iridocorneal endothelial syndrome

Grouping of three closely linked conditions: I: iris nevus (or Cogan-Reese) syndrome; C: Chandler's syndrome; E: Essential (progressive) iris atrophy Etiology: idiopathic Epidemiology: 1) more common in women 2) generally diagnosed between ages 30-50 Pathology: 1) corneal edema 2) glaucoma 3) severe vision loss when normal fluid inside the eye cannot drain properly 4) movement of endothelial cells off the cornea onto the iris 5) loss of cells from the cornea often leads to a) corneal edema b) distortion of the iris c) variable degrees of distortion of the pupil 6) movement of cells onto the iris plugs the fluid outflow channels of the eye, causing glaucoma Clinical manifestations: 1) visible changes in the iris 2) severe vision loss 3) generally present in only one eye Management: 1) treat glaucoma 2) corneal transplantation

General

corneal disease (keratopathy) syndrome

References

Iridocorneal endothelial syndrome http://www.nei.nih.gov/health/cornealdisease/index.asp#g