Non-Infectious Corneal Disorders: Dellen, Subepithelial Infiltrates, Metabolic Keratopathies

Question 1

what are the 3 types of iron deposits in cornea?

Answer 1

Hudson Stahli Line, Stocker’s Line, Fleischer’s Ring

Question 2

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?

Answer 2

Hudson Stahli line

Question 3

iron deposits in basal epithelium at leading edge of a pterygium
pts are asymptomatic & no intervention needed

what type of iron deposit is this?

Answer 3

Stocker’s line

Question 4

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?

Answer 4

Fleischer’s ring

Question 5

What systemic medications cause corneal deposits?

What deposits are they?

Answer 5

• amiodarone &chloroquine → Whorl keratopathy (stromal deposits
• chlorpromazine → granular deposits in endothelium, Descemet’s & posterior stroma

Question 6

systemic anti-arrhythmic medication

what drug is this?

Answer 6

amiodarone

Question 7

systemic immunosuppressive & antimalarial medication

what drug is this? what corneal deposit does it cause?

Answer 7

chloroquine → Whorl keratopathy

Question 8

yellow-brown granular deposits in corneal endothelium, Descemet’s membrane, & posterior stroma

assoc. w/ stellate anterior subcapsular cataract

what drug is this?

Answer 8

chlorpromazine

Question 9

enzyme replacement therapy is a treatment for what disease?

Answer 9

Fabry’s

Question 10

how do you diagnose Fabry’s disease?

Answer 10

serum levels of alpha-galactosidase A

Question 11

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

Answer 11

Fabry’s disease

Question 12

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

Answer 12

Fabry’s disease

Question 13

what condition is an x-linked lysosomal storage disease

deficiency in the enzyme results in?

Answer 13

Fabry’s disease

abnormal tissue accumulation of glycolipid

Question 14

what is a rare autosomal recessive condition resulting in decreased biliary copper excretion & subsequent copper deposition throughout body (liver, brain, kidneys, cornea)

Answer 14

Wilson’s disease

Question 15

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?

Answer 15

Wilson’s disease → manifest between 1st & 4th decades of life

Question 16

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?

Answer 16

Wilson’s disease

seen in the posterior stroma

Question 17

these clinical findings are indicative of what condition?

2+ of following findings:

• Kayser-Fleischer rings**
• typical neurological symptoms
• low serum ceruloplasmin levels

Answer 17

Wilson’s disease

Question 18

if Wilson’s disease is left untreated…

Answer 18

disease is fatal

Question 19

what condition is a rare autosomal recessive lysosomal storage disease that causes widespread deposit of cystine crystals throughout body, including cornea, conjunctiva, & retina

Answer 19

cystinosis

Question 20

what does cystinosis frequently result in?

Answer 20

pediatric renal failure

Question 21

when does cystinosis manifest?

Answer 21

in early childhood → keratopathy develops in first year of life in infantile cystinosis & in adolescence in the adolescent form of disease

Question 22

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.

Answer 22

cystinosis

Question 23

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

Answer 23

metabolic keratopathies

Question 24

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?

Answer 24

subepithelial infiltrates

inflammatory cells are recruited from limbal vasculature

Question 25

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?

Answer 25

• 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

Question 26

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

Answer 26

subepithelial infiltrates

Question 27

what is caused by persistent drying of a focal area → usually due to adjacent elevated lesion?

what are some examples of elevated lesions?

Answer 27

Dellen

• pinguecula: usually elevated, benign degeneration of conjunctiva (can be close to limbus)
• large subconjunctival heme

Question 28

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?

Answer 28

Dellen

Question 29

what is the management of dellen?

Answer 29

manage underlying cause;

aggressive lubrication, bandage soft CLs