Iridocorneal Endothelial (ICE) Syndrome, Cogan-Reese/Iris Nevus Syndrome Subtype with Secondary Angle Closure Glaucoma
A patient presented with a 3-day history of unilateral worsening eye pain, blurry vision and irregular pupil in the right eye. Found to have elevated IOP at an outside facility and was started on Timolol, Dorzolamide, and Brimonidine eye drops in the affected eye. No past ocular history was reported. No significant past medical history. Family history significant for glaucoma and blindness in paternal grandfather. Upon presentation, visual acuity was 20/70 in the right eye and 20/25 with pinhole correction to 20/20 in the left eye. IOP was 33mmHg in the right eye and 11mmHg in the left eye. Slit lamp examination disclosed corneal endothelial irregularity, trace MCCE, iridocorneal touch with high PAS at 1:00, 3:00, 6:00 and 9:30, pigmented nodules, and correctopia in the right eye. Fundus exam disclosed increased C:D ratio in the right eye and scattered intraretinal heme in the periphery. Exam of the left eye was normal. IOP improved to 21mmg Hg in the right eye and the patient was discharged with close follow up. At a one week follow up appointment, the patent’s IOP remained elevated at 30mm Hg, and it was decided to proceed with a tube shunt procedure. AGI was completed without complication. On POD1, VA 20/400 pinhole to 20/100 and IOP was 10 mm Hg on no glaucoma drops with tube in good position in the AC. This remained stable at the POW1 visit with IOP 8 mm Hg without pressure drops. However, at POW3 the patient had anterior chamber inflammation and IOP was elevated to 30 mm Hg. It was discovered the patient was using only antibiotic drops since surgery, rather than Prednisolone drops as instructed. The antibiotics were discontinued and the patient started on Prednisolone QID as well as Dorzolamide/ Timolol and Brimonidine drops BID. POM2 visit complicated by further drop non-compliance, persistent anterior inflammation and large CME of right eye. IOP improved to 21 mg Hg. The patient was restarted on Prednisolone QID, Ketorolac QID, Dorzolamide/ Timolol BID and Brimonidine drops BID in the right eye and will be seen again in clinic for close follow up.
Presentation Date: 08/04/2022
Issue Date: 08/19/2022
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