Iridocorneal endothelial syndrome
 

There are iris atrophy with corectopia and polycoria. The contralateral eye is normal. The eye  may have previous glaucoma operation. The cornea may show signs of decompensation with  corneal oedema. The endothelium shows guttate-like changes. Further examination: look for tube (Molteno's) in the anterior chamber  posterior segment for signs of glaucoma

Questions:

1. What is the pathophysiology of ICE syndrome?

Answer
The main abnormality is in the corneal endothelium which unlike normal cornea appears like the epithelium. The endothelium usually becomes several layer thick and spread over the trabecular meshwork and the iris causing glaucoma and iris distortion respectively. The cause is unknown.

2. What are the different varieties of ICE syndrome?

Answer
Essential iris atrophy. There is progressive angle closure by peripheral anterior synechiae with corectopia, polycoria and iris atrophy. The changes are the results of pulling by the endothelium.
Iris naevus syndrome (Cogan-Reese). Angle changes are as above with diffuse naevus covering the anterior iris. Iris nodules may or may not be present. The nodules are the results of iris stroma protruding through the abnormal endothelium growing over the iris.
Chandler's syndrome falls between the above two entities.

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