Cornea: Dystrophies

Question 1

Describe map dot fingerprint corneal dystrophy?

Answer 1

• Epithelial/Anterior corneal dystrophy
• Dystrophy involving corneal basement epithelium
• Manifests self in different ways
• Px do not often have signs of inflammation
• Slow, degenerative process

Question 2

Describe Meesmann’s epithelial corneal dystrophy?

Answer 2

• Epithelial/Anterior corneal dystrophy
• Involves epithelium
• Autosomal dominant dystrophy
• Characterised by multiple, small cystic lesions in epithelium
• Can see on retro illumination
• Px do not often have signs of inflammation
• Slow, degenerative process

Question 3

Describe Bowman’s layer corneal dystrophy?

Answer 3

Reis-Bücklers’ corneal dystrophy:
* Autosomal dominant
* Involves Bowman’s layer
Formation of reticular opacities w/ eventual breakdown of overlying epithelium which can lead to corneal erosions – can be quite painful

Question 4

Describe Granular corneal dystrophy (Groenouw Type I)?

Answer 4

• Type of stromal dystrophy
• Autosomal dominant
• Deposition of hyaline bodies – develop over many years – start as small opacities before developing into crumb like opacities
• Diff diagnosis is infective keratitis – but a px with that is going to have a red, sore eye – this px may be asymptomatic
• Often presents routinely
• Eventually opacities can become dense and effect px’s vision but not acutely
• May see deposition of hyaline bodies

Question 5

Describe macular corneal dystrophy?

Answer 5

• Stromal dystrophy
• Autosomal dominant

Question 6

Describe central cloudy corneal dystrophy (posterior crocodile shagreen)?

Answer 6

• Greyish haze with darker lines through it
• Px’s are often asymptomatic
• Very common
• Does not need referred and does not need treatment

Question 7

Describe posterior polymorphous corneal dystrophy?

Answer 7

• Can be associated with glaucoma
• Malformation of inner lining of cornea and can also affect malformation of trab meshwork
• Lines on endothelium – criss-cross across the endothelial surface

Question 8

Describe Guttata/Fuchs’ Endothelial Dystrophy (corneal)?

Answer 8

• Disfunction of endothelium
• Endothelium works as pump to keep cornea clear & if endothelium becomes disfunctional, whole cornea can fail & cornea can become cloudy
• Dystrophies where number of endothelial cells dies off & get cell disfunction
• Seen on retro illumination with high magnification – dappled, orange peel effect
• Difficulty comes when endothelium becomes so disfunctional that the cornea becomes oedematous, stroma becomes hazy and px’s VA reduces
• Nothing can really be done to make endothelium work again
• In early form can give hypertonic saline 5% eyedrops – salty water to dehydrate cornea – only works for so long
• Definitive tx is corneal transplantation
• Risk with cataract surgery: Increased risk of cornea becoming oedematous as result to surgery – do cataract surgery sooner rather than later
  o Px who are at increased risk of endothelial failure whenever do cataract surgery – they should be aware of it

Question 9

Describe Congenital Hereditary Endothelial Dystrophy (CHED)?

Answer 9

• Congenital Hereditary Endothelial Dystrophy
• Autosomal dominant or recessive
• Presents at or soon after birth
• Causes significant visual loss from early age
• Risk of amblyopia
• Pxs can have glaucoma
• Look for hazy cornea at early age
• Best if picked up early