Fuchs Endothelial Dystrophy

Question 1

Aetiology

Answer 1

Bilateral, progressive, non-inflammatory endothelial corneal dystrophy where there is endothelial dysfunction that leads to oedema, reduced vision, and epithelial bullae.

usually sporadic but can be autosomal dominant inheritance

Question 2

Predisposing factors

Answer 2

Females
4th decade
White people
Smoking
Genetics

Question 3

Symptoms

Answer 3

Asymptomatic in early stages

Blurred vision, glare, reduced contrast worse on waking, tends to improve as the day goes on - more corneal oedema on waking.

Diurnal variations in refraction - more myopic on waking.

Reduced vision in more severe cases.

If epithelial bull rupture, they may experience sharp pain, photophobia, watering.

Question 4

Signs

Answer 4

Signs begin centrally before extending into peripheral cornea, bilateral but asymmetric

Guttata on endothelium, beaten-metal appearance.

Fine pigment on endothelium.

Thickened DM. DM folds.

Microcystic epithelial oedema -> epithelial bull, ground glass appearance

Increased CCT. Stromal oedema and thickening.

Subepithelial/posterior stromal scarring from chronic oedema

Peripheral neovascularisation and pannus in advanced disease

Question 5

Non-pharmacological management

Answer 5

Specular microscopy and other imaging to monitor.
CCT as baseline to check for progression

If there is pain, therapeutic CL to protect exposed corneal nerves

Sunglasses / tint to help with glare

If referring for cataract, include Fuch’s diagnosis as risk of corneal decompensation is greater in a PX with Fuch’s who has cataract surgery

Question 6

Pharmacological management

Answer 6

Lubricants
Sodium Chloride eye drops

Question 7

Refer?

Answer 7

Initial measures then ROUTINE referral

Question 8

Management at hospital?

Answer 8

Monitoring with CCT, endothelial cell counts, specular microscopy

Cataract counselling for Px’s with Fuchs.

Various surgeries can be done if symptoms are no longer managed - DSAEK, DSEK, UTDSEK, DMEK, PK