Curvilinear streaks in multifocal choroiditis.

PURPOSE: Discreet chorioretinal spots, neovascular maculopathy, peripapillary atrophy and peripheral curvilinear pigmentary-atrophic streaks are characteristic of Multifocal Choroiditis. We report the presence of curvilinear streaks in the posterior pole. METHODS: Observational series. RESULTS/CONCLUSIONS: Like chorioretinal discreet spots, peripheral curvilinear atrophic-pigmentary streaks may also appear in the posterior fundus, including the macular region, in Multifocal Choroiditis.

The nature and frequency of neovascular age-related macular degeneration.

PURPOSE: This study was designed to evaluate the frequency and nature of neovascularization in age-related macular degeneration (ARMD) utilizing the combination of digital imaging techniques, fluorescein angiography (FA), indocyanine green (ICG) angiography, and optical coherence tomography (OCT). METHODS: A complete clinical examination was performed on 100 eyes of 93 consecutive newly diagnosed patients with neovascular ARMD. Digital fluorescein angiography, ICG angiography, and OCT were also used in evaluating those patients. Comparison of the imaging techniques to determine their value in studying the nature of the lesions. RESULTS: On the basis of existing fluorescein standards, 15 eyes were diagnosed with classic choroidal neovascularization (CNV), 15 with minimally classic CNV, and 70 with occult CNV. ICG angiography was superior for detecting the active vascular component in polypoidal CNV (16 eyes) and retinal angiomatous proliferation (14 eyes). OCT was more sensitive than FA for determining the presence of cystoid macular edema evident in the vast majority of eyes with retinal angiomatous proliferation (RAP). CONCLUSIONS: These results suggest that FA, ICG angiography, and OCT, when used in combination, will assist clinicians in best determining the precise nature of the neovascular process in ARMD.

Late effect of external eye irradiation on choroidal circulation.

PURPOSE: To report two cases of atypical late onset radiation chorioretinopathy occurring 15 and 25 years post exposure and the indocyanine green (ICG) angiographic findings in these patients. METHODS: Clinical examination and imaging including fluorescein and ICG angiography were performed. RESULT: Fundus examination of the first patient revealed microangiopathy with intraretinal hemorrhages, lipid exudation, telangiectatic and aneurysmal capillary changes. Indocyanine green angiography showed an apparent chorioretinal anastomosis and delayed perfusion of the choriocapillaris. Fundus examination of the second patient revealed a pigment epithelial detachment and retinal pigment epithelial changes. Indocyanine green angiography showed atypical, tortuous, dilated, choroidal vessels as well as areas of hypoperfusion. Both patients had multiple dot-like hyperfluorescent spots in the midphase of the ICG angiogram. CONCLUSIONS: External radiation exposure may lead to both retinal and choroidal alterations which may be independent events and which may manifest after a long period of quiescence. Furthermore, ICG angiography appears to be a useful diagnostic tool to study the alterations of the choroid following external eye irradiation.

Curvilinear pigmentary lesions in a rod-cone dystrophy.

PURPOSE: To report a peculiar curvilinear pigmentary lesion in the peripheral fundus in a rod-cone dystrophy. METHODS: Observational case report. Fundus examination of a 57-year-old woman who was known to have a generalized rod-cone dystrophy since she was 8 years old. RESULTS: The peripheral fundus examination revealed a curvilinear lesion which resembles a well-known finding associated with a presumed ocular histoplasmosis syndrome or multifocal choroiditis. CONCLUSIONS: The differential diagnosis of a peculiar curvilinear pigmentary lesion in the peripheral fundus may be expanded to include a generalized rod-cone dystrophy.

Ocular Whipple's disease: earlier definitive diagnosis.

PURPOSE: Whipple's disease is a rare, chronic, and multiorgan bacterial disease that predominantly involves the gut and its lymphatic drainage in middle-aged Caucasian men but may involve the eye. It is often difficult to diagnose and treat. We report results of one more polymerase chain reaction (PCR) analysis-diagnosed ocular Whipple's disease (OWD) case and the care of three new patients with ocular inflammation caused by OWD. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Three patients. METHODS: Diagnostic PCR was performed on a vitreous sample, medical records of three patients with OWD were reviewed, and a literature review (1907 to present) located 74 OWD cases. Analysis of published OWD cases to seek out factors that contribute to earlier, definitive diagnosis and treatment of OWD is made. MAIN OUTCOME MEASURES: Response of ocular inflammation and vision to treatment. RESULTS: One of 3 new and 4 of 74 published OWD cases reviewed were diagnosed with PCR on vitreous samples and subsequently successfully treated with antibiotics without devastating central nervous system (CNS) sequelae. CONCLUSION: Diagnostic PCR on vitreous samples and recognition of key presenting symptoms in patients with OWD allows earlier definitive diagnosis, when the disease may be more amenable to antibiotic treatment, compared with "late" CNS Whipple's disease in the cases reviewed.

Retinal angiomatous proliferation in age-related macular degeneration.

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.

Locally invasive tumors arising from hyperplasia of the retinal pigment epithelium.

PURPOSE: Focal hyperplasia of the retinal pigment epithelium (RPE) is a common fundus condition that is generally stationary, with little or no tendency to enlarge or spawn neoplasms. The purpose of this report is to describe the unusual clinical features of two similar cases in which a nodular tumor of the RPE was documented to arise from a small focus of hyperplasia of the RPE. METHODS: Clinical and cytopathologic observations of two patients. RESULTS: Both patients were observed for approximately 25 years with an unusual progressive fundus tumor that originally arose from a small, flat, irregular focus of hyperplasia of the RPE. The originally observed pigmented lesion was attributed to toxoplasmosis in one patient and laser treatment for central serous chorioretinopathy in the other. In both patients, the tumor enlarged, invaded through the full-thickness sensory retina, and produced a characteristic retinal perforation with apposition of the mass to the vitreous. In both instances, fine-needle aspiration biopsy showed scant pigmented cells, but a definite diagnosis was not made. However, clinical observations in both patients suggested that these tumors were acquired neoplasms that arose from small foci of hyperplasia of the RPE. CONCLUSION: Focal hyperplasia of the RPE can give rise to unusual invasive tumors that invade and replace the overlying sensory retina. These tumors have unique clinical features that differentiate them from uveal melanoma and other pigmented fundus lesions.

Central serous chorioretinopathy after epidural corticosteroid injection.

PURPOSE: To report three patients who developed central serous chorioretinopathy after epidural corticosteroid injection for treatment of back pain. DESIGN: Interventional case series. METHODS: Three men, aged 73, 52, and 73 years, presented with bilateral central serous chorioretinopathy after corticosteroid injection in the epidural space for treatment of back pain. In all three cases, we did not initially elicit the history of corticosteroid use. RESULTS: Two of the three patients, aged 52 and 73 years, had diffuse retinal pigment epitheliopathy and one, aged 73 years, had classic central serous chorioretinopathy. Two patients had a spontaneous resolution of the subretinal fluid in both eyes. One patient had laser photocoagulation in both eyes but continued to have diffuse leakage in one eye. CONCLUSIONS: A careful history to determine corticosteroid use, including possible intrajoint and epidural injection, should be performed in older people with serous detachment of the macula, particularly when bilateral.

Optical coherence tomography of bleb-like subretinal lesions after retinal reattachment surgery.

PURPOSE: To report the optical coherence tomographic characteristics of persistent bleb-like subretinal lesions after retinal reattachment surgery, including scleral buckling and cryopexy. METHODS: Case reports. RESULTS: In two eyes of two patients yellow to yellowish-orange bleb-like lesions were found in the posterior pole after retinal reattachment surgery. The common aspects of the surgery were scleral buckling procedures and cryopexy. There were subretinal precipitates in some of the lesions. The lesions showed no associated leakage during fluorescein or indocyanine green angiography. Optical coherence tomography demonstrated that the bleb-like lesions were tiny retinal detachments. CONCLUSION: Optical coherence tomography examination of bleb-like subretinal lesions showed that they were actually retinal detachments. Our patients and previously reported patients developed these lesions after scleral buckling and cryopexy. These lesions may represent persistent retinal detachment because of the presence of protein in the subretinal fluid.

Polypoidal choroidal vasculopathy in Italy.

PURPOSE: To report on the frequency and clinical features of polypoidal choroidal vasculopathy (PCV) in a consecutive series of elderly Italian patients presenting with macular exudation. METHODS: The authors conducted a retrospective study on a series of 194 consecutive patients 50 years or older with newly diagnosed exudative maculopathy and the presumed diagnosis of age-related macular degeneration (ARMD). Color and/or red-free photographs and fluorescein and indocyanine green angiography were performed in all patients. RESULTS: Of the 194 patients, 19 (9.8%) were diagnosed with PCV. The remaining 175 (90.2%) patients had ARMD complicated by choroidal neovascularization. No age or sex differences were observed between the two groups. The disease was unilateral in 103 (58.9%) of 175 ARMD cases compared with 15 (78.9%) of 19 PCV cases (P = 0.09). Nine (47.3%) of 19 patients with PCV had an extramacular choroidal neovascularization, compared with only 5 (2.9%) of 175 patients with ARMD (P < 0.0001). Significant drusen were present in the fellow eyes of 66 (64.1%) of 103 unilateral cases in the ARMD group and in 4 (26.7%) of 15 patients with unilateral disease in the PCV group (P = 0.006). CONCLUSIONS: Polypoidal choroidal vasculopathy is not an uncommon disease in Italy and should be suspected in patients presenting with extramacular lesions and no large drusen in the fellow eye.

Endoscopic vitreoretinal surgery for complicated proliferative diabetic retinopathy.

PURPOSE: To evaluate the indication for endoscopic vitreoretinal surgery in proliferative diabetic retinopathy (PDR). METHODS: Chart review of consecutive cases of vitreoretinal surgery for PDR performed by one of the authors (Y.L.F.) over a 2-year period. RESULTS: Endoscopic vitreoretinal surgery was performed in 8 of 41 (19.5%) eyes. The surgical indications were small pupil (3), hyphema (3), pseudophakia with fibrotic posterior capsule (1), and pars plana neovascularization with anterior tractional retinal detachment (6). CONCLUSION: Endoscopic vitreoretinal surgery, by enhancing the visualization of the retroirideal space, is a useful technique in PDR with opaque ocular media and/or neovascularization of the pars plana and ciliary body.

Differentiation between presumed ocular histoplasmosis syndrome and multifocal choroiditis with panuveitis based on morphology of photographed fundus lesions and fluorescein angiography.

OBJECTIVE: To evaluate whether inactive cases of presumed ocular histoplasmosis syndrome (POHS) and multifocal choroiditis with panuveitis (MFC) can be differentiated from each other by their appearance on fundus photography and fluorescein angiography. METHODS: Two masked observers classified 50 patients' photographs (27 with fluorescein angiograms) as POHS, MFC, or "indeterminate." Twenty-five patients had known POHS and 25 had known MFC. Statistical analysis was performed to assess agreement and interrater reliability. RESULTS: Observer A classified 33 patients and was indeterminate on 17. Of the 33, he was correct on 26 (79% crude accuracy; kappa = 0.560; 95% confidence interval [CI], 0.286-0.834). Observer B classified 40 patients and was indeterminate on 10. Of the 40, he was correct on 33 (82% crude accuracy; kappa = 0.650; 95% CI, 0.422-0.878). Both observers ventured a diagnosis on 28 common patients. Of these, they selected the same diagnosis on 26 (93% crude agreement). When the 2 observers' diagnoses were compared and indeterminate patients were factored in, the kappa value was 0.408 (95% CI, 0.215-0.601). When the indeterminate patients are excluded, the kappa agreement increased to 0.825 (95% CI, 0.592-1). When pictures only were available, observer A and observer B kappa values against the gold standard were 0.625 (95% CI, 0.270-0.980) and 0.588 (95% CI, 0.235-0.940), respectively. The pictures-only kappa values for observer A vs observer B were 0.582 (95% CI, 0.316-0.848) with indeterminate patients factored in and 1.0 (95% CI, 1.0-1.0) when indeterminate patients were excluded. Pictures and fluorescein angiogram kappa values were 0.493 (95% CI, 0.076-0.909) for observer A and 0.706 (95% CI, 0.413-0.999) for observer B against the gold standard. For observer A vs observer B, the kappa value was 0.261 (95% CI, -0.002 to 0.524) with indeterminate patients factored in and 0.567 (95% CI, 0.032-1) excluding indeterminate patients. Sensitivity for all cases for observer A was 60% (+/-13%) for POHS and 94% (+/-6%) for MFC. For observer B, the sensitivity for all cases was 70% (+/-10%) for POHS and 95% (+/-5%) for MFC. CONCLUSIONS: Given adequate funduscopic information, the experienced observer can often accurately distinguish between POHS and MFC without the need for ancillary testing. Angiography in addition to fundus photography does not appear to increase diagnostic ability. There appears to be a higher sensitivity for MFC than for POHS.

Relentless placoid chorioretinitis: A new entity or an unusual variant of serpiginous chorioretinitis?

OBJECTIVE: To characterize an unusual clinical entity resembling acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and serpiginous choroiditis but with an atypical clinical course. PATIENTS: We describe 6 patients, aged 17 through 51 years, exhibiting this unusual entity who were seen at 6 different centers from 1984 to 1997. RESULTS: The acute retinal lesions in this series were similar to those of APMPPE or serpiginous choroiditis, both clinically and on fluorescein and indocyanine green angiography. However, the clinical course, number of lesions, and location of these lesions were atypical. These patients had evidence of numerous posterior and peripheral retinal lesions predating or occurring simultaneously with macular involvement. Older, healing pigmented lesions were often accompanied by the appearance of new active white placoid lesions. Additionally, these cases all demonstrated prolonged periods of activity resulting in the appearance of more than 50 and sometimes hundreds of lesions scattered throughout the fundus. Growth of subacute lesions and the appearance of new lesions continued for 5 to 24 months after initial examination, and relapses were common. CONCLUSIONS: This entity has clinical features similar to APMPPE and serpiginous choroiditis but has a prolonged progressive clinical course and widespread distribution of lesions. It may represent a variant of serpiginous choroiditis or may be a new entity. We call it relentless placoid chorioretinitis. Arch Ophthalmol. 2000;118:931-938

Retinal choroidal anastomoses and occult choroidal neovascularization in age-related macular degeneration.

OBJECTIVE: This study was designed to identify the incidence of retinal choroidal anastomoses in patients with occult choroidal neovascularization (CNV) and focal hot spots on indocyanine green (ICG) angiography, to identify the clinical and angiographic features that would assist in their identification, and to determine if the presence of these anastomotic lesions affect the outcome of laser therapy. DESIGN: Combined prospective and retrospective cross-sectional study. PARTICIPANTS: One hundred fifty consecutive patients with newly diagnosed occult CNV secondary to exudative age-related macular degeneration and focal hot spots on ICG angiography were evaluated prospectively. In addition, a retrospective review was performed on 79 eyes previously reported to have undergone laser photocoagulation treatment with ICG guidance. METHODS AND TESTING: In all cases, stereo color and red-free photographs, and stereo fluorescein and digital ICG angiograms were obtained for evaluation. MAIN OUTCOME MEASURES: Images obtained by all four techniques were evaluated for the presence of a retinal choroidal anastomosis. Associated clinical and angiographic findings were noted. In the retrospective review, the success rate of laser treatment was correlated with the presence or absence of a retinal choroidal anastomosis. RESULTS: Of the 150 eyes evaluated prospectively, 31 (21%) were found to have a retinal choroidal anastomosis. Retinal choroidal anastomoses were found in 27% of patients with associated serous pigment epithelial detachment (PED), whereas 13% were found in those without an associated elevation of the retinal pigment epithelium. Seventy-one percent of eyes had multiple anastomotic connections. Ninety percent of eyes had at least one retinal vein involved in the anastomotic connection. Clinical evidence of preretinal and intraretinal hemorrhage and cystic edema coupled with angiographic evidence of intraretinal dye leakage were key features of retinal choroidal anastomoses. In the retrospective review, seven patients were found to have retinal choroidal anastomoses with associated serous PED and demonstrated a very low (14%) success rate for laser treatment. CONCLUSIONS: Retinal choroidal anastomoses can present as a primary manifestation of the exudative process in age-related macular degeneration. They may be seen in eyes with and without detachment of the retinal pigment epithelium. Specific clinical and angiographic features have been identified that can aid in the diagnosis of these vascular anomalies. Their presence represents a poor prognostic sign for successful ICG-guided laser treatment.

Polypoidal choroidal vasculopathy masquerading as central serous chorioretinopathy.

OBJECTIVE: To differentiate polypoidal choroidal vasculopathy (PCV) from central serous chorioretinopathy (CSC). DESIGN: A retrospective, observational case series. PARTICIPANTS: Thirteen patients originally diagnosed with CSC proved to have PCV after more extensive evaluation and follow-up. METHODS: A clinical and angiographic review of patients with manifestations of CSC, including macular detachment. MAIN OUTCOME MEASURES: Demographic data, funduscopic examination, and fluorescein and indocyanine green (ICG) angiographic findings. RESULTS: Thirteen patients initially suspected of having CSC were ultimately diagnosed as having PCV. These eyes had exudative macular detachments secondary to a small caliber, polypoidal choroidal vascular abnormality or so-called polypoidal choroidal neovascularization. The clinical manifestations in the fundus varied. They included multiple, variably sized serous pigment epithelial detachments, neurosensory retinal detachment, lipid deposition, patchy atrophy of the pigment epithelium and indistinct staining from decompensation of the posterior blood-retinal barrier on fluorescein angiography. In reality, the suspected PEDs proved to be polypoidal lesions of PCV when imaged with ICG angiography. CONCLUSIONS: The clinical diagnosis of CSC or PCV generally poses little challenge to the experienced retinal specialist. However, in CSC with persistent and/or recurrent exudation, a myriad of retinal pigment epithelial changes may evolve that make it difficult to differentiate these two entities. In such patients, ICG angiography is useful in differentiating CSC from PCV. An accurate clinical diagnosis is important since each of these entities, CSC and PCV, may differ in terms of their risk factors, natural course, and visual prognosis.