WHAT’S YOUR DIAGNOSIS? MORNING ROUNDS

Foggy With a Chance of Hemorrhage

atiana Ivanov,* a 79-year-old and her IOP improved to 26 mm Hg Albanian woman now living in prior to discharge from the ED. 1 TNew York City, grimaced and held her hand over her right eye. She We Get a Look reported that the vision in that eye had The following day, Ms. Ivanov presented become blurry 4 days earlier. Two days at our office feeling much better; her after the onset of blurred vision, she eye pain had resolved, and IOP was began to experience severe pulsating 12 mm Hg in the right eye. Additional eye pain and worsening vision. Now, history revealed that she had systemic she said that she could see only “fog” hypertension, dyslipidemia treated with her right eye. with statins, and exfoliation syndrome Ms. Ivanov described several episodes (XFS). of self-limited painless vision loss in the showed an open angle right eye that had occurred over the past in the right eye with hyphema inferiorly. year. Each of these lasted a few days We saw no neovascularization of the IRIS TRANSILLUMINATION. Slit-lamp before her vision returned to normal. angle. The anterior chamber was filled exam 1 month after presentation reveals Apart from these episodes, she had with 4+ cells; although both pigmented crescent-shaped iris trans­illumination enjoyed excellent vision in her right and nonpigmented types were present, defects nasally and temporally (arrow). eye since undergoing cataract surgery the pigmented cells were more numer- We also observed pseudophacodonesis. 5 years ago. ous. A well-centered single-piece pos- terior chamber intraocular lens (IOL) work, and red cells predominate in the Emergency Department was noted. anterior chamber. In ghost cell glau- Findings coma, degenerated red blood cells that Ms. Ivanov was first seen in the ED, Differential Diagnosis have lost their intracellular hemoglobin where her visual acuity was recorded The profusion of pigmented cells in cause the outflow obstruction, and as light perception in the right eye and the anterior chamber coupled with the small, khaki-colored “ghost cells” are 20/25 in the left eye. Intraocular pres- vitreous hemorrhage suggested that noted in the anterior chamber. Vitreous sure (IOP) was 70 mm Hg in the right intraocular bleeding was the most likely hemorrhage is usually present for 1 to 3 eye and 12 mm Hg in the left eye. Slit- cause of the marked IOP elevation. Our months before red blood cells degen- lamp examination showed profound differential diagnosis included hemo- erate into ghost cells. Given the sudden microcystic edema of the right , lytic and ghost cell glaucoma, onset of Ms. Ivanov’s blurry vision limiting visualization of intraocular both of which can develop following 4 days prior to presentation and the structures. B-scan ultrasonography vitreous hemorrhage. predominance of pigmented cells in the revealed vitreous hemorrhage. In hemolytic glaucoma, hemo- anterior chamber, hemolytic glaucoma Intravenous acetazolamide and serial globin-laden macrophages produce was the most likely explanation for the IOP-lowering drops were administered, obstruction of the trabecular mesh- elevated IOP. However, the etiology of the vitreous hemorrhage remained unclear. Although BY RICHMOND WOODWARD, BA, ABDALLAH MAHROUS, MD, MRINALI Ms. Ivanov had a history of hyperten- P. GUPTA, MD, AND SARAH H. VAN TASSEL, MD. EDITED BY STEVEN J. sion, there were no signs of hyperten-

Sarah H. Van Tassel, MD Tassel, H. Van Sarah GEDDE, MD. sive retinopathy in the fellow eye. Our

EYENET MAGAZINE • 39 working diagnoses were acute hemor- any IOL can cause UGH syndrome, rhagic posterior vitreous detachment or 2 including modern anterior chamber retinal vein occlusion, and consultation IOLs, sulcus IOLs (both 3-piece and with a retina specialist was arranged. In single-piece IOLs, which may be in- the meantime, IOP-lowering therapy advertently or purposely placed in the was continued. ciliary sulcus2), and IOLs placed in the capsular bag.3,4 Next Steps Blood-aqueous barrier compromise Retina consult. Ms. Ivanov saw the due to the chafing results in the classic retina specialist 8 days after presenta- triad of , glaucoma, and hyphema. tion. The vision in her right eye had IOP elevation can be caused by inflam- improved to 20/50, and the IOP was mation and resultant scarring of the 12 mm Hg. The anterior chamber was , by pigment and still filled with pigmented cells, and the hyphema clogging the meshwork, and vitreous hemorrhage was clearing. Ms. by direct injury to the aqueous drain- UBM. Ultrasound biomicroscopy 4 Ivanov’s daughter, who accompanied age complex. Iris transillumination weeks after initial presentation shows her to the appointment, recalled that defects, secondary neovascularization single-piece IOL in the capsular bag and her mother’s blood pressure had been of the iris, and cystoid macular edema a Soemmering ring (arrow). There was very high in the weeks before the severe may also occur. The term “UGH Plus suspicion that a haptic was abutting the eye pain started. However, Ms. Ivanov’s syndrome” has been used to describe ciliary body nasally, but it was poorly retinal examination and fluorescein the condition of patients such Ms. visualized by UBM. angiography showed no evidence of Ivanov, who, in addition, have vitreous retinal vascular occlusion to implicate hemorrhage.5 hypertension as the etiology of the ring (Fig. 2). No vitreous hemorrhage In-the-bag UGH. Single-piece bleeding. The hyphema was followed to was noted. IOLs in the capsular bag are a rare but resolution, and the vision in the right We strongly suspected uveitis-glau- increasing cause of UGH syndrome.4 eye improved to 20/25. coma-hyphema (UGH) syndrome at Zhang et al. reported a patient with Optic nerve head exam. Once the this point. We discussed the options of XFS and UGH syndrome 3 years after vitreous hemorrhage had cleared, we ongoing observation versus surgery to uneventful placement of a single-piece could examine the optic nerve head. remove the IOL. The decision was made IOL in the capsular bag. They posit that In the right eye, we found a vertical to moni­tor the condition, with consid- the haptic-capsule complex chafed the cup-to-disc ratio of 0.6 with thinning eration of surgery if significant or re- posterior iris in the setting of subclini- of the inferior neuroretinal rim; in the current hemorrhages or IOP elevation cal pseudophacodonesis.4 left eye, we observed a cup-to-disc ratio were noted. IOP-lowering medications Bryant et al. observed extensive of 0.3. Optical coherence tomography and topical steroids were continued. fibrosis (Soemmering ring) around the (OCT) demonstrated intact and sym- haptics of the single-piece IOL in the metric retinal nerve fiber layer thick- Making the Diagnosis capsular bag in a patient with UGH ness and inferior ganglion cell loss in Eight weeks after initial presentation, syndrome. They suspected that the the right eye. Automated perimetry was Ms. Ivanov returned acutely for de- fibrosis caused the IOL to tilt out of performed reliably in both eyes. Several creased vision. In the right eye, her the iris plane, leading to haptic-iris superonasal pixels were present in the visual acuity was hand motions, and and haptic–ciliary body chafing.3 right eye, raising concern for an early IOP was 32 mm Hg despite good Single-piece IOLs are now the most nasal step. The left eye testing was full. compliance with IOP-lowering drops. commonly implanted lenses in the A new microhyphema and diffuse vit­ United States. Given the aging of the New Findings reous hemorrhage were present, and U.S. population, ophthalmologists may About 1 month after the initial pre- the pseudophacodonesis appeared more encounter a growing number of cases sentation, Ms. Ivanov returned for dramatic than at the last examination, of in-the-bag UGH syndrome in the follow-up, reporting another episode confirming the diagnosis of UGH syn­ future. of vision loss—which had since re- drome in the setting of XFS and late Treatment. Topical therapy with solved—in the previous week. Slit-lamp zonular weakness. steroids and IOP-lowering medication, exam now showed crescentic, haptic- with or without cycloplegia, is the shaped iris transillumination defects Discussion first-line treatment. Photocoagulation nasally and temporally (Fig. 1) and UGH syndrome was initially described to ablate leaking vessels, intravitreal pseudophacodonesis. Ultrasound bio- in 1978 as a of first-gen- anti–vascular endothelial growth factor microscopy (UBM) revealed a normal eration anterior chamber IOLs that medications, and placement of a capsu- iris configuration, a single-piece IOL caused chafing of intraocular struc- lar tension ring to adjust IOL position- 1 in the capsular bag, and a Soemmering tures. It is now known that virtually ing have all been described as therapies MD Tassel, H. Van Sarah

40 • SEPTEMBER 2018 for UGH syndrome.5 However, removal of the IOL is often required, as in our patient’s case. In a series of 109 eyes at a tertiary referral center, UGH syndrome was among the most common rea- sons for IOL exchange, accounting for 11.9% of cases.6 Key considerations. The presence of seemingly spontaneous hyphema along with elevated IOP and inflam- mation should prompt clinicians to consider UGH syndrome,­ even if the patient has a single-piece IOL in the capsular bag. This consideration is particularly important in eyes that are susceptible to zonular weakness, such High-quality as those with XFS or a history of ocular research from the trauma. Careful slit-lamp examination, gonioscopy, and UBM are important world’s leading tools in making the diagnosis of UGH ophthalmic journal syndrome.

Our Patient Ms. Ivanov underwent pars plana vitrectomy, IOL removal, and place- ment of a scleral-sutured Akreos AO60 IOL. At 4 months following surgery, best-corrected visual acuity was 20/25 in the right eye, and IOP was in the midteens on a prostaglandin analogue and fixed-dose combination drops. Serial OCT and visual field testing were stable. No further episodes of decreased vision were reported. The Academy’s newest scientific * Patient name is fictitious. publication, ® 1 Ellingson FT. J Am Intraocul Implant Soc. 1978; 4(2):50-53. Retina, focuses on advances in 2 Chang DF et al. J Cataract Refract Surg. 2009; medical drug treatment, surgery 35(8):1445-1458. 3 Bryant TK et al. J Cataract Refract Surg. 2017; and technology for retina-related 43(7):985-987. disesases and conditions. 4 Zhang L et al. J Cataract Refract Surg. 2014; 40(3):490-492. Subscribe at aao.org/store. 5 Sousa DC et al. J Curr Glaucoma Pract. 2016; 10(2):76-78. 6 Davies EC, Pineda R. J Cataract Refract Surg. 2016;42(9):1262-1267.

Mr. Woodward is a third-year medical student at Weill Cornell Medical College. Dr. Mahrous is a third-year ophthalmology resident at Weill Cornell/New York Presbyterian Hospital. Dr. Gupta is a vitreoretinal specialist, and Dr. Van Tassel is a glaucoma specialist; both are at Weill Cornell Medicine. All authors are in New York City. Financial disclosures: None.

EYENET MAGAZINE • 41