Non-Infectious Corneal Disorders: Dellen, Subepithelial Infiltrates, Metabolic Keratopathies Flashcards
what are the 3 types of iron deposits in cornea?
Hudson Stahli Line, Stocker’s Line, Fleischer’s Ring
iron deposition line in basal epithelium where tear film becomes stagnant
usually located on inferior ⅓ of cornea, asymptomatic
what type of iron deposit is this?
Hudson Stahli line
iron deposits in basal epithelium at leading edge of a pterygium
pts are asymptomatic & no intervention needed
what type of iron deposit is this?
Stocker’s line
iron deposits in basal epithelium in pts w/ keratoconus
usually seen at base of cone, but can be present 360
pts are asymptomatic & no intervention needed
what type of iron deposit is it?
Fleischer’s ring
What systemic medications cause corneal deposits?
What deposits are they?
- amiodarone &chloroquine → Whorl keratopathy (stromal deposits
- chlorpromazine → granular deposits in endothelium, Descemet’s & posterior stroma
systemic anti-arrhythmic medication
what drug is this?
amiodarone
systemic immunosuppressive & antimalarial medication
what drug is this? what corneal deposit does it cause?
chloroquine → Whorl keratopathy
yellow-brown granular deposits in corneal endothelium, Descemet’s membrane, & posterior stroma
assoc. w/ stellate anterior subcapsular cataract
what drug is this?
chlorpromazine
enzyme replacement therapy is a treatment for what disease?
Fabry’s
how do you diagnose Fabry’s disease?
serum levels of alpha-galactosidase A
what are these ocular manifestations indicative of?
- white to golden-brown corneal stromal deposits in a vortex pattern
- wedge-shaped posterior cataract
- conjunctival vascular tortuosity (aka corkscrew vessels) & aneurysms
- retinal vascular tortuosity
Fabry’s disease
what are systemic signs/symptoms indicative of?
- acroparesthesia: periodic burning pain in extremities & GI tract
- angiokeratomas: benign cutaneous capillary lesions
- cardiomyopathy, hypertension, stroke, renal disease, hypohidrosis, tinnitus & hearing loss
Fabry’s disease
what condition is an x-linked lysosomal storage disease
deficiency in the enzyme results in?
Fabry’s disease
abnormal tissue accumulation of glycolipid
what is a rare autosomal recessive condition resulting in decreased biliary copper excretion & subsequent copper deposition throughout body (liver, brain, kidneys, cornea)
Wilson’s disease
these systemic signs are indicative of what disease?
- chronic liver disease & hepatic failure
- acute renal failure
- neuropsychiatric disorders: tremors, behavioral changes, seizures
when do these signs manifest?
Wilson’s disease → manifest between 1st & 4th decades of life
what are these ocular signs indicative of?
- copper deposition in peripheral Descemet membrane known as Kayser Fleischer Ring (best seen in gonioscopy)
- no interval b/w limbus & Kayser Fleischer Ring
where can they be seen during an optic section?
Wilson’s disease
seen in the posterior stroma
these clinical findings are indicative of what condition?
2+ of following findings:
- Kayser-Fleischer rings**
- typical neurological symptoms
- low serum ceruloplasmin levels
Wilson’s disease
if Wilson’s disease is left untreated…
disease is fatal
what condition is a rare autosomal recessive lysosomal storage disease that causes widespread deposit of cystine crystals throughout body, including cornea, conjunctiva, & retina
cystinosis
what does cystinosis frequently result in?
pediatric renal failure
when does cystinosis manifest?
in early childhood → keratopathy develops in first year of life in infantile cystinosis & in adolescence in the adolescent form of disease
these signs/symptoms are indicative of what condition?
- deposition of crystals in all layers of cornea (doesn’t pick up dye)
- can lead to epithelial erosions & photophobia
- crystals have no effect on VA.
cystinosis
what are corneal deposits caused by inherited metabolic disorders of protein & amino acid metabolism or inherited lysosomal storage diseases called?
ex: cystinosis, Wilson’s disease, Fabry’s disease
metabolic keratopathies
what disorder occurs when there is penetration of WBCs into anterior stroma due to an inflammatory response to:
- surface toxins (bacterial medication)
- hypoxia (CL overwear)*
- conjunctivitis from infection (chlamydia, viral)
- corneal surgery
where are the inflammatory cells recruited from?
subepithelial infiltrates
inflammatory cells are recruited from limbal vasculature
Which subepithelial infiltrate (active or chronic) will & will not stain with NaFl?
Which infiltrate can lead to permanent scrarring?
Which infiltrate has little breaks in the epithelium?
- chronic will not stain w/ NaFl
- active infiltrates will stain w/ NaFl
- chronic infiltrates can lead to permanent scarring
- active infiltrates have little breaks in the epithelium
these treatments are for what disorder?
- bacterial toxin: antibiotic steroid combo
- medication toxicity: discontinue offending agent -> initial topical steroid
- chlamydia infection: oral antibiotic
- adenoviral infection: topical steroid
- HSV: antiviral w/ topical steroid
- hypoxia due to CL overwear: discontinue CL wear, antibiotic-steroid combo
subepithelial infiltrates
what is caused by persistent drying of a focal area → usually due to adjacent elevated lesion?
what are some examples of elevated lesions?
Dellen
- pinguecula: usually elevated, benign degeneration of conjunctiva (can be close to limbus)
- large subconjunctival heme
a localized area of corneal stromal dehydration & thinning w/ intact epithelium → area will not stain w/ NaFl → NaFl will pool in area
what disorder is this indicative of?
Dellen
what is the management of dellen?
manage underlying cause;
aggressive lubrication, bandage soft CLs
