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Posterior amorphous corneal dysgenesis

B B Grimm1, G O Waring, S B Grimm

  • 1EJ-Maghraby Eye Hospital, Jeddah, Saudi Arabia.

American Journal of Ophthalmology
|October 1, 1995
PubMed
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Posterior amorphous corneal dysgenesis, a rare eye disorder, presents with diffuse corneal opacities and thinning. This study expands its known characteristics and ethnic prevalence.

Area of Science:

  • Ophthalmology
  • Genetics
  • Corneal Diseases

Background:

  • Posterior amorphous corneal dysgenesis (PACD) is a rare inherited corneal disorder.
  • Previous descriptions were limited to only four families worldwide.

Observation:

  • Seven new cases from three distinct ethnic backgrounds (Saudi Arabian, Egyptian, Indian) were identified.
  • Affected individuals presented with bilateral, diffuse, sheet-like opacities in the posterior corneal stroma.
  • Clinical findings included corneal thinning, cornea plana, astigmatism, and progressive ectasia.

Findings:

  • PACD is characterized by posterior stromal opacities extending to the limbus.
  • The condition exhibits a variable clinical spectrum, including iridocorneal adhesions.
  • Corneal thinning and ectasia are noted in more severe presentations.

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Implications:

  • This study expands the known spectrum and ethnic diversity of posterior amorphous corneal dysgenesis.
  • The findings support classifying PACD as a corneal dysgenesis due to its anatomical abnormalities.
  • Further research is needed to differentiate dysgenesis from dystrophy and investigate associated abnormalities.