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Dystrophies of Cornea

December 21, 2011  by: Dr. Simran Kaur  Points: 12   Category: Health  Earning $0.35   Views: 312

These are inherited disorders of cornea in which the cells have some inborn defects due to which pathological changes may occur with passage of time leading to development of corneal haze.

         

These are inherited disorders of cornea in which the cells have some inborn defects due to which pathological changes may occur with passage of time leading to development of corneal haze.

Types-

1. Anterior dystrophies.

2. Stromal dystrophies.

3. Posterior dystrophies.


1. Anterior dystrophies
- Anterior dystrophies are further of five types which are as follows-

a) Epithelial basement membrane dystrophy.

b) Reis- Buckler's dystrophy.

c) Meesman's dystrophy.

d) Recurrent corneal erosion syndrome.

e) Stocker-Holt dystrophy.


a) Epithelial basement membrane dystrophy - is seen in working age adults. Lesions involve corneal epithelium are bilateral dot like opacities.

b) Reis-Buckler's dystrophy - primarly involve bowman's layer. It occurs in childhood. It has autosomal dominant inheritance. There are recurrent erosions which lead to diffuse anterior scarring.

c) Meesman's dystrophy - is usually asymptomatic and has autosomal dominant inheritance.

d) Recurrent corneal erosion syndrome - occurs after injury to cornea by finger nail or any other sharp edge object.

e) Stocker-Holt dystrophy - is grey dots and lines between epithelium and Bowman's layer.


2. Stromal dystrophies
- are further of four types-

a) Granular dystrophy.

b) Lattice dystrophy.

c) Macular dystrophy.

d) Crystalline dystrophy.


a) Granular dystrophy - is an autosomal dominant disorder. There are milky granular hyaline deposits in anterior stroma.

b) Macular dystrophy - is an autosomal recessive disorder results from local enzyme deficiency.

c) Lattice dystrophy - is also an autosomal dominant disorder characterized by appearance of recurrent erosions and progressive clouding of central cornea.

d) Schnyder's crystalline dystrophy - is an autosomal dominant dystrophy is a ring shaped central corneal stromal opacity.


3. Posterior dystrophies
- are of four types-

a) Cornea guttata.

b) Fuch's epithelial endothelial dystrophy.

c) Posterior polymorphous dystrophy.

d) Congenital hereditary endothelial dystrophy.


a) Cornea guttata - is a drop like excrescences involving the entire posterior surface of Descemet's membrane. It usually occurs in old age.

b) Fuch's epithelial-endothelial dystrophy - usually affects females.

c) Posterior polymorphous dystrophy - is a dominantly inherited dystrophy of endothelium and Descemet's membrane. Opacities are found at the level of Descemet's membrane.

d) Congenital hereditary endothelial dystrophy - is very rare.




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