iridocorneal endothelial syndrome

Introduction

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

• more common in women
• generally diagnosed between ages 30-50

Pathology

• corneal edema
• glaucoma
• severe vision loss when normal fluid inside the eye cannot drain properly
• movement of endothelial cells off the cornea onto the iris
• loss of cells from the cornea often leads to
  • corneal edema
  • distortion of the iris
  • variable degrees of distortion of the pupil
• movement of cells onto the iris plugs the fluid outflow channels of the eye, causing glaucoma

Clinical manifestations

• visible changes in the iris
• severe vision loss
• generally present in only one eye

Management

• treat glaucoma
• corneal transplantation

More general terms

• corneal disease (keratopathy)
• syndrome