does a Krukenberg spindle

Iris pigmentation liberated after lens zonules rub on iris

What condition is this?

Krukenberg spindle

Found in pigmentary glaucoma/pigment dispersion syndrome

• pigment may precede the glaucoma by as much as 20y

Krukenberg spindle

What condition is this?

Krukenberg spindle

• form during / after inflammatory events (e.g. uveitis)

  • lymphocytes (WBCs) on corneal endothelium

• may become pigmented with age

Keratic precipitates

What condition is this?

Keratic precipitates

• Epithelial cell proliferation

• Common after LASIK

  • At flat interface

• Risk factors

  • Epithelial defects at/around the time of Sx

  • EBMD

  • hypertonic Tx

  • Repeat Tx

Epithelial ingrowth

Hos do we treat epithelial ingrowths?

If affecting VA, lift flap and irrigate

What condition is this?

Epithelial ingrowth

• subepithelial open nerve endings

• associated with a variety of ocular and systemic diseases

  • keratoconus

  • Fuchs dystrophy

  • Reis-Buckler

  • multiple endocrine neoplasia

    • MEN Type lib

  • neurofibromatosis

    • NF-I

• also easily seen in normal, young patients

Prominent corneal nerves

What condition is this?

Prominent corneal nerves