Cystoid macular edema, rebound iritis persist after cataract surgery
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A 72-year-old woman was referred to the uveitis clinic at the New England Eye Center for recurrent bilateral iritis and persistent cystoid macular edema 6 months after cataract surgery in both eyes.
She initially presented with decreased vision bilaterally for 2 years and was found to have significant nuclear sclerotic cataracts and mildly elevated IOP of 22 mm Hg in the right eye and 25 mm Hg in the left eye. No angle structures were visible on gonioscopy in either eye, and the optic nerves were both cupped with thin superior and inferior rims. She was diagnosed with bilateral chronic angle-closure glaucoma and subsequently underwent cataract surgery with Kahook Dual Blade goniotomy (New World Medical).
Postoperatively, she had intermittent photopsias, floaters and photosensitivity. On exam, she was found to have 1+ cells in the anterior chamber of both eyes and cystoid macular edema (CME) in the right eye. The anterior chamber inflammation initially improved with prednisolone but recurred when topical steroids were tapered.
Ultimately, the patient had three episodes of rebound iritis in the right eye and two episodes in the left eye, with persistent CME in the right eye for 6 months.
Because of her bilateral recurrent inflammation, she was referred to the uveitis clinic for further workup and management.
Her medical history was significant for osteoporosis and chronic abdominal pain. She did not take any medications. The patient drank socially but did not smoke cigarettes or use recreational drugs. She recently moved from the Dominican Republic to the United States and lived with her son.
Examination
Upon presentation to the uveitis clinic, the patient’s visual acuity was 20/40 in the right eye and 20/20 in the left. The pupils were briskly reactive bilaterally with no afferent pupillary defect. IOP was 13 mm Hg in the right eye and 14 mm Hg in the left. Extraocular movements were full. There was inferior constriction in the left eye on confrontation visual field testing consistent with severe glaucoma.
Anterior segment examination was notable for trace cells in the anterior chamber of the right eye and small granulomas at the inferior palpebral conjunctiva of the left eye. No keratic precipitates or iris nodules were noted. Dilated fundus examination showed a moderate epiretinal membrane and temporal laser scars in the right eye and diffuse, coalescent basal laminar drusen in both eyes. Upon a detailed review of systems, the patient reported lower back pain, knee pain and an itchy rash that developed over the past few months on her palms and soles (Figure 1).
Source: Yi Ling Dai, MD, and Lana Rifkin, MD
What is your diagnosis?
See answer below.
Iritis, cystoid macular edema
In this patient who presented with iritis and CME after cataract surgery, Irvine-Gass syndrome was the initial working diagnosis. However, when inflammation is recurrent, bilateral or refractory to treatment, one must consider other infectious or inflammatory causes of uveitis such as retained lens particles, uveitis-glaucoma-hyphema (UGH) syndrome, chronic postoperative endophthalmitis or ocular lymphoma. The patient must also be evaluated for common causes of uveitis such as syphilis, sarcoidosis and tuberculosis.
Retained lens cortex can cause low-grade inflammation, as seen in our patient, and can sometimes be managed medically. Nuclear fragments, on the other hand, cause a strong inflammatory response and almost always require surgical intervention. Repeat gonioscopy and dilated fundus examination did not reveal any retained lens particle in our patient. UGH syndrome, although commonly seen with poorly fitted anterior chamber lenses, can occur with posterior capsular lenses. Chafing of the iris against the lens can cause chronic inflammation and lead to uveitis, glaucoma and hyphema. An ultrasound biomicroscopy was performed in our patient and did not show any iris-lens touch. Chronic postoperative endophthalmitis may present with indolent intraocular inflammation that is persistent despite topical steroid therapy; however, it is less likely to present in both eyes unless it is endogenous. Our patient did not have fungemia or bacteremia. Ocular lymphoma is rare and presents with variable symptoms, making diagnosis challenging. However, given its life-threatening nature, it should be on the differential for any elderly patient with retinal lesions.
Clinical course
OCT in the right eye revealed intraretinal fluid, subretinal fluid, patchy loss of the inner segment/outer segment (IS/OS) junction and an epiretinal membrane (Figure 2a). Fluorescein angiography of the right eye demonstrated leakage at the macula consistent with CME (Figure 2b). The patient’s reported rash was concerning for syphilis, which was possibly exacerbated by the cataract surgery. Loss of the IS/OS junction on OCT further raised concern for syphilis, as this finding can be seen in placoid syphilis. Systemic workup was initiated, including treponemal antibody, QuantiFERON Gold and chest radiograph, as well as lysozyme and angiotensin-converting enzyme. Treponemal antibody was positive with an RPR titer of 1:8. The patient was subsequently started on intravenous penicillin G for treatment of ocular syphilis.
Discussion
Syphilis is a sexually transmitted disease caused by the spirochete bacteria Treponema pallidum. Syphilis infection is one of the great masqueraders and presents with periods of activity and inactivity. Active disease is staged as primary, secondary and tertiary syphilis.
Although rare, ocular syphilis can occur at any stage of the disease. It can manifest in every ocular structure in a myriad of ways. The majority of patients present with posterior uveitis (60.8%) and panuveitis (22.5%). Notably, ocular symptoms may be the only presenting symptom in patients; an epidemiologic study in Great Britain found that only 40% to 69% of patients with ocular syphilis had systemic symptoms. Hence, a detailed history and review of systems are crucial.
Dark-field microscopy remains the gold standard in diagnosing syphilis; however, as it is not readily available, serologic testing has been the practical method of diagnosis. The traditional screening algorithm consists of an initial screening test with a nontreponemal test followed by a second confirmatory treponemal test, which is more specific and sensitive. However, a reverse screening algorithm, in which a treponemal test is performed first, is usually preferred in cases of high clinical suspicion. If both treponemal and nontreponemal tests are positive, the patient is diagnosed with active syphilis and should undergo lumbar puncture for cerebrospinal fluid (CSF) analysis and HIV testing.
Ocular syphilis is treated as neurosyphilis with a 10- to 14-day course of systemic antibiotics (eg, intravenous penicillin G). Repeat lumbar puncture at 6 months may be considered if initial CSF studies show pleocytosis or elevated protein. Sexual partners also need to be verified.
Case resolution
Our patient underwent an additional workup and received the full course of treatment for ocular syphilis. HIV testing was negative, and lumbar puncture was deferred. Follow-up testing 1 month after treatment demonstrated complete resolution of the subretinal fluid and iritis as well as restoration of the IS/OS junction (Figure 3). The patient also reported subjective improvement of her photosensitivity and injection. The rash on the soles of her feet and palms improved dramatically as well.
- References:
- Centers for Disease Control and Prevention. MMWR Morb Mortal Wkly Rep. 2006;55(10):269-273.
- Mathew RG, et al. Invest Ophthalmol Vis Sci. 2014;doi:10.1167/iovs.14-14559.
- Oliver GF, et al. Br J Ophthalmol. 2019;doi:10.1136/bjophthalmol-2018-313207.
- Syphilis – 2015 STD treatment guidelines. www.cdc.gov/std/tg2015/syphilis.htm. Updated June 4, 2015. Accessed Feb. 23, 2021.
- For more information:
- Yi Ling Dai, MD, and Lana Rifkin, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.
- Edited by Christine Benador-Shen, MD, and Malgorzata Dymerska Peterson, MD. They can be reached at New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.