Congenital hereditary corneal oedema of Maumenee: its clinical features, management, and pathology

Br J Ophthalmol. 1987 Feb;71(2):130-44. doi: 10.1136/bjo.71.2.130.

Abstract

The clinical and histological features of congenital hereditary corneal oedema in 23 patients are presented. The series includes cases of both recessive and dominant inheritance. Although the condition is present at birth or in early childhood, visual development appears to be little impaired, if at all. Penetrating keratoplasty carries a relatively good surgical prognosis and can produce a substantial visual gain even when carried out late in life.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Cornea / pathology
  • Cornea / ultrastructure
  • Corneal Dystrophies, Hereditary / genetics*
  • Corneal Dystrophies, Hereditary / pathology
  • Corneal Dystrophies, Hereditary / surgery
  • Corneal Transplantation
  • Descemet Membrane / ultrastructure
  • Edema / pathology
  • Endothelium / ultrastructure
  • Female
  • Genes, Dominant
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Microscopy, Electron
  • Pedigree
  • Postoperative Complications