Woman presents with unilateral blurred vision and eye pain
Exam shows anterior chamber inflammation, iris transillumination defects and vitreous hemorrhage.
A 53-year-old Hispanic woman with a history of rheumatoid arthritis, iritis and a mechanical heart valve presented with complaints of blurry vision in her left eye for 1 week. She described a sharp pain as well as a burning sensation in the left eye. Additionally, she had intermittent headaches, flashes, floaters and a “blob” in her vision.
She was diagnosed 1 week prior with recurrence of iritis by an outside ophthalmologist, who started her on oral prednisone and prednisolone eye drops three times a day in the left eye. Her eye pressure was also high at her visit with that physician, so she was started on topical latanoprost and dorzolamide-timolol in the left eye. She then developed new floaters, was diagnosed by another outside ophthalmologist with a vitreous hemorrhage, and was referred urgently to the retina clinic at Tufts Medical Center/New England Eye Center to rule out a possible retinal tear. She was a nonsmoker and nondrinker and had no significant family history of medical or eye disease. Her systemic medications included Coumadin (warfarin sodium, Bristol-Myers Squibb) (most recent INR was 2.8), metoprolol, ranitidine and adalimumab.
Upon examination, visual acuities were 20/25+3 in the right eye and 20/25-2 in the left eye. Both pupils were briskly reactive to light, and there was no relative afferent pupillary defect. Confrontational visual fields were full bilaterally. Extraocular movements were grossly full in both eyes. IOPs were 12 mm Hg and 8 mm Hg in the right and left eyes, respectively.
Anterior segment examination was significant for trace nuclear sclerosis in the right eye. Examination of the left eye revealed temporal and nasal transillumination defects of the iris, 1+ anterior chamber cells with a mix of white and red blood cells, and a posterior chamber IOL, slightly decentered inferonasally, with the temporal haptic visible through the transillumination defects temporally (Figure 1). Fundus examination demonstrated normal optic nerves in both eyes with symmetric cup-to-disc ratios of 0.3. The right eye had a clear vitreous, normal course and caliber of vessels, and a flat retina. The left eye had a mild vitreous hemorrhage, posterior vitreous detachment (PVD), and no signs of retinal tear or detachment on scleral depression exam. The retinal vasculature appeared normal.
What is your diagnosis?
See answer on next page.
Blurred vision, eye pain
This patient was found to have unilateral blurry vision with elevated IOP, an anterior chamber reaction, microhyphema and a vitreous hemorrhage. Differential diagnosis of unilateral elevated IOP with vitreous and anterior chamber hemorrhage includes PVD with vitreous hemorrhage, retinal or iris neovascularization due to retinal vascular inflammation or occlusion with associated vitreous hemorrhage, or uveitis-glaucoma-hyphema syndrome. The vitreous hemorrhage in this patient was also probably made worse by the Coumadin.
Initially, the patient was referred for evaluation of possible retinal tear or detachment. While a hemorrhagic PVD with or without a retinal tear could be the cause of some of this patient’s findings, it is unlikely that such an etiology would cause an anterior chamber reaction, microhyphema and elevated IOP. As this patient had a history of rheumatoid arthritis as well as iritis, we considered that there could have been retinal vasculitis that led to retinal or iris neovascularization; however, careful fundus examination of both eyes did not reveal any retinal vascular abnormalities, and there was no iris neovascularization on examination, making this diagnosis unlikely. Similarly, other causes of retinal vascular occlusion, such as retinal vein occlusion, diabetic retinopathy or retinal arterial occlusion, were not apparent on examination. With findings of iritis, microhyphema, elevated IOP and vitreous hemorrhage in addition to the iris defects and an inferonasally displaced IOL, a diagnosis of uveitis-glaucoma-hyphema syndrome was deemed most likely. Ultrasound biomicroscopy was performed in the left eye and demonstrated that the IOL was displaced nasally and appeared to be in contact with the ciliary body on the nasal side (Figure 2).
Uveitis-glaucoma-hyphema (UGH) syndrome was first described in 1978 by Ellington. At the time, anterior chamber IOLs were more common, and he noted that footplates caused mechanical irritation of anterior chamber angle structures as well as the iris. This chafing often leads to iris transillumination defects, pigmentary dispersion, hyphema and elevated IOP. The disease has been found to occur in any age group, and although it has mostly been associated with anterior chamber lenses, it can be seen with any IOL that makes contact with uveal tissue. As posterior chamber IOLs have become more standard and lens design has advanced, the incidence of UGH has dramatically decreased since first described.
Patients with UGH syndrome initially may complain of blurry vision, transient vision loss, pain, redness and/or photophobia. Examination reveals IOL contact with uveal tissue, and there may be resultant transillumination defects. Anterior chamber cell, hyphema and corneal endothelial pigment deposits may also be noted. Gonioscopy may demonstrate blood in the angle, increased pigmentation of the trabecular meshwork or, in the case of an anterior chamber IOL, poorly positioned haptics. There have also been reported cases demonstrating iris neovascularization. Fundus examination may show vitreous hemorrhage, and some patients have been found to have cystoid macular edema. Although UGH syndrome is a clinical diagnosis, imaging can be used to aid in the diagnosis. Ultrasound biomicroscopy can help visualize the position of IOLs in relation to the ciliary body and iris. Posterior segment OCT can be used to detect cystoid macular edema when it is suspected based on physical exam.
Management of UGH syndrome is based around medical treatment of the underlying signs and symptoms until definitive surgical repair can be undertaken. Topical corticosteroids should be initiated to control anterior chamber inflammation. Just as in this patient, IOP can be elevated and can be controlled through the use of topical IOP-lowering medications. Pilocarpine and other cholinergic medications are best avoided due to their miotic effects, which may induce further iris chafing. Finally, the hyphema can be managed with head elevation, decreased physical activity and cycloplegia for ciliary spasm and any present photophobia. Eventually, surgery is often required to either reposition or exchange the IOL, which will both remedy the current situation as well as prevent future recurrences.
Clinical course continued
The patient initially developed worsening vision after 1 week due to continued bleeding and presented with 3+ anterior chamber red blood cells in the left eye, as well as persistent mild vitreous hemorrhage. She continued to take topical IOP-lowering medications as well as prednisolone eye drops. Topical atropine was added for cycloplegia. She was encouraged to continue close monitoring with her local ophthalmologist for improvement of symptoms and to monitor the IOP, with a long discussion regarding a potential IOL repositioning or exchange surgery. She is currently weighing her options but has not yet committed to surgery at this time.
- Apple DJ, et al. Surv Ophthalmol. 1984;doi:10.1016/0039-6257(84)90113-9.
- Asaria RH, et al. Eye (Lond). 1997;doi:10.1038/eye.1997.213.
- Ellingson FT. J Am Intraocul Implant Soc. 1978;4(2):50-53.
- Piette S, et al. Am J Ophthalmol. 2002;doi:10.1016/S0002-9394(02)01386-7.
- Sharma A, et al. Am J Ophthalmol. 2003;doi:10.1016/S0002-9394(02)02054-8.
- Zemba M, et al. Rom J Ophthalmol. 2017;61(1):11-17.
- Zhang L, et al. J Cataract Refract Surg. 2014;doi:10.1016/j.jcrs.2013.12.002.
- For more information:
- Omar Dajani, MD, and Andre J. Witkin, 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 Adam T. Chin, MD, and Omar Dajani, 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.