Issue: June 10, 2014
May 01, 2014
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PK for iridocorneal endothelial syndrome improves vision but may increase IOP

Visual acuity was 20/40 or better in 33% of patients at 6 months and 50% at 1 year.

Issue: June 10, 2014

PHILADELPHIA — Penetrating keratoplasty improved vision in patients with iridocorneal endothelial syndrome, according to a study presented here.

“ICE syndrome and corneal decompensation can benefit from transplantation, but you have to be careful about intraocular pressure,” Ajay B. Shalwala, MD, said at the Wills Eye Annual Conference. “Endothelial keratoplasty has been shown to be effective in ICE syndrome. The largest series to demonstrate that is three patients. So, if we did larger studies of that, maybe we’d find that the results are even better than PK since it’s primarily an endothelial problem.”

Iridocorneal endothelial (ICE) syndrome, an abnormal proliferation in the anterior segment, is a unilateral disease that primarily affects women ages 30 to 50 years. Abnormal endothelial cells can form a membrane over the angle, increasing IOP. The membrane can also cause corneal edema (Chandler’s syndrome), iris nevus syndrome, corectopia and pseudopolycoria, Shalwala said.

Previous studies of PK for ICE syndrome yielded mixed results, Shalwala said.

“Two early reports from the 1980s showed relatively good results, the first report showing postop vision of 20/15 to 20/30 and another report showing 100% graft clarity,” he said. “But in the 1990s, a study was produced that showed that PK did poorly in ICE syndrome. Five of six patients had poor vision and failed grafts.”

Patients and methods

The retrospective study included 13 patients with ICE syndrome who underwent PK between 2000 and 2013. Investigators evaluated graft survival, visual acuity and IOP. Ten of the patients were female.

A previous study of PK for ICE syndrome conducted at Wills Eye Hospital had varied endpoints, Shalwala said.

“They just aggregated all of the data at final follow-up. What we wanted to do was take specific time intervals after surgery,” he said. “Not everybody has data at all time points, but we thought that would be a better way to look at it. We wanted to look at graft survival. We wanted to look at visual acuity outcomes. We wanted to look at associated glaucoma issues.”

Preoperative data were available for 10 patients.

“We’re trying to get the [data for] three other patients out of the archives,” Shalwala said.

Preoperatively, five patients had counting fingers or worse vision, and five had visual acuity of 20/200 to 20/400.

Mean IOP was 18 mm Hg, with about 1.3 glaucoma drops. Five patients had previous glaucoma surgery.

Visual acuity, IOP, graft clarity

At 6 months postoperatively, three of nine patients (33%) had visual acuity of 20/40 or better; one patient had visual acuity of counting fingers or worse.

No grafts failed. IOP remained stable, but patients required slightly more medication to manage pressure. One patient required subsequent glaucoma surgery.

At 1 year, four of eight patients (50%) had visual acuity of 20/40 or better, and all patients had clear grafts. Two patients experienced graft rejection that ultimately resolved with topical steroids.

“So, vision definitely improves. There’s good graft survival. Intraocular pressure issues are definitely a concern,” Shalwala said.

Limitations of the study included its retrospective design and small patient sample, Shalwala said.

“Because it’s retrospective, there’s a lack of complete data at all time points, but we’re trying to get some data out of the archives that is not available in the electronic medical record. And [there is] a small number of patients, 13 patients, but of the previous studies in the literature, only one was larger, with 14 patients,” he said. – by Matt Hasson

  • Ajay B. Shalwala, MD, can be reached at Wills Eye Hospital, 840 Walnut St., Suite 920, Philadelphia, PA 19107; 215-928-3180; fax: 215-928-3854; email: ajaybshalwala@gmail.com.
  • Disclosure: Shalwala has no relevant financial disclosures.