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.

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.

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.

Retinal pigment epitheliopathy, macular telangiectasis, and intraretinal crystal deposits in HIV-positive patients receiving ritonavir.

PURPOSE: The purpose of this study was to describe the occurrence of a retinal pigment epitheliopathy associated with macular telangiectasis and intraretinal crystal deposits in three human immunodeficiency virus-positive patients receiving long-term ritonavir as part of highly active antiretroviral therapy. METHODS: The patient's records were reviewed. RESULTS: The CD4 T-cell counts at presentation were 163 cells per microliter, 464 cells per microliter, and 349 cells per microliter, and viral loads were undetectable in all patients. None of the patients had a concurrent AIDS-defining illness. Other significant medical history included hyperlipidemia in one patient and a remote history of lymphoma and tuberculosis in a second patient. Initial visual acuity ranged from 20/32 to 20/400, with a median of 20/150. Anterior segment examination and intraocular pressures were normal in all eyes. Posterior segment examination revealed bilateral macular retinal pigment epitheliopathy with intraretinal crystalline deposits. No hemorrhage or cotton wool spots were seen consistent with human immunodeficiency virus retinopathy, and there was no evidence of previous or active cystomegalovirus retinitis. Fluorescein angiography revealed parafoveal telangiectasis with late leakage in two of the three patients. Optical coherence tomography showed thickening of the macula in three eyes and inner foveal cysts in two eyes. Autofluorescence performed on one patient revealed complete loss of normal retinal pigment epithelium autofluorescence corresponding to the area of retinal pigment epitheliopathy bilaterally. The only medicine common to all 3 patients was ritonavir, and the duration of ritonavir therapy before presentation was 19 months in one patient, 30 months in the second patient, and 5 years in the third patient. CONCLUSION: Retinal changes characterized by retinal pigment epitheliopathy, parafoveal telangiectasias, and intraretinal crystal deposits occurred in three human immunodeficiency virus-positive patients on long-term ritonavir as part of highly active antiretroviral therapy.

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.

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.

Macular fibrosis in Coats disease.

PURPOSE: The purpose of this study was to describe the angiographic features and visual impact of macular fibrosis in patients with Coats disease. METHODS: This is a single institution retrospective case series. Charts of patients diagnosed with Coats disease between 1973 and 2007 were reviewed. Data collected included patient demographics, treatment method, initial and final logarithm of the minimum angle of resolution visual acuity, photographic findings, angiographic characteristics, and anatomical outcome. RESULTS: Forty-seven patients were identified with adequate imaging and posttreatment follow-up. Average age at presentation was 38 years (4-82 years). Average follow-up was 4.9 years (0-17 years). Macular fibrosis was identified in 11 patients (23%). At presentation, the average logarithm of the minimum angle of resolution visual acuity was as follows: all patients, 0.67; patients with macular fibrosis, 1.14; and patients without macular fibrosis, 0.50 (P = 0.01, 2-tailed Student's t-test). The average posttreatment logarithm of the minimum angle of resolution visual acuity was as follows: all patients, 0.78; patients with macular fibrosis, 0.97; and patients without macular fibrosis, 0.70 (P = 0.26). Macular fibrosis was associated with a pigmented spot at the point of apparent intraretinal vascular anastamosis. Fluorescein angiography showed leakage consistent with neovascularization that appeared to be intraretinal. CONCLUSION: Macular fibrosis is a common finding in Coats disease, occurring in 23% of our patients. It may be a result of intraretinal neovascularization and is associated with a worse vision outcome.

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.

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.

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.

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.

Pupil ovalization after phakic intraocular lens implantation is associated with sectorial iris hypoperfusion.

OBJECTIVE: To investigate iris perfusion in patients with and without pupil ovalization after phakic intraocular lens implantation. METHODS: Comparative retrospective randomized case series of 6 participants, each with a regular pupil, and 6 participants with pupil ovalization after phakic intraocular lens implantation for high myopia were included in the study. Indocyanine green angiography was performed between 20 and 40 months (mean +/- SD, 26 +/- 6.1 months) after lens implantation. RESULTS: Iris perfusion defects were found in 5 of 6 patients with pupil ovalization. No perfusion deficits were noted in patients with round pupils. CONCLUSION: Iris ovalization after phakic intraocular lens implantation may be associated with a lack of iris perfusion and with secondary ischemia. Patients with these lenses and pupil ovalization should be followed regularly.

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.