Faculty Bibliography

PURPOSE: To review the pathophysiologic principles underlying increased autofluorescence from the outer retina and subretinal space using selected diseases as examples. METHODS: The ocular imaging information and histopathologic features, when known, were integrated for diseases causing increased autofluorescence from the outer retina and subretinal space. Inferences were taken from this information and used to create a classification scheme. RESULTS: These diseases are principally those that cause separation of the outer retina from the retinal pigment epithelium, thereby preventing proper phagocytosis of photoreceptor outer segments. The separation can arise from increased exudation into the subretinal space or inadequate removal of fluid from the subretinal space. Lack of normal outer segment processing initially leads to increased accumulation of outer segments on the outer retina and subretinal space. Over time, this material is visible as an increasingly thick coating on the outer retina, is yellow, and is autofluorescent. Over time, atrophy develops with thinning of the deposited material and decreasing autofluorescence. The accumulated material is ultimately capable of inducing damage to the retinal pigment epithelium. Diseases causing accumulation of the material include central serous chorioretinopathy, vitelliform macular dystrophy, acute exudative polymorphous vitelliform maculopathy, choroidal tumors, and vitreomacular traction syndrome. CONCLUSION: The physical separation of the retinal outer segments from the retinal pigment epithelium hinders proper phagocytosis of the outer segments. Accumulation of the shed but not phagocytized outer segments plays a role in disease manifestations for a number of macular diseases

PURPOSE: To evaluate the autofluorescence findings of acute syphilitic posterior placoid chorioretinitis (ASPPC). METHODS: Patients with ASPPC underwent complete ophthalmologic examinations, including biomicroscopy, fundus color photography, autofluorescence photography, and fluorescein angiography. RESULTS: As expected, the patients had a yellowish lesion in the posterior pole. However, the lesion appeared to have two components, a diffuse placoid yellow discoloration at the level of the retinal pigment epithelium (RPE) and focal yellow spots that appeared to lie on the RPE. The diffuse placoid yellow color change at the RPE level showed a slight generalized increase in autofluorescence admixed with tiny spots of decreased autofluorescence. The yellow collections in front of the RPE were intensely hyperautofluorescent. The placoid area showed fluorescein staining, while the yellow deposits blocked the background fluorescence. Treatment of syphilis caused resolution of the placoid changes with associated improvement in the autofluorescence and fluorescein abnormalities. CONCLUSION: Patients with ASPPC have autofluorescence abnormalities during the acute manifestations of the disorder that help in interpretation of the fluorescein angiographic findings. We found evidence that the RPE is primarily involved and there does not appear to be choriocapillaris occlusion in early lesions.

PURPOSE: To determine the results of intravitreal bevacizumab injections for the management of choroidal neovascularization (CNV) in patients with pseudoxanthoma elasticum (PXE)-associated angioid streaks. METHODS: A consecutive series of patients with PXE and CNV were managed with intravitreal bevacizumab injection (1.25 mg per 0.05 cc). The main outcome measures were visual acuity and greatest lesion height as measured by optical coherence tomography (OCT). RESULTS: Nine eyes of nine consecutive patients received intravitreal bevacizumab (1.25 mg/0.05 mL) injections. The mean follow-up time was 6 months, during which eyes received an average of 1.8 injections. The baseline visual acuity was a mean of 20/368 and improved to 20/289 at the last visit (P = 0.056). Visual acuity either improved or stabilized in all 9 eyes (100%). Serial OCT measurements in 8 eyes showed a mean of 353 microm at baseline, which decreased to 201 mum at the last visit (P = 0.012). No complications were noted. CONCLUSIONS: These short-term results support the use of intravitreal bevacizumab for the management of CNV in patients with PXE. Continued experience with intravitreal bevacizumab in this population will help establish its longer-term efficacy and better define the potential need for serial injections to maintain these results

PURPOSE: To evaluate the short-term outcomes after intravitreal ranibizumab (Lucentis; Genentech, Inc., South San Francisco, CA) injection in patients with neovascular age-related macular degeneration. METHODS: A review of data for consecutive patients who received intravitreal ranibizumab injection was conducted. The main outcome measures were mean visual acuity and central macular thickness at 3 months compared with those at baseline. Response to ranibizumab therapy was evaluated with particular attention to prior treatment with bevacizumab (Avastin; Genentech, Inc.). RESULTS: Mean baseline visual acuity of 231 eyes of 231 patients was 20/152, and 189 patients (81.8%) had undergone prior treatment, with 153 (65.4%) having received intravitreal bevacizumab. Mean visual acuity at 3 months, available for 203 patients (88%), was 20/126 (P = 0.004). Mean visual acuity for 98 patients treated with bevacizumab within 3 months before ranibizumab injection was 20/100 at baseline and 20/98 at 3 months (P = 0.35). Mean baseline central macular thickness was 278 microm for all patients and improved to 211 microm at 3 months (P < 0.001). Macular thickness decrease was noted irrespective of previous bevacizumab therapy. CONCLUSION: Ranibizumab therapy was associated with significant improvements in mean visual acuity and central macular thickness for the group of all patients. Patients who had received bevacizumab treatment within 3 months before initiating ranibizumab treatment had stability of, but no improvement in, visual acuity

PURPOSE: To describe the macular holes in patients with idiopathic parafoveal telangiectasis (IPT) and to propose a pathophysiologic explanation for their formation. METHODS: Four eyes of two patients with IPT were evaluated with biomicroscopy and optical coherence tomography (OCT). RESULTS: One patient had a nearly full-thickness hole with preservation of only the internal limiting membrane (ILM), but had a 20/60 visual acuity. The other patient had a large full-thickness macular hole, but retained 20/40 visual acuity. Each patient had a fellow eye showing prominent central inner foveal cavitation under a very thin ILM, which was devoid of associated tissue. CONCLUSIONS: This report describes the findings of two patients with IPT who developed pronounced central foveal structural abnormalities. The induced anatomic changes noted in our patients suggest that there is a loss of the structural aspects afforded by Muller cells, particularly the Muller cell cone, in the central macula in patients with IPT. The preservation of good visual acuity in our patients implies that the holes were the result of lateral separation of the photoreceptors within the fovea and that there could not have been profound atrophy of the photoreceptors

OBJECTIVE: To evaluate the short-term visual acuity and anatomic responses after intravitreal bevacizumab (Avastin, Genentech) treatment in patients with retinal angiomatous proliferation (RAP). METHODS: The authors conducted a retrospective review of consecutive patients with newly diagnosed or recurrent RAP treated with intravitreal bevacizumab (1.25 mg) during a 3-month period. Complete ocular examination was performed at baseline and follow-up visits. Interval data were analyzed statistically at 1 and 3 months follow-up. RESULTS: Twenty-three eyes of 23 patients underwent intravitreal bevacizumab treatment. The mean age of patients was 81.1 years, median baseline visual acuity of treated eyes was 20/80 (range 20/25-20/800), and mean baseline central macular thickness was 335 mum (optical coherence tomography was available for 22 eyes). Nine eyes had retinal pigment epithelial detachments (PEDs) at baseline. At 1-month follow-up, the median acuity improved to 20/60 (range 20/30-20/400) (P < 0.001), mean central macular thickness decreased to 202 microm (P < 0.001), and PED was present in only 2 eyes (P = 0.016). Seven of 23 eyes at 1 month (30.4%) had improved visual acuity, defined as halving of the visual angle, and no eyes had worse acuity, defined as doubling of the visual angle. Of the 17 eyes available for 3-month follow-up, 5 eyes (29.4%) had better visual acuity, 1 eye (5.9%) had worse acuity, and the remaining 11 (64.7%) had the same acuity. The median visual acuity at month 3 was 20/60 (range 20/25-20/400). There were no thromboembolic phenomena, endophthalmitis cases, retinal detachments, or any other adverse events. CONCLUSION: Treatment of RAP with intravitreal bevacizumab during this retrospective review resulted in a significant decrease in macular thickness and improvement or stabilization of visual acuity. Further long-term investigation is warranted given the promising short-term results