Simple Vision Function Tests that Distinguish Eyes with Early to Intermediate Age-related Macular Degeneration.

PURPOSE: To present and compare baseline vision findings in eyes with early age-related macular degeneration (E-AMD), intermediate AMD (I-AMD), and age-similar participants with normal aging changes to the retina (No-AMD). METHODS: Two hundred and thirty-seven eyes of 125 individuals (66.4% female, mean age 75.3 years) were tested monocularly using several simple, rapid psychophysical tests: high contrast visual acuity, low contrast visual acuity at reduced luminance, contrast sensitivity, shape discrimination hyperacuity, colour vision, reading rate, and glare recovery. Retinal status was determined using colour fundus photographs that were graded according to the Beckman Initiative for Macular Research Classification Committee scale. Logistic regression analyses with generalized estimating equations were used to assess the association between each vision variable and AMD category, while taking into account the correlation between the two eyes. RESULTS: Three vision measures (contrast sensitivity [CS], shape discrimination hyperacuity [SDH], and colour discrimination [DesatCCS]) were significantly and independently associated with intermediate AMD. Relative Risk Ratios (RRR), 95% Confidence Intervals (in parentheses), beta coefficients, and significance (p) for the I-AMD vs. No-AMD model are: CS: RRR = 6.5 (1.91-22.0), beta = 1.87, p < .01; SDH: RRR = 2.34 (1.24-4.44), beta = 0.85, p < .001; DesatCCS: RRR = 1.43 (1.22-1.68), beta = 0.36, p < .001. Performance on these measures was significantly poorer for participants with I-AMD vs. No-AMD. CONCLUSIONS: Simple screening tests distinguish eyes with intermediate AMD from eyes with less severe AMD or normal aging changes. This suggests that these vision measures may be significant predictors of which participants will go on to develop advanced AMD.

RETINAL DYSTROPHY IN A PATIENT WITH McARDLE DISEASE.

PURPOSE: To report a case of pattern dystrophy in a patient with McArdle disease, a rare autosomal recessive disorder of glycogen metabolism. METHODS: Case report. RESULTS: A 29-year-old woman with a history of muscle biopsy-confirmed McArdle disease presented with fundus findings consistent with pattern dystrophy. Multimodal imaging, including optical coherence tomography and fundus autofluorescence, was performed. CONCLUSION: To our knowledge, this is the third reported case of pattern dystrophy in a patient with McArdle disease.

FINGERPRINT SIGN OF THE HENLE FIBER LAYER.

PURPOSE: To describe the appearance of concentric, fingerprint-like waves within the Henle fiber layer (HFL) using en face optical coherence tomography in patients with tractional pathologies of the retina. METHODS: Retrospective analysis of six eyes of six patients imaged by optical coherence tomography with volumetric slabs positioned at the level of the HFL. RESULTS: Optical coherence tomography data from six patients with tractional vitreoretinal pathology were reviewed. Concentric, fingerprint-like microwaves were visualized through en face optical coherence tomography in all six study eyes at the level of the HFL. This finding resembled the finding of HFL waves previously noted histopathologically from force exerted on this layer. CONCLUSION: In retinal pathologies in which specific physical forces act on the retina, volumetric optical coherence tomography may permit visualization of en face concentric, fingerprint-like hyperreflective rings within the HFL. This "fingerprint sign" may represent a biomechanical consequence of traction on the retina and allow clinical decision making based on improved recognition of the existence of such traction.

AUTOSOMAL RECESSIVE BESTROPHINOPATHY: MULTIMODAL IMAGING UPDATE.

BACKGROUND: Autosomal recessive bestrophinopathy is part of the diverse spectrum of retinal diseases caused by mutations in the BEST1 gene. METHODS: A case report. RESULTS: We present a case that highlights the classic retinal findings of autosomal recessive bestrophinopathy with an emphasis on modern multimodal imaging. CONCLUSION: We describe modern multimodal imaging in an individual with a BEST1 gene mutation and clinical findings consistent with an autosomal recessive bestrophinopathy.

Neovascularization of the iris in retinoschisis.

PURPOSE: To report the association of rubeosis iridis with chronic bullous degenerative peripheral retinoschisis. OBSERVATIONS: A 63-year-old female presented with acute hyphema and neovascularization of the iris in association with elevated intraocular pressure. Posterior segment examination including imaging revealed no vascular occlusion as a potential cause. However, large, peripheral bullous retinoschisis was noted in the right eye. No nonperfusion aside from that seen within the schism detachment, or neovascularization of the retina on wide-field fundus photography or fluorescein angiography was noted. Bullous retinoschisis was also found in the left eye. The patient was treated conservatively with prednisolone acetate and timolol eye drops. CONCLUSIONS AND IMPORTANCE: Chronic bullous retinoschisis can be associated with anterior segment neovascularization such as rubeosis iridis, presumably due to non-perfusion within the retinoschisis cavity.

ACUTE MACULAR NEURORETINOPATHY WITHOUT NEAR-INFRARED REFLECTANCE-AN ATYPICAL CASE PRESENTATION.

BACKGROUND: Acute macular neuroretinopathy is a rare disease that with the help of multimodal imaging is being diagnosed more frequently. METHODS: An atypical case is presented and followed by multimodal imaging. RESULTS: A typical acute macular neuroretinopathy lesion was seen on biomicroscopic examination and spectral domain optical coherence tomography examination. However, near-infrared imaging revealed a lack of the characteristic finding along with a relative afferent pupillary defect and a widespread depressed multifocal electroretinogram which has not been reported previously. CONCLUSION: This patient exhibits photoreceptor and outer retinal findings commonly described in acute macular neuroretinopathy lesion without classic near-infrared findings. This case may represent a severe form of acute macular neuroretinopathy.

Rhegmatogenous Retinal Detachment in Patients with Acute Syphilitic Panuveitis.

PURPOSE: To describe the clinical characteristics and surgical management of rhegmatogenous retinal detachment (RD) in patients with acute syphilitic panuveitis. METHODS: Retrospective case series and comprehensive literature review. RESULTS: Including present and previously reported cases, we identified 11 eyes in 8 patients with acute syphilitic panuveitis that developed a rhegmatogenous RD. Seven of 11 eyes (63.6%) were repaired with a combined scleral buckling, vitrectomy, and endolaser photocoagulation surgery; 1 eye (9.1%) was repaired with scleral buckling only; and 2 eyes (18.2%) with vitrectomy only. Cryotherapy was used to treat a giant retinal tear in 1 eye (9.1%). Four eyes (36.4%) redetached and 3 underwent a second vitrectomy surgery. CONCLUSIONS: Although uncommon, rhegmatogenous RD can occur in patients with moderate to severe acute syphilitic panuveitis. We believe scleral buckling, vitrectomy, endolaser photocoagulation, and silicone oil tamponade give the best chance for successful retinal reattachment.

Spectral domain optical coherence tomography findings in eyes with acute ischaemic retinal whitening.

Acute retinal ischaemia presents in various forms depending on the type and location of the associated vascular occlusion. Cotton wool spots have been considered one manifestation of ischaemia and represent swelling in the nerve fibre layer. However, clinical retinal whitening also occurs in areas not affected by cotton wool spots, and has distinguishing spectral domain optical coherence tomography (SD-OCT) features. We present SD-OCT findings of hyper-reflectivity and thickening in four eyes with representative retinal arterial or retinal venous occlusions, specifically branch retinal artery occlusion, central retinal vein occlusion, Purtscher-like retinopathy and ophthalmic artery occlusion. The spectrum of retinal ischaemia from various causes was found to manifest in inner nuclear layer hyper-reflectivity and thickening on SD-OCT. En Face OCT imaging further characterises the topographical distribution of ischaemia, and reveals patterns which provide insight into the pathological processes involved.

Central serous chorioretinopathy after trabeculectomy in a patient with microphthalmos and congenital rubella syndrome.

PURPOSE: To describe a case of central serous chorioretinopathy after trabeculectomy surgery in an eye with microphthalmos in the setting of congenital rubella syndrome. METHODS: A Case report with color fundus photographs, fluorescein angiography, and spectral domain optical coherence tomography. RESULTS: A 46-year-old African American man, with a history of congenital heart disease and bilateral hearing loss, developed persistent vision loss in the left eye after trabeculectomy surgery. Ocular examination revealed bilateral salt-and-pepper retinopathy and a serous detachment in the macula of the left eye. Fluorescein angiography showed an early "smoke-stack" pattern of hyperfluorescence with progressive filling of the subretinal space consistent with central serous chorioretinopathy. Spectral domain optical coherence tomography documented both size and extent of the serous retinal detachment and showed several pigment epithelial detachments. B-scan ultrasonography confirmed the serous retinal detachment on the left but showed no evidence of posterior eye wall thickening or of retrobulbar fluid. An A-scan revealed an axial length of 21.8 mm on the right and 19.7 mm on left eye, confirming microphthalmos and supporting the suspected diagnosis of congenital rubella syndrome. CONCLUSION: Although uncommon, central serous chorioretinopathy can occur in anatomically small eyes after trabeculectomy surgery.

Acute retinal necrosis secondary to multidrug-resistant herpes simplex virus 2 in an immunocompetent adolescent.

PURPOSE: To report the clinical course of a patient with acute retinal necrosis resulting from a multidrug-resistant strain of herpes simplex virus 2. METHODS: Observational case report. RESULTS: A 17-year-old man with no identifiable immune deficiency presented with pain and decreased vision in his left eye. He had dense anterior and posterior segment inflammation with retinal whitening suggestive of acute retinal necrosis, which progressed despite treatment with intravenous acyclovir, methylprednisolone, and ganciclovir. A transition to intravitreal and intravenous foscarnet led to clinical improvement. Genetic analysis revealed the etiology to be a multidrug-resistant strain of herpes simplex virus 2. CONCLUSION: Antiviral resistance is an uncommon finding among viruses causing acute retinal necrosis in immunocompetent patients. Patients with these infections may be adequately treated with prompt recognition and a change in therapy to alternative antiviral agents such as foscarnet.

Chronic exogenous exophiala dermatitidis endophthalmitis.

PURPOSE: To describe a case of chronic exogenous Exophiala dermatitidis endophthalmitis. METHODS: Retrospective chart review and case report. RESULTS: A 60-year-old man with history of chronic herpes zoster keratitis complicated by secondary fungal keratitis treated with penetrating keratoplasty presented with a white cataract, chronic anterior uveitis, and counting fingers vision in the left eye. Combined cataract extraction and diagnostic vitrectomy revealed positive cultures and polymerase chain reaction-based testing for E. dermatitidis-the same organism responsible for the keratitis. The patient was treated with multiple oral, intravenous, and intravitreal antifungal agents. Ultimately, the corneal infection recurred and the patient elected to undergo enucleation. Filamentous fungi consistent with E. dermatitidis infection were identified in the cornea of the enucleation specimen. CONCLUSION: Although rare, Exophiala species can cause exogenous endophthalmitis. Chronic endophthalmitis should be suspected in patients who develop persistent intraocular inflammation after infectious keratitis.

Paraneoplastic vitelliform retinopathy in a patient with treated choroidal melanoma.

PURPOSE: To describe a peculiar case of paraneoplastic vitelliform maculopathy/retinopathy in a patient with treated choroidal melanoma. METHODS: A case report of a 58-year-old woman with a history of treated choroidal melanoma 16 years before developing visual changes in the setting of metastatic choroidal melanoma. RESULTS: We demonstrate bilateral, multifocal vitelliform subretinal lesions and focal, neurosensory retinal detachment associated with metastatic melanoma. CONCLUSION: Paraneoplastic vitelliform maculopathy/retinopathy should be considered in patients with or without a history of melanoma, who have unexplained serous, vitelliform retinal detachments, especially with atypical or absence of leakage on fluorescein angiography.

En face spectral domain optical coherence tomography analysis of lamellar macular holes.

PURPOSE: To analyze the anatomical characteristics of lamellar macular holes using cross-sectional and en face spectral domain optical coherence tomography. METHODS: Forty-two lamellar macular holes were retrospectively identified for analysis. The location, cross-sectional length, and area of lamellar holes were measured using B-scans and en face imaging. The presence of photoreceptor inner segment/outer segment disruption and the presence or absence of epiretinal membrane formation were recorded. RESULTS: Forty-two lamellar macular holes were identified. Intraretinal splitting occurred within the outer plexiform layer in 97.6% of eyes. The area of intraretinal splitting in lamellar holes did not correlate with visual acuity. Eyes with inner segment/outer segment disruption had significantly worse mean logMAR visual acuity (0.363 ± 0.169; Snellen = 20/46) than in eyes without inner segment/outer segment disruption (0.203 ± 0.124; Snellen = 20/32) (analysis of variance, P = 0.004). Epiretinal membrane was present in 34 of 42 eyes (81.0%). CONCLUSION: En face imaging allowed for consistent detection and quantification of intraretinal splitting within the outer plexiform layer in patients with lamellar macular holes, supporting the notion that an area of anatomical weakness exists within Henle's fiber layer, presumably at the synaptic connection of these fibers within the outer plexiform layer. However, the en face area of intraretinal splitting did not correlate with visual acuity, disruption of the inner segment/outer segment junction was associated with significantly worse visual acuity in patients with lamellar macular holes.

Sensitivity of fluid detection in patients with neovascular amd using spectral domain optical coherence tomography high-definition line scans.

PURPOSE: To determine the sensitivity of the Cirrus high-definition (HD) 5-line raster scans for detecting retinal fluid in neovascular age-related macular degeneration when using the spectral domain optical coherence tomography macular cubes as a gold standard. METHODS: Patients were retrospectively identified from their initial follow-up visit after being newly diagnosed with neovascular age-related macular degeneration in at least one eye. Patients were imaged with Cirrus spectral domain optical coherence tomography using the 512 × 128 macular cube scan and HD 5-line raster scan settings. Patients with other diseases that cause subretinal or intraretinal fluid, or who had an epiretinal membrane causing macular traction were excluded from the analysis. We recorded the presence or absence of subretinal or intraretinal fluid in the macular cube and on the HD 5-line raster scans. RESULTS: Seventy-nine patients met the study requirements. Of the 63 patients who had fluid present on the macular cube, 1 did not seem to have fluid on the HD 5-line raster scans. Taking the macular cube as a gold standard, the sensitivity of the HD 5-line raster scans for detecting retinal fluid in this cohort was 98.4%. CONCLUSION: The Cirrus HD 5-line raster scans have a high sensitivity for detecting fluid in the macular cube in patients with neovascular age-related macular degeneration.

Autofluorescence and spectral domain OCT findings in Alport syndrome.

PURPOSE: The purpose of this study was to report novel autofluorescence and spectral domain optic coherence tomography findings in a case of Alport syndrome. METHODS: Case report and literature review. RESULTS: A 30-year-old woman with a history of Alport syndrome presented with a full-thickness macular hole in her right eye and evidence of vitreofoveal traction in her asymptomatic left eye. Both eyes had temporal macular thinning. She had anterior lenticonus and perimacular flecks characteristic of Alport syndrome. In addition, fundus autofluorescence revealed an interesting pattern of splotchy hypoautofluorescence in the periphery (in the absence of any peripheral retinopathy on examination), which has not been described previously. CONCLUSION: Macular hole in Alport syndrome results from basement membrane weakness and an abnormal vitreoretinal interface. Although this makes surgery in these cases challenging, careful separation of the posterior hyaloid and internal limiting membrane peeling with fluid gas exchange can lead to successful closure. Alport syndrome can also cause abnormalities in the retinal pigment epithelium/Bruch membrane leading to abnormal autofluorescence.

Intravitreous ranibizumab for persistent macular edema in retinal vein occlusion unresponsive to bevacizumab.

PURPOSE: To report successful treatment of persistent retinal edema associated with retinal vascular occlusion with ranibizumab after initial failure with bevacizumab intravitreal injections. METHODS: A retrospective chart review of all patients undergoing treatment with intravitreal bevacizumab for retinal vascular occlusion was undertaken. Clinical features of patients who were switched to monthly ranibizumab because of persistent and/or worsening retinal edema despite multiple (six or more) monthly intravitreal bevacizumab injections were selected for review. The type of retinal vascular occlusion, age of the patient, visual acuity at each visit, number of intravitreal bevacizumab injections, optical coherence tomography findings at each visit, fluorescein angiography, and number of intravitreal ranibizumab injections with optical coherence tomography findings at each subsequent visit were recorded. RESULTS: Six patients (five women and one man) were identified. Age ranged from 56 years to 74 years. Five had central retinal vein occlusion and one a branch retinal vein occlusion. All were treated with at least 6 sequential intravitreal bevacizumab injections (range, 6-13) before treatment with ranibizumab. All demonstrated an immediate and marked reduction in retinal edema that had not occurred with any of the previous treatments. The average central foveal thickness on optical coherence tomography was 592 µm before ranibizumab injection and 346 µm after the injection. Visual acuity improved in four of the six patients and was stable in the remaining two patients. CONCLUSION: Eyes of patients with persistent macular edema secondary to retinal vein occlusion despite bevacizumab may experience prompt improvement after initiation of intravitreal ranibizumab injections.