Faculty Bibliography

PURPOSE: To report four examples of a novel optical coherence tomography finding, which appears to be characteristic of inflammatory choroidal neovascularization. METHODS: Retrospective observational case series. RESULTS: Four eyes of four patients were diagnosed clinically with inflammatory choroidal neovascularization and underwent optical coherence tomography. In each case, imaging revealed multiple, distinctive finger-like projections extending from the area of active choroidal neovascularization into the outer retina-the "pitchfork sign"--a finding not typically seen in Type 2 neovascularization due to other etiologies. CONCLUSION: The pitchfork sign may help distinguish inflammatory choroidal neovascularization from other causes of Type 2 neovascularization.

PURPOSE: To describe a series of previously normotensive eyes experiencing sustained elevated intraocular pressure (IOP) associated with long-term intravitreal antivascular endothelial growth factor (VEGF) therapy for neovascular age-related macular degeneration (AMD). PATIENTS AND METHODS: Clinical data were reviewed for 25 eyes of 23 patients with neovascular AMD who had increased IOP while receiving interval doses of intravitreal ranibizumab and/or bevacizumab. All eyes had tolerated multiple anti-VEGF injections in the past without IOP elevations. RESULTS: After a mean of 20.0 anti-VEGF injections (range, 8-40 injections), the mean IOP was 29.8 mm Hg (range, 22-58 mm Hg), compared with a baseline of 16.9 mm Hg (range, 14-21 mm Hg). The mean highest IOP while receiving intravitreal anti-VEGF therapy was 35.8 mm Hg (range, 23-58 mm Hg). Overall, 23 of 25 cases required IOP management. In the remaining 2 cases, anti-VEGF dosing was switched from regular interval dosing to an optical coherence tomography-guided variable regimen, with subsequent improvement in IOP without antiglaucoma treatment. CONCLUSIONS: Serial injections of anti-VEGF agents may lead to persistent IOP elevations that require glaucoma therapy. The clinician should recognize this phenomenon, as it can occur even if the patient has tolerated multiple prior injections without IOP elevation. Further exploration of the relationship between anti-VEGF therapy and IOP is needed

This is a report of nine patients who experienced sudden, severe, unilateral central vision loss following a flulike illness. Each patient had an exudative detachment of the macula. All patients experienced a spontaneous resolution of the acute macular manifestations with near-complete recovery of vision. A characteristic "bull's-eye" appearance in the macula persisted. The acute manifestations of the disorder did not recur in any of the patients during the period of follow-up. The constellation of findings was suggestive of an inflammatory disease of the retinal pigment epithelium, but a specific causative agent could not be identified. The acute clinical and angiographic features, the natural course, and the residual pigment epithelial derangement were not consistent with any previously described disorder.

PURPOSE: To study the effects of photodynamic therapy using verteporfin in the treatment of patients with subfoveal polypoidal choroidal vasculopathy (PCV). METHODS: A retrospective chart review of 16 consecutive patients with subfoveal PCV treated with photodynamic therapy using verteporfin was performed. RESULTS: The mean age of the patients involved was 70.5 years. The mean follow-up time was 12 months. The visual acuity improved in 9 (56.3 %), remained the same in 5 (31.3 %), and decreased in 2 (12.5 %). The mean change in visual acuity was an improvement of 2.38 lines, a difference that was highly significant (P = 0.004). The change in visual acuity was negatively correlated with increasing age. The final visual acuity was positively correlated with initial acuity and negatively correlated with age. These results were confirmed by multiple linear regression. No patient had any lasting complication from the treatment. CONCLUSIONS: Subfoveal PCV has no proven method of treatment. Although the follow-up time and the number of patients in this pilot study were limited, the encouraging results and lack of complications suggest that further study is indicated.

BACKGROUND: It is known that choroidal neovascularization (CNV) in age-related macular degeneration (ARMD) may erode through the retinal pigment epithelium, infiltrate the neurosensory retina, and communicate with the retinal circulation in what has been referred to as a retinal-choroidal anastomosis (RCA). This is extremely common in the end stage of disciform disease. In recent years, the reverse also seems to be possible, as angiomatous proliferation originates from the retina and extends posteriorly into the subretinal space, eventually communicating in some cases with choroidal new vessels. This form of neovascular ARMD, termed retinal angiomatous proliferation (RAP) in this article, can be confused with CNV. Purpose: The purpose of this article is 1) to review the clinical and angiographic characteristics of a series of patients with RAP and 2) to propose a theoretical sequence of events that accounts for the neovascularized process. METHODS: In this retrospective clinical and angiographic analysis, 143 eyes with RAP (108 patients) were reviewed and classified based on their vasogenic nature and course. Clinical biomicroscopic examination, fluorescein angiography, and indocyanine green angiography were used to evaluate patients. RESULTS: The results of this series suggest that angiomatous proliferation within the retina is the first manifestation of the vasogenic process in this form of neovascular ARMD. Dilated retinal vessels and pre-, intra-, and subretinal hemorrhages and exudate evolve, surrounding the angiomatous proliferation as the process extends into the deep retina and subretinal space. One or more dilated compensatory retinal vessels perfuse and drain the neovascularization, sometimes forming a retinal-retinal anastomosis. Fluorescein angiography in these patients usually revealed indistinct staining simulating occult CNV. Indocyanine green angiography was useful to make an accurate diagnosis in most cases. It revealed a focal area of intense hyperfluorescence corresponding to the neovascularization ("hot spot") and other characteristic findings. Based on understanding of the nature and progression of the neovascularized process, patients with RAP were classified into three vasogenic stages. Stage I involved proliferation of intraretinal capillaries originating from the deep retinal complex (intraretinal neovascularization [IRN]). Stage II was determined by growth of the retinal vessels into the subretinal space (subretinal neovascularization [SRN]). Stage III occurred when CNV could clearly be determined clinically or angiographically. A vascularized pigment epithelial detachment and RCA were inconsistent features of this stage. CONCLUSIONS: Retinal angiomatous proliferation appears to be a distinct subgroup of neovascular ARMD. It may present in one of three vasogenic stages: IRN, SRN, or CNV. Whereas ICG angiography is helpful in diagnosing RAP and in documenting the stage of the neovascularized process, it is frequently difficult to determine the precise nature and location of the new vessel formation. It is important for clinicians to recognize the vasogenic potential and the associated manifestations of this peculiar form of neovascular ARMD so that a proper diagnosis can be made, and when possible, an appropriate management administered.

This report describes a new system for digital indocyanine green videoangiography (ICGV) that provides enhanced imaging of the choroidal circulation. This newly assembled system was used to study a consecutive series of 129 patients with exudative age-related macular degeneration (AMD), and ill-defined or occult choroidal neovascularization (CNV). Overall, 39% of the patients in this study with occult CNV could be reclassified as having well-delineated or so-called classic CNV by virtue of the additional findings provided by ICGV. In this series, ICGV was particularly useful in identifying occult CNV in eyes with a large, serous pigment epithelial detachment (PED) and in eyes with recurrent CNV after previous laser photocoagulation treatment. Some of these patients were selected for laser photocoagulation of the abnormal choroidal vessels in order to evaluate the feasibility of this form of treatment on the basis of combined clinical, fluorescein angiographic, and ICGV findings. The results of this study suggest that ICGV is an important adjunct in the evaluation, classification, and laser treatment of patients with occult CNV secondary to AMD.

Eleven patients, 40 to 71 years old, had a choroidal vasculopathy that led to hemorrhagic and exudative macular degeneration. The patients had peculiar polypoidal, subretinal, vascular lesions associated with serous and hemorrhagic detachments of the retinal pigment epithelium. This macular disorder, which we have named idiopathic polypoidal choroidal vasculopathy (IPCV), appears to represent a distinct entity that differs clinically and demograph-ically from age-related macular degeneration (AMD) and other macular diseases associated with subretinal neovascularization. Recognition of this condition is important because it may have specific risk factors, natural course, and management considerations that differ from those of age-related macular degeneration

PURPOSE:: To report three cases of solitary, focal retinal phlebitis. METHODS:: An observational case series. RESULTS:: Three eyes in three patients were noted to have unilateral decreased vision, macular edema, and a focal retinal phlebitis, which was not at an arteriovenous crossing. All three patients developed a branch retinal vein occlusion at the site of inflammation. These patients had no other evidence of intraocular inflammation, including vitritis, retinitis, retinal vasculitis, or choroiditis, nor was there any systemic disorder associated with inflammation, infection, or coagulation identified. CONCLUSION:: Focal retinal phlebitis appears to be an uncommon and unique entity that produces macular edema and ultimately branch retinal vein occlusion. In our patients, the focal phlebitis and venous occlusion did not occur at an arteriovenous crossing, which is the typical site for branch retinal venous occlusive disease. This suggests that our cases represent a distinct clinical entity, which starts with a focal abnormality in the wall of a retinal venule, resulting in surrounding exudation and, ultimately, ends with branch retinal vein occlusion