Alice T. Lyon

Idiopathic polypoidal choroidal vasculopathy of the macula

OBJECTIVE The authors evaluated the clinical, fluorescein, and indocyanine green (ICG) angiographic characteristics of the macular variant of idiopathic polypoidal choroidal vasculopathy (IPCV). DESIGN Observational case series. PARTICIPANTS The records, photographs, and fluorescein and ICG angiograms of eight eyes of seven patients with IPCV lesions confined to the macula were reviewed. MAIN OUTCOME MEASURES The visual acuity, fundus examination, fluorescein and ICG angiographic characteristics, and clinical course were compared. RESULTS All patients demonstrated polypoidal lesions arising from macular choroidal vessels on ICG angiography. One patient had bilateral lesions. These lesions appeared hyperfluorescent in the early phases of both fluorescein and ICG angiography. Late-phase leakage was seen in cases associated with subretinal fluid or exudate. None of these patients demonstrated polypoidal lesions arising from the peripapillary choroidal circulation or peripapillary choroidal neovascularization. Three eyes with polypoidal lesions that were associated with subretinal fluid and exudates were treated with photocoagulation. Five eyes were not treated. Final visual acuity ranged from 20/20 to hand motions. Severe visual loss was associated with vitreous and subretinal hemorrhage, but this resolved without permanent severe visual loss in several cases. CONCLUSIONS In the macular variant of IPCV, ICG and fluorescein angiography demonstrate characteristic macular polypoidal lesions without evidence of peripapillary lesions. The vascular origin of these polypoidal lesions appears to be the macular choroidal circulation. This is distinguished from classic IPCV, in which lesions appear to arise from the peripapillary choroidal circulation. Visual prognosis appears to be good, with most patients retaining visual acuity of 20/80 or better. If subretinal fluid or exudates reduce visual acuity, photocoagulation should be considered.

Rhegmatogenous retinal detachments in children ☆: Risk factors and surgical outcomes

PURPOSE To describe the presenting features and surgical outcomes in a series of children with rhegmatogenous retinal detachments. DESIGN Retrospective, noncomparative, interventional case series. PARTICIPANTS Thirty-nine eyes of 34 children 18 years of age or younger undergoing surgery for rhegmatogenous retinal detachment. METHODS Patients were identified by chart review at two affiliated tertiary care centers. Risk factors for retinal detachment were classified into four categories: (1). congenital or developmental structural ocular abnormalities, (2). trauma, (3). previous ophthalmologic surgery, and (4). preceding uveitis. RESULTS Median age was 10 years, and 79% of patients were boys. Nine patients (26%) had bilateral retinal detachment at presentation, or experienced a detachment in their second eye before their nineteenth birthday. Every eye had at least one risk factor for retinal detachment, and more than half had risk factors in two or more categories. Structural abnormalities were most common (56%). Fifty-one percent of eyes underwent previous surgery, 36% experienced trauma, and 15% had uveitis. Detachments tended to be complex. Median follow-up was 24 months. Retinal reattachment was achieved in 79% of eyes; however, visual recovery was modest. Median preoperative and postoperative visual acuities were counting fingers and 20/400, respectively. Predictors of a poor visual outcome were: unmeasurable or light perception-only preoperative vision (P = 0.0001), macula-off retinal detachment (P = 0.01), the need for vitrectomy surgery (P = 0.01), the presence of proliferative vitreoretinopathy grade C or worse (P = 0.02), and the use of silicone oil (P = 0.02). CONCLUSIONS Predisposing factors in pediatric retinal detachments, particularly congenital and developmental structural abnormalities, may be more common than previously reported. Modern vitreoretinal surgical techniques can help achieve retinal reattachment in most cases. Many factors contribute to the limited visual recovery in this patient population. Predictors of visual outcomes are similar to those observed in adults. Inability of the clinician to determine confidently the preoperative visual acuity is a newly identified predictor of poor visual outcomes.

Course of Cytomegalovirus Retinitis in the Era of Highly Active Antiretroviral Therapy: Five-Year Outcomes

PURPOSE To describe the 5-year outcomes of patients with cytomegalovirus (CMV) retinitis and AIDS in the era of highly active antiretroviral therapy (HAART). DESIGN Prospective, multicenter, observational study. PARTICIPANTS A total of 503 patients with AIDS and CMV retinitis. METHODS Follow-up every 3 months with medical history, ophthalmologic examination, laboratory testing, and retinal photographs. Participants were classified as having previously diagnosed CMV retinitis and immune recovery (CD4+ T cells ≥ 100 cells/μl), previously diagnosed retinitis and immune compromise, and newly diagnosed CMV retinitis (diagnosis <45 days before enrollment). MAIN OUTCOME MEASURES Mortality, retinitis progression (movement of the border of a CMV lesion ≥ ½ disc diameter or occurrence of a new lesion), retinal detachment, immune recovery uveitis (IRU), and visual loss (< 20/40 and ≥ 20/200). RESULTS Overall mortality was 9.8 deaths/100 person-years (PY). Rates varied by group at enrollment from 3.0/100 PY for those with previously diagnosed retinitis and immune recovery to 26.1/100 PY for those with newly diagnosed retinitis. The rate of retinitis progression was 7.0/100 PY and varied from 1.4/100 PY for those with previously diagnosed retinitis and immune recovery to 28.0/100 PY for those with newly diagnosed retinitis. The rate of retinal detachment was 2.3/100 eye-years (EY) and varied from 1.2/100 EY for those with previously diagnosed retinitis and immune recovery to 4.9/100 EY for those with newly diagnosed retinitis. The rate of IRU was 1.7/100 PY and varied from 1.3/100 PY for those with previously diagnosed retinitis and immune recovery at enrollment to 3.6/100 PY for those with newly diagnosed retinitis who subsequently experienced immune recovery. The rates of visual loss to < 20/40 and to ≤ 20/200 were 7.9/100 EY and 3.4/100 EY, respectively; they varied from 6.1/100 EY and 2.7/100 EY for those with previously diagnosed retinitis and immune recovery to 11.8/100 EY and 5.1/100 EY for those with newly diagnosed retinitis. Although the event rates tended to decline with time, in general, at no time did they reach zero. CONCLUSIONS Despite the availability of HAART, patients with AIDS and CMV retinitis remain at increased risk for mortality, retinitis progression, complications of the retinitis, and visual loss over a 5-year period. FINANCIAL DISCLOSURE(S) Proprietary or commercial disclosure may be found after the references.

Visual and anatomical outcomes following intravitreal aflibercept in eyes with recalcitrant neovascular age-related macular degeneration: 12-month results

PurposeTo describe the efficacy of intravitreal aflibercept on 12-month visual and anatomical outcomes in patients with neovascular age-related macular degeneration (AMD) recalcitrant to prior monthly intravitreal bevacizumab or ranibizumab.MethodsNon-comparative case series of 21 eyes of 21 AMD patients with evidence of persistent exudation (intraretinal fluid/cysts, or subretinal fluid (SRF), or both) on spectral domain OCT despite ≥6 prior intravitreal 0.5 mg ranibizumab or 1.25 mg bevacizumab (mean 29.8±17.1 injections) over 31.6±17.4 months who were transitioned to aflibercept.ResultsAt baseline, best-corrected visual acuity (BCVA) was 0.42±0.28 logarithm of minimum-angle of resolution (logMAR), central foveal thickness (CFT) was 329.38±102.67 μm and macular volume (MV) was 7.71±1.32 mm3. After 12 months of aflibercept (mean 10.2±1.2 injections), BCVA was 0.40±0.28 logMAR (P=0.5), CFT decreased to 292.71±91.35 μm (P=0.038) and MV improved to 7.33±1.27 mm3 (P=0.003). In a subset of 15 eyes with a persistent fibrovascular or serous pigment epithelial detachment (PED), mean baseline PED greatest basal diameter (GBD) was 2350.9±1067.6 μm and mean maximal height (MH) was 288.7±175.9 μm. At 12 months, GBD improved to 1896.3±782.3 μm (P=0.028), while MH decreased to 248.27±146.2 μm (P=0.002).ConclusionIn patients with recalcitrant AMD, aflibercept led to anatomic improvement at 12 months, reduction in proportion of eyes with SRF and reduction in PED, while preserving visual acuity.

Laser treatment in fellow eyes with large drusen: Updated findings from a pilot randomized clinical trial

PURPOSE To update the findings from the Choroidal Neovascularization Prevention Trial (CNVPT) with respect to resolution of drusen, incidence of choroidal neovascularization, and visual function. DESIGN A multicenter, randomized, controlled, pilot clinical trial. PARTICIPANTS The 120 patients enrolled in the CNVPT. Patients had signs of choroidal neovascularization or retinal pigment epithelial detachment in 1 eye and had >/=10 large (>63- micro m) drusen in the contralateral, or fellow, eye. INTERVENTION The fellow eye of 59 patients was assigned randomly to argon green laser treatment consisting of multiple 100- micro m spots at least 750 micro m from the center of the fovea. The fellow eye of the remaining 61 patients was assigned randomly to observation. MAIN OUTCOME MEASURES Change in visual acuity was the primary outcome measure. Incidence of choroidal neovascularization, resolution of drusen, change in contrast threshold, change in critical print size for reading, and incidence of geographic atrophy were secondary outcome measures. RESULTS Throughout 4 years of follow-up, there were no statistically significant differences in change in visual acuity, contrast threshold, critical print size, or incidence of geographic atrophy. With additional follow-up, the large increase in the incidence of choroidal neovascularization observed within 18 months of treatment was maintained; however, by 30 months, the incidence in the two treatment groups was the same. Most drusen resolution in treated eyes occurred within 24 months of the initial treatment. Treated eyes that received higher-intensity laser burns had an increased risk of choroidal neovascularization. Among eyes developing choroidal neovascularization in each treatment group, most lesions (two thirds or more) were composed of occult neovascularization only. CONCLUSIONS Laser treatment as applied in the CNVPT caused an excess risk of choroidal neovascularization in the first year or so after treatment. The increased early incidence of choroidal neovascularization was not associated with either a harmful or beneficial effect in this pilot study.

Association Between Abnormal Contrast Sensitivity and Mortality Among People With Acquired Immunodeficiency Syndrome

PURPOSE To investigate the relationship between contrast sensitivity (CS) and mortality among people with acquired immunodeficiency syndrome (AIDS); and to explore the hypothesis that abnormal CS is a marker of systemic, life-threatening microvascular disease. DESIGN Longitudinal, observational cohort study. METHODS We evaluated 3395 eyes of 1706 individuals enrolled in the Longitudinal Study of the Complications of AIDS (1998-2008). CS was evaluated as a risk factor for death, and was compared to the presence of systemic diseases characterized by microvasculopathy (diabetes, cardiovascular disease, stroke, renal disease) and to laboratory markers of those diseases. Abnormal CS was defined as logCS <1.5 (lower 2.5th percentile for a normal control population). RESULTS CS was abnormal in 284 of 1691 (16.8%) study participants at enrollment. There was a positive relationship between the presence of abnormal CS at study entry and mortality (relative risk 2.0, 95% confidence interval 1.7-2.3, P < .0001). Abnormal CS was related to the presence of cardiovascular disease, stroke, and renal disease (all P values <or= .01), but abnormal CS remained associated with death even after adjustment for these diseases and for other known predictors of death among people with AIDS. Diseases characterized by microvasculopathy were more often identified as causes of death among individuals with abnormal CS than among those with normal CS, although the strength of the association was moderate (P = .06). CONCLUSIONS Abnormal CS among people with AIDS is associated with increased mortality, and is independent of other risk factors for death that are monitored routinely. The relationship may indicate life-threatening microvascular disease in other organs.

Imaging characteristics of neovascular pigment epithelial detachments and their response to anti-vascular endothelial growth factor therapy

Purpose To evaluate the imaging characteristics of macular neovascular pigment epithelial detachments (PEDs) and their response to anti-vascular endothelial growth factor (VEGF) therapy. Methods Patients with exudative age-related macular degeneration (AMD), idiopathic polypoidal choroidal vasculopathy, presumed ocular histoplasmosis syndrome (POHS) and central serous retinopathy (CSR) with choroidal neovascularisation (CNV) were included in the study. A retrospective chart review of 72 eyes of 64 patients was performed. Results Three types of PEDs were identified based on reflectivity of the material under the retinal pigment epithelium on optical coherence tomography: hollow (26 eyes with primarily hyporeflectivity under the PED), solid (30 eyes with primarily hyperreflective signal under the PED) and mixed (8 eyes with mixed reflectivity). The average number of anti-VEGF injections was 7 per eye and the average duration of follow-up was 16 months. Among eyes with exudative AMD, 7/21 hollow PEDs flattened, 1/19 solid PEDs flattened and 2/6 mixed PEDs flattened after anti-VEGF therapy. POHS and CSR with CNV were associated with subfoveal solid PEDs and were unchanged after therapy. Overall, 46% (12/26) with hollow PEDs, 25% (2/8) with mixed PEDs and 3% (1/30) with solid PEDs had flattening after anti-VEGF therapy. Conclusions The likelihood of PED flattening was inversely related to the reflectivity of the PED. The more reflective the PED, the less likely resolution with anti-VEGF therapy occurred.

Long-term Outcomes of Cytomegalovirus Retinitis in the Era of Modern Antiretroviral Therapy: Results from a United States Cohort

PURPOSE To describe the long-term outcomes of patients with cytomegalovirus (CMV) retinitis and AIDS in the modern era of combination antiretroviral therapy. DESIGN Prospective, observational cohort study. PARTICIPANTS Patients with AIDS and CMV retinitis. METHODS Immune recovery, defined as a CD4+ T-cell count >100 cells/μl for ≥3 months. MAIN OUTCOME MEASURES Mortality, visual impairment (visual acuity <20/40), and blindness (visual acuity ≤20/200) on logarithmic visual acuity charts and loss of visual field on quantitative Goldmann perimetry. RESULTS Patients without immune recovery had a mortality of 44.4/100 person-years (PYs) and a median survival of 13.5 months after the diagnosis of CMV retinitis, whereas those with immune recovery had a mortality of 2.7/100 PYs (P < 0.001) and an estimated median survival of 27.0 years after the diagnosis of CMV retinitis. The rates of bilateral visual impairment and blindness were 0.9 and 0.4/100 PYs, respectively, and were similar between those with and without immune recovery. Among those with immune recovery, the rate of visual field loss was approximately 1% of the normal field per year, whereas among those without immune recovery it was approximately 7% of the normal field per year. CONCLUSIONS Among persons with CMV retinitis and AIDS, if there is immune recovery, long-term survival is likely, whereas if there is no immune recovery, the mortality rate is substantial. Although higher than the rates in the population not infected by human immunodeficiency virus, the rates of bilateral visual impairment and blindness are low, especially when compared with rates in the era before modern antiretroviral therapy.

Original articleLong-term Outcomes of Cytomegalovirus Retinitis in the Era of Modern Antiretroviral Therapy: Results from a United States Cohort

PURPOSE To describe the long-term outcomes of patients with cytomegalovirus (CMV) retinitis and AIDS in the modern era of combination antiretroviral therapy. DESIGN Prospective, observational cohort study. PARTICIPANTS Patients with AIDS and CMV retinitis. METHODS Immune recovery, defined as a CD4+ T-cell count >100 cells/μl for ≥3 months. MAIN OUTCOME MEASURES Mortality, visual impairment (visual acuity <20/40), and blindness (visual acuity ≤20/200) on logarithmic visual acuity charts and loss of visual field on quantitative Goldmann perimetry. RESULTS Patients without immune recovery had a mortality of 44.4/100 person-years (PYs) and a median survival of 13.5 months after the diagnosis of CMV retinitis, whereas those with immune recovery had a mortality of 2.7/100 PYs (P < 0.001) and an estimated median survival of 27.0 years after the diagnosis of CMV retinitis. The rates of bilateral visual impairment and blindness were 0.9 and 0.4/100 PYs, respectively, and were similar between those with and without immune recovery. Among those with immune recovery, the rate of visual field loss was approximately 1% of the normal field per year, whereas among those without immune recovery it was approximately 7% of the normal field per year. CONCLUSIONS Among persons with CMV retinitis and AIDS, if there is immune recovery, long-term survival is likely, whereas if there is no immune recovery, the mortality rate is substantial. Although higher than the rates in the population not infected by human immunodeficiency virus, the rates of bilateral visual impairment and blindness are low, especially when compared with rates in the era before modern antiretroviral therapy.

Quantification of Change in Pigment Epithelial Detachment Volume and Morphology After Transition to Intravitreal Aflibercept in Eyes With Recalcitrant Neovascular AMD: 18-Month Results

BACKGROUND AND OBJECTIVE To quantitatively evaluate the change in pigment epithelial detachment (PED) morphology on spectral-domain optical coherence tomography (SD-OCT) 18 months after the transition to intravitreal aflibercept in patients with neovascular age-related macular degeneration (AMD) with PED recalcitrant to monthly intravitreal bevacizumab or ranibizumab. PATIENTS AND METHODS Retrospective case series examining patients with neovascular AMD who had a persistent fibrovascular or serous PED on SD-OCT. PED volume was calculated by manually outlining the PED on individual OCT slices of the raster scan and multiplying by the pixel dimensions. RESULTS Eleven eyes of 10 patients who had received an average of 25.7 ± 20.1 (range: 6 to 70) prior bevacizumab or ranibizumab injections over a period of 26.6 ± 19.8 months (range: 4 to 63) were included. PED volume decreased with aflibercept from 0.687 ± 0.837 mm(3) to 0.562 ± 0.705 mm(3) (P = .02), a decrease of 19% ± 12.27%. CONCLUSION After 18 months of aflibercept, recalcitrant PED volumes were reduced by 19% while preserving visual acuity in eyes with neovascular AMD.