Surgical Management of Advanced Coats Disease in 32 Eyes: A 20-Year Study

2020 ◽  
Vol 4 (6) ◽  
pp. 467-471
Author(s):  
Pukhraj Rishi ◽  
Ekta Rishi ◽  
Yamini Attiku ◽  
Mahesh Uparkar ◽  
Pramod Bhende ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Subretinal Fluid ◽  
Vitreous Hemorrhage ◽  
Disease Stage ◽  
Final Visit ◽  
Kaplan Meier ◽  
Coats Disease ◽  
End Stage ◽  
Anatomical Outcome

Purpose: This work studies outcomes of external subretinal fluid (SRF) drainage in management of eyes with advanced Coats disease. Methods: Patients with advanced-stage Coats disease (≥stage 3B), who were younger than 12 years and underwent external SRF drainage from 1996 to 2016, were included in this retrospective study. Surgical intervention involved external drainage of SRF and cryotherapy. SRF drainage was performed by lamellar scleral dissection or by external needle drainage. Favorable anatomical outcome was defined as retinal reattachment with normal intraocular pressure (IOP). IOP greater than 24 mm Hg was considered raised. Univariate and multivariate analyses were performed to measure the association between preoperative or intraoperative factors and retinal status at final follow-up. Outcome measures evaluated included visual acuity, IOP, retinal status, globe status, and complications of surgery. Kaplan-Meier analysis was performed for globe salvage without pain. Results: Thirty-two eyes of 32 patients were included in the study. Mean age at surgery was 3.8 ± 3 years. The mean duration of follow-up was 7 years (range, 6 months-15.7 years). Improvement in visual acuity was seen in 5 eyes. Retina was attached at final visit in 6 eyes. IOP in the range of 8 to 24 mm Hg was noted in 16 eyes. Favorable anatomical outcome was achieved in 3 (9%) eyes. Globe salvage was achieved in 84% of eyes. Complications included intraoperative vitreous hemorrhage (n = 1) and postoperative inflammation (n = 1). Kaplan-Meier ocular survival rate without pain at 10 years was 76%. Conclusions: SRF drainage and cryotherapy in eyes with advanced Coats disease favorably alter the natural history of the disease and prevent end-stage complications. Visual outcomes remain poor.

Longitudinal ellipsoid zone and subretinal fluid mapping following ocriplasmin injection in the prospective observational ORBIT trial

2019 ◽  
Vol 104 (3) ◽  
pp. 410-415
Author(s):  
Jeremy A Lavine ◽  
Sunil K Srivastava ◽  
Neeley Dukles ◽  
Jamie L Reese ◽  
Justis P Ehlers
Keyword(s):  
Optical Coherence Tomography ◽  
Visual Acuity ◽  
Subretinal Fluid ◽  
Final Visit ◽  
Volume Loss ◽  
Ellipsoid Zone ◽  
Retrospective Reports ◽  
Baseline Levels ◽  
Feature Extraction Software

BackgroundOcriplasmin is approved for the treatment of symptomatic vitreomacular traction (VMT). However, several retrospective reports have identified ellipsoid zone (EZ) alterations on spectral domain optical coherence tomography (SDOCT) after ocriplasmin injection. This report quantitatively analysed outer retinal changes after intravitreal ocriplasmin.MethodsOcriplasmin Research to Better Inform Treatment is a prospective, observational phase IV clinical study where subjects received a single intravitreal injection of ocriplasmin for symptomatic VMT. Macular cube scans were imported into a semiautomated EZ mapping and fluid feature extraction software for SDOCT analysis. Change in visual acuity, VMT release, macular hole (MH) closure, EZ integrity/volume and subretinal fluid (SRF) volume on SDOCT macular cube scans were recorded and analysed.ResultsThis analysis included 55 participants with 6 months of follow-up. Intravitreal ocriplasmin injection caused VMT release in 67% and MH closure in 82% of participants. Visual acuity improved by 4.5 letters (p<0.05) in the whole cohort and by 6.0 letters (p<0.05) in participants with VMT release. EZ volume was reduced by 23.4% at week 1 (p<0.001) and recovered to baseline by between months 3 and 6. EZ volume loss at week 1 did not correlate with ETDRS acuity at final visit.ConclusionOcriplasmin treatment resulted in VMT release, MH closure and visual acuity gains in a significant portion of eyes. EZ volume was significantly reduced at week 1, but recovered to baseline levels by final follow-up and was not associated with final visual acuity.

Visual acuity outcomes in Coats disease by classification stage in 160 patients

2019 ◽  
Vol 104 (3) ◽  
pp. 422-431 ◽  
Author(s):  
Carol L Shields ◽  
Sanika Udyaver ◽  
Lauren A Dalvin ◽  
Li-Anne S Lim ◽  
Hatice T Atalay ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Laser Photocoagulation ◽  
Disease Stage ◽  
Final Visit ◽  
Advanced Stage ◽  
Snellen Visual Acuity ◽  
Stage 4 ◽  
Before And After ◽  
Coats Disease ◽  
Stage 1

PurposeTo assess visual outcomes of Coats disease by classification stage.MethodsA retrospective review was conducted on consecutive patients with Coats disease, classified according to Shields classification and with available Snellen visual acuity before and after treatment.ResultsThere were 160 eyes with Coats disease (stage 1 (n=2) vs stage 2A (n=17) vs stage 2B (n=22) vs stage 3A1 (n=26) vs stage 3A2 (n=40) vs stage 3B (n=42) vs stage 4 (n=9) vs stage 5 (n=2)). By comparison, more advanced stage showed greater frequency of poor presenting visual acuity (<20/200) (0% vs 0% vs 50% vs 35% vs 38% vs 83% vs 100% vs 100%, p<0.001) and higher mean intraocular pressure (17 vs 15 vs 15 vs 15 vs 15 vs 15 vs 37 vs 26, p<0.001). More advanced stage was less likely managed with laser photocoagulation (100% vs 87% vs 48% vs 62% vs 74% vs 35% vs 0% vs 0%, p<0.001) and more likely with cryotherapy (0% vs 47% vs 81% vs 81% vs 82% vs 88% vs 50% vs 100%, p=0.001). More advanced stage was associated with lower frequency of visual acuity ≥20/40 (100% vs 71% vs 5% vs 42% vs 23% vs 5% vs 0% vs 0%, p<0.001) and greater frequency of visual acuity <20/200 (0% vs 12% vs 36% vs31 % vs 45% vs 88% vs 0% vs 100%, p<0.001).ConclusionVisual acuity in eyes with Coats disease parallels staging with more advanced stage demonstrating poorer visual acuity at presentation and final visit.

scholarly journals Characteristics of patients with coexisting DNAJB9-associated fibrillary glomerulonephritis and IgA nephropathy

2020 ◽  
Author(s):  
Samar M Said ◽  
Alejandro Best Rocha ◽  
Anthony M Valeri ◽  
Mohamad Sandid ◽  
Anhisekh Sinha Ray ◽  
...  
Keyword(s):  
Iga Nephropathy ◽  
Immunoglobulin A ◽  
Renal Survival ◽  
Hepatitis C Infection ◽  
Clinicopathologic Characteristics ◽  
Fibrillary Glomerulonephritis ◽  
Kaplan Meier ◽  
Dense Deposits ◽  
End Stage

Abstract Background Coexistence of fibrillary glomerulonephritis (FGN) and immunoglobulin A (IgA) nephropathy (IgAN) in the same kidney biopsy (FGN–IgAN) is rare, and the clinicopathologic characteristics and outcome of this dual glomerulopathy are unknown. Methods In this study, 20 patients with FGN–IgAN were studied and their characteristics were compared with 40 FGN and 40 IgAN control patients. Results Concurrent IgAN was present in 1.8% of 847 consecutive FGN cases and was the second most common concurrent glomerulopathy after diabetic nephropathy. FGN–IgAN patients were overwhelmingly White (94%) and contrary to FGN patients were predominantly (60%) males. Compared with IgAN patients, FGN–IgAN patients were older, had higher proteinuria, a higher incidence of renal insufficiency, and a lower incidence of microhematuria and gross hematuria at diagnosis. Six (30%) patients had malignancy, autoimmune disease or hepatitis C infection, but none had a secondary cause of IgAN or clinical features of Henoch–Schonlein purpura. Histologically, all cases exhibited smudgy glomerular staining for immunoglobulin G and DnaJ homolog subfamily B member 9 (DNAJB9) with corresponding fibrillary deposits and granular mesangial staining for IgA with corresponding mesangial granular electron-dense deposits. On follow-up (median 27 months), 10 of 18 (56%) FGN–IgAN patients progressed to end-stage kidney disease (ESKD), including 5 who subsequently died. Serum creatinine at diagnosis was a poor predictor of renal survival. The proportion of patients reaching ESKD or died was higher in FGN–IgAN than in IgAN. The median Kaplan–Meier ESKD-free survival time was 44 months for FGN–IgAN, which was shorter than IgAN (unable to compute, P = 0.013) and FGN (107 months, P = 0.048). Conclusions FGN–IgAN is very rare, with clinical presentation and demographics closer to FGN than IgAN. Prognosis is guarded with a median renal survival of 3.6 years. The diagnosis of this dual glomerulopathy requires careful evaluation of immunofluorescence findings, and electron microscopy or DNAJB9 immunohistochemistry.

Retinal Vasculitis Associated With Dermatomyositis Treated With Intravenous Steroids

2021 ◽  
pp. 247412642110189
Author(s):  
Austen N. Knapp ◽  
Jawad I. Arshad ◽  
Daniel F. Martin ◽  
Rula Hajj-Ali ◽  
Kimberly Baynes ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Macular Edema ◽  
Retinal Vasculitis ◽  
Subretinal Fluid ◽  
Retrospective Case ◽  
Hand Motion ◽  
Frosted Branch Angiitis ◽  
Intravenous Steroids

Purpose: This work aims to present treatment and long-term follow-up of a 31-year-old woman with dermatomyositis who presented with hemorrhagic retinal vasculitis and macular edema. Methods: A retrospective case report is presented. Results: A 31-year-old woman with dermatomyositis treated with systemic immunosuppression was evaluated for acute, reduced vision. Best-corrected visual acuity was hand motion in the right eye and 20/200 in the left eye. Fundus examination revealed diffuse intraretinal hemorrhages, cotton-wool spots, and vascular sheathing with a frosted branch angiitis–like appearance. Optical coherence tomography revealed significant macular edema and subretinal fluid that quickly resolved after admission and treatment with intravenous steroids. Multimodal imaging at 7-year follow-up disclosed long-term sequelae including peripheral nonperfusion and retinal neovascularization. Conclusions: When vasculitis associated with dermatomyositis is treated aggressively with intravenous steroids at initial presentation, good visual acuity outcomes can be achieved, but long-term consequences of retinal nonperfusion and neovascularization persist.

scholarly journals Polypoidal choroidal vasculopathy in pachychoroid: combined treatment with photodynamic therapy and Aflibercept

2021 ◽  
Author(s):  
Maria Vadalà ◽  
Massimo Castellucci ◽  
Giulia Guarrasi ◽  
Giovanni Cillino ◽  
Vincenza Maria Elena Bonfiglio ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Photodynamic Therapy ◽  
Polypoidal Choroidal Vasculopathy ◽  
Combined Therapy ◽  
Combined Treatment ◽  
Subretinal Fluid ◽  
Clinical Activity ◽  
Monthly Basis ◽  
Choroidal Vasculopathy

Abstract Introduction To evaluate the effects of combined therapy using intravitreal Aflibercept (IVA) and photodynamic therapy (PDT) on polypoidal choroidal vasculopathy related to pachychoroid disease (PPCV).Methods Patients with PPCV were treated with PDT combined with 3 IVA injections on a monthly basis, followed by pro re nata criteria. The 12-month follow-up consisted of multiple revaluations of visual acuity and SSOCT parameters of clinical activity.Results Nineteen eyes were included in the study; mean age was 65.5 years. Visual acuity improved after 12 months (0.35 ± 0.25 to 0.2 ± 0.20 logMAR, p = 0.005). Percentage of eyes with intraretinal and subretinal fluid reduced from baseline to the 12-month follow-up (from 52.6–10.5%, p = 0.12, and from 89.5–5.3% p = 0.0009, respectively). Central retinal and mean macular thicknesses reduced (258 ± 39.6 to 204.8 ± 38.8 µm p = 0.04 and 293.8 ± 32.1 to 248.1 ± 29.6 µm p = 0.017 respectively). Central choroidal and mean choroidal thicknesses also displayed a reduction (328.6 ± 54.9 to 289.8 ± 44.6 µm p = 0.001 and 314.5 ± 55.3 to 287.9 ± 47.6 µm p = 0.015 respectively). The mean number of injections was 4.6/year.Conclusion The results support the use of a combined therapy with Aflibercept and PDT in PPCV. This treatment would act in synergy, with anti-VEGF controlling exudation and PDT closing the aneurysmal vessel and reducing choroidal congestion.

Twenty-year outcomes in patients with newly diagnosed glaucoma: mortality and visual function

2018 ◽  
Vol 102 (12) ◽  
pp. 1663-1666
Author(s):  
Christopher King ◽  
Justin C Sherwin ◽  
Gokulan Ratnarajan ◽  
John F Salmon
Keyword(s):  
Visual Acuity ◽  
Visual Field ◽  
Open Angle Glaucoma ◽  
Survival Curve ◽  
Visual Outcome ◽  
Field Analysis ◽  
Time To Death ◽  
Kaplan Meier ◽  
Two Stages

Background/aimsTo determine the mortality within 20 years of diagnosis of chronic open-angle glaucoma (COAG) and visual acuity and visual field progression of a cohort followed for 20 years.MethodsTwenty years following the diagnosis of COAG in 68 of 436 (16%) patients seen in a glaucoma case-finding clinic, visual and mortality outcomes were audited from medical records. Causes of death were obtained from general practitioner records and death certificates. Probability of death was calculated using a Kaplan-Meier survival curve. The visual field of each eye of survivors was graded using a nine-stage severity scale. Visual outcome was analysed at the 20-year follow-up visit.ResultsFrom 68, 14 (21%) were lost to follow-up. In the remaining 54, 20 (37%) were alive 20 years after diagnosis. Of 63% who died, mean age of death was 84 years, most commonly due to vascular disease. Mean age at presentation of those who died was 73.7 years versus 63.2 years for survivors (P=0.001). The median time to death was 16 years. On visual field analysis, nearly half (48.9%) of eyes did not deteriorate, but 28.3% eyes deteriorated by more than two stages. Those who died had worse final visual acuity than survivors (P<0.001). Three who died were registered severely visually impaired mainly from macular disease, but no survivors were registered (P<0.001).ConclusionIn this cohort, approximately two-thirds of patients with glaucoma died within 20 years of diagnosis. In most older patients with glaucoma, the overall goal of preventing visual handicap and blindness is achievable 20 years after diagnosis.

Long-term functional outcomes of different subtypes of primary congenital glaucoma

2019 ◽  
pp. bjophthalmol-2019-315131 ◽  
Author(s):  
Richard Sher Chaudhary ◽  
Amisha Gupta ◽  
Ajay Sharma ◽  
Shikha Gupta ◽  
Rayees Ahmad Sofi ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Congenital Glaucoma ◽  
Cumulative Probability ◽  
Primary Congenital Glaucoma ◽  
Visual Outcomes ◽  
Post Surgery ◽  
Kaplan Meier ◽  
Significant Difference

AimTo analyse long-term visual outcomes across different subtypes of primary congenital glaucoma (PCG).MethodsPatients with PCG with a minimum of 5-year follow-up post surgery were included in the study. Snellen visual acuity recordings taken at their last follow-up were analysed. We evaluated the results using Kaplan-Meier curves to predict the probability of maintaining good vision (as defined by a visual acuity of 6/18 or better) in our patients after 30-year follow-up. The results were also analysed to determine whether there were any differences in the long-term visual acuities with time between the neonatal and infantile PCG. We also analysed the reasons for poor visual outcomes.ResultsWe assessed a cohort of 140 patients with PCG (235 eyes) with an average follow-up of 127±62.8 months (range 60–400 months). Overall, the proportion of eyes with good visual acuity was 89 (37.9%), those with fair visual acuity between 6/60 and 6/18 was 41 (17.4%), and those with poor visual acuity (≤6/60) was 105 (44.7%). We found a significant difference (p=0.047) between neonatal and infantile patients with PCG whereby the neonatal cohort fared worse off in terms of visual morbidity. On Kaplan-Meier analysis, the cumulative probability of survival of a visual acuity of 6/18 or better was more among the infantile PCG in comparison to the neonatal PCG (p=0.039) eyes, and more among the bilateral than the unilateral affected eyes (p=0.029). Amblyopia was the most important cause for poor visual acuity as shown on a Cox proportional-hazards regression model .ConclusionsLong-term visual outcomes of infantile are better than neonatal PCG. Eyes with unilateral have worse visual outcomes compared with those with bilateral PCG because of the development of dense amblyopia.

scholarly journals Genetic defects of CHM and visual acuity outcome in 24 choroideremia patients from 16 Japanese families

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Takaaki Hayashi ◽  
Shuhei Kameya ◽  
Kei Mizobuchi ◽  
Daiki Kubota ◽  
Sachiko Kikuchi ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Survival Curve ◽  
Japanese Patients ◽  
Outcome Data ◽  
High Rate ◽  
Exon 2 ◽  
Pathogenic Variants ◽  
Kaplan Meier ◽  
Whole Exome

Abstract Choroideremia (CHM) is an incurable progressive chorioretinal dystrophy. Little is known about the natural disease course of visual acuity in the Japanese population. We aimed to investigate the genetic spectrum of the CHM gene and visual acuity outcomes in 24 CHM patients from 16 Japanese families. We measured decimal best-corrected visual acuity (BCVA) at presentation and follow-up, converted to logMAR units for statistical analysis. Sanger and/or whole-exome sequencing were performed to identify pathogenic CHM variants/deletions. The median age at presentation was 37.0 years (range, 5–76 years). The mean follow-up interval was 8.2 years. BCVA of the better-seeing eye at presentation was significantly worsened with increasing age (r = 0.515, p < 0.01), with a high rate of BCVA decline in patients > 40 years old. A Kaplan–Meier survival curve suggested that a BCVA of Snellen equivalent 20/40 at follow-up remains until the fifties. Fourteen pathogenic variants, 6 of which were novel [c.49 + 5G > A, c.116 + 5G > A, p.(Gly176Glu, Glu177Ter), p.Tyr531Ter, an exon 2 deletion, and a 5.0-Mb deletion], were identified in 15 families. No variant was found in one family only. Our BCVA outcome data are useful for predicting visual prognosis and determining the timing of intervention in Japanese patients with CHM variants.

scholarly journals Changes in Metamorphopsia, Visual Acuity, and Central Macular Thickness after Epiretinal Membrane Surgery in Four Preoperative Stages Classified with OCT B-Scan Images

2019 ◽  
Vol 2019 ◽  
pp. 1-8
Author(s):  
Jianbo Mao ◽  
Hanfei Wu ◽  
Chenyi Liu ◽  
Chenting Zhu ◽  
Jimeng Lao ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Macular Thickness ◽  
Final Visit ◽  
Central Macular Thickness ◽  
Epiretinal Membranes ◽  
Stage 4 ◽  
Significant Difference ◽  
Three Stages ◽  
B Scan Images

Purpose. To observe the changes in metamorphopsia, visual acuity, and central macular thickness (CMT) in patients undergoing vitrectomy for idiopathic epiretinal membranes (iERM); all of which were preoperatively stratified into 4 stages according to the anatomical structure of the macula seen on the optical coherence tomography (OCT) b-scan images. Methods. A total of 108 eyes of 106 patients were included. We evaluated and classified the severity of each preoperative ERM based on OCT. Changes in the best-corrected visual acuity (BCVA), metamorphopsia, and CMT were studied by comparing the pre- and postoperative measurements. The follow-up time was at least 6 months. Results. There were 41 eyes at stage 2, 35 at stage 3, 32 at stage 4, and none at stage 1. BCVA and metamorphopsia significantly improved at the final visit in all patients (P<0.01). However, comparing the pre- and postoperative measurements at each stage, only the BCVA and CMT improved significantly for all stages (P<0.001). For stages 2 and 3 ERMs, the horizontal (MH) and vertical (MV) metamorphopsia scores decreased significantly after surgery (P<0.05). No significant difference was found in either MH or MV for stage 4 ERMs (P both >0.05). The preoperative BCVA, MH, and CMT had significant difference among the three stages (P<0.05). Similarly, the postoperative values in the three variables mentioned above also had significant difference among the three stages (P<0.05). For stage 2 ERMs, the baseline MH and MV were positively correlated with the baseline CMT. The MH and MV at the final follow-up also presented a significant positive correlation with the baseline CMT. For stage 3 ERMs, only the baseline MV showed significant correlation with the CMT. Conclusion. Categorization of the preoperative ERMs is a useful method to predict the postoperative improvement in metamorphopsia, which would aid in surgical decisions for patients with ERMs.

Phase 1b/2, open label, multicenter, study of the combination of SD-101 and pembrolizumab in patients with advanced melanoma who are naïve to anti-PD-1 therapy.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. 9534-9534 ◽  
Author(s):  
Mohammed M. Milhem ◽  
Georgina V. Long ◽  
Christopher J. Hoimes ◽  
Asim Amin ◽  
Christopher D. Lao ◽  
...  
Keyword(s):  
Disease Stage ◽  
Open Label ◽  
Stage Iiic ◽  
Kaplan Meier ◽  
Unresectable Stage ◽  
Treatment Naïve ◽  
Phase 1B ◽  
Grade 3 ◽  
Post Hoc

9534 Background: SD-101 is a synthetic CpG TLR9 agonist. Pembrolizumab is a PD-1 inhibitor. SYNERGY-001/KEYNOTE-184 assesses the safety and preliminary efficacy of the combination of SD-101 and pembrolizumab in patients naïve to anti-PD-1/L1 therapy with unresectable stage IIIC-IV melanoma. Methods: SD-101 was evaluated as 2 mg/lesion injected into 1 to 4 lesions and 8 mg/lesion in 1 lesion as 4 weekly doses followed by 7 doses Q3W. Pembrolizumab was administered as 200 mg IV Q3W. CT scans were performed every 9 weeks. Responses were assessed per investigator using RECIST v1.1. Responses and post-hoc Kaplan-Meier analyses of PFS in the ITT population were compared for patients who received 2 mg/lesion with 8 mg/lesion. Results: 86 patients (2 mg: N = 45; 8 mg: N = 41) have been enrolled with similar baseline characteristics: median age = 66 years; male = 67%; ECOG stage 0 = 67%; disease stage: IIIC = 21%; IVM1a/b = 50%; IVM1c = 28%; LDH ≤ ULN = 71%; treatment naïve = 73%. Median follow up to date is 8.1 months in 2 mg group and 8.3 months in 8 mg group. SD-101 safety profile comprises flu-like symptoms with most frequent grade ≥3 SD-101-related AEs of headache (7%), fatigue (7%), malaise (5%), myalgia (4%), and chills (4%). Immune-related AEs were reported in 19%. ORR in 2 mg group = 71% (95% CI: 57, 82) (CR: 13%) and in 8 mg group = 49% (95% CI: 33, 65) (CR: 7%) with responses in both injected and non-injected lesions, including visceral. DOR = not reached (NR) in either group. ORR by baseline PD-L1 expression in 62 patients, 53% of whom were PD-L1 positive: 2 mg = 80%/79% (PD-L1 positive/negative); 8 mg = 62%/40% (PD-L1 positive/negative). PFS was higher in 2 mg group with median PFS in 2 mg = NR (95% CI: Not estimable [NE], NE) and in 8 mg = 10.4 months (95% CI: 4.2, NE), HR = 0.45 (95% CI: 0.21, 0.98), p = 0.036. 6 month PFS rate in 2 mg = 81% and in 8 mg = 60%. 6 month OS rate in 2 mg = 98% and in 8 mg = 92%. Conclusions: The TLR9 innate immune stimulant, SD-101, in combination with pembrolizumab has been well-tolerated, and is showing promising high response rates and PFS, regardless of PD-L1 expression, particularly in patients who received 2 mg SD-101. Clinical trial information: NCT02521870.

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