Man presents with two episodes of unilateral transient vision loss
IOP was 47 mm Hg in the right eye 4 hours after symptom onset.
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A 77-year-old man presented to the Lahey Medical Center emergency department with a chief complaint of right eye vision loss for several hours.
On the morning of his presentation, the patient experienced sudden painless vision loss in the right eye as if “a curtain was dropping down” over his vision. The blurred vision gradually improved over a period of 4 hours and was nearly back to baseline at the time of his ophthalmology evaluation. The patient described a similar episode in his right eye 3 months earlier, which lasted several hours before resolving. He was evaluated by an outside ophthalmologist 2 days after that episode, and the ocular examination was reportedly normal. The patient was diagnosed with amaurosis fugax and underwent a full stroke workup, which was unremarkable. The patient’s review of systems was negative for scalp tenderness, jaw claudication, fevers or weight loss, and he had no other systemic or neurologic symptoms.
The patient’s medical history included aortic stenosis, type 2 diabetes, hypertension, hyperlipidemia, peripheral arterial disease and chronic obstructive pulmonary disease. He was a current smoker. His ocular history was notable for cataract extraction in the right eye 3 years earlier complicated by a retained lens fragment causing elevated IOP and inflammation. The patient was treated with pressure-lowering drops and topical steroids for several months until the lens fragment had completely absorbed. He had undergone uncomplicated cataract extraction in the left eye.
Source: Chelsea Gottschalk, MD, and Paul Cotran, MD
Examination
On initial examination 4 hours after symptom onset, the patient’s best corrected visual acuity was 20/40 in the right eye and 20/20 in the left eye. Pupils were reactive with no afferent pupillary defect. Visual fields were full to confrontation. IOPs were measured at 47 mm Hg in the right eye and 14 mm Hg in the left eye using an Icare tonometer. Limited anterior segment evaluation using a portable slit lamp in the emergency department revealed mild conjunctival injection and a deep anterior chamber in the right eye.
Multiple rounds of pressure-lowering drops were administered to the right eye, and the patient was transferred to the ophthalmology clinic for a full evaluation. A few hours later, the right eye IOP had improved to 10 mm Hg, and the visual acuity was measured at 20/20-3 in the right eye. Repeat slit lamp examination of the right eye revealed a quiet conjunctiva and clear cornea (Figure 1) but was notable for 2+ mixed pigmented and white cells in the anterior chamber and a superonasal arcuate transillumination defect in the iris (Figure 2). There was no evidence of iris neovascularization, and the posterior chamber IOL appeared to be well-positioned in the right eye. Anterior segment examination of the left eye was unremarkable. Gonioscopic examination of the right eye revealed an open angle with 2+ pigmentation of the trabecular meshwork. There was an area of peripheral anterior synechiae at 6 o’clock and a small amount of hemorrhage inferiorly with no neovascularization of the angle. Gonioscopic exam of the left eye revealed an open angle with no hemorrhage. Fundoscopic examination was within normal limits in each eye with no evidence of embolus or retinal whitening.
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Transient vision loss
The constellation of findings in this pseudophakic patient, including elevated IOP, iris transillumination defects, blood in the angle and pigmented cells in the anterior chamber, is most suggestive of uveitis-glaucoma-hyphema syndrome.
Other diagnostic considerations in this setting include herpetic uveitis, which can cause IOP elevation that generally does not self-resolve over the course of several hours, and pigment dispersion syndrome, which typically affects a younger demographic and is not associated with hemorrhage on gonioscopic exam.
The patient reported a prior episode of vision loss lasting several hours that was diagnosed as amaurosis fugax. Generally, amaurosis fugax resolves over seconds to minutes, so this diagnosis is less likely. It is more likely that this episode was the patient’s first presentation of uveitis-glaucoma-hyphema (UGH) syndrome.
Workup and management
The patient underwent additional in-office testing including OCT of the retinal nerve fiber layer (RNFL) to detect thinning from potentially recurrent episodes of elevated IOP in the right eye (Figure 3). The testing demonstrated no thinning of the nerve fiber layer in the right eye. There was mild thinning inferiorly in the nerve fiber layer in the left eye.
The patient was prescribed pressure-lowering drops and topical steroids for the right eye. One week later, his IOPs were normal and the anterior chamber reaction had improved. Gonioscopic examination showed absence of blood in the angle. On the patient’s most recent visit to clinic, the anterior segment inflammation had completely resolved and his IOPs remained normal. The patient will be scheduled for ultrasound biomicroscopy, which was not available at our center but could confirm IOL malposition in the right eye before any planned surgical intervention.
Discussion
UGH syndrome is a relatively rare complication of cataract surgery caused by a malpositioned IOL. UGH syndrome is typically seen with anterior chamber IOLs but can occur with any lens implant that causes chafing of anterior segment structures. The IOP elevations seen in UGH syndrome are caused by dispersed iris pigment, which clogs the trabecular meshwork, and hemoglobin-laden macrophages, which can block the angle in the setting of hyphema or microhyphema.
On examination, signs of UGH syndrome include elevated IOP, anterior chamber inflammation and iris transillumination defects from IOL chafing. Gonioscopic examination may reveal blood in the angle and hyperpigmentation of the trabecular meshwork from dispersed iris pigment. An obviously dislocated or malpositioned IOL can confirm the diagnosis of UGH syndrome. Ultrasound biomicroscopy may be used to support the diagnosis if necessary. OCT RNFL can also be useful to detect glaucomatous changes from IOP spikes.
Initial management of UGH syndrome includes treatment of intraocular inflammation and pressure elevation. Pilocarpine should be avoided as it can cause increased inflammation and potentially increased contact between the iris and IOL. Ultimately, the definitive treatment for UGH syndrome is IOL repositioning or exchange.
- References:
- Badakere SV, et al. BMJ Case Rep. 2016;doi:10.1136/bcr-2015-213745.
- Bothun ED, et al. Am J Ophthalmol. 2018;doi:10.1016/j.ajo.2017.12.016.
- Bryant TK, et al. J Cataract Refract Surg. 2017;doi:10.1016/j.jcrs.2017.07.002.
- Cates CA, et al. J Neurol Neurosurg Psychiatry. 1998;doi.org/10.1136/jnnp.65.1.131.
- Ellingson FT. J Am Intraocul Implant Soc. 1978, doi:10.1016/s0146-2776(78)80054-8.
- Sousa DC, et al. J Curr Glaucoma Pract. 2016;doi:10.5005/jp-journals-10008-1205.
- Zemba M. Rom J Ophthalmol. 2017;doi:10.22336/rjo.2017.3.
- For more information:
- Chelsea Gottschalk, MD, and Paul Cotran, MD, can be reached at New England Eye Center, Tufts University School of Medicine. 800 Washington Street, Box 450, Boston, MA 02111; website: www.neec.com.
- Edited by Alison J. Lauter, MD, and Sarah E. Thornton, MD. They can be reached at the New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.