Faculty Bibliography

BACKGROUND/PURPOSE: To describe the detection of multiple choroidal lesions in a patient with Type 1 neurofibromatosis using multispectral imaging. METHODS: A 25-year-old woman diagnosed with Type 1 neurofibromatosis was examined with the Annidis-RHA system (Annidis Corp.). RESULTS: While conventional ophthalmoscopic imaging showed an unremarkable examination, multispectral imaging with red and infrared wavelengths evidenced numerous deep choroidal lesions. CONCLUSION: Multispectral imaging may detect the choroidal lesions of Type 1 neurofibromatosis that are not easily seen with clinical examination or with other imaging modalities.

Purpose. To describe the complete sequence of the progressive vasculopathy in macular telangiectasia type 2.Methods. This is a report of a case demonstrating the complete vasogenic sequence in macular telangiectasia type 2 over the course of 15 years, and representative images from a collective of 150 patients with macular telangiectasia type 2 employing fundus photography, fluorescein angiography, and optical coherence tomography.Results. Macular telangiectasia may progress along a predictable vasogenic sequence which consists of nonproliferative stages, characterized by temporal loss of macular luteopigment and inner retinal volume loss in the absence of vascular changes, followed by a progressive proliferative vasculopathy, first involving the deep capillary plexus with eventual extension of the vascular changes circumferentially in the inner retinal capillary plexus. Late proliferative stages may become indistinguishable from advanced neovascular age-related macular degeneration.Conclusions. While it is rare to observe the complete vasogenic sequence of macular telangiectasia type 2, a classification into nonproliferative and proliferative stages can be established, and may prove helpful as the mechanisms driving the pathogenic process through those stages are identified.

PURPOSE: To describe the clinical and angiographic features of a series of patients with acute syphilitic posterior placoid chorioretinitis (ASPPC) in the context of previously published cases. METHODS: A retrospective, noncomparative, multicenter chart review was performed on 16 patients with active ASPPC. Positive serologic tests supported the diagnosis in all patients. Color and red-free photographs as well as fluorescein angiography were obtained in each case. Indocyanine green angiography, optical coherence tomography, and fundus autofluorescence were performed on selected patients. A total of 44 previously published cases of ASPPC were identified using both a Medline Search and references listed in articles identified. RESULTS: Ocular involvement was bilateral in 9 of our 16 patients (56.3%). The mean and median ages at presentation were 40 and 38 years, respectively (range 28-57 years). Nine patients (56.3%) were human immunodeficiency virus positive, with most recent CD4 cell counts ranging from 160 cells/muL to 450 cells/muL and a median CD4 cell count of 250 cells/muL. Seven of 16 patients (43.8%) had a history of mucocutaneous manifestations of secondary syphilis, whereas 4 (25.0%) had evidence of neurosyphilis. Anterior chamber and/or vitreous inflammation was evident in 13 patients (81.3%). Fifteen of 16 patients had positive venereal disease research laboratory or rapid plasma regain titers, and 13 of 13 tested patients had a positive serum fluorescent treponemal antibody absorption. The initial vision in the 25 affected eyes ranged from 20/20 to counting fingers, with a median of 20/80. In all patients, posterior segment examination in the involved eyes revealed a large, yellowish, placoid, outer retinal lesion. Fluorescein angiography showed progressive hyperfluorescence in the area of the lesion, often with scattered focal hypofluorescence, or leopard spotting. Inflammation subsided, the yellowish lesions resolved, and vision improved shortly after antibiotic therapy in 20 of 25 affected eyes. Visual acuity at last visit ranged from 20/20 to 20/150, with a median final vision of 20/25. A review of the literature revealed 44 previously reported cases of ASPPC. Shared demographic, clinical, and angiographic features were summarized. CONCLUSION: Acute syphilitic posterior placoid chorioretinitis is an uncommon but clinically and angiographically distinct manifestation of ocular syphilis. All patients with characteristic clinical and angiographic findings of ASPPC should be tested for both neurosyphilis and human immunodeficiency virus coinfection. Vision recovery typically followed completion of appropriate antibiotic therapy.

PURPOSE: The purpose of this study was to describe clinical and multimodal imaging features of patients with Type 1 neovascularization who lack findings of age-related macular degeneration but instead have features consistent with long-standing central serous chorioretinopathy (CSC). METHODS: Nonconsecutive, retrospective, observational case series. Two groups of patients were identified and analyzed. Group 1 included patients presenting with Type 1 neovascularization who at the time of diagnosis were found to have findings more consistent with long-standing CSC than age-related macular degeneration. Group 2 included patients with a known history of CSC who developed Type 1 neovascularization over their course of follow-up. Clinical histories and multimodal imaging findings (color and red-free photography, fundus autofluorescence imaging, fluorescein angiography, indocyanine green angiography, spectral domain optical coherence tomography, and enhanced depth imaging optical coherence tomography) were analyzed. RESULTS: Twenty-seven eyes of 22 patients were identified. Thirteen patients presented with Type 1 neovascularization thought to be secondary to CSC (Group 1), and 9 patients with CSC were observed to develop Type 1 neovascularization over their course of follow-up (Group 2). Eight patients (36%) had polypoidal neovascular structures within their Type 1 neovascular lesions, of which 4 (18% of all patients) had bilateral Type 1 neovascularization. The mean age of patients was 61 years (range, 48-76 years), and the median age was 58.5 years. Thirteen patients (59%) were men. For those patients in Group 2, the mean duration between diagnosis of CSC and detection of Type 1 neovascularization was 139 months (range, 7-365 months). The mean subfoveal choroidal thickness was 354 mum (range, 186-666 mum). CONCLUSION: Some patients presenting with Type 1 neovascularization may have clinical and multimodal imaging findings more consistent with long-standing CSC than with age-related macular degeneration. These patients are more likely to be younger, men, have thicker choroids, and have a higher prevalence of polypoidal neovasculopathy than those patients with Type 1 neovascularization secondary to age-related macular degeneration. Proper identification of these patients may have implications for their natural course and management.

PURPOSE: To report the presence of a hyperautofluorescent ring and corresponding spectral-domain optical coherence tomography (SD-OCT) features seen in patients with autoimmune retinopathy. METHODS: All eyes were evaluated by funduscopic examination, full-field electroretinography, fundus autofluorescence, and SD-OCT. Further confirmation of the diagnosis was obtained with immunoblot and immunohistochemistry testing of the patient's serum. Humphrey visual fields and microperimetry were also performed. RESULTS: Funduscopic examination showed atrophic retinal pigment epithelium (RPE) associated with retinal artery narrowing but without pigment deposits. The scotopic and photopic full-field electroretinograms were nondetectable in three patients and showed a cone-rod pattern of dysfunction in one patient. Fundus autofluorescence revealed a hyperautofluorescent ring in the parafoveal region, and the corresponding SD-OCT demonstrated loss of the photoreceptor inner segment-outer segment junction with thinning of the outer nuclear layer from the region of the hyperautofluorescent ring toward the retinal periphery. The retinal layers were generally intact within the hyperautofluorescent ring, although the inner segment-outer segment junction was disrupted, and the outer nuclear layer and photoreceptor outer segment layer were thinned. CONCLUSION: This case series revealed the structure of the hyperautofluorescent ring in autoimmune retinopathy using SD-OCT. Fundus autofluorescence and SD-OCT may aid in the diagnosis of autoimmune retinopathy and may serve as a tool to monitor its progression

PURPOSE: The purpose of this study was to describe the association of acquired vitelliform lesion (AVL) and large drusen in patients with non-neovascular age-related macular degeneration. METHODS: A retrospective review of clinical examination and multimodal imaging data of patients with AVL and large drusen seen over a 12-month period was performed. Acquired vitelliform lesion was defined as subretinal accretion of hyperautofluorescent yellowish material within the macular region not due to vitelliform macular dystrophy. Large drusen were diagnosed by the presence of mounded deposits in the subretinal pigment epithelial space between the retinal pigment epithelium and the Bruch membrane using multimodal imaging analysis (color photography, autofluorescence, and spectral domain optical coherence tomography). RESULTS: Thirteen eyes of 9 white patients with a mean age of 74 years were observed to have AVL associated with large drusen. The median visual acuity was 20/60. All AVLs were hyperautofluorescent and were located in the subretinal space between the retinal pigment epithelium and the photoreceptor inner segment/outer segment junction. The AVL in this series had similar color, autofluorescence, and optical coherence tomographic findings as the AVL seen in association with cuticular drusen and subretinal drusenoid deposits. CONCLUSION: Acquired vitelliform lesions, which have previously been related to cuticular drusen and subretinal drusenoid deposits, can occur in association with large drusen. Abnormalities leading to drusen formation or processes that function in parallel to these may be causative in AVL formation.