Iridocorneal Endothelial (ICE) Syndrome, with Secondary Angle Closure Glaucoma
Section outline
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A patient with no previous ocular history presented to the ED with concern for irregular pupil shape in the right eye. The pupil had been noted to be irregular for the last month. Patient denied changes in vision, eye pain, redness. No significant past medical history or trauma. No family history significant for ocular disease, glaucoma and blindness. Visual acuity was 20/20 in the right eye and 20/20 in the left eye. IOP was 17 mm Hg OD and 18 mm Hg OS. The right pupil was irregular, without an APD. Motility and confrontational visual field were full in both eyes. Slit lamp examination of the right eye showed a unilateral irregular pupil and quiet anterior chamber. Gonioscopy in the right eye was notable for PAS crossing Schwalbe’s line, and sectoral pigmentary changes ST and SN areas. Dilated fundus exam was notable for small chorioretinal scar in right eye, there was no optic nerve cupping. Patient was seen once at follow up and obtained baseline RNFL OCT which was normal before being lost to follow up. After 2.5 years, the patient represented to the BPEI ED with eye pain. Visual acuity was found to be 20/20 in both eyes and IOP elevated at 44 mm Hg in the right eye. Slit lamp exam showed progressive iris atrophy and corneal guttae in right eye. Patient was then started on Brimonidine and dorzolamide/timolol twice per day in the right eye. At subsequent follow ups, IOP remained elevated despite escalation of medical therapy to include oral agents. Given young age and phakic status, trabeculectomy was performed. POD1 visual acuity was 20/50 and IOP was 15. At POW1, visual acuity was 20/20 and IOP was 14 mm Hg. At POM2, the IOP was 5 mm Hg on no additional glaucoma drops. Patient is currently awaiting further follow up.
Presentation Date: 05/21/2026
Issue Date: 05/22/2026
