Grand Rounds Joseph Reck VAMC Wilkes-Barre, PA November 3, 2006

Clinical Presentation Seen in Texas six weeks ago; GAT- 54 Seen in Texas six weeks ago; GAT- 54 Current Medications: Current Medications: – Diamox 500mg b.id.; ran out 5d ago – Cosopt b.id. – Atropine b.id. – Brimonidine b. id. Ocular history: Ocular history: – Cataract extraction without implantation, – Anterior chamber IOL implantation, 1987.

Acuity and Externals VA cc: O.D. 20/60, PH 20/40 VA cc: O.D. 20/60, PH 20/40 O.S. 20/20 O.S. 20/20 Pupils: O.D. pharm fixed; O.S. RRL –APD Pupils: O.D. pharm fixed; O.S. RRL –APD EOM: Full and smooth, O.U. EOM: Full and smooth, O.U. Confrontation fields; Full, O.U. Confrontation fields; Full, O.U.

Clinical Findings Slit Lamp Exam: Slit Lamp Exam: – 1+ injection. – Diffuse microcysts and SPK. – 1+ AC Reaction. – Iris atrophy with exposed iris vessels near ACIOL haptic foot.

Tonometry O.D O.D O.S.- 13 O.S.- 13

Uveitic Glaucoma Unilateral, red eye. Unilateral, red eye. Pain and photosensitivity. Pain and photosensitivity. Corneal edema. Corneal edema. AC reaction. AC reaction. Increased IOP. Increased IOP.

Inflammatory Cells Decrease aqueous outflow Decrease aqueous outflow – Physically obstruct trabecular meshwork.

Synechiae

Inflammation, then Pressure Topical steroid. Topical steroid. – Pred Forte q15min; then taper. Strong cycloplegia. Strong cycloplegia. – Atropine 1% b.id. Break synechiae. Break synechiae. – Phenylephrine 10%. Beta-blocker Beta-blocker Alpha-agonist Alpha-agonist CAI CAI Avoid Prostaglandins. Avoid Prostaglandins.

Assessment/Plan: Lotemax q2h Lotemax q2h Atropine t.id. Atropine t.id. Cosopt b.id. Cosopt b.id. Diamox 500mg, b.id. Diamox 500mg, b.id. Follow-up in 1 week. Follow-up in 1 week.

One Week Follow-Up VA cc: O.D. 20/100, PH 20/40 VA cc: O.D. 20/100, PH 20/40 O.S. 20/20 O.S. 20/20 GAT: O.D. 52, O.S. 12 GAT: O.D. 52, O.S AC Reaction 1+ AC Reaction

Updated Treatment Plan Continue meds as scheduled. Continue meds as scheduled. – Add Alphagan t.id. Run full uveitis work-up. Run full uveitis work-up. Follow-up next day. Follow-up next day.

Return Visit Patient experiencing some pain. Patient experiencing some pain. VA cc: O.D. 20/80, ph 20/30 VA cc: O.D. 20/80, ph 20/30 O.S. 20/20 O.S. 20/20 GAT: O.D. 55, O.S. 11. GAT: O.D. 55, O.S AC reaction. 1+ AC reaction.

Differential Unilateral increase in IOP Unilateral increase in IOP  Steroid response  PAS  Endopthalmitis  Chronic inflammation  Retained lens material

Gonioscopy Lens position in iris; not angle Lens position in iris; not angle Small areas of synechiae. Small areas of synechiae. Small areas of bleeding. Small areas of bleeding. Peripheral rubeosis, superiorly. Peripheral rubeosis, superiorly. Dilated iris tissue rolled into angle. Dilated iris tissue rolled into angle. ACIOL haptics appear to have pushed peripheral iris directly into angle ACIOL haptics appear to have pushed peripheral iris directly into angle Discontinue Atropine. Discontinue Atropine.

U VEITIS U VEITIS G LAUCOMA G LAUCOMA H YPHEMA H YPHEMA

UGH Syndrome Inflammation after anterior chamber IOL implantation, caused by the haptics of the IOL. Inflammation after anterior chamber IOL implantation, caused by the haptics of the IOL. Misplaced or misdirected haptics from the anterior chamber IOL erode the tissues of the angle, causing bleeding and inflammation. Misplaced or misdirected haptics from the anterior chamber IOL erode the tissues of the angle, causing bleeding and inflammation.

UGH Syndrome – Excessive lens movement Small size Small size Decentration or dislocation Decentration or dislocation – Poorly manufactured edges – Iris-clipped IOL – Rigid, closed loop haptics

Open v. Closed Loop Open Loop IOL -good finish/polish -easy to size -less area of contact Closed Loop IOL -difficult to fit -erosion chaffing -large contact zone -poorly finished/ sharp edges

UGH with PCIOL’s Unstable sulcus fixation Unstable sulcus fixation PCIOL decentration PCIOL decentration – zonular weakness – trauma

UGH Etiology; Uveitis Activation of innate immunity. Activation of innate immunity. Theories Theories – Cytokine and eicosanoid synthesis triggered by mechanical excoriation of the angle or iris by the haptics or optic – Plasma-derived enzymes (especially complement or fibrin) activated by the surface of the IOLs – Adherence of bacteria and leukocytes to the IOL surface – Toxicity caused by contaminants on the IOL surface during manufacturing or implantation

Post-Operative Timing UGH Development UGH Development – Usually weeks to months. – Literature suggests 1-8 yrs. This patient; 1987 to 2006 – 19 years. This patient; 1987 to 2006 – 19 years.

Clinical Spectrum Iris pigment epithelial defects Iris pigment epithelial defects Pigment dispersion Pigment dispersion Microhypema Microhypema Macrohyphema I Macrohyphema I Increase in IOP Increase in IOP

Presenting Symptoms Intermittant blurring Intermittant blurring ‘Redness’ to vision ‘Redness’ to vision Eye pain Eye pain Red eye Red eye Photophobia Photophobia

UGH Complications Pseudophakic bullous keratopathy Pseudophakic bullous keratopathy Corneal staining; recurrent hyphema Corneal staining; recurrent hyphema Chronic inflammation Chronic inflammation Cystoid macular edema Cystoid macular edema Glaucoma Glaucoma

UGH Management Bed rest with elevated head position to encourage hyphema settling Bed rest with elevated head position to encourage hyphema settling Topical steroid Topical steroid Reduce increased IOP Reduce increased IOP Ultimately, the lens may have to be repositioned or removed. Ultimately, the lens may have to be repositioned or removed.

UGH Treatment Options Observe, treat episodes individually. Observe, treat episodes individually. Pharmacologically reposition IOL Pharmacologically reposition IOL IOL rotation IOL rotation IOL explanation +/- replacement. IOL explanation +/- replacement.

Patient Returns VA cc: O.D. 20/50, ph 20/30 VA cc: O.D. 20/50, ph 20/30 O.S. 20/20 O.S. 20/20 GAT: 22, O.D.; 13 O.S. GAT: 22, O.D.; 13 O.S. 2+ AC reaction. 2+ AC reaction. Patient scheduled for IOL removal. Patient scheduled for IOL removal.

STUDY:Indications for IOL Explanation (FL) The majority of the removed IOLs were anterior chamber styles (53.9%), followed by iris-fixated lenses (33.7%) The majority of the removed IOLs were anterior chamber styles (53.9%), followed by iris-fixated lenses (33.7%) The most common indications for surgery included: The most common indications for surgery included: Pseudophakic bullous keratopathy, 69% Pseudophakic bullous keratopathy, 69% UGH syndrome, 9% UGH syndrome, 9% IOL instability, 7%. IOL instability, 7%.

Surgical Timing with ACIOL Time between implantation and explanation with ACIOL complications: Time between implantation and explanation with ACIOL complications: – 1 to 8 years.

Surgical Outcome The poorest visual outcome was seen in patients with the UGH syndrome. The poorest visual outcome was seen in patients with the UGH syndrome. – 83% had a final acuity of 20/200 or worse. – Resolution of pain and inflammation – Better control of their IOP as a result of the surgery.

1- Day Post-Operative Surgery without incident Surgery without incident VA- 20/400, PH 20/100 VA- 20/400, PH 20/100 Some corneal edema; 3+ AC reaction. Some corneal edema; 3+ AC reaction. GAT- 13. GAT- 13. Continue with meds: Continue with meds: – Cosopt b.id.- Tobradex ung q.id. – Alphagan P b.id.- Atropine b.id. – Diamox 500mg b.id.

Follow-Ups Seen on Day 2, 4, then 1 week, 2 week. Seen on Day 2, 4, then 1 week, 2 week. VA improves to 20/100 with pinhole and +15D lens. VA improves to 20/100 with pinhole and +15D lens. Cornea improves; AC reaction diminishes to grade 1. Cornea improves; AC reaction diminishes to grade 1. IOP in mid to low teens. IOP in mid to low teens. Continuing all meds. Continuing all meds.

3 Week Follow-Up VA- 20/80 VA- 20/80 Refracts to 20/30. Refracts to 20/30. Trace AC reaction. Trace AC reaction. GAT- 13. GAT- 13. SLOW taper off all meds. SLOW taper off all meds.

Review: Key Points Be suspicious of misplaced IOL Be suspicious of misplaced IOL ACIOL with Uveitis ACIOL with Uveitis Gonioscopy Gonioscopy

THE END