Search

Purpose To report two cases of cytomegalovirus (CMV) retinitis in renal transplant patients after conversion to belatacept immunosuppression. Methods Case report. Results Case A was a 41-year-old African American man with no ocular history, chronic hepatitis B infection on entecavir, status-post living relative renal transplant for end-stage renal disease secondary to IgA nephropathy presented with a chief complaint of "blurry vision" in his right eye. Fundoscopic examination of the right eye revealed perivascular retinitis of the superior arcade extending temporally and a lesion nasal to the optic disk. The patient was diagnosed with CMV retinitis with macular involvement. Case B was a 52-year-old woman with an ocular history significant for bilateral vein occlusions treated with laser, and a medical history of hypertension, hyperlipidemia, and end-stage renal disease secondary to hypoplastic kidney status-post deceased donor renal transplant presented with one week of blurred vision in the right eye. Fundoscopic examination of the right eye revealed macular star configuration of exudates, as well as subretinal fibrosis temporal to the macula. Fundoscopic examination of the left eye showed Grade 4 vitreous haze and multiple areas of retinal whitening and hemorrhage, consistent with CMV retinitis infection. The patient was diagnosed with bilateral CMV retinitis, with macular involvement in the right eye. Conclusion Renal transplant patients converted to belatacept immunosuppression may be at an increased risk for herpes virus infection, and thus herpes virus retinitis. Although the degree of risk remains uncharacterized, patients on belatacept therapy who are at the high risk of CMV infection (i.e., donor-positive/recipient-negative patients) should be counseled on the presenting signs and symptoms of CMV retinitis. In these cases, clinicians should also consider regular monitoring of serum CMV titers or continuation of antiviral prophylaxis.

Purpose: The temporary cessation and profound changes in ophthalmic care delivery that occurred as a result of the coronavirus disease 2019 (COVID-19) pandemic have yet to be fully understood. Our objective is to assess patients’ self-reported impact of health care lockdown measures on their fears and anxieties during the crisis period of the COVID-19 pandemic in New York City. Methods: We conducted a digital, self-reported, patient care survey distributed by an e-mail at Columbia University’s Department of Ophthalmology outpatient faculty practice. Inclusion criteria were age greater than or equal to 18 years, a diagnosis of either retinal disease or glaucoma, and a canceled or rescheduled ophthalmology established patient appointment during the acute phase of the COVID-19 pandemic in New York City. Patients without an e-mail address listed in their electronic medical records were excluded. The survey occurred between March 2, 2020, to May 30, 2020. Primary measures were survey responses to assess key areas of patient anxiety or concern during the pandemic including the safety of care delivery in a COVID pandemic, difficulties contacting or being seen by their ophthalmologist, concern of vision loss or disease progression, and concern over missed or access to treatments. Secondary measures were correlating survey response to factors such as visual acuity, intraocular pressure, diagnosis, disease severity, follow-up urgency, recent treatments, and diagnostic testing data. Results: Of the 2594 surveys sent out, 510 (19.66%) were completed. Over 95% of patients were at least as concerned as in normal circumstances about their ocular health during the peak of the pandemic. Overall, 76% of respondents were more concerned than normal that they could not be seen by their ophthalmologist soon enough. Increased concern over ocular health, disease progression, and access to care all showed positive correlations (P

PURPOSE: The novel coronavirus, SARS-CoV-2 (COVID-19), has disrupted the practice of ophthalmology and threatens to forever alter how we care for our patients. Physicians across the country encounter unique clinical dilemmas daily. This manuscript presents a curated set of ethical dilemmas facing ophthalmologists both during and following the pandemic. DESIGN: Perspective METHODS: Case presentations drawn from actual clinical scenarios were presented during a virtual Ophthalmology Grand Rounds and discussed with the Director of Clinical Ethics at Columbia University Irving Medical Center. RESULTS: It has become routine to expect an ophthalmologist to be involved in many levels of care for critically ill patients with COVID-19. Ophthalmology patients - even those with chronic, progressive conditions - are being triaged, and vision-saving interventions are being postponed. Four questions were applied to each scenario allowing for ethical conclusions to be reached. These questions were; What is the imminence and the severity of the harm expected without intervention? What is the efficacy of the intervention under consideration? What are the risks of treatment for the patient? What are the risks of treating the patient for the healthcare team? CONCLUSION: During this pandemic and for months, perhaps years, to come, it is critical to reconsider the ethical principles underlying modern medicine and ophthalmic care as well as the ramifications of our decisions and actions. ABSTRACT: Perspective.

Background In resident family medicine practices, a >20% no-show rate is common, often resulting in suboptimal care. Few studies have evaluated resident ophthalmology practices in a similar fashion. The objective of this study was to determine no-show rates and factors related to missed appointments in a resident-based ophthalmology clinic. Methods We reviewed 19,237 de-identified appointments over one academic year. Appointments were divided into general clinic visits and subspecialty visits (these two clinics function separately at our institution). The number of days between an appointment being made for a patient and the scheduled visit was defined as “lead time.” Each appointment was labeled “show” or “no-show.” Pertinent factors available in the analysis included lead time to appointment, patients age, month, time of day, practitioner training level (postgraduate years 2 to 4, fellow), and proximity to holiday. Subspecialty clinics included oculoplastics clinic, strabismus clinic, neuro-ophthalmology clinic, uveitis clinic, cornea clinic, glaucoma clinic, and retina clinic. Generalized linear mixed effects models were fit on the no-show status with these factors. Results The overall no-show rate for general clinic was 31.4%. Longer lead time to appointment, younger age, afternoon appointments, new patients, December appointments, and lower residency level were associated with higher odds of no-show (p ≤ 0.05). The overall no-show rate for subspecialty clinic was 21.8%. Longer lead time to appointment, younger age, and lower residency level were associated with higher odds of no-show (p ≤ 0.05). Patients were significantly less likely to show up for retina clinic, oculoplastics clinic, and glaucoma clinic. Conclusion The overall no-show rate in our clinic was on the higher end of reported no-show values from other specialties. Patient status and the lead time to appointment play a significant role in patient compliance. The significance of other factors (patient age, subspecialty, time of day, appointment month, and resident level) have been found in previous studies but are not consistent factors across all clinical environments.

Purpose To describe the first known case of an extracorporeal membrane oxygenation (ECMO)-related complication in an adult. Methods Case report. Results A 34-year-old man awaiting a lung transplant for interstitial lung disease was connected to an ECMO circuit as a bridge to lung transplant, with a drainage catheter attached to his right internal jugular vein. Shortly after he was cannulated, he developed blurred vision in his right eye and was found to have a progressively worsening bullous exudative retinal detachment. After receiving a lung transplant and getting decannulated from ECMO, his bullous detachment rapidly improved. The patient's clinical course as well as his ophthalmic testing showed findings inconsistent with alternative diagnoses such as central serous chorioretinopathy. His findings were best explained as a complication of ECMO cannulation. Conclusion Extracorporeal membrane oxygenation may be associated with bullous exudative retinal detachment in rare cases where there is a possible anatomical or physiological predisposition.

Marlene D Wang, Cecile Truong, Zaid Mammo, Syed Amal Hussnain, Royce WS Chen Department of Ophthalmology, Edward S. Harkness Eye Institute, Columbia University Irving Medical Center, New York, NY, 10032, USACorrespondence: Royce WS ChenEdward S. Harkness Eye Institute, Columbia University Irving Medical Center, 635 W 165th St, New York, NY, 10032, USATel +1 212-342-4570Email Rc2631@cumc.columbia.eduBackground: Biomicroscopy, B-scan ultrasound imaging, and SD-OCT are all modalities used to characterize a posterior vitreous detachment (PVD). Our objective is to assess the precision of the diagnosis of PVD by SS-OCT.Methods: This prospective observational study examines ninety-five eyes of forty-nine patients with biomicroscopy, B-scan ultrasound, and SS-OCT for the presence or absence of a complete PVD. All SS-OCT images were reviewed by two retina specialists (RWSC, ZM). All three diagnostic methods were evaluated for agreement by Cohen’s kappa statistic.Results: The inter-rater reliability between retina specialists reading the SS-OCT images was 97.9% (κ = 0.957). Agreement on PVD status between SS-OCT and biomicroscopy was 85.3% (κ = 0.711). Agreement between SS-OCT and B-scan ultrasound was 83.2% (κ = 0.667). Agreement between B-scan ultrasound and biomicroscopy was 87.4% (κ = 0.743).Conclusion: For the diagnosis of complete PVD, SS-OCT allows for high accuracy and agreement between graders.Keywords: optical coherence tomography, swept source, posterior vitreous detachment

PurposeTo identify the role of gender and other factors in influencing ophthalmologists' compensation.DesignCross-sectional study.ParticipantsU.S. practicing ophthalmologists.MethodsBetween January and March 2020, an anonymous survey was sent to U.S. residency program directors and practicing ophthalmologists who recently completed residency training. Respondents who completed residency ≤ 10 years ago and responded to questions about gender, fellowship training, state of practice, and salary were included. Propensity score match (PSM) analysis was performed with age, academic residency, top residency, fellowship, state median wage, practice type, ethnicity, and number of workdays. Multivariate linear regression (MLR) analysis controlled for additional factors along with the aforementioned variables.Main outcome measuresBase starting salary with bonus (SWB) received in the first year of clinical position was the main outcome measure. A multiplier of 1.2 (20%) was added to the base salary to account for bonus.ResultsOf 684 respondents, 384 (56% were female, 44% were male) from 68 programs were included. Female ophthalmologists received a mean initial SWB that was $33 139.80 less than that of their male colleagues (12.5%, P= 0.00). The PSM analysis showed an SWB difference of-$27 273.89 (10.3% gap, P= 0.0015). Additionally, SWB differences were calculated with the number of workdays substituted by operating room (OR) days (-$27 793.67 [10.5% gap, P= 0.0013]) and clinic days (-$23 597.57 [8.90% gap, P= 0.0064]) in separate PSM analyses. The SWB differences between genders were significant using MLR analyses, which also controlled for work, clinic, and OR days separately (-$22 261.49, $-18 604.65, and $-16 191.26, respectively; P= 0.017, P= 0.015, P= 0.002, respectively). Gender independently predicted income in all 3 analyses (P&nbsp

BACKGROUND: From March 2, 2020, to April 12, 2020, New York City (NYC) experienced exponential growth of the COVID-19 pandemic due to novel coronavirus (SARS-CoV-2). Little is known regarding how physicians have been affected. We aimed to characterize the COVID-19 impact on NYC resident physicians. METHODS: IRB-exempt and expedited cross-sectional analysis through survey to NYC residency program directors April 3–12, 2020, encompassing events from March 2, 2020, to April 12, 2020. RESULTS: From an estimated 340 residency programs around NYC, recruitment yielded 91 responses, representing 24 specialties and 2306 residents. In 45.1% of programs, at least 1 resident with confirmed COVID-19 was reported. One hundred one resident physicians were confirmed COVID-19–positive, with an additional 163 residents presumed positive for COVID-19 based on symptoms but awaiting or unable to obtain testing. Two COVID-19–positive residents were hospitalized, with 1 in intensive care. Among specialties with more than 100 residents represented, negative binomial regression indicated that infection risk differed by specialty (P = 0.039). In 80% of programs, quarantining a resident was reported. Ninety of 91 programs reported reuse or extended mask use, and 43 programs reported that personal protective equipment (PPE) was suboptimal. Sixty-five programs (74.7%) redeployed residents elsewhere to support COVID-19 efforts. CONCLUSION: Many resident physicians around NYC have been affected by COVID-19 through direct infection, quarantine, or redeployment. Lack of access to testing and concern regarding suboptimal PPE are common among residency programs. Infection risk may differ by specialty. FUNDING: National Eye Institute Core Grant P30EY019007; Research to Prevent Blindness Unrestricted Grant; Parker Family Chair; University of Pennsylvania.

Background The American Academy of Ophthalmology, in conjunction with the Accreditation Council of Graduate Medical Education (ACGME), has mandated the creation of a linked ophthalmology internship year for all programs by 2021. This mandate provides a unique opportunity to tailor a curriculum specific to the ophthalmology trainee. This study reports the results of a national survey of ophthalmology residents and fellows regarding the relevance and applicability of extramural content to their ophthalmology training. Methods Using the online portal SurveyMonkey, an anonymous survey was sent to 119 ACGME-accredited ophthalmology residencies. It consisted of nine questions formulated to measure the perceived applicability and relevance of other specialties to ophthalmology training. Responses were summarized with descriptive statistics. Outcomes were compared by one-way analysis of variance with Tukey's post hoc analysis for between group differences and Benjamini–Hochberg for multiple comparisons. Results Ninety-four respondents (residents and fellows) completed the survey (January–February 2020); 52.1% completed an internal medicine internship, 42.6% transitional, and 5.3% general surgery. Overall, prior internship experience was rated favorably. Otolaryngology and radiology were deemed the most relevant to ophthalmology training. Intensive care unit (ICU) was considered the least beneficial, followed by general surgery, primary care, and pediatrics. Neuroradiology was overwhelmingly preferred over general radiology. The majority of respondents reported minimal exposure to oral and maxillofacial surgery (OMFS) in medical school/internship. When choosing a reason for inclusion in the new curriculum, respondents reported a high level of surgical overlap with otolaryngology, OMFS, and plastic surgery; clinical overlap with neurology; and ophthalmic consultation with emergency medicine. Conclusion Several specialties—notably otolaryngology, radiology, and neurology—were considered highly relevant but lacking in preresidency exposure. The preference for neuroradiology was almost unanimous. In contrast, general surgery and ICU were found to be minimally relevant. Overall, ophthalmology residents reported a high level of satisfaction with their internship experience, an important consideration when building a curriculum that will appeal to potential applicants. The findings of this survey provide a framework to build a curricular plan based on current resident and fellow experience.

BackgroundFrom March 2-April 12, 2020, New York City (NYC) experienced exponential growth of the COVID-19 pandemic due to novel coronavirus (SARS-CoV-2). Little is known regarding how physicians have been affected. We aimed to characterize COVID-19 impact on NYC resident physicians.MethodsIRB-exempt and expedited cross-sectional analysis through survey to NYC residency program directors (PDs) April 3–12, 2020, encompassing events from March 2–April 12, 2020.FindingsFrom an estimated 340 residency programs around NYC, recruitment yielded 91 responses, representing 24 specialties and 2,306 residents. 45.1% of programs reported at least one resident with confirmed COVID-19: 101 resident physicians were confirmed COVID-19-positive, with additional 163 residents presumed positive for COVID-19 based on symptoms but awaiting or unable to obtain testing. 56.5% of programs had a resident waiting for, or unable to obtain, COVID-19 testing. Two COVID-19-positive residents were hospitalized, with one in intensive care. Among specialties with >100 residents represented, negative binomial regression indicated that infection risk differed by specialty (p=0.039). Although most programs (80%) reported quarantining a resident, with 16.8% of residents experiencing quarantine, 14.9% of COVID-19-positive residents were not quarantined. 90 programs, encompassing 99.2% of the resident physicians, reported reuse or extended mask use, and 43 programs, encompassing 60.4% of residents, felt that personal protective equipment (PPE) was suboptimal. 65 programs (74.7%) have redeployed residents elsewhere to support COVID-19 efforts.InterpretationMany resident physicians around NYC have been affected by COVID-19 through direct infection, quarantine, or redeployment. Lack of access to testing and concern regarding suboptimal PPE are common among residency programs. Infection risk may differ by specialty.FundingAHA, MPB, RWSC, CGM, LRDG, JDH are supported by NEI Core Grant P30EY019007 and an unrestricted grant from RPB. ACP and JS are supported by the Parker Family Chair. SXX is supported by the University of Pennsylvania.

Purpose Central retinal vein occlusions (CRVO) are relatively common; however, they are rare in young, otherwise healthy individuals. We report a case of CRVO associated with creatine supplementation and dehydration in a 25-year-old man. Observations A 25-year-old man developed a non-ischemic CRVO in the right eye. Comprehensive thrombophilia screening was unrevealing. Further questioning revealed that the patient was an avid weightlifter and had been taking creatine as a nutrition supplement daily for the past 5 years at a higher than recommended dose. At the time of CRVO onset, he was also restricting water intake in order to lose weight. Conclusions and Importance We conclude that the CRVO occurred in the context of creatine use and water restriction, leading to increased risk for thrombosis. Given the increased popularity for nutritional supplements to enhance fitness, it is important for individuals to recognize the association between CRVO, creatine supplementation, and hydration status.

Purpose Based on standard screening techniques, sickle retinopathy reportedly occurs in 10% of adolescents with sickle cell disease (SCD). We performed a prospective, observational clinical study to determine if ultra-widefield fluorescein angiography (UWFA), spectral-domain optical coherence tomography (SD-OCT), and optical coherence tomography angiography (OCT-A) detect more-frequent retinopathy in adolescents with SCD. Design Cross-sectional study. Methods Setting : Institutional. Subjects : Sixteen adolescents with SCD, aged 10–19 years (mean age 14.9 years), and 5 age-equivalent controls (mean age 17.4 years). Observation Procedures : Examinations including acuity, standard slit-lamp biomicroscopy, UWFA, SD-OCT, and OCT-A were performed. Main Outcome Measures : Sickle retinopathy defined by biomicroscopic changes, Goldberg stages I-V, Penman scale, flow void on OCT-A, or macular thinning on SD-OCT. Results While 22 of 32 SCD eyes (68.8%) had retinopathy on biomicroscopy, by UWFA 4 of 24 (16.7%) SCD eyes had peripheral arterial occlusion (Goldberg I), and 20 of 24 eyes (83.3%) had peripheral arteriovenous anastomoses (Goldberg II) in addition. No patients had Goldberg stages III-V. By SD-OCT and OCT-A, thinning of the macula and flow voids in both the superficial and deep retinal capillary plexus were found in 6 of 30 (20%) eyes. Conclusions All 24 eyes with adequate UWFA studies demonstrated sickle retinopathy. SD-OCT and OCT-A, which have not been previously reported in the adolescent population, detected abnormal macular thinning and flow abnormalities undetected by biomicroscopy. These findings suggest that pediatric sickle retinopathy may be more prevalent than previously suspected. If these findings are confirmed with larger cross-sectional and prospective analyses, these approaches may enhance early screening for sickle retinopathy.

Sickle retinopathy reflects disease-related vascular injury of the eye, which can potentially result in visual loss from vitreous hemorrhage or retinal detachment. Here we review sickle retinopathy among children with sickle cell disease, describe the epidemiology, pediatric risk factors, pathophysiology, ocular findings, and treatment. Newer, more sensitive ophthalmological imaging modalities are available for retinal imaging, including ultra-widefield fluorescein angiography, spectral-domain optical coherence tomography, and optical coherence tomography angiography. Optical coherence tomography angiography provides a noninvasive view of retinal vascular layers that could previously not be imaged and can be quantified for comparative or prospective analyses. Ultra-widefield fluorescein angiography provides a more comprehensive view of the peripheral retina than traditional imaging techniques. Screening for retinopathy by standard fundoscopic imaging modalities detects a prevalence of approximately 10%. In contrast, these more sensitive methods allow for more sensitive examination that includes the retina perimeter where sickle retinopathy is often first detectable. Use of these new imaging modalities may detect a higher prevalence of early sickle pathology among children than has previously been reported. Earlier detection may help in better understanding the pathogenesis of sickle retinopathy and guide future screening and treatment paradigms.

PURPOSE To describe findings in a patient with choroideremia (CHM) and his mother, an obligate CHM carrier. METHODS Case report. RESULTS A 25-year-old man with nyctalopia and poor peripheral vision since childhood, as well as a family history consistent with an X-linked retinal disorder was diagnosed with CHM. His asymptomatic 50-year-old mother, an obligate carrier, was also examined. Fundus examination of the affected man showed significant atrophy of the choroid and retinal pigment epithelium, whereas the carrier woman showed patchy pigmentary mottling. Imaging of the affected man showed diffuse retinal pigment epithelium atrophy on optical coherence tomography and extensive areas of decreased choriocapillaris flow on optical coherence tomography angiography. By contrast, the carrier woman showed subtle retinal pigment epithelium changes on optical coherence tomography and mild flow alterations on optical coherence tomography angiography. CONCLUSION This case demonstrates the findings seen in CHM, and while CHM carrier women are often asymptomatic, they may exhibit a mosaic pattern of pigmentary change on fundus examination. Optical coherence tomography angiography may show mild choroidal flow abnormalities. This finding serves to further characterize the extent of dysfunction in CHM and its carrier state.

IMPORTANCE: Although almost equal numbers of male and female medical students enter into ophthalmology residency programs, whether they have similar surgical experiences during training is unclear. OBJECTIVE: To determine differences for cataract surgery and total procedural volume between male and female residents during ophthalmology residency. DESIGN, SETTING, PARTICIPANTS: This retrospective, longitudinal analysis of resident case logs from 24 US ophthalmology residency programs spanned July 2005 to June 2017. A total of 1271 residents were included. Data were analyzed from August 12, 2017, through April 4, 2018. MAIN OUTCOMES AND MEASURES: Variables analyzed included mean volumes of cataract surgery and total procedures, resident gender, and maternity or paternity leave status. RESULTS: Among the 1271 residents included in the analysis (815 men [64.1%]), being female was associated with performing fewer cataract operations and total procedures. Male residents performed a mean (SD) of 176.7 (66.2) cataract operations, and female residents performed a mean (SD) of 161.7 (56.2) (mean difference, −15.0 [95% CI, −22.2 to −7.8]; P

Many conditions, both infectious and noninfectious, may lead to inflammation of the retina and choroid. The entities discussed in this chapter are presented to provide a broad survey of conditions encountered by the uveitis and retinal specialist, but this list is by no means comprehensive. A nuanced approach that considers medical and ocular history, constitutional symptoms, ocular manifestations, and targeted ancillary testing is required to provide patients with appropriate therapies and follow-up. Close collaboration with infectious disease specialists, rheumatologists, and other medical professionals is usually a critical aspect of care in these often complicated cases.

Purpose Knowledge on the utility of prophylactic 360° laser retinopexy before pars plana vitrectomy in the absence of peripheral retinal pathology is limited. This study compares the occurrence of rhegmatogenous events in the setting of small-gauge pars plana vitrectomy with and without prophylactic preoperative laser. Methods Our multicenter, retrospective case-control analysis reviewed patients who underwent epiretinal membrane removal or macular hole repair through 23- or 25-gauge pars plana vitrectomy: 205 controls who did not receive prophylactic laser and 176 cases who received preoperative prophylactic laser retinopexy anterior to the equator. Main outcome measures were the rate and characteristics of postoperative retinal tears and detachments. Patients with previous pars plana vitrectomy or significant retinal disease were excluded. Results Of those patients with prophylactic laser and those without, there was no significant difference in the number of retinal breaks (1.7% vs. 0.49%, respectively; P = 0.339) or retinal detachments (0% vs. 0.49%, respectively; P = 1.00). Of the lasered group, there was one sclerotomy-related retinal break and two non-sclerotomy-related retinal breaks. Of the nonlasered group, there was one non-sclerotomy-related retinal break and one sclerotomy-related retinal detachment. Conclusion Preoperative prophylactic peripheral laser retinopexy does not seem to offer an added benefit in the prevention of intraoperative and postoperative rhegmatogenous events.

Purpose: To describe a case of intermediate uveitis caused by chikungunya virus infection in the Western Hemisphere.Methods: Case report of a patient diagnosed with chikungunya infection presenting with blurry vision and floaters.Results: Exam revealed a unilateral intermediate uveitis, with an extensive work-up positive for chikungunya virus immunoglobulin M and G titers. The patient responded to oral corticosteroids with signs and symptoms resolving over the course of 3 months’ treatment.Conclusions: While anterior uveitis and retinitis are the most common ocular manifestations of chikungunya infection, we report here a case of chikungunya infection presenting as an intermediate uveitis, responding well to oral corticosteroids. This case demonstrates the varied presentation of chikungunya-related uveitis and highlights its consideration in the differential diagnoses of those who have had preceding systemic viral symptoms and uveitis.

A 17-year-old man had acute vision loss OS (visual acuity 20/40), having lost vision OD months before (20/400). There were cecocentral scotomas OU. Mitochondrial DNA testing revealed a T14484C mutation, suggestive of Leber hereditary optic neuropathy (LHON), which results in painless sequential vision loss. Acutely, there is hyperemia of the optic nerve, circumpapillary microangiopathy, and dilated, tortuous vasculature1; the pathognomonic finding is nerve swelling without leakage on fluorescein angiography. Chronically, the optic nerve develops atrophy, sometimes with cupping.2 Optical coherence tomography angiography provides rapid, high-resolution visualization of retinal vasculature without dye injection, and is a novel imaging modality in LHON (figure).

Purpose: To report a case of full-thickness macular hole in a patient with X-linked retinoschisis and the outcome after surgical repair. Methods: A 15-year-old boy with a history of X-linked retinoschisis presented to his ophthalmologist for routine follow-up and was found to have a Stage-3 macular hole in his left eye. His vision was 20/200. The patient underwent pars plana vitrectomy and internal limiting membrane peeling, and he received long-acting gas. Color fundus photography and spectral domain optical coherence tomography (Cirrus; Carl Zeiss Meditech Inc, Dublin, CA) recorded images at office visits, before and after surgical repair. Results: The initial spectral domain optical coherence tomography of the left eye showed a full-thickness macular hole of 1,370 μm in diameter as well as schisis cavities in the macula. After pars plana vitrectomy and repeat fluid–gas exchange, the hole was closed at the sixth-week follow-up visit. The patient noted a subjective decrease in the size of a central scotoma. Best-corrected visual acuity returned to the baseline of 20/80. Conclusion: A large full-thickness macular hole can develop in X-linked retinoschisis, and pars plana vitrectomy with internal limiting membrane peeling may be helpful for successful surgical closure.

Aims: To demonstrate ultrahigh-resolution, three-dimensional optical coherence tomography (3D-OCT) and projection OCT fundus imaging for enhanced visualisation of outer retinal pathology in non-exudative age-related macular degeneration (AMD). Methods: A high-speed, 3.5 μm resolution OCT prototype instrument was developed for the ophthalmic clinic. Eighty-three patients with non-exudative AMD were imaged. Projection OCT fundus images were generated from 3D-OCT data by selectively summing different retinal depth levels. Results were compared with standard ophthalmic examination, including fundus photography and fluorescein angiography, when indicated. Results: Projection OCT fundus imaging enhanced the visualisation of outer retinal pathology in non-exudative AMD. Different types of drusen exhibited distinct features in projection OCT images. Photoreceptor disruption was indicated by loss of the photoreceptor inner/outer segment (IS/OS) boundary and external limiting membrane (ELM). RPE atrophy can be assessed using choroid-level projection OCT images. Conclusions: Projection OCT fundus imaging facilities rapid interpretation of large 3D-OCT data sets. Projection OCT enhances contrast and visualises outer retinal pathology not visible with standard fundus imaging or OCT fundus imaging. Projection OCT fundus images enable registration with standard ophthalmic diagnostics and cross-sectional OCT images. Outer retinal alterations can be assessed and drusen morphology, photoreceptor impairment and pigmentary abnormalities identified.

A 42-year-old man with Hunter syndrome developed bilateral visual field loss. Visual field testing demonstrated bilateral ring scotomata that corresponded to areas of thinning seen on standard resolution optical coherence tomography. High-speed, ultrahigh resolution optical coherence tomography, capable of 3.5-micron axial resolution, showed a loss of photoreceptors outside the fovea and cystoid spaces within the inner nuclear, ganglion cell, and outer nuclear layers. These results were consistent with histopathologic features that have been reported previously in patients with Hunter syndrome. Optical coherence tomography could be used as a diagnostic modality to monitor patients with Hunter syndrome and to detect subclinical forms of disease. [Ophthalmic Surg Lasers Imaging 2007;38:423-428.] AUTHORS From New England Eye Center (MKY, RWC, TRH, IG, MW, JSD), Tufts-New England Medical Center, Tufts University, Boston, Massachusetts; the Department of Electrical Engineering and Computer Science and Research Laboratory of Electronics (VJS, IG, JGF, MW), Massachusetts Institute of Technology, Cambridge, Massachusetts; and UPMC Eye Center and the Eye and Ear Institute (JSS), Ophthalmology and Visual Science Research Center, Department of Ophthalmology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania Accepted for publication April 20, 2007. Supported in part by National Institutes of Health RO1-EY13178-06, RO1-EY11289-20, P30-EY08098, P30-EY13078, Air Force Office of Scientific Research, Medical Free Electron Laser Program contract FA9550-040- 1-0046, FA9550-040-1-0011, National Science Foundation ECS-0119452, BES-0522845, The Eye and Ear Foundation (Pittsburgh, PA), and Research to Prevent Blindness Unrestricted Grant and Medical Student Eye Research Fellowship. Drs. Fujimoto and Schuman receive royalties from intellectual property licensed by the Massachusetts Institute of Technology to Carl Zeiss Meditec, and Drs. Duker and Schuman receive research support from Optovue, Inc. Dr. Schuman also receives research support from Alcon Laboratories, Inc., Allergan, Inc., SOLX, and Clarity, and receives lecture fees from Alcon Laboratories, Inc., Allergan, Inc., Carl Zeiss Meditec, Merck U.S. Human Health, the National Eye Institute, Heidelberg Engineering, Inc., and Optovue, Inc. Dr. Schuman did not participate in the editorial review of this manuscript. Address correspondence to Thomas R. Hedges III, MD, Ophthalmology Department, Tufts-New England Medical Center, 750 Washington Street, Boston, MA 02111.

IMPORTANCE Evidence is mounting that achromatopsia is a progressive retinal degeneration, and treatments for this condition are on the horizon. OBJECTIVES To categorize achromatopsia into clinically identifiable stages using spectral-domain optical coherence tomography and to describe fundus autofluorescence imaging in this condition. DESIGN, SETTING, AND PARTICIPANTS A prospective observational study was performed between 2010 and 2012 at the Edward S. Harkness Eye Institute, New York-Presbyterian Hospital. Participants included 17 patients (aged 10-62 years) with full-field electroretinography-confirmed achromatopsia. MAIN OUTCOMES AND MEASURES Spectral-domain optical coherence tomography features and staging system, fundus autofluorescence and near-infrared reflectance features and their correlation to optical coherence tomography, and genetic mutations served as the outcomes and measures. RESULTS Achromatopsia was categorized into 5 stages on spectral-domain optical coherence tomography: stage 1 (2 patients [12%]), intact outer retina; stage 2 (2 patients [12%]), inner segment ellipsoid line disruption; stage 3 (5 patients [29%]), presence of an optically empty space; stage 4 (5 patients [29%]), optically empty space with partial retinal pigment epithelium disruption; and stage 5 (3 patients [18%]), complete retinal pigment epithelium disruption and/or loss of the outer nuclear layer. Stage 1 patients showed isolated hyperreflectivity of the external limiting membrane in the fovea, and the external limiting membrane was hyperreflective above each optically empty space. On near infrared reflectance imaging, the fovea was normal, hyporeflective, or showed both hyporeflective and hyperreflective features. All patients demonstrated autofluorescence abnormalities in the fovea and/or parafovea: 9 participants (53%) had reduced or absent autofluorescence surrounded by increased autofluorescence, 4 individuals (24%) showed only reduced or absent autofluorescence, 3 patients (18%) displayed only increased autofluorescence, and 1 individual (6%) exhibited decreased macular pigment contrast. Inner segment ellipsoid line loss generally correlated with the area of reduced autofluorescence, but hyperautofluorescence extended into this region in 2 patients (12%). Bilateral coloboma-like atrophic macular lesions were observed in 1 patient (6%). Five novel mutations were identified (4 in the CNGA3 gene and 1 in the CNGB3 gene). CONCLUSIONS AND RELEVANCE Achromatopsia often demonstrates hyperautofluorescence suggestive of progressive retinal degeneration. The proposed staging system facilitates classification of the disease into different phases of progression and may have therapeutic implications.

Purpose To report the outcomes of current vitreoretinal surgical management of proliferative sickle retinopathy and to compare current methods to previous studies. Design A retrospective, interventional case series. Methods Fifteen eyes of 14 patients with proliferative sickle retinopathy were managed with vitreoretinal surgery over a 12-year period at a single institution. Results Nine patients had a sickle cell–hemoglobin C (SC) profile, 1 was sickle cell–beta + thalassemia (S beta + -thal), and 4 were sickle cell trait (AS). All 15 eyes underwent pars plana vitrectomy (PPV): 6 for vitreous hemorrhage (VH), 1 for epiretinal membrane (ERM), and an additional 8 for tractional retinal detachment (RD) and/or rhegmatogenous RD. In addition, an encircling scleral buckle (SB) was used in 2 cases. In 7 cases, 20 gauge PPV was used; 23 gauge was used in 3; and 25 gauge was used in 5. All 7 eyes with VH or ERM had improved vision postoperatively. Four of the 8 patients with traction and/or rhegmatogenous RD developed recurrent detachments and required a second operation. All retinas were attached at last follow-up, and visual acuity was 20/400 or better in all eyes. No cases of anterior segment ischemia were encountered. Conclusions Anterior segment ischemia is no longer a common occurrence in eyes undergoing surgery for proliferative sickle retinopathy. Although PPV has replaced the use of SB in many situations, an encircling SB may still be used in this population when necessary. Surgery for VH and ERM generally results in favorable outcomes, but eyes undergoing surgery for traction/rhegmatogenous RD carry a more guarded prognosis.

To demonstrate the utility and characteristics of fundus autofluorescence in late-onset retinitis pigmentosa.Observational case series. Patients diagnosed with late-onset retinitis pigmentosa were identified retrospectively in an institutional setting. Twelve eyes of six patients were identified and medical records were reviewed.All patients presented with slowly progressive peripheral field loss and initial clinical examination revealed only subtle retinal changes. There was a notable lack of intraretinal pigment migration in all patients. Five out of six patients underwent magnetic resonance imaging of the brain to rule out intracranial processes and all were referred from another ophthalmologist for further evaluation. Fundus autofluorescence was ultimately employed in all patients and revealed more extensive retinal pathology than initially appreciated on clinical examination. Fundus autofluorescence directed the workup toward a retinal etiology in all cases and led to the eventual diagnosis of late-onset retinitis pigmentosa through electroretinogram testing.Fundus autofluorescence may be a more sensitive marker for retinal pathology than stereo fundus biomicroscopy alone in late-onset retinitis pigmentosa. Early use of fundus autofluorescence imaging in the evaluation of patients with subtle retinal lesions and complaints of peripheral field loss may be an effective strategy for timely and cost-efficient diagnosis.

Purpose: To evaluate the findings in a case of acute macular neuroretinopathy involving sudden development of scotomas accompanied by rapid focal increases in fundus autofluorescence. Methods: The clinical presentation of the patient was documented by color fundus photographs, fundus autofluorescence, infrared imaging, and high-resolution spectral domain optical coherence tomography. The scotomas were assessed by Humphrey visual field 10-2 and MP-1 microperimetry. Results: Visual field defects exhibited spatial correspondence with wedge-shaped lesions demonstrable in color fundus photographs and infrared imaging. It was notable that the lesions exhibited increased intensity on autofluorescence images obtained within 3 weeks of presentation. Optical coherence tomography revealed focal loss of photoreceptor inner segment/outer segment junctions in both eyes. Conclusion: This case was distinguished by the relative rapidity with which the lesions became hyperautofluorescent in fundus autofluorescence images. Given that the bisretinoids that are the source of autofluorescence form in photoreceptor cells and are transferred to retinal pigment epithelium secondarily, the rapid increase in autofluorescence is unlikely to only reflect retinal pigment epithelium status and is more likely to be indicative of photoreceptor cell dysfunctioning and loss of structural integrity.

Introduction: Sickle cell retinopathy (SCR) reflects disease-related vascular injury. Previous studies of pediatric SCR using standard imaging approaches reported retinopathy in Results: Eight children (mean age 16.3 years; 5 HbSS, 2 HbSC,1 HbS-Beta thalassemia0) were evaluated. Three were on hydroxyurea therapy, 1 on chronic transfusion, 1 on both and 3 on no therapy. No patient had vision disturbance at the time of examination. Biomicroscopic examination revealed sunburst lesions in the mid-peripheral retina in 3 patients and subtle vascular changes in the far periphery of all patients. In contrast, UWFA detected extensive capillary non- perfusion of the peripheral retina with abnormal vascular loops (Goldberg Stage I) in all 8 patients; 3 patients had peripheral arteriovenous anastomoses (Goldberg Stage II) that were undetectable by biomicroscopy. No patient had Goldberg Stages III-V. SD-OCT demonstrated thinning of the temporal macula in 2 patients, and OCT-A of both patients revealed flow voids in both the superficial and deep retinal capillary plexus in the area of thinned retina. Both the SD- OCT and OCT-A abnormalities were undetectable with standard biomicroscopy. Conclusions: All 8 children with SCD in this case series demonstrated evidence of SCR using the combination of standard biomicroscopy, UWFA, SD-OCT, and OCT-A imaging. Findings from these sensitive imaging modalities suggest that pediatric SCR is more prevalent than previously suspected. Additional patients and age-equivalent controls will be included in this report to validate the specificity of findings to SCD. Larger cross-sectional and prospective analyses using sensitive retina imaging are needed to confirm our findings and identify their significance for detecting pathology. If confirmed, these approaches may enhance early screening for patients at risk of vision-threatening consequences of SCR. Supported by the Irving Institute CTSA at CUMC and by 2T35HL007616 (DP). Retinal findings in pediatric sickle cell disease patients SD-OCT: Spectral-domain optical coherence tomography; OCT-A: Optical coherence tomography angiography; UWFA: Ultra-widefield fluorescein angiography. Retinal findings in pediatric sickle cell disease patients. / SD-OCT: Spectral-domain optical coherence tomography; OCT-A: Optical coherence tomography angiography; UWFA: Ultra-widefield fluorescein angiography. Disclosures No relevant conflicts of interest to declare.

Objective: To determine whether unihemispheral hemodynamic failure is independently associated with cognitive impairment among participants in the National Institute of Neurological Disorders and Stroke–sponsored, multicenter, randomized clinical trial, Randomized Evaluation of Carotid Occlusion and Neurocognition (RECON). Methods: Forty-three patients were randomized into RECON after recent symptomatic carotid artery occlusion and asymmetrically increased oxygen extraction fraction (OEF) by PET (OEF ratio >1.13), indicating stage II hemodynamic failure on the side of occlusion. The PET-positive patients were compared with 28 RECON-enrolled patients who met all clinical and radiographic inclusion/exclusion criteria but had no OEF asymmetry. A multivariable regression compared patients with PET OEF >1.13 or ≤1.13, stratifying by TIA vs stroke as the qualifying event. The dependent variable was a composite neurocognitive score derived from averaging age-normalized z scores on a test battery that included global and internal carotid artery (ICA) side-relevant hemisphere-specific tests. Results: There were no differences in demographic, clinical, or radiologic characteristics between the PET-positive and PET-negative patients except for PET OEF asymmetry. The unadjusted average neurocognitive z score was −1.45 for the PET-positive and −1.25 for the PET-negative patients, indicating cognitive impairment in both groups but no difference between them ( p = 0.641). After adjustment for age, education, side of occlusion, depression, and previous stroke, there was a significant difference between PET-positive and PET-negative patients among those with TIA as a qualifying event (average z score = −1.41 vs −0.76, p = 0.040). Older age and right ICA side were also significant in this model. Conclusion: Hemodynamic failure is independently associated with cognitive impairment in patients with carotid occlusion. This finding establishes the physiologic parameter upon which the extracranial-intracranial bypass will be tested. Neurology ® 2012;78:250–255

Purpose To test the hypothesis that the evaluation of retinal structure can have diagnostic value in differentiating between incomplete congenital stationary night blindness (CSNB2) and retinitis pigmentosa (RP). To compare retinal thickness differences between patients with CSNB2 and myopic controls. Design Prospective cross-sectional study. Methods Ten eyes of 5 patients diagnosed with CSNB2 (4 X-linked recessive, 1 autosomal recessive) and 6 eyes of 3 patients with RP (2 autosomal dominant, 1 autosomal recessive) were evaluated with spectral-domain optical coherence tomography (SD OCT) and fundus autofluorescence (FAF). Diagnoses of CSNB2 and RP were confirmed by full-field electroretinography (ERG). Manual segmentation of retinal layers, aided by a computer program, was performed by 2 professional segmenters on SD OCT images of all CSNB2 patients and 4 age-similar, normal myopic controls. Seven patients were screened for mutations with congenital stationary night blindness and RP genotyping arrays. Results Patients with CSNB2 had specific findings on SD OCT and FAF that were distinct from those found in RP. CSNB2 patients showed qualitatively normal SD OCT results with preserved photoreceptor inner segment/outer segment junction, whereas this junction was lost in RP patients. In addition, CSNB2 patients had normal FAF images, whereas patients with RP demonstrated a ring of increased autofluorescence around the macula. On SD OCT segmentation, the inner and outer retinal layers of both X-linked recessive and autosomal recessive CSNB2 patients were thinner compared with those of normal myopic controls, with means generally outside of normal 95% confidence intervals. The only layers that demonstrated similar thickness between CSNB2 patients and the controls were the retinal nerve fiber layer and, temporal to the fovea, the combined outer segment layer and retinal pigment epithelium. A proband and his 2 affected brothers from a family segregating X-linked recessive CSNB2 had a mutation, p.R614X, in the gene encoding calcium channel, α 1F subunit. Conclusions CSNB2 patients (X-linked recessive and autosomal recessive) had significantly thinner retinas than myopic controls. However, they demonstrated qualitatively normal SD OCT and FAF images, and therefore can be differentiated from RP patients with these techniques. Although ERG testing remains the gold standard for the diagnosis of these conditions, FAF and SD OCT systems are more widely available to community ophthalmologists, offer shorter acquisition times, and, unlike ERG, can be performed on the same day as the initial clinic visit. Therefore, as a supplement to ERG and genetic testing, we advocate the use of FAF and SD OCT in the examination of patients with CSNB2 and RP.

A benign orbital perineurioma was discovered just posterior to the lacrimal sac during surgery for recurrent bacterial dacryocystitis in an 83-year-old woman. The tumor was circumscribed but nonencapsulated and located exterior to the lacrimal sac mucosa. It was composed of cytologically bland spindle cells organized in graceful, elongated, and twisted bundles reminiscent of a storiform pattern. Immunohistochemical staining displayed a diagnostic pattern of Claudin-1, GLUT-1, and epithelial membrane antigen positivity. Malignant transformation is virtually unknown, even with incomplete excision. The differential diagnosis includes neurofibroma, schwannoma, leiomyoma, solitary fibrous tumor (CD34 positive), and low-grade fibromyxoid sarcoma.

The Parkes pulsar data archive currently provides access to 144044 data files obtained from observations carried out at the Parkes observatory since the year 1991. Around 10^5 files are from surveys of the sky, the remainder are observations of 775 individual pulsars and their corresponding calibration signals. Survey observations are included from the Parkes 70cm and the Swinburne Intermediate Latitude surveys. Individual pulsar observations are included from young pulsar timing projects, the Parkes Pulsar Timing Array and from the PULSE@Parkes outreach program. The data files and access methods are compatible with Virtual Observatory protocols. This paper describes the data currently stored in the archive and presents ways in which these data can be searched and downloaded., Accepted by PASA