Efficacy of plasmapheresis as treatment for bilateral hyperviscosity syndrome related retinopathy in multiple myeloma.

PURPOSE: To report a case of good clinical response to plasmapheresis as therapy for a bilateral hyperviscosity syndrome related retinopathy in a young patient with undiagnosed multiple myeloma (MM). METHODS: Case report. RESULTS: A 48-year-old caucasian man, previously diagnosed with monoclonal gammopathy of undetermined significance (MGUS), presented for medical attention for back ache and vision decrease, worst in the left eye. Ophthalmological examination revealed presence of bilateral CRVO-like retinopathy which raised the hypothesis of hyperviscosity syndrome (HVS). After confirmation of MM diagnosis, plasmapheresis were promptly begun. The right eye regained a good visual acuity and an anatomical restoration already notable 5 days from the first plasmapheresis. CONCLUSIONS: Fundoscopy lead to diagnosis of HVS, it is therefore advisable in all patients with MM in order to perform plasmapheresis as soon as possible if necessary. In case of atypical CRVO, haematological and inflammatory causes, should be always ruled out.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY CHARACTERISTICS OF POLYPOIDAL CHOROIDAL VASCULOPATHY SECONDARY TO CHRONIC CENTRAL SEROUS CHORIORETINOPATHY.

PURPOSE: To report diagnostic capability of optical coherence tomography angiography (OCTA) in detecting polypoidal choroidal vasculopathy and its morphologic characteristics in white patients with chronic central serous chorioretinopathy. METHODS: Retrospective consecutive series of 20 eyes (17 consecutive patients) with a diagnosis of polypoidal choroidal vasculopathy secondary to chronic central serous chorioretinopathy based on clinical and multimodal imaging were included. All patients underwent a complete ophthalmologic examination, including best-correct visual acuity, fundus autofluorescence, fluorescein and indocyanine green angiography, spectral-domain optical coherence tomography, and OCTA. RESULTS: In all eyes (100%), OCTA revealed the branching vascular network as a hyperflow lesion in both choriocapillaris and outer retina layers. Segmentation of the choriocapillaris in OCTA, in correspondence of the polypoidal dilation detected at indocyanine green angiography, showed a hyperflow round structure in 75% of cases and hypoflow round structure in 15%. Two of 20 eyes (10%) had no detectable polyps on OCTA. CONCLUSION: Optical coherence tomography angiography is a noninvasive imaging modality allowing a good visualization of both branching vascular network and polyp dilations. Our findings suggest that OCTA is a useful tool in the investigation of chronic central serous chorioretinopathy complicated by polypoidal choroidal vasculopathy.

Comparison of inverted flap and subretinal aspiration technique in full-thickness macular hole surgery: a randomized controlled study.

PURPOSE: To compare the inverted flap and the subretinal aspiration technique for full-thickness macular hole (FTMH) surgery. METHODS: Forty consecutive eyes with a stage IV FTMH were randomly assigned into 2 treatment groups. After core vitrectomy and perimacular internal limiting membrane (ILM) peeling, in group A, the subretinal remnant macular fluid was aspirated with a 41-G cannula after the air-fluid exchange procedure, while in group B, the technique of an inverted ILM flap was completed. Differences in postoperative best-corrected visual acuity (BCVA) and occurrence of intraoperative or postoperative complications between the 2 groups were evaluated. RESULTS: All FTMHs were closed after the first surgery with no intraoperative or postoperative complications. In group A, 16 patients (80%) showed improvement of BCVA and 4 (20%) showed stabilization. In group B, 12 patients (60%) had improved BCVA, while 6 (30%) remained stable and 2 (10%) worsened. Postoperative BCVA for group A was significantly better than for group B (p = 0.022). CONCLUSIONS: The surgical techniques had similar rates of closure of FTMH, although BCVA outcomes were significantly better in the subretinal aspiration group.

ANTI-VASCULAR ENDOTHELIAL GROWTH FACTOR THERAPY VERSUS PHOTODYNAMIC THERAPY IN THE TREATMENT OF CHOROIDAL NEOVASCULARIZATION SECONDARY TO CENTRAL SEROUS CHORIORETINOPATHY.

PURPOSE: To evaluate the efficacy of photodynamic therapy (PDT) compared with intravitreal vascular endothelial growth factor (VEGF) inhibitors alone or combined with PDT in the treatment of choroidal neovascularization (CNV) secondary to chronic central serous chorioretinopathy (CSCR). METHODS: Retrospective study of a consecutive series of 34 white patients (34 eyes) with angiographic evidence of choroidal neovascularization secondary to chronic central serous chorioretinopathy, divided into two groups followed for at least 12 months: 16 were treated with full fluence PDT (PDT group) and 18 with vascular endothelial growth factor inhibitors (intravitreal [IVT] group). RESULTS: In the PDT group, mean best-corrected visual acuity (BCVA) was 20/40 (0.30 ± 0.69 logMAR) at baseline and did not change after 12 months (20/40; 0.30 ± 0.49 logMAR [P = 0.49]). In the IVT group mean, best-corrected visual acuity was 20/40 (0.30 ± 0.69 logMAR) at baseline and also did not change at the 12-month follow-up (20/32; 0.20 ± 0.49 logMAR [P = 0.20]). There was no statistically significant difference between the two groups in terms of best-corrected visual acuity. A statistical significance difference was found in central macular thickness between the two groups with a better reduction of the thickening in the PDT group (P = 0.05); moreover, indocyanine green angiography analysis revealed that 50% of the polypoidal lesions were closed after PDT and 25% of the polypoidal lesions disappeared after anti-vascular endothelial growth factor therapy at 12-month follow-up. CONCLUSION: PDT and IVT inhibitors alone or combined show similar clinical effects in chronic central serous chorioretinopathy eyes with choroidal neovascularization.

Optical coherence tomography evaluation of patients with macula-off retinal detachment after different postoperative posturing: a randomized pilot study.

PURPOSE: To assess the presence of outer and inner retinal folds (RFs) and drop-out of the ellipsoid zone (EZ) occurring after surgical repair of macula-off rhegmatogenous retinal detachment (RRD) with different postoperative posture and preoperative use of adjuvant perfluorocarbon liquid (PFCO). METHODS: In this prospective study, 56 eyes of 56 consecutive patients affected by RRD were subjected to 23- or 25-gauge pars plana vitrectomy (PPV). The patients were randomized in four groups (14 prone 5 hr without PFCO, 14 supine 5 hr without PFCO, 14 prone 5 hr with PFCO and 14 supine 5 hr with PFCO) and followed up with spectral domain optical coherence tomography (SD-OCT). RESULTS: Spectral domain optical coherence tomography (SD-OCT) was recorded before surgery, at days 30 and 90 to detect the presence of outer RFs, inner RFs and drop-out of EZ and to follow their variation over time. No statistical significance was found in our groups for outer RFs, inner RFs, drop-out of EZ formation and evolution. The postoperative best-corrected visual acuity (BCVA) improved in all groups (mean preoperative BCVA 1.47 logMar ± 0.19, mean postoperative BCVA 0.27 logMar ± 0.11, p < 0.01), without statistical variations between the four groups in BCVA after surgery. CONCLUSION: The use of adjuvant and variation in postoperative position did not change the risk of presenting outer RFs, inner RFs and drop-out of EZ after RRD.

A NOVEL P.ASP304GLY MUTATION IN BEST1 GENE ASSOCIATED WITH ATYPICAL BEST VITELLIFORM MACULAR DYSTROPHY PHENOTYPE AND HIGH INTRAFAMILIAL VARIABILITY.

PURPOSE: To report the atypical phenotypic characteristics of patients with a novel p.Asp304Gly mutation in BEST1. METHODS: Affected individuals underwent a complete ophthalmic examination, including best-corrected visual acuity, fundus autofluorescence, spectral domain optical coherence tomography, and electrophysiologic testing. All individuals were screened for mutations in the BEST1 gene. RESULTS: Five patients of the same Italian family were clinically examined. All patients complained of decreased vision as the initial symptom. Best-corrected visual acuity ranged from 20/800 to 20/32. On fundus examination, all patients showed atypical Best vitelliform macular dystrophy phenotype with multifocal macular and extramacular involvement. The spectral domain optical coherence tomography characteristics of central macular and extramacular lesions varied in each patient and included "giant" choroidal excavation, extensive flat macular elevation with hyporeflective subretinal material accumulation surrounded by hyperautofluorescent spots/annulus, and extensive hypoautofluorescent extramacular atrophic areas. Electrooculogram was always abnormal with Arden ratio lower than 1.55, whereas electroretinogram was normal in the two younger patients and abnormal (low amplitude) in the three older patients. Genetic analysis revealed a novel missense mutation in BEST1, substituting aspartate for glycine at amino acid 304. CONCLUSION: We describe the atypical phenotype and high intrafamilial variability associated with a new mutation in the BEST1 gene in an Italian family affected with Best vitelliform macular dystrophy. Clinicians should consider screening the BEST1 gene even in the absence of the typical phenotype and in case of high intrafamilial variability.

CHOROIDAL NEOVASCULARIZATION IN CAUCASIAN PATIENTS WITH LONGSTANDING CENTRAL SEROUS CHORIORETINOPATHY.

PURPOSE: To report the frequency of choroidal neovascularization (CNV) in Caucasian patients with chronic central serous chorioretinopathy (CSC). METHODS: Retrospective consecutive series of 272 eyes (136 patients) who were diagnosed as having chronic CSC based on clinical and multimodal fundus imaging findings and documented disease activity for at least 6 months. The CNVs were mainly determined by indocyanine-green angiography. RESULTS: Patients were evaluated and followed for a maximum of 6 years, with an average follow-up of 14 ± 12 months. Distinct CNV was identified in 41 eyes (34 patients). Based on fluorescein angiography, 37 eyes showed occult with no classic CNV, 3 eyes showed predominantly classic and 1 eye had a disciform CNV. Furthermore, indocyanine-green angiography revealed polypoidal choroidal vasculopathy lesions, in 27 of the 37 eyes, classified as occult CNV on fluorescein angiography. In total, 17.6% of our patients with chronic CSC were found to have CNV that upon indocyanine-green angiography were recognized as being polypoidal choroidal vasculopathy. CONCLUSION: In our series of Caucasian patients, we found a significant correlation between chronic CSC and CNV, in which the majority of patients with CNV were found to have polypoidal choroidal vasculopathy. Our findings suggest that indocyanine-green angiography is an indispensable tool in the investigation of chronic CSC.

Cognitive Impairment and Age-Related Vision Disorders: Their Possible Relationship and the Evaluation of the Use of Aspirin and Statins in a 65 Years-and-Over Sardinian Population.

Neurological disorders (Alzheimer's disease, vascular and mixed dementia) and visual loss (cataract, age-related macular degeneration, glaucoma, and diabetic retinopathy) are among the most common conditions that afflict people of at least 65 years of age. An increasing body of evidence is emerging, which demonstrates that memory and vision impairment are closely, significantly, and positively linked and that statins and aspirin may lessen the risk of developing age-related visual and neurological problems. However, clinical studies have produced contradictory results. Thus, the intent of the present study was to reliably establish whether a relationship exist between various types of dementia and age-related vision disorders, and to establish whether statins and aspirin may or may not have beneficial effects on these two types of disorders. We found that participants with dementia and/or vision problems were more likely to be depressed and displayed worse functional ability in basic and instrumental activities of daily living than controls. Mini mental state examination scores were significantly lower in patients with vision disorders compared to subjects without vision disorders. A closer association with macular degeneration was found in subjects with Alzheimer's disease than in subjects without dementia or with vascular dementia, mixed dementia, or other types of age-related vision disorders. When we considered the associations between different types of dementia and vision disorders and the use of statins and aspirin, we found a significant positive association between Alzheimer's disease and statins on their own or in combination with aspirin, indicating that these two drugs do not appear to reduce the risk of Alzheimer's disease or improve its clinical evolution and may, on the contrary, favor its development. No significant association in statin use alone, aspirin use alone, or the combination of these was found in subjects without vision disorders but with dementia, and, similarly, none in subjects with vision disorders but without dementia. Overall, these results confirm the general impression so far; namely, that macular degeneration may contribute to cognitive disorders (Alzheimer's disease in particular). In addition, they also suggest that, while statin and aspirin use may undoubtedly have some protective effects, they do not appear to be magic pills against the development of cognitive impairment or vision disorders in the elderly.

Influence of macular choroidal thickness on visual function in highly myopic eyes.

PURPOSE: To explore the retinal and choroidal thicknesses (RT, CT), as measured using enhanced depth imaging (EDI) optical coherence tomography (OCT, Spectralis) in highly myopic eyes and its relationship with visual function. MATERIALS AND METHODS: Prospective, case-control, noninterventional clinical study. CT was measured by EDI-OCT in highly myopic eyes (≥6 dpt) without any macular diseases and age-matched control eyes. A complete ophthalmological examination, visual acuity assessment and MP1 microperimetry were obtained. RESULTS: 38 myopic (15 M/23 F, mean age 51 ± 8.9 years) and 21 control eyes (5 M/16 F, mean age 50 ± 5.4 years) were included. The myopic mean refractive error was -13.3 ± 4.9 dpt and axial length 29.2 ± 2.2 mm. The mean best-corrected visual acuity (BCVA) was lower in highly myopic than in control eyes (77.3 ± 9.25 vs. 84.8 ± 0.6 letters, p = 0.0001, respectively) as was the mean retinal sensitivity (MRS; 16.32 ± 2.6 vs. 19.9 ± 0.2 dB, p < 0.0001). While RT was similar between groups (291.5 ± 24.2 vs. 283.6 ± 13.9 µm, p = 0.06, respectively), subfoveal CT was thinner in highly myopic compared to control eyes (114.3 ± 78.5 vs. 272.6 ± 110.2 µm, p < 0.0001). A significant relationship was found between subfoveal CT and MRS (R(2) = 0.22; p = 0.003) and BCVA (R(2) = 0.13; p = 0.027). CONCLUSIONS: Macular function is reduced in highly myopic eyes without any visible macular diseases compared to controls, and a significant proportion of the macular function variability seems to be related to a reduced CT.

Age-related macular degeneration and cognitive impairment show similarities in changes of neutral lipids in peripheral blood mononuclear cells.

Starting from previous studies showing that patients with cognitive deficit present neutral lipids (NLs) accumulation in cytoplasm of their peripheral blood mononuclear cells (PBMCs) and considering that there is epidemiological evidence linking age-related macular degeneration (AMD) to cognitive deficit, the first purpose of this study was to test whether neutral lipids also accumulated in PBMCs from AMD subjects. Moreover, the impact of statin use on AMD was explored and whether such use in AMD subjects was associated with NLs accumulation in PBMCs. The study was conducted on 222 subjects: 136 AMD (36 of which - 26.5% - using statins], 48 cognitive deficit (20 of which - 41.7% - using statins) and 38 healthy controls (4 of which -10.1% - using statins), AMD lesions were assessed from color fundus photographs. Mini-mental state examination (MMSE), demographics, lifestyle factors and medical history were collected at interview. MMSE score was categorized as normal (24-30), and impaired (<24), NLs content was evaluated by oil red 0 (ORO) staining method. ORO determination showed that neutral lipids were generally absent or very low (score between 0 and 1) in healthy controls while most of PBMCs from cognitive deficit and AMD had ORO staining levels scoring 2-4. Post hoc analysis (Bonferroni) in a one-way ANOVA revealed that ORO score was significantly higher in cognitive deficit and AMD subjects compared to healthy controls and in cognitive deficit compared to AMD. Bonferroni-test also showed that AMD subjects had significantly lower total cholesterol (TC) levels compared to healthy controls while high density lipoprotein-cholesterol (HDL-C) did not reach statistical significance. The results also revealed a significant higher number of statin-users in AMD compared to healthy controls. Likewise when cognitive deficit vs healthy controls was analyzed, the number of statin users were found to be significant higher in cognitive deficit than in healthy controls. There were no significant differences in statin use between AMD and cognitive deficit. Compared to healthy controls, statin use in cognitive deficit and AMD groups was significantly associated with ORO scores of 2-4. This data supports the hypothesis that AMD and cognitive deficit share similar complex pathophysiology and risk factors including NLs accumulation in their PBMCs, although this does not necessarily imply that one disease causes the other. In addition, they provide further evidence that statin use may increase the risk of AMD.