Corneal Dystrophies

CORNEAL DYSTROPHIES


CORNEAL DYSTROPHIES

  • Group of disorders, characterised by a non-inflammatory, inherited, bilateral opacity of the cornea.
  • There is no vascularisation of cornea.
  • Dystrophies are classified according to anatomical involvement.

Anterior corneal Dystrophies

Stromal corneal Dystrophies

Posterior Corneal Dystrophies

Epithelium:

  • Meesamnn’s dystrophy
  • Epithelium Basement membrane dystrophy

Bowmnann’s layer:

  • Reis- Bucklers dystrophy
  • Anterior membrane dystrophy of Grayson-Wilbrandt
  • Honycomb dystrophy of Thiel & Behnke
  • Subepithelial mucinous corneal dystrophy
Stroma:

  • Granular dystrophy (Groenouw’s type 1)
  • Lattice dystrophy (Type I, Type III, IIIA & IV)
  • Macular dystrophy
  • Gelatinous droplike dystrophy
  • Fleck dystrophy
  • Pre- Descement dystrophy
  • Avellino’s dystrophy
  • Meretoja syndrome (Lattice type II)
  • Marginal crystalline dystrophy of Bietti
Endothelial dystrophy:

  • Fuch’s endothelial dystrophy
  • Congenital hereditary endothelial dystrophy
  • Posterior polymorphous dystrophy
  • Epithelial basement membrane dystrophy is the most common Anterior Corneal Dystrophy
  • Lattice dystrophy is the most common Stromal Corneal dystrophy.
  • Lattice Type I is the commonest type of dystrophy.
  • Endothelial corneal Dystrophy is the least common of the classical stromal dystrophies
  • Fuch’s Endothelial Dystrophy is the most common Posterior/Lattice Dystrophy

Fuch’s epithelial-endothelial dystrophy

  • Fuch’s dystrophy-slowly progressive bilateral condition.
  • Affecting females more than males.
  • Primary open angle glaucoma is associated with this cobdition.

Stages & Clinical features

1. Stage of cornea guttata

  • Hassal-Henle type of excrescences in the central part of cornea.
  • A gradual increase of central guttae with peripheral spread & confluence gives rise to the so called ‘beaten-metal appearance’.
  • This stage is asymptomatic.

2.Oedematous stage or Stage of endothelial decompensation

  • Early stromal edema & epithelial dystrophy
  • Patients complain of blurring of vision.

3. Stage of Bullous keratopathy

  • Follows long standing stromal edema.
  • Marked epithelial edema with formation of bullae, 

4. Stage of scarring

  • Cornea becomes opaque & vascularised.
  • May be complicated by secondary infection or glaucoma.

Treatment

  1. Edematous stage: 5% Sodium chloride (Hypertonic saline).
  2. Bullous keratopathy: Bandage soft contact lenses.
  3. Penetrating keratoplasty: treatment of choice

Exam Important

  • Corneal dystrophies are degenerations that are usually Primary and bilateral.

Anterior corneal Dystrophies

Stromal corneal Dystrophies

Posterior Corneal Dystrophies

Epithelium:

  • Meesamnn’s dystrophy
  • Epithelium Basement membrane dystrophy

Bowmnann’s layer:

  • Reis- Bucklers dystrophy
  • Anterior membrane dystrophy of Grayson-Wilbrandt
  • Honycomb dystrophy of Thiel & Behnke
  • Subepithelial mucinous corneal dystrophy

 

Stroma:

  • Granular dystrophy (Groenouw’s type 1)
  • Lattice dystrophy (Type I, Type III, IIIA & IV)
  • Macular dystrophy
  • Gelatinous droplike dystrophy
  • Fleck dystrophy
  • Pre- Descement dystrophy
  • Avellino’s dystrophy
  • Meretoja syndrome (Lattice type II)
  • Marginal crystalline dystrophy of Bietti
Endothelial dystrophy:

  • Fuch’s endothelial dystrophy
  • Congenital hereditary endothelial dystrophy
  • Posterior polymorphous dystrophy
  • Epithelial basement membrane dystrophy is the most common Anterior Corneal Dystrophy
  • Lattice dystrophy is the most common Stromal Corneal dystrophy.
  • Lattice Type I is the commonest type of dystrophy.
  • Endothelial corneal Dystrophy is the least common of the classical stromal dystrophies
  • Fuch’s Endothelial Dystrophyis the most common Posterior/Lattice Dystrophy
Don’t Forget to Solve all the previous Year Question asked on CORNEAL DYSTROPHIES

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