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To investigate ocular anomalies in joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type (JHS/EDS-HT).Prospective, cross-sectional study.Forty-four eyes of 22 consecutive patients with an established diagnosis of JHS/EDS-HT and 44 eyes of 22 age- and gender-matched control subjects. Administration of a standardized questionnaire (Ocular Surface Disease Index) and a complete ophthalmologic examination, including assessment of best-corrected visual acuity, slit-lamp biomicroscopy, intraocular pressure measurement, indirect ophthalmoscopy, tear-film break-up time, Schirmer I testing, axial length and anterior chamber depth measurement, corneal topography, corneal pachymetry, and confocal microscopy. Main outcome measures included comparing ocular anomalies in JHS/EDS-HT and control eyes.JHS/EDS-HT patients reported dry eye symptoms more commonly than controls (P.0001). Scores of tear-film break-up time and Schirmer I test were significantly lower in JHS/EDS-HT eyes (P.0001). Minor lens opacities were significantly more common in the JHS/EDS-HT group (13.6%; P.05). Pathologic myopia with abnormal vitreous was found in 7 JHS/EDS-HT eyes (15.9%) and 0 controls (P = .01). Corneas were significantly steeper and the best-fit sphere index was significantly higher in JHS/EDS-HT group (P.01). By confocal microscopy, the JHS/EDS-HT group showed lower density of cells in the superficial epithelium (P.001) and higher density of stromal keratocytes in anterior and posterior stroma (P.0001).The most consistent association of eye anomalies in the JHS/EDS-HT group included xerophthalmia, steeper corneas, pathologic myopia, and vitreous abnormalities, as well as a higher rate of minor lens opacities. These findings indicate the need for ophthalmologic survey in the assessment and management of patients with JHS/EDS-HT.

To correlate the postoperative visual outcome with the spectral-domain optical coherence tomography (SD-OCT) findings in the fovea after successful rhegmatogenous retinal detachment repair. Cross-sectional, observational study.Thirty-five patients with preoperative macula-on rhegmatogenous retinal detachment (12 eyes) and macula-off rhegmatogenous retinal detachment (23 eyes) who underwent scleral buckling surgery for primary rhegmatogenous retinal detachment were recruited. Early Treatment Diabetic Retinopathy Study best-corrected visual acuity measurement, microperimetry, and SD-OCT examination were performed on the same day. Foveal center retinal thickness, central 1-mm subfield thickness, and outer nuclear layer thickness were measured using SD-OCT. The presence or absence of epiretinal membrane, intraretinal fluid, and subretinal fluid was assessed. The status of the external limiting membrane, inner/outer segment junction, and intermediate line was also evaluated and judged as disrupted or complete. The correlations between SD-OCT findings and either postoperative best-corrected visual acuity or retinal sensitivities for central 12° were analyzed.The outer nuclear layer thickness was the only significant SD-OCT retinal measurement strongly correlated with both postoperative best-corrected visual acuity (r = 0.61; P0.001) and retinal sensitivities for central 12° (r = 0.53; P = 0.001). Among the SD-OCT imaging findings, status of the external limiting membrane, inner/outer segment junction, and intermediate line and the presence of intraretinal fluid showed a significantly high correlation either with best-corrected visual acuity outcome (r = -0.60; P0.001, r = -0.63; P0.001, r = -0.66; P0.001, and r = -0.50; P = 0.002, respectively) or with postoperative retinal sensitivities (r = -0.59; P0.001, r = -0.61; P0.001, r = -0.66; P0.001, r = -0.50; P = 0.002, respectively). Multivariate analysis showed that the outer nuclear layer thickness and the status of the intermediate line were the most important predictors of visual outcome (P0.001 and P0.001, respectively).This study showed that not only the status of the external limiting membrane and the inner/outer segment junction but also the integrity of the intermediate line and the outer nuclear layer thickness changes may be important predictors of postoperative visual outcome after anatomically successful rhegmatogenous retinal detachment repair.

Purpose To compare the short-term efficacy and safety of intravitreal ranibizumab versus bevacizumab in treating myopic choroidal neovascularization (CNV). Design Prospective, comparative, randomized, interventional study. Methods Thirty-two eyes from 32 patients with myopic CNV were consecutively enrolled and randomly treated, in a 1:1 ratio, with intravitreal ranibizumab (0.5 mg) or bevacizumab (1.25 mg) as needed, after the first injection. ETDRS best-corrected visual acuity (BCVA), foveal center thickness (FCT) on optical coherence tomography (OCT), and fluorescein angiographic findings were examined before and after treatment. Patients were followed up for 6 months. Results No statistically significant difference in the BCVA improvement, as well as in the FCT reduction, was found between groups during follow-up ( P value at 1, 3, 6 months > .05). Complete resolution of fluorescein leakage was observed in all 16 bevacizumab-treated eyes and in 15 out of 16 (93.7%) ranibizumab-treated eyes. No ocular or systemic adverse effects from treatment were encountered. Conclusion This randomized clinical study cannot determine a statistically significant difference in anti-VEGF treatment effect between ranibizumab and bevacizumab for the treatment of CNV secondary to pathologic myopia. A larger study is required to determine the relative efficacy and duration of action of these drugs.

Purpose The authors report the cases of two patients affected with idiopathic choroidal neovascularisation studied with combined fluorescein angiography and indocyanine green (ICG) angiography. In particular the presence of choroidal abnormalities at ICG angiography which could not be detected by fluorescein angiography was studied. Methods Both patients underwent a complete systemic and ocular assessment. Fluorescein angiography and ICG angiography were performed in a routine fashion at the time of presentation in both cases and after 14 months in the second patient. Results Results of the systemic investigations were unremarkable. A distinct dark rim surrounding the choroidal neovascular net was evident until the late phases of ICG angiography despite the presence of subretinal blood. Dilated choroidal vessels were observed beneath the neovascular membrane in both cases. In the first patient a hyperfluorescent area beyond the primary lesion was detected in the affected eye and a distinct leaking subfoveal choroidal venous vessel was found in the fellow eye. The second patient never showed other angiographic alterations either in the affected or in the fellow eye. Conclusions ICG angiography has proved to be useful, both to better define and follow up the true extent of the pigment halo (healing response) around the neovascular membrane when subretinal blood and dye leakage at fluorescein angiography prevent its full appreciation, and to rule out other causes of choroidal neovascularisation in young healthy adults associated with either choroidal inflammatory focal lesions or choroidal vascular dynamic or inflammatory alterations.

To evaluate the efficacy and safety of intravitreal bevacizumab for the treatment of myopic choroidal neovascularization (CNV).Prospective, non-randomized, interventional clinical study.Twenty eyes from 20 patients with CNV secondary to pathologic myopia participated in this study. These patients had already completed 12 months of follow-up. All patients were scheduled for 3 monthly intravitreal bevacizumab 1.25 mg injections. ETDRS best-corrected visual acuity (BCVA), foveal center thickness (FCT) on OCT and CNV size as assessed by fluorescein angiography were examined before and after treatment. Patients were followed up for 24 months.Mean BCVA (+/- SD) at baseline was 24.8 (+/- 11.86) letters (Snellen equivalent: 20/80). At 24 months after treatment the mean BCVA (+/- SD) improved significantly (p less than 0.05) to 44 (+/- 13.99) letters (Snellen equivalent: 20/33). At 24 month follow-up, BCVA improved of 10 letters or more in 17 (85%) out of 20 treated eyes and improved of 15 letters or more in 15 (75%) eyes. No treated eyes experienced a worsening of BCVA from baseline. Mean foveal center thickness (FCT) (+/- SD) at baseline was 223 (+/- 47,43) microns. By month 24, mean FCT (+/-SD) reduced to 190 (+/- 29.01) microns (p less than 0.05). Mean area of the CNVs at baseline was 0.77 (+/- 0.78) mm2, which decreased to 0.31 +/- (0.51) mm2 and 0.30 (+/- 0.50) mm2 at 12 (p less than 0.05) and 24 months (p less than 0.05), respectively. At 24 months follow-up absence of fluoresce in leakage from the CNV was demonstrated in 18 (90%) out of 20 treated eyes. No ocular or systemic adverse effects from treatment were encountered.Eyes with myopic CNV treated with intravitreal bevacizumab over 2 years had significant anatomic and functional improvement. Further studies will be needed to confirm the long-term efficacy and safety of this treatment.

We report an undocumented association of macula halo syndrome and mechanical ptosis secondary to non-pitting oedema in two members of an Italian family. Macula halo syndrome is universally recognised to be variant of Niemann-Pick disease type B, a lipid storage disorder which is ultimately diagnosed by measuring sphingomyelinase activity levels. Although the diagnosis could not be confirmed because of our patients' refusal to undergo enzymatic investigation, the clinical picture proved to be highly compatible with the suggested diagnosis. We discuss the possible clinical differential diagnosis of lipid storage diseases involving the retina.