A 72-year-old female patient had a longstanding history of a very problematic left eye. Throughout the years, her left eye has experienced iridocorneal endothelial syndrome (ICE), glaucoma, trabeculotomy, a total iridectomy, cataract extraction, and three penetrating keratoplasty procedures. Her right eye is completely normal.

Her primary diagnosis was ICE at age 30. ICE is a spectrum of diseases that includes Chandler syndrome, progressive iris atrophy, and Cogan-Reese syndrome; common to all of these is an alteration of the corneal endothelium. In many cases, the endothelial disorder gives rise to corneal edema of varying degrees. Also common throughout this spectrum of diseases is peripheral anterior synechiae, which often extends to or beyond Schwalbe’s line. These synechiae typically spread around the anterior chamber angle, eventually leading to secondary glaucoma in a high percentage of cases. In addition, ICE characteristically causes changes in the iris.

In Chandler syndrome, the iris abnormalities are either absent or limited to mild pupillary distortion, whereas progressive iris atrophy is characterized by marked decentration of the pupil and iris atrophy with hole formation. Cogan-Reese syndrome may include any degree of iris changes but also has the additional feature of nodules on the stromal surface of the iris.

ICE is almost always unilateral and typically becomes apparent during young adulthood. There is usually no family history of the disease or associated systemic diseases. Typical presenting complaints range from visual disturbance, such as a mild blurring in the morning (secondary to corneal edema), to marked persistent reductions and alterations in the pupil or iris.

Case Specifics

This patient came to our clinic for a contact lens to manage her left eye aphakia and irregular astigmatism (Figure 1). She had failed twice with scleral fittings and was referred for a corneal contact lens fitting. Her entering manifest refraction was OD –0.25 –0.75 x 045, 20/15 and OS +14.50 –3.50 x 75, 20/100. Her corneal topography showed a moderate degree of against-the-rule corneal astigmatism, and the simulated keratometric readings were 45.62 @ 031 / 42.87 @ 121 with 2.75D of corneal toricity (Figure 2).

She was diagnostically fitted with a 9.5mm bitoric lens, and an over-refraction was performed. Figure 3 shows the final lens on the patient’s left eye with a best-corrected visual acuity of 20/80. She did report a significant improvement in her peripheral vision, and her comfort with the lens was very good. This “never-give-up” patient is now ready for the last leg of her long journey, which is referral to the strabismus surgeon to correct her 35-prism-diopter left exotropia. CLS