This article is more than 5 years old. Information may no longer be current.
Woman presents with bilateral eye pain and unilateral hyphema
The left eye also had 4+ pigmented and non-pigmented cells in the anterior chamber.
Click Here to Manage Email Alerts
A 64-year-old Hispanic woman presented to the emergency department with bilateral pain that started 2 days before presentation. She reported that her symptoms first started in the right eye and then in the left. She described a dull pain in both eyes, increased light sensitivity and redness. She denied similar episodes in the past. She denied any history of trauma, and to her knowledge, she did not have any anticoagulative disorders.
Her medical history was significant for hypertension and hyperlipidemia but no diabetes, arthritis or thyroid problems. Her surgical history included elbow surgery, cesarean section, bilateral pterygium removal (3 years before presentation) and cataract surgery bilaterally (4 years before presentation). Her ophthalmic history was remarkable for refractive error and recurrent corneal abrasions in both eyes for which she was seen in the clinic 4 months before presentation. She was taking antihypertensive and antihyperlipidemic medications.
Examination
At the initial examination, the patient’s vision was 20/40 bilaterally, and her pupils were round, equal and reactive without an afferent pupillary defect. Extraocular movements were full and intact. Pressures were 10 mm Hg and 11 mm Hg in the right and left eyes, respectively, with color plates intact. She did not have any neovascularization of the iris or the angle. The exam was significant for conjunctival irritation bilaterally and 4+ pigmented and non-pigmented cells in the anterior chamber in the left eye along with a 1-mm hyphema; the right eye had only trace pigmented cells in the anterior chamber. She had bilateral posterior chamber IOLs. Careful examination of the left eye demonstrated narrowing of the IOL iris interface superotemporally. There were no transillumination defects. There was no phacodonesis or iridodonesis. On gonioscopy, there was no abnormal vasculature in the angle.
Fundus examination demonstrated normal optic nerves in both eyes with symmetric cup-to-disc ratios of 0.4. Both eyes had a clear vitreous, normal course and caliber of vessels, and flat retinas.
What is your diagnosis?
See answer on next page.
Bilateral pain
The patient was found to have bilateral pain, blurry vision with inflammation, 4+ pigmented and non-pigmented cells in the anterior chamber as well as hyphema in the left eye. Differential diagnosis of hyphema includes trauma, intraocular surgery, trabeculoplasty, iridotomy, anticoagulation and other medications. Infectious causes such as herpes simplex and herpes zoster can also sometimes lead to hyphema, even in the absence of other corneal findings (dendritic ulcer, stromal keratitis). Inflammatory processes such as iritis or Fuchs’ heterochromic iridocyclitis can also be in the differential. Our patient did not have any heterochromia. Neoplasms such as melanoma as well as vascular abnormalities such as rubeosis iridis, ocular ischemic syndrome, retinal artery occlusion, retinal vein occlusions, iris varices and iris hemangioma may also lead to hyphema. Lastly, systemic disorders such as sickle cell trait or disease and coagulation disorders are also included in the differential.
Initially, the patient was referred for evaluation of nontraumatic hyphema. Careful fundus examination of both eyes did not reveal any retinal vascular abnormalities, and there was no iris neovascularization on examination. Vascular occlusion, such as retinal vein occlusion, diabetic retinopathy or retinal arterial occlusion, were not apparent on examination. With findings of iritis, microhyphema, otherwise normal-appearing fundus and a centered-placed IOL with temporal haptic displaced anteriorly forward in the superior and temporal quadrants, a diagnosis of uveitis-glaucoma-hyphema syndrome was deemed most likely. Ultrasound biomicroscopy was performed in the left eye and showed half the IOL optic and one haptic were displaced into the sulcus superotemporally scraping against the iris.
Discussion
Uveitis-glaucoma-hyphema (UGH) syndrome was first described in 1978 by Ellington. It is a post-surgical complication caused by mechanical chafing of anterior segment structures by an IOL. It has a wide range of clinical manifestations, including anterior chamber inflammation and pigment dispersion, increased IOP, hyphema or microhyphema, and vitreous hemorrhage. Pain out of proportion due to iridociliary chafing can be subtle, and often the patient’s ocular discomfort may be out of proportion to ocular findings. The syndrome was most commonly associated with poorly positioned first-generation anterior chamber IOLs; more recently, it has been associated with single-piece acrylic IOLs placed in the ciliary sulcus. But recently there were cases that reported UGH due to in-the-bag IOLs secondary to fibrosis around the foot plates. It can be challenging to diagnose and often results in significant ocular morbidity.
The mechanism involves subluxated IOL chafing leading to a breakdown of the blood-aqueous barrier, causing a triad of uveitis, glaucoma and hyphema. Glaucoma can be caused by direct mechanical injury, with clogging of the trabecular meshwork, by pigment or scarring, or by the inflammatory response itself. Medical therapy with topical steroids and pressure-lowering agents is the first-line treatment, but often the offending IOL must be removed or repositioned to preserve retinal and corneal function. Studies have estimated that UGH syndrome accounts for approximately 10% of IOL exchanges.
Clinical course and management
The patient received topical steroids to treat the uveitis and cyclopentolate to help with the pain and avoid the formation of posterior synechiae. She was followed closely until clearance of the inflammation and corneal edema and eventually underwent IOL exchange. The single-piece acrylic IOL was carefully freed from the capsular bag using viscodissection and brought into the anterior chamber. There it was bisected with intraocular scissors and removed through a 3-mm clear corneal incision. A three-piece acrylic IOL was then injected into the sulcus, and irrigation and aspiration were used to clear the anterior chamber of viscoelastic material. The surgery was uneventful, and at 1 month postop the patient was doing well. Repositioning the old IOL in the posterior chamber would not be ideal in this case because the fibrotic material around the haptic of the old lens would make it prone to further displacement.
- References:
- 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.
- Bryant TK, et al. J Cataract Refract Surg. 2017;doi:10.1016/j.jcrs.2017.07.002.
- 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:
- Eleni Konstantinou, MD, Lisa Sitterson, MD, and Michael B. Raizman, 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.