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To describe the features of peripapillary pachychoroid syndrome (PPS), a novel pachychoroid disease spectrum (PDS) entity.Medical records of 31 eyes (16 patients) with choroidal thickening associated with intraretinal and/or subretinal fluid in the nasal macula extending from the disk were reviewed (patients with PPS). Choroidal thickness was compared with 2 age-matched cohorts: typical PDS (17 eyes with central serous chorioretinopathy or pachychoroid neovasculopathy) and 19 normal eyes.The patients with PPS were 81% men aged 71 ± 7 years. Peripapillary pachychoroid syndrome eyes displayed thicker nasal versus temporal macular choroids, unlike PDS eyes with thicker temporal macular choroids (P0.0001). Peripapillary intraretinal and/or subretinal fluid was often overlying dilated Haller layer vessels (pachyvessels). Fundus autofluorescence and fluorescein angiography illustrated peripapillary pigmentary mottling without focal leakage. Most PPS eyes (70%) exhibited other PDS findings including serous pigment epithelial detachment or gravitational tracks. Indocyanine green angiography illustrated dilated peripapillary pachyvessels and choroidal hyperpermeability. The disk was usually crowded, with edema noted in 4/31 (13%) eyes and mild late fluorescein disk leakage identified in half of the cases. Choroidal folds (77%), short axial lengths (39% less than 23 mm), and hyperopia (86%) were common.Peripapillary pachychoroid syndrome is a distinct PDS variant, in which peripapillary choroidal thickening is associated with nasal macular intraretinal and/or subretinal fluid and occasional disk edema. Recognition of PPS is important to distinguish it from disorders with overlapping features such as posterior uveitis and neuro-ophthalmologic conditions.

Purpose To investigate vascular endothelial growth factor (VEGF) level according to the clinical and imaging features, and to explore its relationship with the responsiveness to anti-VEGF treatment in eyes with polypoidal choroidal vasculopathy. Methods Aqueous samples were collected from 62 eyes of 62 patients with treatment-naive polypoidal choroidal vasculopathy. Vascular endothelial growth factor levels were measured using enzyme-linked immunosorbent assay. Baseline best-corrected visual acuity, central macular thickness, subfoveal choroidal thickness, greatest linear dimension of the lesion, and the presence of hemorrhage were included in the analysis. The effects of 3 monthly intravitreal ranibizumab injections on best-corrected visual acuity and central macular thickness were assessed. Results Baseline VEGF level was negatively correlated with subfoveal choroidal thickness (r = -0.33, P = 0.01). Other variables had no correlation with VEGF level. The mean change in central macular thickness after anti-VEGF treatment was -51 ± 64 μm, which is positively correlated with VEGF concentration (r = 0.30, P = 0.04) and negatively correlated with subfoveal choroidal thickness (r = -0.35, P = 0.02). Conclusion Vascular endothelial growth factor level demonstrated a negative correlation with baseline subfoveal choroidal thickness and was associated with response to anti-VEGF treatment. These findings suggest that VEGF has a variable contribution to the pathogenesis of polypoidal choroidal vasculopathy depending on choroid thickness.

PURPOSE To subsegment the choroid in patients with polypoidal choroidal vasculopathy and to determine whether the ratio of choriocapillaris/Sattler layer thickness to total choroidal thickness is decreased at sites of polypoidal pathology. METHODS Retrospective, observational, cross-sectional study. A total of 320 eyes of 305 patients with polypoidal choroidal vasculopathy were studied with optical coherence tomography and dye angiography. The ratio of choriocapillaris/Sattler layer thickness to total choroidal thickness was calculated at polypoidal lesion sites in eyes with subfoveal choroidal thickness (SFCT) ≤200 μm. RESULTS Mean SFCT was 267.7 ± 118.5 μm for the entire cohort. Mean SFCT was 151.2 ± 35.0 μm in eyes with SFCT ≤200 μm (n = 124, 39%). In this subgroup, dilated Haller vessels (pachyvessels) were identified under the site of neovascular ingrowth in 117 eyes (94%). Choroidal thickness in the pachyvessel zone was greater (213.3 ± 52.2 μm) than SFCT (P < 0.001) with a significantly lower choriocapillaris/Sattler layer to total thickness ratio (P < 0.001). Qualitative alterations of the retinal pigment epithelium were observed in 60 eyes (51%). CONCLUSION Eyes with normal or subnormal SFCT exhibited extrafoveal choroidal thickening at sites of polypoidal disease. The choriocapillaris and Sattler layers were attenuated at these locations, but Haller vessels were markedly dilated. These changes were topographically associated with sites of neovascular ingrowth and support the classification of polypoidal choroidal vasculopathy as a pachychoroid disorder.

To evaluate the 1-year results of adjunctive photodynamic therapy in patients with Type 1 neovascularization associated with thickened choroid who were refractory to anti-vascular endothelial growth factor monotherapy.Data on 28 eyes of 28 patients with Type 1 neovascularization with a minimum subfoveal choroidal thickness of 300 μm were reviewed. All showed persistent subretinal and/or intraretinal fluid after at least 4 anti-vascular endothelial growth factor injections in the 6 months before adjunctive photodynamic therapy. No eyes had certain polypoidal lesions on indocyanine green angiography.At 3 months, complete fluid absorption was observed in 24 eyes (85.7%). The mean best-corrected visual acuity significantly improved (20/53-20/44, P = 0.040) and mean subfoveal choroidal thickness reduced (386 to 350 μm, P0.001) compared with baseline. Over a 1-year follow-up period, 17 eyes (60.7%) were free from recurrent fluid accumulation. Anti-vascular endothelial growth factor injection was mainly used in retreatment (mean: 1.5). At 12 months, best-corrected visual acuity improved by ≥0.3 logMAR or maintained compared with baseline in 27 eyes (96.4%).Adjunctive photodynamic therapy in eyes with Type 1 neovascularization with thickened choroid that were refractory to anti-vascular endothelial growth factor monotherapy resulted in complete fluid absorption in most eyes, which translated to visual improvement until 1 year.

Preoperatively predicting postoperative kidney function is an essential step to achieve improved renal function and prevent chronic kidney disease. We introduce a novel formula especially to calculate resected and ischemic volume before partial nephrectomy. We examined whether resected and ischemic volume would have value for predicting postoperative renal function.We performed a retrospective cohort study in 210 patients who underwent robotic partial nephrectomy between September 2006 and October 2013 at a tertiary cancer care center. Based on abdominopelvic computerized tomography and magnetic resonance imaging we calculated resected and ischemic volume by the novel mathematical formula using integral calculus. We comparatively analyzed resected and ischemic volume, and current nephrometry systems to determine the degree of association and predictability regarding the severity of the postoperative functional reduction.On multivariable analysis resected and ischemic volume showed a superior association with the absolute change in estimated glomerular filtration rate/percent change in estimated glomerular filtration rate (B = 6.5, p = 0.005/B = 6.35, p = 0.009). The ROC AUC revealed accurate predictability of resected and ischemic volume on the stratified event of an absolute change in estimated glomerular filtration rate/event of percent change in estimated glomerular filtration rate compared to 3 representative nephrometry systems. The calibration plot of this model was excellent (close to the 45-degree line) within the whole range of predicted probabilities.We report a method of preoperatively calculating resected and ischemic volume with a novel formula. This method has superior correlation with the absolute and percent change in estimated glomerular filtration rate compared to current nephrometry systems. The predictive model achieved a strong correlation for the absolute and percent change in estimated glomerular filtration rate.

To evaluate the efficacy of intravitreal triamcinolone injection in diabetic macular edema unresponsive to intravitreal bevacizumab.Patients with diabetic macular edema unresponsive to at least three monthly intravitreal bevacizumabs were included. At least 2 months after the last intravitreal bevacizumab, intravitreal triamcinolone was performed after obtaining an aqueous humor sample. Multiplex cytokine array was used to assay vascular endothelial growth factor, interleukin (IL)-2, IL-6, IL-8, tumor necrosis factor-α, and transforming growth factor-β2. Best-corrected visual acuity and central subfield thickness were evaluated from Month 0 to 3.Twenty eyes were enrolled. The mean best-corrected visual acuity was 47.1 ± 18.9 letters at baseline, and significantly increased to 53.3 ± 19.7 letters at 1 month (P = 0.002) and 52.4 ± 19.1 letters at 2 months (P = 0.041). These visual gains were not sustained at 3 months (50.9 ± 18.6; P = 0.204). A decrease in central subfield thickness more than 11% of baseline occurred in 12 eyes at 1 month. Multivariate analysis showed that intraocular levels of IL-8 (β = 0.538 P = 0.006) was an independent factor for anatomic response at 1 month.Intravitreal triamcinolone has a role in patients who are unresponsive to intravitreal bevacizumab over the short-term. Elevated intraocular IL-8 levels were related to the efficacy.

Although cytomegalovirus (CMV) retinitis is usually diagnosed in allogeneic hematopoietic cell transplantation recipients among patients with hematologic and oncologic disease, it can also occur in acute leukemia patients who have not received hematopoietic cell transplantation. However, CMV retinitis diagnosed after completion of chemotherapy for acute leukemia has not previously been reported. A 17-year-old boy was diagnosed with CMV retinitis 3 months after completion of chemotherapy for acute lymphoblastic leukemia, and his retinitis was assumed to be caused by a delayed immune reconstitution after chemotherapy. The patient was treated with intravenous and intravitreous ganciclovir therapy, and subsequently underwent surgery for retinal detachment.

Polypoidal choroidal vasculopathy (PCV) is an exudative maculopathy affecting vision, with clinical features distinct from neovascular age-related macular degeneration. Currently, no evidence-based guidelines exist for its diagnosis and treatment.A panel of experts analyzed a systematic literature search on PCV together with results of the EVEREST trial, the only published randomized controlled clinical trial in PCV. At a subsequent Roundtable meeting, recommendations for the management of PCV were agreed based on this analysis and their own expert opinion.Diagnosis of PCV should be based on early-phase nodular hyperfluorescence from choroidal vasculature visualized using indocyanine green angiography. Recommended initial treatment of juxtafoveal and subfoveal PCV is either indocyanine green angiography-guided verteporfin photodynamic therapy or verteporfin photodynamic therapy plus 3 × 0.5 mg ranibizumab intravitreal injections 1 month apart. If there is incomplete regression of polyps by indocyanine green angiography, eyes should be retreated with verteporfin photodynamic therapy monotherapy or verteporfin photodynamic therapy plus ranibizumab. If there is complete regression of polyps by indocyanine green angiography, but there is leakage on fluorescein angiography and other clinical or anatomical signs of disease activity, eyes should be retreated with ranibizumab.Practical guidance on the clinical management of PCV is proposed based on expert evaluation of current evidence.

The purpose of this study was to evaluate the safety and efficacy of combination therapy with intravitreal ranibizumab and photodynamic therapy in the treatment of retinal angiomatous proliferation (RAP) with serous pigment epithelial detachment.Ten eyes of nine consecutive patients with newly diagnosed RAP were enrolled in this prospective pilot study. A course of combination therapy consisted of three ranibizumab injections at monthly intervals and a single photodynamic therapy, guided by indocyanine green angiography, about 1 week after the first injection. The patients were followed every month for 12 months. Retreatment was administered when a persistent, recurrent, or new RAP lesion was confirmed.Eight of the 9 patients (9 eyes) completed 12 months of follow-up. At the 3-month visit, 8 of the 9 eyes (89%) showed favorable initial responses. After 6 months, recurrent lesions developed in 2 eyes (25%) and a new lesion in one other eye; all showed favorable responses to retreatment. At the 12-month visit, 7 eyes (78%) showed regression of the RAP lesions, among which 5 eyes (56%) required only a single session of combination treatment. The mean best-corrected visual acuity was improved from 20/125 at baseline to 20/63 (P = 0.021), and the mean central foveal thickness was reduced from 353 μm at baseline to 169 μm (P = 0.017). The mean improvement in the best-corrected visual acuity was 3.86 lines. No patient had vision-threatening adverse events.Ranibizumab and photodynamic therapy combination therapy appears to be safe and effective for anatomical and functional improvement in patients with RAP with pigment epithelial detachment. Further evaluation with a larger patient sample and a long-term controlled study is required to compare treatment efficacy with antivascular endothelial growth factor monotreatment.

PURPOSE To evaluate the efficacy of photodynamic therapy (PDT) with verteporfin for avascular serous pigment epithelial detachment (PED) in elderly Korean patients. METHODS We retrospectively analyzed the medical records of 5 consecutive patients (7 eyes) who presented with a single large (>1 disk diameter) serous PED without clinical signs of soft drusen. Evident or faint choroidal hyperfluorescence was observed around the PED on indocyanine green angiogram (ICGA). Standard PDT was performed with laser spot covering the PED area. RESULTS The mean follow-up period was 17 months (range, 12-31 months). Five eyes (71%) of 4 patients showed complete resolution of the PED with visual improvement 1 month post-PDT, and these results were sustained during the follow-up period. In the remaining 2 eyes (29%) of 1 patient, there was no change in PED with stable or decreased vision. Vision-threatening adverse events did not occur. CONCLUSION In elderly Asians, it is uncertain whether large serous PED without soft drusen is associated with age-related macular degeneration. Most of these PEDs may represent a variant of central serous chorioretinopathy in the elderly and PDT for these lesions showed encouraging results.