Reduced corneal swelling and deswelling response in the iridocorneal endothelial syndrome.

Purpose. Despite severe abnormalities of the corneal endothelium in the iridocorneal endothelial (ICE) syndrome, the cornea can remain clear and maintain its normal thickness for years before corneal decompensation occurs. The aim of this study is to analyze this discrepancy by studying corneal hydration control in the ICE syndrome. Methods. In four subjects with unilateral ICE syndrome, without signs of corneal decompensation, a "corneal stress test" was performed in both the affected and the unaffected eye. The stress test measures the recovery of corneal thickness after swelling induced by wearing a soft contact lens with the eyes closed. Corneal thickness was measured by noncontact pachymetry. Results. Mean baseline corneal thickness of affected eyes (535 +/- 56 mu m) was not different from unaffected eyes (526 +/- 39 mu m). On the other hand, the corneal stress test showed a smaller induced swelling in affected eyes (33 +/- 15 mu m) compared with unaffected eyes (67 +/- 11 p,m) and a slower recovery of corneal thickness in affected eyes. In one of four subjects, the cornea of the affected eye was calculated not yet to have recovered its original thickness before midnight. After this finding, although the cornea of this subject did not show any signs of clinical decompensation, the study was discontinued. Conclusions. Corneal hydration control is altered in the ICE syndrome. The ICE cornea can maintain its normal thickness despite severe morphologic abnormalities of the endothelium. Because we found that recovery of corneal thickness can be extremely slow, it cannot be guaranteed that such corneal "stress tests" are completely safe for subjects with the ICE syndrome, and we therefore feel that they should no longer be performed in these subjects.