Category: Peer-reviewed

Wide-Field Fundus Autofluorescence Imaging of Retinitis Pigmentosa – Corrected Proof

Purpose: To evaluate the clinical usefulness of wide-field fundus autofluorescence (FAF) imaging in patients with retinitis pigmentosa (RP).Design: Cross-sectional case series.Participants: Seventy-five eyes of 75 patients with RP.Methods: We examined the eyes of the RP patients using the Optos 200Tx imaging system (Optos PLC, Dunfermline, United Kingdom) and identified abnormal FAF patterns such as ring hyperautofluorescence and patchy hypoautofluorescent areas. Patients with hyperautofluorescent rings or foveal hyperautofluorescence were compared with those without such findings. We determined the percentage area occupied by the FAF abnormalities within a defined region of the eye and examined the relationship between the percentage area of these abnormalities and the visual field area. Moreover, we categorized the patients into 3 different groups based on the presence of a patchy hypoautofluorescent lesion larger than 1 disc diameter: Group A consisted of those with patchy lesions smaller than 1 disc diameter, group B consisted of those with patchy lesions larger than 1 disc diameter but present in only 1 quadrant, and group C consisted of those with patchy lesions larger than 1 disc diameter and present in more than 1 quadrant. In addition, various clinical characteristics were compared among these 3 groups.Main Outcome Measures: Predicting the visual field size and duration of the disease in RP patients based on FAF patterns.Results: Patients without hyperautofluorescent rings or foveal hyperautofluorescence had better visual acuity or mean deviation measured with a Humphrey perimeter. The total area of the abnormal FAF image correlated with the visual field area measured with a Goldmann perimeter (R = −0.64, P<0.001). The individuals with the large patchy hypofluorescent areas (i.e., larger than 1 disc diameter) were older than those with small patchy hypofluorescent areas (group A vs. groups B and C, P = 0.002 and P<0.001, respectively) and had experienced the symptoms for longer durations (group A vs. groups B and C, P<0.05 and P<0.001, respectively).Conclusions: We can estimate the visual field in patients with RP using the objective measurements from wide-field FAF. The presence of patchy hypofluorescent lesions can be used an indicator of the duration of RP.Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Characterization of a Clinician–Scientist Cohort in Ophthalmology: A Demographic Analysis of K Grant Awardees in Ophthalmology – Corrected Proof

Objective: To analyze ophthalmologists who are National Institutes of Health (NIH) K grant awardees to characterize clinician–scientists in ophthalmology.Design: Cohort study.Participants: Ophthalmologists who have received a K award from 1996 through 2010.Methods: K08 and K23 grant awardees were identified through the NIH Research Portfolio Online Reporter database. Information including gender, institution, educational degrees, and success in obtaining an R01 grant was analyzed.Main Outcome Measures: Receipt of an R01 grant.Results: One hundred five ophthalmologists were identified who received K08 or K23 grants from the National Eye Institute from 1996 through 2010. Overall, 75% of these were male, although 43% of K awardees were women from 2006 through 2010. Sixty-five percent (68/105) of individuals came from ophthalmology departments that ranked in the top 20 of NIH funding in 2010. The most predominant subspecialties represented were retina (32%), cornea (22%), and glaucoma (15%). Among the K awardees, 40% (42/105) had a doctor of philosophy (PhD) degree in addition to their medical doctor degree. From 1996 through 2000, 61% (23/38) were successful in obtaining an R01 grant, whereas only 13% (5/39) from 2001 through 2005 obtained R01 grants (P<0.001).Conclusions: Gender disparities exist among K awardees in ophthalmology, but these seem to be improving over time. None of the other variables studied—gender, PhD degree, specialty, or funding tier—was associated with obtaining R01 funding. This study helps to characterize the clinician–scientist cohort in ophthalmology and to identify areas to improve the recruitment of these individuals.Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Cataract Extraction Outcomes and the Prevalence of Zonular Insufficiency in Retinitis Pigmentosa – Corrected Proof

Purpose: To examine surgical outcomes in patients with retinitis pigmentosa (RP) undergoing phacoemulsification cataract extraction.Design: Retrospective observational case series.Patients And Methods: In this single-institution study of consecutive RP patients who underwent phacoemulsification cataract extraction and intraocular lens implantation by a single surgeon between 2002 and 2012, preoperative, intraoperative, and postoperative records were analyzed with attention to best-corrected visual acuity (BCVA), lens and zonular status, capsular tension ring use, incidence of posterior capsular opacification and neodymium-doped yttrium-aluminum-garnet (YAG) laser capsulotomy, and surgical complications.Results: Eighty eyes of 47 RP patients (21 male) underwent cataract surgery during the study period at an average age of 48.9 years (range, 31-78 years). Mean follow-up time was 23.3 months (range, 1 day – 95 months). Posterior subcapsular cataracts were present in 97.5% of patients. Mean BCVA improved from 20/340 (logarithm of the minimal angle of resolution [logMAR] 1.23) to 20/129 (0.81) within 3 months of surgery, P < .0001. Eyes with a preoperative vision of 20/40 to 20/200 (47 eyes) improved from a mean of 20/81 (logMAR 0.61) preoperatively to 20/43 (0.33), P < .0001, postoperatively. Posterior capsule opacification occurred in 66 eyes (82.5%), and 42 eyes (52.5%) underwent a YAG laser capsulotomy at a mean of 10.8 months after surgery. Fifteen eyes (18.8%) of 10 patients (21.3%) had signs of phacodonesis (3 eyes noted preoperatively, 8 intraoperatively, and 4 postoperatively). One patient had bilateral dislocated in-the-bag intraocular lenses at 5.5 years and 6 years after surgery.Conclusion: Cataract surgery yields significantly improved Snellen visual acuity in a majority of RP patients with a preoperative vision of 20/200 or better. Conversely, patients with a preoperative visual acuity of 20/400 or worse generally have more limited objective improvements, likely because of macular involvement, but usually report noticeable subjective improvement. A high prevalence of zonular instability is seen in RP patients undergoing cataract extraction. It is therefore important to conduct a careful preoperative assessment of lens stability with preparation for adjunctive methods that augment intraoperative and postoperative lens stabilization.

Incidence and Progression of Epiretinal Membranes in Eyes After Cataract Surgery – Corrected Proof

Purpose: To assess eye-specific epiretinal membrane (ERM) incidence 3 years after phacoemulsification surgery, and ERM detection bias attributable to cataract.Design: Cohort study.Methods: We recruited 1932 cataract surgical patients aged ≥64 years at Westmead Hospital (2004-2007). The surgical eye of each patient was assessed for presence of cellophane reflex or preretinal fibrosis at preoperative and 1-month-postoperative visits, and annually thereafter, using retinal image grading. Agreement on ERM detection between preoperative and 1-month-postoperative visits was assessed using kappa statistics. Cumulative incidence of ERM from 1 month to 3 years postoperatively was estimated using Kaplan-Meier methods and compared to the 5-year incidence of idiopathic ERM in right eyes of age-matched Blue Mountains Eye Study (BMES) participants.Results: ERM prevalence was 13.9% among 1394 participants with retinal photographs taken 1 month postoperatively. Of 1040 participants with retinal photographs from both preoperative and 1-month-postoperative visits, ERM was detected in 3.1% and 14.8%, respectively, with low diagnostic agreement (kappa = 0.17). Of 1119 subjects without ERM 1 month post surgery, the 3-year cumulative incidence of ERM was 11.2% (95% confidence interval [CI], 9.4%-13.4%; cellophane reflex 6.6%; preretinal fibrosis 4.2%). The age-standardized 3-year incidence of ERM in the surgical cohort (12.1%, 95% CI 8.6%-16.9%) was higher than the 5-year incidence of the BMES subsample (4.4%, 95% CI 3.0%-6.0%).Conclusions: A substantial under-detection of ERM in eyes before cataract surgery could incorrectly contribute to ERM incidence after surgery. Over 3 years, ERM developed in >10%, including preretinal fibrosis in 4%, of surgical eyes free of ERM 1 month post surgery.

Comparisons of Outcomes With Different Intervals Between Adjunctive Ranibizumab and Photodynamic Therapy for Polypoidal Choroidal Vasculopathy – Corrected Proof

Purpose: To determine the optimal time for administration of intravitreal ranibizumab injections before photodynamic therapy (PDT) as combined therapy to treat polypoidal choroidal vasculopathy (PCV).Design: Retrospective, comparative, interventional case series.Methods: The study included 99 eyes (98 patients) with treatment-naïve subfoveal PCV treated with an intravitreal ranibizumab injection followed by PDT. The combination therapy included 1 ranibizumab injection administered 7 days before PDT (7-day group) or 2 days before PDT (2-day group). All eyes were followed for over 12 months.Results: Intravitreal ranibizumab was administered 7 days before PDT in 59 eyes and 2 days before PDT in 40 eyes. In the 7-day group, the best-corrected visual acuity (BCVA) did not improve significantly at 3 months (P = .086) or 12 months (P = .259) compared with baseline. In the 2-day group, BCVA improved significantly at 3 months (P < .001) and 12 months (P < .001). The polypoidal lesions regressed completely in 46 eyes (78.0%) in the 7-day group and in 34 eyes (85.0%) in the 2-day group; 38 eyes (64.4%) and 35 eyes (87.5%), respectively, did not require additional treatment, which differed significantly (P = .008) between the 2 groups. Subretinal hemorrhages did not develop in either group within 1 month after the combined therapy.Conclusions: Administration of an intravitreal ranibizumab injection 2 days before PDT achieves significantly better visual outcomes and requires fewer additional treatments compared with administration of the injection 7 days before PDT.

Ocular Biometric Parameters Associated With Intraocular Pressure Reduction After Cataract Surgery in Normal Eyes – Corrected Proof

Purpose: To evaluate the ocular biometric parameters associated with intraocular pressure (IOP) reduction after phacoemulsification.Design: Prospective, observational case series.Methods: The study included 999 patients who had undergone uncomplicated phacoemulsification. IOP and ocular biometric parameters were checked preoperatively and 3 months postoperatively using anterior segment optical coherence tomography, optical biometry, and ultrasonic biomicroscopy. The relationship between IOP change and the parameters, including preoperative IOP, anterior chamber depth, axial length, angle opening distance at 500 μm, anterior chamber area, corneal thickness, lens thickness, and iris thickness at 750 μm, was evaluated.Results: The mean patient age was 67.1 ± 4.3 years. The average change in IOP was −1.6 mm Hg (−11.8%). In univariate analysis, axial length, corneal thickness, and iris thickness were not significantly associated with IOP reduction. However, preoperative IOP, anterior chamber depth, angle opening distance, anterior chamber area, and lens thickness were significantly associated with IOP change (P < .05). Furthermore, changes in anterior chamber depth (standardized coefficient beta [B] = −0.082), angle opening distance (B = −0.095), and anterior chamber area (B = −0.380) were more strongly correlated with IOP change than were preoperative factors (B = −0.078, B = −0.071, and B = −0.067, respectively). In multivariate analysis, preoperative IOP, lens thickness, angle opening distance change, and anterior chamber area change were significantly associated with IOP change (P < .005).Conclusion: In addition to preoperative IOP and lens thickness, parameters such as changes in anterior chamber area and angle opening distance were significantly associated positively with reduced IOP after phacoemulsification.

The Value of Combining Autofluorescence and Optical Coherence Tomography in Predicting the Visual Prognosis of Sealed Macular Holes – Corrected Proof

Purpose: To investigate the autofluorescence patterns of sealed macular holes and determine the correlations of the postoperative changes in spectral-domain optical coherence tomography and fundus autofluorescence (FAF) with the best-corrected visual acuity (BCVA) of patients with sealed idiopathic macular holes.Design: Retrospective consecutive observational case series.Methods: Seventy-seven eyes from 75 consecutive patients who had undergone successful vitrectomies for idiopathic macular holes by a single surgeon with postoperative follow-ups for over 1 year were included. FAF imaging studies were performed 1 month and 6 months post surgery, and optical coherence tomography (OCT) was performed at regular intervals after surgery. The main outcome measures were macular hole size, FAF patterns, retina outer segment from OCT, and BCVA before and after operation.Results: The patients who had intact external limiting membranes (ELMs) at 1 month after surgery had better BCVAs than those without (P < .001), regardless of the photoreceptor inner segment/outer segment (IS/OS) condition. The FAF patterns were divided into 3 groups: homogenous hyperfluorescence, patchy hyperfluorescence, and normal hypofluorescence. No significant BCVA differences were noted (P = .28) among the 3 groups. However, significantly better BCVAs were noted in the patients who had both intact ELMs and normal hypofluorescence patterns (P = .011).Conclusions: In sealed macular holes, intact ELMs predicted good postoperative BCVA. In this subgroup, a hypofluorescence pattern on the FAF imaging predicted the best postoperative BCVA.

Characteristics of Incident Geographic Atrophy in the Complications of Age-Related Macular Degeneration Prevention Trial – Corrected Proof

Objective: To characterize the size, location, conformation, and features of incident geographic atrophy (GA) as detected by annual stereoscopic color photographs and fluorescein angiograms (FAs).Design: Retrospective cohort study within a larger clinical trial.Participants: Patients with bilateral large drusen in whom GA developed during the course of the Complications of Age-related Macular Degeneration Prevention Trial (CAPT).Methods: Annual stereoscopic color photographs and FAs were reviewed from 114 CAPT patients in whom GA developed in the untreated eye during 5 to 6 years of follow-up. Geographic atrophy was defined according to the Revised GA Criteria for identifying early GA.23 Color-optimized fundus photographs were viewed concurrently with the FAs during grading.Main Outcome Measures: Size and distance from the fovea of individual GA lesions, number of areas of atrophy, and change in visual acuity (VA) when GA first developed in an eye.Results: At presentation, the median total GA area was 0.26 mm2 (0.1 disc area). Geographic atrophy presented as a single lesion in 89 (78%) eyes. The median distance from the fovea was 395 μm. Twenty percent of incident GA lesions were subfoveal and an additional 18% were within 250 μm of the foveal center. Development of GA was associated with a mean decrease of 7 letters from the baseline VA level compared with 1 letter among matched early age-related macular degeneration eyes without GA. Geographic atrophy that formed in areas previously occupied by drusenoid pigment epithelial detachments on average were larger (0.53 vs. 0.20 mm2; P = 0.0001), were more central (50 vs. 500 μm from the center of the fovea; P<0.0001), and were associated with significantly worse visual outcome (20/50 vs. 20/25; P = 0.0003) than GA with other drusen types as precursors.Conclusions: Incident GA most often appears on color fundus photographs and FAs as a small, singular, parafoveal lesion, although a large minority of lesions are subfoveal or multifocal at initial detection. The characteristics of incident GA vary with precursor drusen types. These data can facilitate design of future clinical trials of therapies for GA.Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Evaluation of Lamina Cribrosa in Pseudoexfoliation Syndrome Using Spectral-Domain Optical Coherence Tomography Enhanced Depth Imaging – Corrected Proof

Purpose:
To evaluate the features of the lamina cribrosa (LC) in pseudoexfoliation glaucoma (PXG) patients using enhanced depth imaging (EDI) of spectral-domain optical coherence tomography (SD OCT). The results were compared with those of patients with primary open-angle glaucoma (POAG).

Design:
Cross-sectional study.

Participants:
Patients with PXG (n = 21) and POAG (n = 35) matched for age and visual field mean deviation (VF MD).

Methods:
Participants were imaged using SD OCT. Lamina cribrosa thickness (LT) and anterior lamina cribrosa depth (ALD) were determined at 3 areas (mid superior, center, and mid inferior) by 2 examiners using an EDI mode of the optic nerve head.

Main Outcome Measures:
The LT and ALD were compared between PXG and POAG eyes.

Results:
Mean ± standard deviation baseline untreated intraocular pressure was not significantly different between the 2 groups (PXG, 18.3±8.2 mmHg; POAG, 15.3±3.4 mmHg; P = 0.310). The mean VF MD was −12.7±9.0 dB in the PXG group versus −11.6±9.1 dB in the POAG group (P = 0.643). When compared with the POAG group, the PXG group demonstrated a significantly thinner LT in all 3 areas and a thinner mean LT (133.4±14.5 μm in the POAG group vs. 121.3±13.0 μm in the PXG group; P<0.001). Anterior lamina cribrosa depth did not demonstrate a significant difference in any of the 3 areas between both groups (mean ALD, 324.3±91.9 μm in the POAG group vs. 358.7±142.7 μm in the PXG group; P = 0.470). Of 21 eyes in the PXG group, 9 eyes demonstrated a unilateral clinical presentation. When we compared the PXG eyes and the apparently normal-looking fellow eyes of those 9 eyes, neither the LT nor ALD demonstrated a significant difference (P = 0.223 and P = 0.079, respectively).

Conclusions:
Eyes with PXG demonstrate a thinner LC compared with POAG eyes at similar levels of glaucoma severity.

Financial Disclosure(s):
The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Pseudoexfoliation: Normative Data and Associations: The Beijing Eye Study 2011 – Corrected Proof

Objective: To assess the prevalence of pseudoexfoliation syndrome (PEX) and its associations in a population-based setting.Design: Population-based, cross-sectional cohort study.Participants: Of 4403 eligible subjects with an age of ≥50 years, 3468 individuals (78.8%) participated in the Beijing Eye Study 2011 (mean age, 64.6±9.8 years; range, 50–93 years).Methods: All study participants underwent a detailed ophthalmologic examination. After medical pupil dilation, PEX was assessed by an experienced ophthalmologist using slit-lamp-based biomicroscopy.Main Outcome Measures: Prevalence and associations of PEX.Results: Slit-lamp examination results were available for 3022 study participants (87.1%). Definite pseudoexfoliation was observed in 72 of the 3022 subjects, with a prevalence of 2.38% (95% confidence interval [CI], 1.84–2.93). Suspected PEX was detected in 104 of the subjects (3.44%; 95% CI, 2.8–4.1). The overall prevalence of PEX (definite and suspected) was 176 of 3022 or 5.82% (95% CI, 4.99–6.66). In 80 subjects (45.5%), PEX was detected in both eyes, whereas it was detected only in the right eye in 42 subjects (23.9%) and only in the left eye in 54 (30.7%). The prevalence of PEX increased from 1.1% in among those 50 to 54 years old, to 3.5%, 5.7%, and 11.8% among those 60 to 64 years, 70 to 74 years, and ≥80 years, respectively. In multivariate analysis, presence of PEX was significantly associated with older age (P<0.001; odds ratio [OR], 1.08; 95% CI, 1.04–1.10), shorter axial length (P = 0.03; OR, 0.82; 95% CI, 0.68,0.98), and shallower anterior chamber (P = 0.03; OR, 0.59; 95% CI, 0.36–0.95). We found that PEX was not associated (all P>0.05) with sex, diabetes mellitus, blood pressure, psychological depression, smoking, dyslipidemia, body mass index, central corneal thickness, corneal diameter, optic nerve head measurements, choroidal thickness, retinal vessel diameters, early age-related macular degeneration, or retinal vein occlusion.Conclusions: In a North Chinese population aged ≥50 years, the prevalence of definite PEX was 2.38% (95% CI, 1.84–2.93), suspect PEX was 3.4% (95% CI, 2.8–4.1) and overall PEX was 5.82% (95% CI, 4.99–6.66). We found PEX to be associated with older age, shorter axial length, and shallower anterior chamber. The relationship between PEX and glaucomatous optic neuropathy remained inconclusive among our population.Financial Disclosure(s): The authors have no proprietary or commercial interest in any materials discussed in this article.

A National Survey of Practice Patterns: Temporal Artery Biopsy – Corrected Proof

Objective: To assess current clinical practice patterns for temporal artery biopsy (TAB) among clinicians in establishing the diagnosis of giant cell arteritis.Design: A survey was sent via e-mail using the Survey Monkey website (www.surveymonkey.com; accessed January 24, 2013). The survey initially was sent in July 2010 and continued through October 2010.Participants: The survey was sent via e-mail to the members and affiliates of the American Society of Ophthalmic Plastic and Reconstructive Surgery, the North American Neuro-Ophthalmology Society, and the American College of Rheumatology.Methods: Data from the survey were collected via Survey Monkey and data analysis was performed using the Fisher exact test and Wilcoxon rank-sum test.Main Outcome Measures: Response to the survey questions on primary unilateral versus bilateral biopsy, performing second-side biopsy if first side results were negative, and the duration for which biopsy findings are reliable after initiating immunosuppressive therapy.Results: The self-described primary subspecialty of the 1074 respondents was as follows: oculoplastic surgery (n = 127), neuro-ophthalmology (n = 119), rheumatology (n = 799), and other (n = 28). Overall, 66% of respondents advocated initial unilateral TAB, 18% advocated bilateral biopsy in all cases, and 16% recommended either unilateral or bilateral TAB depending on the degree of clinical suspicion. Rheumatologists were 4.5 times more likely to advocate initial bilateral biopsy than neuro-ophthalmologists or oculoplastic surgeons (P<0.0001, Fisher exact test). Most respondents believed that biopsy results were accurate for more than 14 days. These results were not affected by stratification of years in practice by the Kruskal-Wallis rank-sum test.Conclusions: Temporal artery biopsy practices vary greatly among treating physicians. This lack of consensus underscores the need for a systematic assessment of varying practice patterns.Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Macular telangiectasia type 2

Publication date: May 2013
Source:Progress in Retinal and Eye Research, Volume 34
Author(s): Peter Charbel Issa , Mark C. Gillies , Emily Y. Chew , Alan C. Bird , Tjebo F.C. Heeren , Tunde Peto , Frank G. Holz , Hendrik P.N. Scholl
Macular telangiectasia type 2 is a bilateral disease of unknown cause with characteristic alterations of the macular capillary network and neurosensory atrophy. Its prevalence may be underestimated and has recently been shown to be as high as 0.1% in persons 40 years and older. Biomicroscopy may show reduced retinal transparency, crystalline deposits, mildly ectatic capillaries, blunted venules, retinal pigment plaques, foveal atrophy, and neovascular complexes. Fluorescein angiography shows telangiectatic capillaries predominantly temporal to the foveola in the early phase and a diffuse hyperfluorescence in the late phase. High-resolution optical coherence tomography (OCT) may reveal disruption of the photoreceptor inner segment–outer segment border, hyporeflective cavities at the level of the inner or outer retina, and atrophy of the retina in later stages. Macular telangiectasia type 2 shows a unique depletion of the macular pigment in the central retina and recent therapeutic trials showed that such depleted areas cannot re-accumulate lutein and zeaxanthin after oral supplementation. There have been various therapeutic approaches with limited or no efficacy. Recent clinical trials with compounds that block vascular endothelial growth factor (VEGF) have established the role of VEGF in the pathophysiology of the disease, but have not shown significant efficacy, at least for the non-neovascular disease stages. Recent progress in structure–function correlation may help to develop surrogate outcome measures for future clinical trials.In this review article, we summarize the current knowledge on macular telangiectasia type 2, including the epidemiology, the genetics, the clinical findings, the staging and the differential diagnosis of the disease. Findings using retinal imaging are discussed, including fluorescein angiography, OCT, adaptive optics imaging, confocal scanning laser ophthalmoscopy, and fundus autofluorescence, as are the findings using visual function testing including visual acuity and fundus-controlled microperimetry. We provide an overview of the therapeutic approaches for both non-neovascular and neovascular disease stages and provide a perspective of future directions including animal models and potential therapeutic approaches.

Highlights

► Macular telangiectasia (MacTel) type 2 is a neurodegenerative disease affecting the central retina. ► Its prevalence has long been underestimated and may be as high as 0.1%. ► There is central depletion of macular pigment and hyporeflective retinal cavities on OCT imaging. ► Vascular endothelial growth factor (VEGF) appears to play a significant role in the pathophysiology. ► Anti-VEGF therapy may show efficacy in neovascular disease only.