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M. Rauen, N. A. Syed, K. M. Goins; Descemet-Stripping Automated Endothelial Keratoplasty (DSAEK) for the Management of Iridocorneal Endothelium (ICE) Syndrome. Invest. Ophthalmol. Vis. Sci. 2009;50(13):2202.
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Metaplastic corneal endothelium has been implicated in the pathogenesis of the iridocorneal endothelial (ICE) syndrome. This typically unilateral condition may manifest with vision threatening glaucoma and corneal edema. This clinicopathologic study evaluates the outcomes of patients with ICE syndrome who underwent Descemet-stripping automated endothelial keratoplasty (DSAEK).
In a retrospective chart analysis, five patients had DSAEK for the treatment of bullous keratopathy associated with ICE syndrome at the University of Iowa since December 2003. The preoperative diagnoses included Cogan-Reese syndrome (3/5) and Chandler’s syndrome (2/5). Two eyes (40%) had DSAEK alone with pre-existing seton placement, two eyes (40%) had DSAEK and phacoemulsification combined with seton placement, and one eye (20%) had DSAEK with phacoemulsification. All Descemet’s membrane specimens acquired at the time of DSAEK were examined and characterized using histologic analysis. The follow-up period ranged from 3 to 36 months (mean, 20 months).
The mean preoperative best corrected visual acuity (BCVA) was 20/400 (range 20/25 to 20/800, n = 5), which improved to 20/160 (range 20/20 to 20/400, n = 3) at 6 and 12 months. One of five patients (20%) had a dislocated DSAEK button after surgery, which necessitated a re-bubble procedure. The mean preoperative donor endothelial cell density (ECD) was 3090 +/- 220 cells/mm2 (n = 5). The postoperative ECD at 6 and 12 months was 1651 +/- 186 and 1000 +/- 932 cells/mm2, which represents a 47 and 67% ECD loss (n = 3). Two eyes (40%) had allograft rejection between 12-24 months after DSAEK, with one necessitating a repeat DSAEK. Non-iatrogenic primary graft failure was observed in one eye (20%) at three months, which required a repeat DSAEK. After initial surgery, three eyes (60%) have remained clear during the postoperative follow-up period. Graft survival ranges from 3 to 36 months (mean 14.4 +/- 13 months). No patients have experienced escalation of intraocular pressure during the postoperative course. The Descemet’s membrane specimens obtained intraoperatively had variable thicknesses, rare endothelial cells, and lacked corneal guttae.
DSAEK for endothelial replacement in ICE syndrome is successful in the treatment of pain and vision loss from bullous keratopathy. However, ICE syndrome patients who require a re-bubble procedure after DSAEK and/or have more severe preoperative glaucoma are more likely to have a higher degree of ECD loss after surgery.
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