Recently, spirited discussions with my colleagues of the American Glaucoma Society sparked questions about the best way surgically to manage glaucoma patients with iridocorneal endothelial (ICE) syndrome. ICE syndrome is a condition that is characterized by unilateral glaucoma, iris changes, and abnormal corneal endothelium. It has been demonstrated in pathologic specimens that these abnormal corneal endothelial cells migrate across the trabecular meshwork onto the iris, and it is hypothesized that resultant membrane formation causes angle closure and glaucoma.¹

Figure 1

In my practice, I have a handful of patients with glaucoma secondary to ICE syndrome. While treatment should include topical glaucoma medications and oral carbonic anhydrase inhibitors, some patients do not respond to medications or the medications lose efficacy over time, thus warranting surgical intervention. Many of these patients have had prior filtering surgeries that have failed, including trabeculectomy and glaucoma drainage device implantation. It is postulated that trabeculectomy fails due to ICE membrane formation over the ostium of the filtration site, whereas glaucoma drainage devices often require repositioning of the tube or multiple surgeries to control intraocular pressure (IOP) adequately.^2–5

The following is a case study of a patient I treated who was diagnosed with ICE syndrome-related glaucoma.

My case study

Figure 2

A 71-year-old man with a diagnosis of pseudophakic bullous keratopathy of the right eye was referred for a glaucoma consultation because of his poorly controlled IOP in both eyes. In the left eye, which was phakic, visual acuity was 20/20 and IOP ranged from 16–22 mmHg on multiple glaucoma medications. Slit lamp examination revealed a clear cornea, corectopia, and areas of iris atrophy (Figure 1). On gonioscopy, scattered peripheral anterior synechiae were noted but the rest of the angle was visible to ciliary body band. The cup to disc ratio was 0.85 with significant temporal sloping and inferior thinning. The rest of the fundus exam was unremarkable. A superior arcuate defect was detected on visual field examination (Figure 2) and specular microscopy demonstrated abnormal corneal endothelium and low endothelial cell counts.

Figure 3

Given the uncontrolled IOP and progressive visual field changes, a trabeculectomy with mitomycin-C and Ex-PRESS shunt device (Optonol) placement was performed in the left eye (Figure 3). His IOP was well-controlled with a patent shunt in a good position and bleb formation until postoperative month six, when bleb needling with 5-FU was performed for an encapsulated bleb. This was repeated again at postoperative year one, with good bleb morphology and an IOP of 12 mmHg at final follow-up.