Faculty Bibliography

PURPOSE: To study eyes with Type 2 (subretinal) neovascularization (NV) secondary to neovascular age-related macular degeneration (nAMD) that shows lesion regression into a Type 1 (subretinal pigment epithelium) pattern after treatment with intravitreal anti-vascular endothelial growth factor (VEGF) therapy. METHODS: Retrospective consecutive case series. Patients showing regression of Type 2 neovascularization into a Type 1 pattern after envelopment by retinal pigment epithelium were included in this analysis. A review of the clinical records and multimodal imaging of these cases was performed at baseline, 1, 3, 6, and 12 months. Demographic data, best-corrected visual acuity (BCVA), color fundus photography, fundus autofluorescence (FAF), fluorescein angiography, near-infrared reflectance (NIR), and structural spectral-domain optical coherence tomography (SD-OCT) were reviewed and analyzed. When available, optical coherence tomography angiography images were analyzed as well. RESULTS: Ten eyes of 9 patients (6 males) diagnosed with treatment-naive pure Type 2 neovascularization secondary to nAMD were included. The mean age was 80.7 years (SD +/- 4.30). Mean best-corrected visual acuity expressed in logMAR (Snellen) was 0.45 +/- 0.20 (20/55) at baseline and significantly improved to 0.22 +/- 0.13 (20/32) at 3-month follow-up (P-value: 0.007). At baseline, color photographs and fundus autofluorescence showed a pigment ring around the neovascular lesion in 6 eyes. A hyperreflective ring was visible on NIR in all eyes at 3-month follow-up. Color photographs showed a tessellated fundus appearance in 9 of the 10 eyes. Serial structural spectral-domain optical coherence tomography scans showed the gradual regression of the Type 2 lesions into a Type 1 pattern with envelopment by the retinal pigment epithelium. En face and cross-sectional optical coherence tomography angiography showed baseline subretinal flow patterns which, after treatment, exhibited reduced flow beneath an intact hyperreflective retinal pigment epithelium (RPE) band. CONCLUSION: Pure Type 2 lesions are infrequent in nAMD, often leading to poor visual outcomes related to subretinal fibrosis. We describe an alternate regression pattern occurring in eyes with early Type 2 lesions treated with intravitreal anti-vascular endothelial growth factor therapy in which the neovascular tissue is enveloped by retinal pigment epithelium producing a Type 1 pattern. These eyes appear to have better visual outcomes than typically seen with Type 2 lesions related to reduced outer retinal damage.

PURPOSE: To study the cross-sectional and en face optical coherence tomography angiography (OCTA) findings in Type 3 neovascularization (NV). METHODS: Optical coherence tomography angiography imaging of 27 eyes of 23 patients with Type 3 NV was analyzed with 9 eyes having consecutive follow-up OCTA studies. RESULTS: Type 3 NV appeared as a linear high-flow structure on cross-sectional OCTA corresponding to a high-flow tuft of vessels seen on en face OCTA. Cross-sectional OCTA seemed to enable the distinction between vascular and nonvascular intraretinal hyperreflective foci. Two patterns of flow were observed; Pattern 1 (11%): a flow signal confined to the neurosensory retina and Pattern 2 (74%): a flow signal extending through the retinal pigment epithelium. No definitive retinal-choroidal anastomosis was observed; however, projection artifacts confounded the interpretation of deeper structures. An increase in the intensity of the high-flow tuft was seen during the progression or recurrence of Type 3 NV. Intravitreal anti-vascular endothelial growth factor therapy caused a reduction in the intensity of the high-flow tuft which was not sustained. CONCLUSION: Compared with conventional imaging, OCTA may improve detection and delineation of vascular changes occurring in Type 3 NV. Cross-sectional and en face OCTA may prove useful in studying the pathogenesis and guiding the management of these lesions.

PURPOSE: To review the literature regarding focal choroidal excavation and show its association with pachychoroid features through case examples. METHODS: The clinical manifestations of focal choroidal excavation are illustrated with various imaging modalities inclusive of fundus photography, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, spectral domain optical coherence tomography (OCT), enhanced depth imaging OCT, and swept-source OCT. RESULTS: Diffuse or focal areas of choroidal thickening with dilated choroidal vessels (pachyvessels) on OCT and choroidal hyperpermeability on indocyanine green angiography are present in many eyes with focal choroidal excavation. Clinical and imaging features of associated comorbidities including central serous chorioretinopathy and choroidal neovascularization are described. CONCLUSION: Focal choroidal excavation appears to be a manifestation of pachychoroid spectrum disease associated with choroidal thickening and pachyvessels on structural OCT and choroidal hyperpermeability on indocyanine green angiography.

PURPOSE: To report an unusual case of delayed-onset bilateral diffuse uveal melanocytic proliferation in a patient with a remote history of gastric adenocarcinoma 17 years earlier. METHODS: Case report of a patient with bilateral diffuse uveal melanocytic proliferation including comprehensive systemic and ocular examinations. RESULTS: A 78-year-old man presented with a history of progressive bilateral vision loss during the 4 previous years associated with fever of unknown origin. He underwent total gastrectomy 17 years earlier as a treatment for gastric adenocarcinoma. Funduscopic examination revealed multiple subretinal pigmented and nonpigmented lesions involving the posterior pole of both eyes. These lesions showed early hyperfluorescence on fluorescein angiography, producing a giraffe pattern. Spectral-domain optical coherence tomography showed intraretinal and subretinal fluid with multiple hyperreflective mounds involving the retinal pigment epithelium. Treatment with the intravitreal anti-vascular endothelial growth factor agent, ranibizumab, produced anatomical improvement in both eyes but visual improvement in just the right eye. CONCLUSION: Although delayed-onset bilateral diffuse uveal melanocytic proliferation may occur, it is important to rule out a second malignancy. To the knowledge of the authors, this is the first report of delayed-onset bilateral diffuse uveal melanocytic proliferation associated with gastric adenocarcinoma. Treatment with intravitreal anti-vascular endothelial growth factor therapy warrants further evaluation.

PURPOSE: To describe the multimodal imaging findings observed unilaterally in a patient with Best disease due to a p.G15D mutation in the BEST1 gene. METHODS: The clinical history of a 62-year-old female patient with unilateral Best disease was reviewed. Retinal findings were documented by clinical examination and multimodal imaging. RESULTS: Posterior segment examination of the patient's right eye demonstrated retinal pigment epithelium hypopigmentation and clumping in the central macula beneath a chronic shallow serous retinal detachment (SRD), confirmed by optical coherence tomography. Fluorescein angiography showed central staining with no evidence of focal leakage or choroidal neovascularization, and correlated with the hypoautofluorescence seen on fundus autofluorescence. There was no evidence of choroidal hyperpermeability on indocyanine green angiography, nor was there any neovascularization detected on optical coherence tomography-angiography. The left eye appeared normal with all imaging modalities. CONCLUSION: Best disease is an autosomal dominant disease that is generally bilateral. We present a case of a unilateral Best disease with serous retinal detachment in a patient with a p.G15D mutation in BEST1. Best disease should be considered in the differential diagnosis of serous retinal detachment and may masquerade as central serous chorioretinopathy.

PURPOSE: To report a case of unilateral stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) in a young male showing structural changes induced by a Valsalva maneuver. METHODS: Case report of a 26-year-old oboist with SNIFR, including multimodal imaging. Eye-tracked spectral-domain optical coherence tomography (SD-OCT) was used to compare the retinal architecture at rest and during a Valsalva maneuver. RESULTS: Spectral-domain optical coherence tomography showed macular and peripapillary retinoschisis with no signs of pathologic myopia, optic pit, or vitreoretinal traction. A full-field electroretinogram showed supranormal responses in the eye studied. Magnetic resonance imaging of the brain showed no abnormalities. Eye-tracked SD-OCT scans showed an increase in retinal thickness reaching 28 microns superior to the disc during an induced Valsalva maneuver. CONCLUSION: Stellate nonhereditary idiopathic foveomacular retinoschisis is a diagnosis made when other known causes of retinoschisis have been excluded. In this patient with unilateral SNIFR, an increase in retinal thickness during a Valsalva maneuver was demonstrated. Further study would be needed to determine the mechanism producing this change and to assess its potential influence on visual prognosis.

PURPOSE: To describe multimodal imaging findings of an evolving case of acute posterior multifocal placoid pigment epitheliopathy occurring in a young healthy male. METHODS: Case report of a patient with acute posterior multifocal placoid pigment epitheliopathy including comprehensive systemic and ocular examinations. Ultra-widefield autofluorescence, fluorescein angiography, indocyanine green angiography, and serial optical coherence tomography angiography were performed. RESULTS: A 34-year-old male presented with acute vision loss in his left eye for 2 weeks. His best-corrected visual acuity was 20/20 in his right eye and 20/200 in his left eye. Dilated funduscopic examination revealed multiple creamy white deep retinal lesions showing macular involvement of the left eye with a diffuse area of pigmentary changes. The presence of multiple areas of hypoperfusion of the inner choroid were demonstrated with fluorescein and indocyanine green angiography. Serial optical coherence tomography angiography showed multiple evolving areas of decreased flow at the level of the inner choroid. CONCLUSION: Although the pathogenesis of acute posterior multifocal placoid pigment epitheliopathy remains unknown, there is growing evidence of a primary choroidal involvement with secondary damage to the overlying retinal pigment epithelium and the outer retinal layers. Optical coherence tomography angiography may provide valuable information for the diagnosis and follow-up of this condition avoiding invasive angiographic procedures.

PURPOSE: To describe two cases of dome-shaped macula (DSM) and serous macular detachment, the diagnosis of which was enhanced with a radial optical coherence tomography (OCT) scanning protocol. METHODS: Retrospective case series of DSM associated with serous macular detachment. Multimodal retinal imaging was performed including spectral domain OCT with a radial scan protocol and en face OCT angiography. Anatomical outcomes before and after therapy are presented. RESULTS: Two cases of DSM associated with serous macular detachment are described. The dome-shaped macular bulge was more clearly elicited as the cause of serous macular detachment with the employment of a radial OCT scanning protocol. Subretinal fluid resolved in both cases using either intravitreal aflibercept injection or half-fluence photodynamic therapy. En face OCT angiography of the choroid demonstrated reduction in the caliber of choroidal vessels after treatment. CONCLUSION: A radial OCT scanning protocol should be considered in eyes with suspicion of DSM, especially in myopic eyes with subretinal fluid. Intravitreal aflibercept therapy or photodynamic therapy may be considered as a treatment for serous macular detachment because of DSM.