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Marilyn Monroe!

Macular Corneal Dystrophy

Autosomal Recessive, CHST6 gene, Chromosome 16q22 - abnormal keratan sulfate

Three types have been defined on the basis of immunoreactivity to specific markers of antigenic keratan sulfates

Type 1 : No AgKS reactivity in the cornea or serum

Type 1A : Keratocytes manifest the AgKS reactivity. Not the serum or corneal extracellular material

Type 2 : The abnormal deposits in the cornea and serum have positive AgKS reactivity.

Symptoms :

Decreased vision & Photophobia

Signs :

  • Anterior stromal white opacities seen in first decade

  • Opacities progress to form a grainy, ground-glass haze between them

  • Extend from limbus to limbus

  • Over time, the cornea thins, endothelium can develop guttae

  • Recurrent corneal erosions

  • Decreased corneal sensations


Glycosaminoglycan accumulation within and outside stromal keratocytes beneath the epithelium and within endothelial cells.

Stains with Alcian Blue, Colloidal Iron or PAS stain

Treatment :

Phototherapeutic keratectomy (high risk of recurrence)

Corneal transplantation

Mnemonic for main corneal dystrophies abnormal deposits and staining

"Marylin Monroe Always Gets Her Men in L. A. County"

Macular dystrophy - Mucopolysaccharide - Alcian blue

Granular dystrophy - Hyaline materials - Masson trichrome

Lattice dystrophy - Amyloid - Congo red

Image from Rajan Eye Care Hospital

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