Lesion evolution and neurodegeneration in RVCL-S: A monogenic microvasculopathy.

OBJECTIVE: To characterize lesion evolution and neurodegeneration in retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) using multimodal MRI. METHODS: We prospectively performed MRI and cognitive testing in RVCL-S and healthy control cohorts. Gray and white matter volume and disruption of white matter microstructure were quantified. Asymmetric spin echo acquisition permitted voxel-wise oxygen extraction fraction (OEF) calculation as an in vivo marker of microvascular ischemia. The RVCL-S cohort was included in a longitudinal analysis of lesion subtypes in which hyperintense lesions on fluid-attenuated inversion recovery (FLAIR), T1-postgadolinium, and diffusion-weighted imaging were delineated and quantified volumetrically. RESULTS: Twenty individuals with RVCL-S and 26 controls were enrolled. White matter volume and microstructure declined faster in those with RVCL-S compared to controls. White matter atrophy in RVCL-S was highly linear (ρ = -0.908, p < 0.0001). Normalized OEF was elevated in RVCL-S and increased with disease duration. Multiple cognitive domains, specifically those measuring working memory and processing speed, were impaired in RVCL-S. Lesion volumes, regardless of subtype, progressed/regressed with high variability as a function of age, while FLAIR lesion burden increased near time to death (p < 0.001). CONCLUSION: RVCL-S is a monogenic microvasculopathy affecting predominantly the white matter with regard to atrophy and cognitive impairment. White matter volumes in RVCL-S declined linearly, providing a potential metric against which to test the efficacy of future therapies. Progressive elevation of white matter OEF suggests that microvascular ischemia may underlie neurodegeneration in RVCL-S.

HEMORRHAGIC RISK OF VITREORETINAL SURGERY IN PATIENTS MAINTAINED ON NOVEL ORAL ANTICOAGULANT THERAPY.

PURPOSE: To evaluate the frequency and type of perioperative hemorrhagic complications associated with vitreoretinal surgery in patients undergoing systemic treatment with the newer anticoagulant and antiplatelet agents including rivaroxaban, apixaban, dabigatran, and prasugrel. METHODS: Retrospective review of a cohort of patients being treated with anticoagulant and antiplatelet drugs, who underwent any vitreoretinal surgical procedure over a 2-year period. RESULTS: Thirty-six eyes of 33 patients were identified who underwent vitreoretinal surgical operations while being treated systemically with anticoagulant and antiplatelet therapy. No eyes suffered perioperative complications of retrobulbar hemorrhage, suprachoroidal hemorrhage, or subretinal hemorrhage. Four eyes (11.1%) experienced postoperative vitreous cavity hemorrhage after which two eyes (5.5%) required repeat surgical intervention and two eyes (5.5%) cleared spontaneously. CONCLUSION: Although there is a relative risk to such surgery in patients who are taking novel oral anticoagulants, these findings suggest that patients may safely undergo vitreoretinal surgery while maintaining therapy with rivaroxaban, apixaban, dabigatran, and prasugrel.

Pars plana vitrectomy versus combined pars plana vitrectomy and scleral buckle for primary repair of rhegmatogenous retinal detachment.

OBJECTIVE: To compare pars plana vitrectomy (PPV) with PPV and scleral buckle (PPV/SB) for repair of rhegmatogenous retinal detachment (RRD). DESIGN: A retrospective chart review. PARTICIPANTS: Patients who underwent PPV or PPV/SB for RRD repair at a single institution. METHODS: A retrospective chart review of patients in two different treatment groups and analysis of the anatomic and functional results. RESULTS: Single-surgery anatomic success was achieved in 31 of 37 (83.8%) phakic eyes that underwent PPV and in 66 of 68 (97.1%) phakic eyes that underwent PPV/SB (p = 0.0216). Among pseudophakic eyes, 42 of 48 (87.5%) in the PPV group and 62 of 66 (93.9%) in the PPV/SB group achieved single-surgery reattachment (p = 0.3175). Visual acuity improvement was marginally greater in the PPV group among phakic (p = 0.4898) and pseudophakic (p = 0.2465) eyes. CONCLUSIONS: PPV/SB may be associated with a decreased risk for retinal redetachment when compared to PPV for repair of phakic RRD. In pseudophakic eyes, the anatomic success rate between the two techniques appears to be similar.

Intravitreal bevacizumab for the treatment of refractory diabetic macular edema.

BACKGROUND AND OBJECTIVE: To report the effect of intravitreal bevacizumab on visual acuity and central retinal thickness (CRT) in refractory diabetic macular edema. PATIENTS AND METHODS: Records of 60 eyes of 54 consecutive patients who underwent intravitreal bevacizumab therapy for refractory diabetic macular edema were reviewed. All eyes received intravitreal bevacizumab 1.25 mg/0.05 mL, and 36 eyes underwent pretreatment and post-treatment optical coherence tomography. Mean follow-up was 7.4 months. RESULTS: Pretreatment mean visual acuity plus or minus standard deviation was 0.71 +/- 0.28 logarithm of the minimum angle of resolution (LogMAR) Snellen letters. At final follow-up, mean visual acuity had improved to 0.66 +/- 0.30 LogMAR (P = .0543). Mean baseline CRT was 440 +/- 106 microm, and follow-up mean CRT was 386 +/- 129 microm (P = .008). Vitrectomized eyes had worse visual acuity and CRT outcomes (P = .002 and P = .028, respectively) compared with nonvitrectomized eyes. CONCLUSION: Intravitreal bevacizumab may provide a functional and anatomic benefit in eyes with persistent diabetic macular edema despite previous treatments.

Surgical outcome of scleral buckling compared with scleral buckling with vitrectomy for treatment of macula-off retinal detachment.

BACKGROUND AND OBJECTIVE: To compare the surgical outcome of scleral buckling (group 1) versus scleral buckling with pars plana vitrectomy (group 2) for the repair of macula-off rhegmatogenous retinal detachment without proliferative vitreoretinopathy. PATIENTS AND METHODS: A retrospective chart review was performed. RESULTS: Eighty-three patients were identified in group 1 and 63 patients in group 2. Presenting visual acuity was 4/200 in group 1 and 3/200 in group 2. Median duration of detachment prior to surgery was 5 days in group 1 and 6 days in group 2. There was no statistical difference in best-corrected (P = .59) or most recent (P = .75) visual acuity between groups. Median best-corrected visual acuity was 20/30 and median most recent visual acuity was 20/40 in both groups. Significantly more additional procedures were performed in group 1 than in group 2 (21.7% vs 7.9%, respectively; P = .024). The final reattachment rate was 96.4% in group 1 and 98.4% in group 2. Proliferative vitreoretinopathy developed in 15.7% of patients in group 1 and 4.8% in group 2 (P= .037). CONCLUSION: Visual outcome of scleral buckling is similar to scleral buckling with pars plana vitrectomy for the treatment of macula-off rhegmatogenous retinal detachment in patients without proliferative vitreoretinopathy. Patients undergoing scleral buckling only are at an increased risk of developing proliferative vitreoretinopathy and requiring additional procedures.

Intravitreal bevacizumab for choroidal neovascularization in ocular histoplasmosis.

PURPOSE: To define the role of intravitreal bevacizumab in individuals with choroidal neovascularization (CNV) resulting from Ocular Histoplasmosis syndrome (OHS). DESIGN: Retrospective chart review of a surgical therapy. METHODS: We reviewed the course of 28 eyes of 28 patients who underwent intravitreal injection of bevacizumab for treatment of CNV secondary to OHS. Outcome was measured by pretreatment and posttreatment visual acuity (VA). RESULTS: The average pretreatment logarithm of the minimum angle of resolution (logMAR) VA was 0.65 (Snellen equivalent of 20/88). Mean follow-up was 22.43 weeks with an average of 1.8 intravitreal injections. Average final logMAR VA was 0.43 (Snellen equivalent of 20/54). Twenty eyes (71%) experienced an increase in central VA, whereas four eyes (14%) were unchanged and four eyes (14%) experienced a decrease in vision. CONCLUSIONS: Intravitreal bevacizumab may improve or stabilize VA in a significant majority of patients with neovascular complications of OHS (24 eyes [85.7%] in our study population).

Treatment of neovascular age-related macular degeneration: past, present and future directions.

PURPOSE OF REVIEW: The treatment options of choroidal neovascularization due to age-related macular degeneration have expanded. Prior to ocular photodynamic therapy the only available treatment was laser photocoagulation. Clinicians and patients were not particularly enthusiastic despite its ability to stabilize vision. The purpose of the review is to review the past and current concepts of neovascular age-related macular degeneration therapy and to provide a short overview of upcoming treatments. RECENT FINDINGS: Photodynamic therapy provided us with the first realistic means to address subfoveal choroidal neovascularization lesions from age-related macular degeneration. Antivascular endothelial growth factors now allow better visual outcomes than mere stabilization of vision and other promising treatments are undergoing study at this time. SUMMARY: Age-related macular degeneration therapy has undergone a significant revolution in recent years. Understanding the historical perspective of treatment provides a better appreciation of current therapies. Still there is no cure for this disease and more promising treatments are currently under investigation.

Maintenance of warfarin anticoagulation for patients undergoing vitreoretinal surgery.

OBJECTIVE: To evaluate the risk of hemorrhagic complications associated with vitreoretinal surgery in patients whose warfarin sodium therapy was continued throughout the surgical period. METHODS: A review of 1737 records of patients undergoing pars plana vitrectomy was conducted. Inclusion criteria included patients receiving warfarin therapy whose international normalized ratios (INRs) were elevated above normal values on the day of surgery. Intraoperative and postoperative hemorrhagic complications were documented. RESULTS: Fifty-four patients underwent 57 vitreoretinal surgical procedures with warfarin therapy and were divided into groups as follows: group S with INRs of 1.20 to 1.49, values considered subtherapeutic; group B with INRs of 1.50 to 1.99, values considered borderline therapeutic; group T with INRs of 2.00 to 2.49, values considered therapeutic; and group HT with INRs of 2.50 or greater, values considered highly therapeutic. No patients experienced anesthesia-related or intraoperative hemorrhagic complications. Two (7.7%) of 26 eyes in group S and 2 (16.7%) of 12 eyes in group HT experienced postoperative hemorrhages. All of the patients with vitreous hemorrhages had spontaneous clearing without additional treatment. CONCLUSIONS: Many patients may safely undergo vitreoretinal surgery while maintaining therapeutic levels of warfarin anticoagulation. We experienced no intraoperative hemorrhagic complications; the 4 postoperative complications resolved spontaneously without persistent visual sequelae or the need for supplemental surgery.

Maintenance of warfarin anticoagulation for patients undergoing vitreoretinal surgery.

PURPOSE: The purpose of this study was to evaluate the risk of hemorrhagic complications associated with vitreoretinal surgery in patients in whom warfarin therapy was continued throughout the surgical period. METHODS: A review of 1,737 consecutive charts of patients undergoing pars plana vitrectomy was conducted. Inclusion criteria included patients on warfarin therapy whose international normalized ratio (INR) was elevated above normal on the day of vitreoretinal surgery. The occurrence of intraoperative and postoperative hemorrhagic complications was documented. RESULTS: The retrospective review detected 54 patients who underwent 57 vitreoretinal surgical procedures while on warfarin therapy. Group S consisted of patients whose INR ranged from 1.2 to 1.49, values that were considered to be subtherapeutic. Group B had INR values ranging from 1.5 to 1.99, values that were considered to be borderline therapeutic. Group T had INRs ranging from 2.0 to 2.49, values that were considered therapeutic. Group HT had INRs of 2.5 or greater, values that were considered highly therapeutic. No patients experienced anesthesia-related or intraoperative hemorrhagic complications. Four procedures (7.0%) were complicated by postoperative hemorrhage. Two of 26 eyes (7.7%) in group S and two of 12 eyes (16.7%) in group HT experienced postoperative hemorrhages. All hemorrhagic complications cleared without additional therapy. CONCLUSIONS: Our findings suggest that many patients may safely undergo vitreoretinal surgery while maintaining therapeutic levels of warfarin anticoagulation. We found no intraoperative hemorrhagic complications. Those hemorrhagic complications that occurred postoperatively resolved spontaneously without persistent visual sequelae or the need for supplemental surgery.

Triamcinolone-assisted internal limiting membrane peeling.

PURPOSE: To review our experience with triamcinolone-assisted pars plana vitrectomy for internal limiting membrane (ILM) peeling for various retinal diseases. METHODS: Twenty-one patients underwent surgery in which intraoperative triamcinolone acetonide (TA) was used. Indications for surgery included epiretinal membrane (3 patients), branch retinal vein occlusion associated with macular edema (2), traction retinal detachment (3), diabetic macular edema (4), vitreous hemorrhage with diabetic macular edema (4), macular hole (4), and cystoid macular edema (1). RESULTS: TA was useful in the removal of the ILM in all cases. There were no intraoperative complications or toxicity. The mean follow-up was 22 weeks (range, 9-30 weeks). Eleven patients improved by >or=2 Snellen lines, 1 lost >or=2 Snellen lines, and 9 were within 2 Snellen lines of preoperative vision at the last follow-up. CONCLUSIONS: The intraoperative use of TA improves visualization of ILMs associated with a variety of conditions. No intraoperative or postoperative complications were observed. TA-assisted removal of the ILM appears to be safe and cost effective. TA-assisted ILM peeling should be considered as an alternative to the use of intraoperative dyes.

Surgical removal of peripapillary choroidal neovascularization associated with age-related macular degeneration.

BACKGROUND AND OBJECTIVE: To describe the results of surgical treatment of peripapillary choroidal neovascularization in age-related macular degeneration as an option to both laser photocoagulation and photodynamic therapy. PATIENTS AND METHODS: Retrospective review of patients with peripapillary choroidal neovascularization secondary to age-related macular degeneration who were not eligible for or refused laser photocoagulation. Patients without the diagnosis of age-related macular degeneration and those who had extension of their neovascularization subfoveally were excluded from the review. RESULTS: Eleven patients total were identified who met the specified inclusion criteria. The male to female ratio was 4:7, with an age range of 63 to 94 years (mean = 78 years). The mean area of involved retina temporal to the optic disc was 5 clock hours, with the distance of the temporal edge of the lesion from the fovea ranging from 100 to 2,000 microm. The mean duration of follow-up was 23 months, with 27% (3 of 11) experiencing recurrent choroidal neovascularization. The preoperative and postoperative visual acuity ranges were both 20/25 to counting fingers. Sixty-four percent (7 of 11) of patients had stable or improved visual acuity postoperatively, with a mean visual acuity change of 1 line visual improvement. CONCLUSION: In cases where photodynamic therapy and laser photocoagulation are not indicated, the surgical treatment of peripapillary choroidal neovascularization secondary to age-related macular degeneration may prove beneficial.

Management of endogenous fungal endophthalmitis with voriconazole and caspofungin.

PURPOSE: Voriconazole, a new generation triazole, has been shown to achieve therapeutic intraocular levels after oral administration. Caspofungin is the first approved agent from a new class of antifungals, the echinocandins. This series describes experience at two centers using these novel antifungals to treat endogenous fungal endophthalmitis. DESIGN: Retrospective review. METHODS: Treatment of five patients with Candida endophthalmitis are reviewed. Postmortem intraocular voriconazole concentrations on a sixth patient are presented as well. RESULTS: All patients had systemic cultures positive for Candida species. Three patients had prompt resolution of intraocular mycosis with intravenous and oral voriconazole, caspofungin, or both. The fourth patient with bilateral disease responded well to i.v. voriconazole and caspofungin but had a recurrence when discharged on oral voriconazole and i.v. caspofungin. This patient had a bowel resection with an ileostomy; therefore, absorption of oral voriconazole may have been inadequate. Bilateral amphotericin B intravitreal injection ultimately treated this patient. The fifth patient received 100 microg/0.1 ml of intravitreal voriconazole (final vitreous concentration approximately 25 microg/ml) followed by oral voriconazole and responded favorably. Our sixth patient had multisystem failure and passed away 1 week after initiating intravenous voriconazole for non-ocular candidemia. Postmortem HPLC analysis of the aqueous and vitreous revealed voriconazole concentrations of 1.52 microg/ml and 1.12 microg/ml, respectively (MIC90 of Candida albicans is 0.06 microg/ml). CONCLUSIONS: Voriconazole and caspofungin appear to be powerful weapons to add to the existing armamentarium against fungal endophthalmitis. Further studies are warranted to define precisely the role of these new agents alone or in combination with other antifungals.

Outcome of macular hole surgery in diabetic patients with nonproliferative retinopathy.

PURPOSE: To determine the outcome of macular hole surgery in a diabetic population with no evidence of proliferative retinopathy. METHODS: This is a retrospective chart review of 183 patients (194 eyes) undergoing pars plana vitrectomy for an idiopathic macular hole. RESULTS: The anatomic closure rate for the diabetic patients without proliferative retinopathy was 93.8% (15/16), compared with 94.9% (169/178) for nondiabetic patients. A best corrected visual acuity of 20/50 or greater was obtained in 50% of diabetic patients (8/16), compared to 58.4% of nondiabetic patients (104/178). There was no difference in postoperative complications between the two groups. CONCLUSION: The anatomic closure rate and visual outcome after macular hole surgery in diabetic patients without proliferative retinopathy is comparable to that of nondiabetic patients.