Woman referred for choroidal neovascularization, worsening vision

Fluorescein angiography revealed multiple areas of patchy, early hyperfluorescence in the posterior pole with moderate leakage.

Issue: March 1, 2007

ByDan S. Landmann, MD

ByJay S. Duker, MD

Grand Rounds at the New England Eye Center

A 25-year-old white woman was referred to the retina service at New England Eye Center by a community ophthalmologist for choroidal neovascularization in the right eye with worsening vision over the past month.

Shazia Ahmed

My Hanh T. Nguyen

History

The patient’s only ocular history included moderate myopia and occasional contact lens wear. She had a medical history of acne for which she took aldactone as well as an oral contraceptive pill. She was allergic to Keflex (cephalexin, Dista) and Biaxin (clarithromycin, Abbott).

Examination

On initial consultation, the patient’s visual acuity was 20/80 in the right eye and 20/20 in the left eye; her vision did not improve with refraction. Pupils were equal, reactive and without afferent pupillary defect. Applanation tonometry revealed IOP of 14 mm Hg in both eyes. Anterior slit lamp exam was unremarkable. There were no signs of anterior chamber or vitreous inflammation. Posterior segment exam was notable for a subretinal lesion with surrounding fluid in the right macula and a normal fundus in the left (Figures 1a, 1b). Fluorescein angiography revealed multiple areas of patchy, early hyperfluorescence in the posterior pole with moderate leakage seen in the late photos (Figures 2a-2c). This was found to be consistent with active choroidal neovascularization.

Color photographs of fundus.

Images: Landmann D, Duker JS


Fluorescein angiography of the right eye. From left, at 29 seconds (Figure 2a), at 1 minute, 4 seconds (Figure 2b) and at 5 minutes, 1 second (Figure 2c).

What is your diagnosis?

Choroidal neovascularization

The differential diagnosis for multiple areas of patchy hyperfluorescence seen primarily limited to the posterior pole with concomitant choroidal neovascular membrane is large. However, in a 25-year-old woman without signs of anterior or posterior inflammation, punctate inner choroidopathy is highest on the differential. Presumed ocular histoplasmosis syndrome should also be considered, although serologies for Histoplasma capsulatum were negative and there was no peripapillary atrophy or characteristic curvilinear chorioretinal atrophy. Multifocal choroiditis with panuveitis (MCP) must be considered in the differential. It is distinguished from this case in that patients with MCP have lesions that are slightly larger, generally bilateral and present with significant vitreous inflammation. Similarly, multiple evanescent white dot syndrome is typically a unilateral condition seen in young female patients. On fundus exam, a granular macular appearance and small yellow lesions were noted at the level of the outer retina. The patient’s presentation was most consistent with punctate inner choroidopathy.

Treatment

Upon the patient’s initial presentation and diagnosis with punctate inner choroidopathy, she was started on a course of oral prednisone with no significant improvement. At that time, the treatment options offered to her included photodynamic therapy, observation and submacular surgery. The patient chose to undergo PDT, and approximately 6 months later, her vision had improved from 20/100 to 20/50 with a persistent but inactive choroidal neovascular membrane.

One year later, she presented with vague visual symptoms of metamorphopsia. Her vision was 20/30 in the right eye and 20/20 in the left. On funduscopic exam, an evolving fibrotic scar temporal to fixation was noted in the right eye, while new retinal pigment epithelial changes were seen in the left eye. A fluorescein angiography showed that there were new lesions now present in the left eye but no active CNV. Optical coherence tomography confirmed the absence of subretinal fluid in both eyes.

Two months later, the patient’s vision declined significantly to 20/40 in the right eye and 20/200 in the left. Fluorescein angiography and OCT confirmed bilateral leakage with subretinal fluid. Given the patient’s marked improvement before, she underwent PDT again, this time in both eyes. Unfortunately, she returned 2 weeks later with a further decline in vision, more so in the right eye. On fluorescein angiography, increased fluid exudation and subretinal fluid was noted in the right eye. The patient was opposed to submacular surgery as a treatment option, and because she was trying to get pregnant, Avastin (bevacizumab, Genentech/Novartis) was not offered. Instead, she underwent an intravitreal injection of Kenalog (triamcinolone acetonide, Squibb) in the right eye. Remarkably, her vision improved from 20/400 to 20/40, as did her clinical and angiographic findings.

Discussion

Punctate inner choroidopathy (PIC) generally presents with blurred vision, flickering lights and metamorphopsia. Most patients described in the literature are young women with mild to moderate myopia. Clinically, it is important to note that there are no signs of vitritis or cystoid macular edema. Acute lesions typically are described as multiple, circular, yellow opacities with moderately well-defined borders. The lesions are located in the posterior pole and midperiphery, and histologically found at the level of inner choroid. Chronic lesions, however, may be variably pigmented and have an atrophic punched-out appearance. They can lie anywhere from the outer retina to the inner choroid. Fluorescein angiography shows early hyperfluorescence and staining of acute PIC lesions. Indocyanine green angiography of PIC lesions is notable for showing several more hypofluorescent areas, which correspond to lesions not easily seen clinically.

The prognosis for punctate inner choroidopathy is generally good, with 60% to 75% of patients not developing choroidal neovascularization. Patients usually maintain a vision of 20/40 or better. However, in 25% to 40% of patients choroidal neovascularization develops, and this has been associated with an increased risk of poor vision in the range of approximately 20/200, particularly if the CNV is subfoveal.

Treatment options for punctate inner choroidopathy include observation because most patients will maintain good vision and do not go on to develop CNV. In the past, laser photocoagulation has been used for extrafoveal and juxtafoveal CNV. Immunosuppressive agents such as prednisone, cyclosporine and tacrolimus have all been used, and most believe that agents such as prednisone may provide more prompt recovery of visual acuity in patients found to have active CNV. This case is notable in that the patient had a marked response to intravitreal injection of Kenalog. Until recently, treatment for subfoveal CNV in PIC was limited to PDT and submacular surgery. Objective outcomes for PDT are limited because the prevalence of PIC is so low and patients are concomitantly treated with other means; however, most agree that PDT is an important part of treatment for subfoveal CNV. The utility of submacular surgery is limited based on availability and because postoperatively there have been high incidences of recurrence. Recently, anti-VEGF medications have been proposed as treatment options, although it is not clear that PIC is a VEGF mediated disease process, as is age-related macular degeneration.

For more information:

Dan Landmann, MD, and Jay S. Duker, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com.

Edited by Shazia Ahmed, MD, and My Hanh T. Nguyen, MD. Drs. Ahmed and Nguyen can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com. Drs. Ahmed and Nguyen have no direct financial interest in the products mentioned in this article, nor are they paid consultants for any companies mentioned.

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