Multimodal imaging of multifocal chorioretinitis secondary to endogenous candida infection.

PURPOSE: To present multimodal imaging of multifocal chorioretinitis secondary to endogenous candida infection in a young adult. METHODS: A 49-year-old woman who presented for evaluation of bilateral endogenous candida chorioretinitis underwent complete ophthalmic examination, in addition to fundus photography (FP), enhanced depth imaging optical coherence tomography, fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA) and optical coherence tomography angiography (OCTA). RESULTS: Multimodal imaging of both eyes of the patient affected by endogenous candida chorioretinitis was performed. FP showed multiple white chorioretinal lesions at the posterior pole, FAF showed dark dot at the posterior pole surrounded by hyperautofluorescence area, FA showed early hyperfluorescence round perifoveal lesion at the posterior pole and small hyperfluorescence dots under the inferior retinal vessels. Early ICGA showed hypofluorescence dots at the posterior pole. Late ICGA showed dark hypofluorescence dots at the posterior pole surrounded by faint hyperautofluorescent ring. OCTA showed dark areas corresponded to hypoperfusion areas seen with early ICGA. CONCLUSION: We reported multimodal imaging of an unusual occurrence of multifocal chorioretinitis due to immunosuppression. These findings suggested that the infection resulted from choroidal infiltration via the short posterior ciliary arteries with resultant breakthrough into the retina, rather than via the central retinal artery. By comparing findings on OCTA with data obtained from traditional systems, we are gaining essential information on the pathogenesis of endogenous candida chorioretinitis.

The overlapping spectrum of flat irregular pigment epithelial detachment investigated by optical coherence tomography angiography.

PURPOSE: To describe the OCT angiography characteristics of flat irregular pigment epithelial detachments (PEDs), an overlapping tomographic feature of various macular disorders. METHODS: Consecutive patients with a dimpled retinal pigment epithelium profile on OCT, referred for a second opinion, were enrolled. Fluorescein (FA) and indocyanine green angiography (ICGA) were performed in all patients and compared to previous examinations, when available. In all patients, enhanced depth imaging-OCT and OCT angiography were performed upon referral and at subsequent follow-ups. RESULTS: Twenty-five eyes from 25 patients (9 women and 16 men, mean age of 63.4 years) were enrolled. The diagnoses of CSCR were already established by the referring physicians in 10 patients, acute in 15% and chronic in 26%; 48% of patients were referred with a diagnosis of type 1 CNV, and 11% of CSCR complicated by CNV. After performing OCT angiography, 2 masked examiner identified 7 type 1 CNV (29%), 18 eyes with pachychoroid disease of which 31% pachychoroid pigment epitheliopathies (PPEs) at baseline evolved to CSCR, 22% PPE at baseline evolved to pachychoroid neovasculopathy, 18% pachychoroid neovasculopathy stable. CONCLUSION: Central serous chorioretinopathy, type 1 CNV, and the pachychoroid spectrum of diseases cause abnormalities in the choroidal circulation that make the overlying RPE dysfunctional, resulting in flat irregular PED. Discrimination between avascular and vascular flat irregular PEDs is crucial for a good visual outcome, but since chronic alterations of the RPE can compromise the diagnostic specificity of FA and ICGA, OCT angiography may become a fundamental tool to differentiate these clinical entities.

Bilateral multiple evanescent white dot syndrome.

PURPOSE: To present a single case of bilateral multiple evanescent white dot syndrome (MEWDS). METHODS: A single case with three months of follow-up using imaging studies including fundus color photography (FP), fluorescein angiography (FA), indocyanine green angiography (ICGA), fundus autofluorescence (FAF), spectral-domain optical coherence tomography (SD-OCT), en face SD-OCT and optical coherence tomography angiography (OCTA) is presented. RESULTS: The patient presented with bilateral MEWDS, ultimately with complete resolution of symptoms. FP revealed foveal granularity and white punctate deep retinal spots, FA found early wreath-like hyperfluorescence, while ICGA showed hypofluorescent dots and spots in the early and late stages. FAF showed areas of hyperautofluorescence. SD-OCT revealed disruption of the ellipsoid zone (EZ) and accumulation of hyperreflective material of variable size and shape. En face SD-OCT demonstrated hyporeflective areas corresponding to areas of EZ disruption as well as hyperreflective dots in the outer nuclear layer. OCTA showed areas of photoreceptor slab black-out corresponding to areas of EZ disruption and light areas of flow void or flow disturbance in the choriocapillaris slab. CONCLUSIONS: This case represents an unusual case of bilateral MEWDS with complete resolution within three months.

Two cases of primary vitreoretinal lymphoma: a diagnostic challenge : The supporting role of multimodal imaging in the diagnosis of primary vitreoretinal lymphoma.

PURPOSE: To report two cases of primary vitreoretinal lymphoma (PVRL), which presented as intermediate and posterior uveitis. METHODS: Combined clinical assessment, multimodal imaging with spectral-domain optical coherence tomography, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, brain magnetic resonance imaging and vitreous and retinal biopsy. Case 1 was a 48-year-old woman who complained of visual loss in her right eye secondary to a diffuse vitreous opacification and multiple chorioretinal lesions. Case 2, a 74-year-old man, presented with low vision in his right eye due to a wide chorioretinal lesion at the posterior pole, vitreous opacification and posterior uveitis in both eyes. RESULTS: Diffuse large B cell lymphoma was histologically diagnosed in the cerebellum in the first case and in chorioretinal tissue in the second patient. Atypical lymphoid cells were detected and allowed to make a diagnosis of primary central nervous system lymphoma in case 1 and PVRL in case 2. CONCLUSION: PVRL often masquerades ad intermediate or posterior uveitis. The management of the patients needed a team of pathologists, haematologists and ophthalmologists to achieve the correct diagnosis and choose the more appropriate therapy. Some peculiar characteristics on multimodal imaging, even in atypical cases of PVRL, should raise suspicious for PVRL and lead to a diagnostic vitrectomy and/or retinal biopsy.

Optical coherence tomography angiography in acute arteritic and non-arteritic anterior ischemic optic neuropathy.

PURPOSE: The purpose of our study was to describe the feature of acute non-arteritic or arteritic anterior ischemic optic neuropathy (NA-AION and A-AION) using optical coherence tomography angiography (OCT-A) and to compare it with fluorescein angiography (FA) and indocyanine green angiography (ICGA). METHODS: In this retrospective, observational case-control study four NA-AION patients and one A-AION patient were examined by FA, ICGA and OCT-A within 2 weeks from disease presentation. The characteristics of the images were analyzed. Optic nerve head (ONH) and radial peripapillary capillaries (RPC) vessel densities (VDs) were compared between NA-AION and controls. RESULTS: In two of four NA-AION cases and in the A-AION patient, OCT-A clearly identified the boundary of the ischemic area at the level of the optic nerve head, which was comparable to optic disc filling defects detected by FA. In the other two NA-AION cases, a generalized leakage from the disc was visible with FA, yet OCT-A still demonstrated sectorial peripapillary capillary network reduction. Both ONH and RPC VDs were reduced in NA-AION patients, when compared to controls. CONCLUSIONS: OCT-A was able to identify microvascular defects and VD reduction in cases of acute optic disc edema due to NA-AION and A-AION. OCT-A provides additional information in ischemic conditions of the optic nerve head.

Spectral-domain optical coherence tomography findings in pediatric tilted disc syndrome.

PURPOSE: To report a novel spectral-domain optical coherence tomography (SD-OCT) finding in children affected by tilted disc syndrome (TDS), and to correlate it with early visual field defects. METHODS: Patients between 5 and 17 years old with TDS were enrolled in this study. The diagnosis of TDS was made by stereoscopic fundus photography, when the upper edge of the optic disc protruded anteriorly relative to its lower edge. All eyes were examined with 12 radial SD-OCT B-scans of 12 mm centered on the optic disc; the fundus area encompassing the optic nerve was additionally scanned using several vertical and horizontal scans.. C-scan SD-OCT were acquired using the Macular Cube 512 x 128 to create the en face image. Standard automated perimetry 24-2 tests were performed on all patients. RESULTS: Thirty-eight eyes of 20 pediatric patients with TDS syndrome were enrolled during this 24-months clinical trial. Their mean age was 10.9 ± 2.7 years (range 7-15 years), 12 (60%) were male and eight (40%) were female. The OCT images of the optic discs showed a protrusion of the upper edge of Bruch's membrane and choroid at the nasal edge of the optic disc in 39.5% of the eyes. The retinal nerve fiber tissue appeared to be herniated into this protrusion and bent superiorly in 15 eyes. This severe bending corresponded to early visual field anomalies that were not reduced by corrective lenses in 46.7% of the eyes. CONCLUSION: Visual field defects that do not improve by increased myopic correction in TDS may be due to the severe bending of the retinal nerve fiber tissue, which would impair axonal flow.

Retinal nerve fiber layer thickness modification after internal limiting membrane peeling.

PURPOSE: To identify early and late retinal nerve fiber layer thickness (RNFLT) modification after internal limiting membrane peeling for idiopathic macular hole or epiretinal membrane and to correlate RNFLT to visual field indices. METHODS: Single-center, prospective, interventional consecutive case series. Complete ophthalmic examination, fundus images, and spectral domain optical coherence tomography were performed in 30 eyes of 30 patients before and 1, 3, and 6 months after surgery. Six peripapillary sectors (superotemporal, temporal, inferotemporal, inferonasal, nasal, superonasal) and global RNFLT were evaluated. Visual field was performed preoperatively and 6 months postoperatively. RESULTS: Significant RNFLT modification was found after surgery (P < 0.0001). Specifically, RNFLT significantly increased in all, but the temporal sectors, 1 month after surgery, and it returned to preoperative values at the third month. Six months after surgery, RNFLT was lower than basal values in the superotemporal, inferotemporal, and temporal sectors (P < 0.001, P < 0.05, and P < 0.001, respectively) with an average reduction of 18.2 ± 9.8 µm. No correlation was found between RNFLT and the visual field indices. CONCLUSION: The diffuse RNFLT increase 1 month postoperatively could be because of inflammatory responses. The reduction of RNFLT in the temporal sectors 6 months postoperatively could indicate damage to the macular retinal nerve fiber layer caused by internal limiting membrane peeling.

Spectral domain optical coherence tomography findings in patients with acute syphilitic posterior placoid chorioretinopathy.

PURPOSE: To describe the appearance of acute syphilitic posterior placoid chorioretinitis, a rare ocular manifestation of syphilis, on spectral domain optical coherence tomography (SD OCT) both before and after treatment. METHODS: Ophthalmic examination and imaging studies of 30 eyes of 19 confirmed cases were analyzed both at the time of presentation and at each follow-up visit. Patients with SD OCT and fluorescein angiography at the time of presentation, and at least three documented follow-up visits after initiation of therapy, were included in the study. Standard treatment of neurosyphilis was given to each patient, including 4 million units of penicillin G administered intravenously every 4 hours for 14 days. RESULTS: Fundus examination and imaging studies were consistent with previous reports and confirmed the diagnosis of acute syphilitic posterior placoid chorioretinitis. In 13 eyes (43.3%), baseline SD OCT scans were performed within 1 to 2 days of presentation and revealed a small amount of subretinal fluid, disruption of the inner segment/outer segment junction, and hyperreflective thickening of the retinal pigment epithelium (RPE). All 30 eyes were again scanned between Days 7 and 9 after presentation and revealed loss of the inner segment/outer segment and OS/RPE bands, and irregular hyperreflectivity of the RPE with prominent nodular elevations but without subretinal fluid. Early disruption of the external limiting membrane and punctate choroidal hyperreflectivity were seen in 1 of the 30 eyes (3.3%) and 14 of the 30 eyes (46.6%), respectively. Vision improved and the outer retinal abnormalities normalized in 28 of the 30 eyes (93.3%) after the treatment of neurosyphilis. The external limiting membrane, inner segment/outer segment band, and/or linear outer segment/RPE junction remained substantially abnormal despite treatment in 2 eyes left with 20/200 vision. CONCLUSION: Patients with acute syphilitic posterior placoid chorioretinitis show characteristic outer retinal abnormalities on SD OCT imaging, including disruption of the inner segment/outer segment band, nodular thickening of the RPE with loss of the linear outer segment/RPE junction, and, in some cases, loss of the external limiting membrane, accumulation of subretinal fluid, and punctate hyperreflectivity in the choroid. Vision improved and these abnormalities reversed after treatment of neurosyphilis in most of the patients. Persistently, poor vision despite treatment was associated with long-term loss or disruption of outer retinal anatomy on SD OCT.

Vitreous hemorrhage secondary to retinal vasculopathy in a patient with dyskeratosis congenita.

The aim of this study was to report a case of vitreous hemorrhage secondary to retinal vasculitis in a patient with dyskeratosis congenita. A 16-year-old white male was referred to the Ophthalmology Clinic due to deterioration of vision in his left eye. Medical history was significant for dyskeratosis congenita associated with thrombocytopenia. General physical examination revealed reticular pigmentation on the upper half of the chest, vertical ridges and splitting of finger nails, and oral mucosal leukoplakia. Ophthalmological examination of the anterior segment was unremarkable. Retinal examination revealed vitreous hemorrhage in the left eye veiling the retinal details. A possible history of trauma was denied. Fundus examination of the right eye showed retinal vascular sheathing with a few dot and blot retinal hemorrhages. Fluorescein angiography revealed extensive areas of non-perfusion beyond the equator in the right eye, later treated with scatter laser photocoagulation. We performed a 23-gauge vitrectomy with endolaser treatment of the new vascularization areas in the left eye. After 6 months, best-corrected visual acuity in the right and left eye was 20/20 and 20/25, respectively. Rather than being confined to anterior segment abnormalities like conjunctivitis, blepharitis and nasolacrimal duct obstruction which are reported in the literature, dyskeratosis congenita can cause significant visual loss due to retinal vasculitis and vitreous hemorrhage. Therefore physicians and ophthalmologists should be aware of this possibility and prompt diagnosis and treatment could prevent further visual loss in such patients.

Early and late inner retinal changes after inner limiting membrane peeling.

Pars plana vitrectomy and inner limiting membrane (ILM) peeling are standard procedures for macular hole and epiretinal membrane surgery. However, ILM peeling is known to cause mechanical traumatic changes to the retinal nerve fiber layer. Recently there have been numerous reports of anatomical changes in the macula after ILM removal. A comprehensive review of the literature. The earliest change in the macula after ILM peeling is post-operative swelling of the arcuate retinal nerve fiber layer (SANFL), which disappears within the 3 month; the swelling is not detected on biomicroscopic fundus examination but appears as hypoautofluorescent arcuate striae in the macular region on infrared and autofluorescence imaging, with corresponding hyperreflectant swelling demonstrated on spectral-domain optical coherence tomography (OCT). SANFL is followed by dissociated optic nerve fiber layer defect, faintly visible on fundus examination and corresponding on OCT to "dimples" in the inner retinal layers. The en face tomographic aspect of this defect appears as concentric macular dark spots. Post-operative foveal displacement toward the optic disc might be responsible for the stretching and thinning of the retinal parenchyma in the temporal subfield and the thickening of the nasal macula. This shortening of the papillofoveal distance after surgery is probably secondary to axonal transport and contractility alterations in the nerve fiber layer, which might also account for apoptotic and atrophic degeneration of the peripapillary retinal nerve fiber layer. Ganglion cells do not seem to be affected by ILM peeling, even if the ganglion cell complex loses some volume because of trauma to the Müller cells contained in the ganglion cell layer. Despite its clear indication in macular hole and epiretinal membrane surgery, ILM peeling is a traumatic procedure that has acute effects on the underlying inner retinal layers. Further investigation of these subclinical changes may assist in aiding the development of minimally traumatic techniques for ILM removal.

Swelling of the arcuate nerve fiber layer after internal limiting membrane peeling.

PURPOSE: The purpose of the study is to report the incidence of changes of the retinal nerve fiber layer in the early postoperative period after internal limiting membrane peeling for idiopathic macular hole and epiretinal membrane surgery. METHODS: Interventional, noncomparative retrospective case series. Fifty-six eyes of 55 patients with an epiretinal membrane and 33 eyes of 31 patients with macular hole underwent pars plana vitrectomy and internal limiting membrane peeling. All patients received a complete ophthalmic examination, infrared and autofluorescence photography, and spectral-domain optical coherence tomography preoperatively and also at approximately 1 week, 1 month, and 3 months postoperatively. Vitrectomy and gas tamponade were performed with internal limiting membrane peeling after staining with Brilliant Peel. The main outcome measures were the presence of postoperative swelling of the arcuate retinal nerve fiber layer on spectral-domain optical coherence tomography, infrared and autofluorescence photographs, and its effect on best-corrected visual acuity. RESULTS: On infrared and autofluorescence photographs, 28 (31.46%) of 89 eyes with internal limiting membrane peeling exhibited swelling of the arcuate retinal nerve fiber layer 1 week to 1 month postoperatively. Swelling of the arcuate retinal nerve fiber layer increased during the first month after surgery and resolved after a mean period of 2 months. These features were best visualized by autofluorescence imaging, as three to five dark striae originating from the optic nerve head, radiating in an arcuate pattern toward the macula. Simultaneous spectral-domain optical coherence tomography scanning through the striae indicated that they correspond to areas of focal swelling of the arcuate nerve fiber layer. No significant differences were found in eyes with or without swelling of the arcuate retinal nerve fiber layer for mean age or pre- and postoperative best-corrected visual acuity (P > 0.05). CONCLUSION: Swelling of the arcuate retinal nerve fiber layer often occurs after internal limiting membrane peeling for macular hole and epiretinal membrane. It is a transient feature after surgery that does not affect visual recovery. It is best visualized using infrared and autofluorescence imaging. Simultaneous infrared and spectral-domain optical coherence tomography imaging after macular surgery can detect transient inner retinal changes, which are not visible on clinical examination.

Standard enucleation with aluminium oxide implant (bioceramic) covered with patient's sclera.

PURPOSE: We describe in our study a modified standard enucleation, using sclera harvested from the enucleated eye to cover the prosthesis in order to insert a large porous implant and to reduce postoperative complication rates in a phthisis globe. METHODS: We perform initially a standard enucleation. The porous implant (Bioceramic) is then covered only partially by the patient's sclera. The implant is inserted in the posterior Tenon's space with the scleral covering looking at front. All patients were followed at least for twelve months (average followup 16 months). RESULTS: We performed nineteen primary procedures (19 patients, 19 eyes, x M; x F) and secondary, to fill the orbital cavity in patients already operated by standard evisceration (7 patients, 7 eyes). There were no cases of implant extrusion. The orbital volume was well reintegrated. CONCLUSION: Our procedure was safe and effective. All patients had a good cosmetic result after final prosthetic fitting and we also achieved good prothesis mobility.

Multimodal ophthalmic imaging of staphylococcus aureus bacteremia associated with chorioretinitis, endocarditis, and multifocal brain abscesses.

PURPOSE: Staphylococcus aureus bacteriemia (SAB) as critical condition for the life and occasionally involves the eyes. The aim of this report is to describe the ocular involvement with multimodal imaging. OBSERVATIONS: A patient admitted for evaluation of acute onset of confusion, disorientation, and generalized malaise and found to have methicillin-resistant staphylococcus aureus (MRSA)-associated endocarditis and multifocal brain abscesses was evaluated by the ophthalmology service. The patient's visual acuity was 20/20 OU without relative afferent pupillary defect and normal intraocular pressures. Bedside anterior segment examination was normal. Posterior segment examination revealed intraretinal hemorrhages and Roth spots in the posterior pole of the right eye, and two deep well-defined focal white chorioretinal infiltrates and a hemorrhagic pigment epithelium detachment in the temporal quadrant of the left eye. Multimodal imaging was utilized to document these findings and ensure adequate antibiotic therapy. CONCLUSION: SAB has the potential for poor visual outcomes as well as significant morbidity and mortality. Multimodal imaging of SAB-related chorioretinitis allows for accurate diagnosis as well as assessment of response to antimicrobial therapy.

Effect of basic fibroblast growth factor in transgenic mice: corneal epithelial healing process after excimer laser photoablation.

PURPOSE: To evaluate the role of preparedbasic fibroblast growth factor (bFGF) eyedrops in corneal epithelial healing of transgenic mice after excimer laser photoablation. MATERIALS AND METHODS: In our prospective case series, 60 eyes of 30 mice had uneventful bilateral excimer laser photoablation. In each mouse, 1 eye received the standard topical postoperative therapy with tobramicin, diclofenac and dexamethasone eyedrops, plusbFGF eyedrops 5 microg/10 microl PBS 3 times a day for 1 week, or until corneal reepithelialization was complete. The fellow eye served as the control and received the standard postoperative therapy plus placebo. The mice were monitored daily, commencing the day after surgery and for 7 days, in order to evaluate the corneal reepithelialization rate by using a video slitlamp camera with a cobalt blue light. The mean diameter of the corneal wounds was measured. Videotaped images were recorded and analyzed by computer planimetry. RESULTS: All the eyes treated withbFGF eyedrops healed completely before day 5 after surgery, with a mean reepithelialization time of 90 +/- 12 h (standard deviation); the mean reepithelialization time was 124 +/- 10 h in those eyes receiving placebo. There were no statistically significant differences between the 2 groups in corneal haze presentation during the follow-up, perhaps because the time period was too brief (7 days). However, corneal clarity on slitlamp biomicroscopy was greater in the study group than in the control group. No side effects or toxic effects were documented. CONCLUSIONS: These data suggest that the bFGF significantly accelerates epithelial healing after excimer photoablation. A further clinical study should be performed to prove the results obtained in this study as well as the long-term efficacy of bFGF to prevent corneal haze.

MULTIMODAL IMAGING IN VORTEX VEIN VARICES.

PURPOSE: The aim of this study is to describe the clinical presentation of vortex vein varices with multimodal imaging. METHODS: The authors carried out a retrospective case series of eight patients (7 female, 1 male) with an average age of 60.2 years (min 8, max 84, median 68.5) presenting with vortex vein varices. All patients were evaluated at the Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy and at Luigi Sacco Hospital, University of Milan, Milan, Italy. Patients underwent complete ophthalmologic examinations, including best corrected visual acuity, intraocular pressure, anterior segment, and fundus examination. Imaging studies, including fundus color photography, near-infrared reflectance imaging, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, and spectral-domain enhanced depth imaging optical coherence tomography were also performed. Ultra-widefield fluorescein angiography and ultra-widefield indocyanine angiography using the Heidelberg Retina Angiograph and the Staurenghi 230 SLO Retina Lens were used to demonstrate the disappearance of all retinal lesions when pressure was applied to the globe. RESULTS: All eight cases initially presented to the emergency room. One patient presented secondary to trauma, two patients presented for suspected hemangioma, whereas the other five were referred to the authors' hospitals for suspected retinal lesions. On examination, retinal abnormalities were identified in all 8 patients, with 7 (87.5%) oculus dexter and 1 (12.5%) oculus sinister, and with 1 (12.5%) inferotemporally, 3 (37.5%) superonasally, 3 (37.5%) inferonasally, and 1 (12.5%) inferiorly. Fundus color photography showed an elevated lesion in seven patients and a nonelevated red lesion in one patient. In all patients, near-infrared reflectance imaging showed a hyporeflective lesion in the periphery of the retina. Fundus autofluorescence identified round hypofluorescent rings surrounding weakly hyperfluorescent lesions in all patients. On fluorescein angiography, all lesions were initially hyperfluorescent with a hypofluorescent ring, with the lesion becoming hyperfluorescent after injection of dye. Indocyanine green angiography demonstrated dilation of the vortex vein ampullae in all patients. Spectral-domain enhanced depth imaging optical coherence tomography demonstrated dilated choroidal vessels and a hyporeflective cavity without subretinal fluid in all patients. Ultra-widefield fluorescein angiography and ultra-widefield indocyanine angiography demonstrated disappearance of all retinal lesions when pressure was applied to the globe. Findings are consistent with the diagnosis of vortex vein varix in all eight patients, with six patients (75%) exhibiting a single varix and two patients (25%) exhibiting a double varix. CONCLUSION: The diagnosis of vortex vein varices can be confirmed through clinical examination through the use of digital pressure to the globe during ophthalmoscopic examination. Adjunctive multimodal imaging (fundus color photography, near-infrared reflectance imaging, fundus autofluorescence, fluorescein angiography, indocyanine angiography, and spectral-domain enhanced depth imaging optical coherence tomography) was useful in the diagnosis of vortex vein varices in the authors' clinical cases. However, in more challenging clinical cases, the authors' novel use of the ultra-widefield contact lens for application of ocular pressure with a resulting resolution of the varix proved to be a useful and easy diagnostic imaging method for confirming the presence of vortex vein varices.

Congenital Retinal Macrovessel and the Association of Retinal Venous Malformations With Venous Malformations of the Brain.

Importance: Congenital retinal macrovessel (CRM) is a rarely reported venous malformation of the retina that is associated with venous anomalies of the brain. Objective: To study the multimodal imaging findings of a series of eyes with congenital retinal macrovessel and describe the systemic associations. Design, Setting, and Participants: In this cross-sectional multicenter study, medical records were retrospectively reviewed from 7 different retina clinics worldwide over a 10-year period (2007-2017). Patients with CRM, defined as an abnormal, large, macular vessel with a vascular distribution above and below the horizontal raphe, were identified. Data were analyzed from December 2016 to August 2017. Main Outcomes and Measures: Clinical information and multimodal retinal imaging findings were collected and studied. Pertinent systemic information, including brain magnetic resonance imaging findings, was also noted if available. Results: Of the 49 included patients, 32 (65%) were female, and the mean (SD) age at onset was 44.0 (20.9) years. A total of 49 eyes from 49 patients were studied. Macrovessel was unilateral in all patients. Color fundus photography illustrated a large aberrant dilated and tortuous retinal vein in all patients. Early-phase frames of fluorescein angiography further confirmed the venous nature of the macrovessel in 40 of 40 eyes. Optical coherence tomography angiography, available in 17 eyes (35%), displayed microvascular capillary abnormalities around the CRM, which were more evident in the deep capillary plexus. Of the 49 patients with CRM, 39 (80%) did not illustrate any evidence of ophthalmic complications. Ten patients (20%) presented with retinal complications, typically an incidental association with CRM. Twelve patients (24%) were noted to have venous malformations of the brain with associated magnetic resonance imaging. Of these, location of the venous anomaly in the brain was ipsilateral to the CRM in 10 patients (83%) and contralateral in 2 patients (17%), mainly located in the frontal lobe in 9 patients (75%). Conclusions and Relevance: Our study has identified an association between macrovessels in the retina and venous anomalies of the brain (24% compared with 0.2% to 6.0% in the normal population). Thus, we recommend new guidelines for the systemic workup of patients with CRM to include brain magnetic resonance imaging with contrast. These lesions may be more accurately referred to as retinal venous malformations, which may raise awareness regarding potential cerebral associations.

Treatment of cystoid macular edema in retinitis pigmentosa with intravitreal triamcinolone.

OBJECTIVE: To evaluate the results of treatment with intravitreal triamcinolone acetonide injection in patients with cystoid macular edema secondary to retinitis pigmentosa. METHODS: This prospective, nonrandomized comparative trial included 20 eyes of 20 patients with cystoid macular edema secondary to retinitis pigmentosa (group A) and 20 eyes of 20 control individuals (group B) with the same characteristics who declined treatment. All treated eyes received an intravitreal injection of 0.1 mL of triamcinolone acetonide (4 mg). The total follow-up was 12 months. The main outcome measures were best-corrected visual acuity, central macular thickness measured by optical coherence tomography, and intraocular pressure. RESULTS: No statistically significant changes were observed in best-corrected visual acuity. Central macular thickness showed statistical differences between the 2 groups. Intraocular pressure showed a statistically significant increase after the first day, at 1 month, and at 3 months in both groups but no significant increase afterward. CONCLUSIONS: Intravitreal triamcinolone administration may be useful for select cases of cystoid macular edema in patients with retinitis pigmentosa but its efficacy seems to be limited over time. Therefore, to obtain a good anatomical result and an improvement of best-corrected visual acuity, further treatment would be necessary after 6 months.