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Purpose: To report a case of a perifoveal exudative vascular anomalous complex (PEVAC) resembling lesion in a patient with multiple myeloma. Observations: A 56-year-old male with multiple myeloma presented with sudden moderate vision loss in the right eye. Best-corrected visual acuity was 20/25 in his right eye. Fundus examination showed a vascular irregularity in the perifoveal region. Fluorescein angiography (FA) revealed an isolated perifoveal aneurysmal lesion with minimal leakage. On optical coherence tomography (OCT) examination, a large oval structure with a hyperreflective wall and exudation was visualised. Three weeks later, spontaneous improvement of the intraretinal fluid was observed on OCT without treatment. However, 3 months later the macular edema recurred. The appearance of the aneurysmal lesion is similar to a PEVAC lesion, which is an isolated well-defined perifoveal intraretinal vascular abnormality presenting on OCT as a round hyperreflective structure with a dark lumen containing variably reflective material and is commonly associated with cystic intraretinal fluid. Conclusions and Importance: PEVAC was originally described as occurring in healthy patients, but recent observations suggest that it also appears in association with other retinal/choroidal vascular abnormalities or underlying cardiovascular abnormalities. Our case supports this hypothesis by demonstrating a PEVAC resembling lesion in a patient with multiple myeloma.
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PURPOSE: To evaluate the clinical course after early surgical treatment with excision of retinal hemangioblastomas (RHs) before development of major complications. METHODS: Interventional case series of four eyes (four patients) with a peripheral RH that had not yet been treated by laser or cryotherapy prior to surgery. All eyes underwent 23-gauge vitrectomy with lesion excision. One patient underwent ligation of the feeder vessel prior to lesion excision. Best-corrected visual acuity and clinical course were assessed during a follow-up period of at least 4 years. RESULTS: Four patients (mean age 27.3 years; range 19-32) were included, of whom two had von Hippel-Lindau syndrome. Visual acuity improved in three patients (mean 4.8 lines; range 3-10) and remained stable at 0.0 logMAR in one patient. There were no intraoperative complications. Postoperative complications included transient mild vitreous haemorrhage (n = 2), and local epiretinal membrane formation at the excision location (n = 1). At 4 years postoperatively, there were no long-term complications. There was one case of a new lesion, which was effectively treated with laser. CONCLUSION: Vitrectomy with RH excision seems to be an effective approach for larger RHs and could be considered an early treatment option in selected cases. Postoperative complications were limited in scope of this case series. Important points to consider during vitrectomy are effective closure of feeder and draining vessels as well as complete removal of posterior hyaloid and epiretinal membranes in order to avoid postoperative vitreous haemorrhage and proliferative vitreoretinopathy.
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Hemangioblastoma/cirugía , Neoplasias de la Retina/cirugía , Vitrectomía , Adulto , Femenino , Hemangioblastoma/patología , Humanos , Complicaciones Intraoperatorias , Neoplasias de la Retina/patología , Agudeza Visual/fisiología , Adulto JovenRESUMEN
PURPOSE: To update the surgical technique in which a vitrectomy is performed and a retinal branch vein is cannulated and infused with recombinant tissue plasminogen activator (RTPA) to treat central retinal vein occlusion (CRVO) in patients who present with very low visual acuity (VA). METHODS: Twelve consecutive patients (12 eyes) with CRVO and low VA (logMAR >1.00) at presentation were treated using this method. RESULTS: Cannulation of a peripapillary retinal vein and stable injection of RTPA was successfully performed without surgery-related complications in all 12 eyes. At 12 months after surgery, 8 of the 12 patients (67%) experienced at least one line of improvement in best corrected visual acuity; 6 of the 12 (50%) improved ≥5 lines and 2 (17%) improved ≥8 lines. After additional grid laser and/or subconjunctival or intravitreal corticosteroids, the mean decrease in central foveal thickness was 260 µm, and the mean total macular volume decreased from 12.10 mm(3) to 9.24 mm(3) . Four patients received panretinal photocoagulation to treat either iris neovascularization (n = 2) or neovascularization of the retina and/or disc (n = 2). CONCLUSION: Administration of RTPA via a peripapillary vein using this updated technique provides an alternative or additional treatment option for patients with very low VA after CRVO.
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Cateterismo/métodos , Fibrinolíticos/administración & dosificación , Oclusión de la Vena Retiniana/tratamiento farmacológico , Vena Retiniana/efectos de los fármacos , Activador de Tejido Plasminógeno/administración & dosificación , Anciano , Anciano de 80 o más Años , Terapia Combinada , Femenino , Angiografía con Fluoresceína , Humanos , Infusiones Intravenosas , Coagulación con Láser , Masculino , Persona de Mediana Edad , Fotograbar , Estudios Prospectivos , Oclusión de la Vena Retiniana/diagnóstico , Oclusión de la Vena Retiniana/fisiopatología , Tomografía de Coherencia Óptica , Agudeza Visual/fisiología , VitrectomíaRESUMEN
BACKGROUND: Paraneoplastic melanocytic proliferation (bilateral diffuse uveal melanocytic proliferation, BDUMP) is a rare but devastating disease that causes progressive visual loss in patients who usually have an occult malignancy. Visual loss occurs as a result of paraneoplastic changes in the uveal tissue. METHODS: In a masked fashion, the serum of two patients with BDUMP was evaluated for the presence of cultured melanocyte elongation and proliferation (CMEP) factor using cultured human melanocytes. We evaluated the efficacy of plasmapheresis as a treatment modality early in the disease in conjunction with radiation and chemotherapy. RESULTS: The serum of the first case patient was investigated after plasmapheresis and did not demonstrate proliferation of cultured human melanocytes. The serum of the second case was evaluated prior to treatment with plasmapheresis and did induce this proliferation. These findings are in accordance with the diminution of CMEP factor after plasmapheresis. Treatment with plasmapheresis managed to stabilise the ocular disease progression in both patients. CONCLUSIONS: In the past, visual loss due to paraneoplastic melanocytic proliferation was considered progressive and irreversible. We treated two patients successfully with plasmapheresis and demonstrated a relation between CMEP factor in the serum of these patients and proliferation of cultured melanocytes.
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Adenocarcinoma/secundario , Neoplasias Pulmonares/patología , Melanocitos/patología , Síndromes Paraneoplásicos Oculares/diagnóstico , Síndromes Paraneoplásicos Oculares/tratamiento farmacológico , Adenocarcinoma/terapia , Anciano , Inhibidores de la Angiogénesis/uso terapéutico , Anticuerpos Monoclonales Humanizados/uso terapéutico , Antineoplásicos/uso terapéutico , Bevacizumab , Proliferación Celular/efectos de los fármacos , Terapia Combinada , Diagnóstico Precoz , Angiografía con Fluoresceína , Humanos , Inyecciones Intravítreas , Neoplasias Pulmonares/terapia , Metástasis Linfática , Masculino , Síndromes Paraneoplásicos Oculares/etiología , Plasmaféresis , Líquido Subretiniano , Tomografía de Coherencia Óptica , Factor A de Crecimiento Endotelial Vascular/antagonistas & inhibidores , Agudeza Visual/fisiología , Pruebas del Campo Visual , Campos VisualesAsunto(s)
Amaurosis Fugax/etiología , Presión Intraocular/fisiología , Complicaciones Posoperatorias , Postura , Cirugía Vitreorretiniana , Adulto , Anciano , Amaurosis Fugax/fisiopatología , Antihipertensivos/uso terapéutico , Humanos , Presión Intraocular/efectos de los fármacos , Persona de Mediana Edad , Desprendimiento de Retina/cirugía , Estudios Retrospectivos , Hemorragia Vítrea/cirugíaRESUMEN
We report macular telangiectasia type 2 (MacTel) in a 34-year-old man, the youngest patient so far published with MacTel type 2. The patient presented with metamorphopsia and impaired reading ability. Diagnosis was based on bilateral abnormal macular autofluorescence, perifoveal telangiectasia with fluorescein angiographic hyperfluorescence without cystoid oedema, a small foveal avascular zone, asymmetric configuration of the foveal pit, disruptions in the inner segment/outer segment layer and hyper-reflective haze and spots in the outer nuclear layer. Although MacTel usually manifests with a slowly progressive decrease in visual acuity in the fifth to seventh decades of life, younger patients may occasionally be diagnosed with the disease. Awareness of subtle signs of the condition is essential for early diagnosis.
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Mácula Lútea , Telangiectasia Retiniana/diagnóstico , Adulto , Diagnóstico Diferencial , Angiografía con Fluoresceína , Humanos , Mácula Lútea/patología , Masculino , Telangiectasia Retiniana/patología , Tomografía de Coherencia ÓpticaRESUMEN
AIMS: To investigate the efficacy and safety of a single intracameral bevacizumab injection to improve the outcome of trabeculectomy. METHODS: A 12-month, prospective, randomised, double-masked, placebo-controlled trial. Patients with medically uncontrolled open-angle glaucoma scheduled for a primary trabeculectomy were recruited and randomised to receive 50 µL of either bevacizumab (1.25 mg) or placebo (balanced salt solution) peroperatively. Absolute success was defined as intraocular pressure (IOP) ≤18 mm Hg and >5 mm Hg with at least 30% reduction from baseline and no loss of light perception. Success through the use of additional medical and/or surgical IOP-lowering treatments was defined as qualified success. RESULTS: 138 patients completed a 12-month follow-up, 69 of whom were in the bevacizumab treated group. IOP at 1 year postoperatively was significantly lower than baseline (placebo: 25.6±9.9 mm Hg vs 11.5±3.9 mm Hg, p<0.01; bevacizumab: 24.8±8.1 mm Hg vs 11.9±3.8 mm Hg, p<0.01), with no difference between treatment groups (p=0.69). However, absolute success was higher in the bevacizumab group (71% vs 51%, p=0.02), with the need for IOP-lowering interventions (needlings) being lower in this group (12% vs 33%, p=0.003). Complication rates were low and comparable between groups. CONCLUSIONS: Peroperative administration of intracameral bevacizumab significantly reduces the need for additional interventions during the follow-up of patients undergoing trabeculectomy.
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Inhibidores de la Angiogénesis/administración & dosificación , Anticuerpos Monoclonales Humanizados/administración & dosificación , Glaucoma de Ángulo Abierto/tratamiento farmacológico , Glaucoma de Ángulo Abierto/cirugía , Trabeculectomía , Anciano , Inhibidores de la Angiogénesis/efectos adversos , Anticuerpos Monoclonales Humanizados/efectos adversos , Bevacizumab , Quimioterapia Adyuvante , Método Doble Ciego , Femenino , Humanos , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Factores de TiempoRESUMEN
We describe long-term fundus changes in a patient with partial lipodystrophy (PL). Retinal pigment alterations, drusen and subretinal neovascularisation were seen without evidence for membranoproliferative glomerulonephritis. Fundus alterations similar to those seen in age-related macular degeneration can occur at an earlier age in patients with PL, even without renal disease. Dysregulation of an alternative complement pathway with low serum levels of C3 has been implicated as a pathogenetic mechanism.
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Fondo de Ojo , Glomerulonefritis Membranoproliferativa/diagnóstico , Lipodistrofia/complicaciones , Degeneración Macular/diagnóstico , Retina/patología , Drusas Retinianas/diagnóstico , Diagnóstico Diferencial , Angiografía con Fluoresceína , Humanos , Masculino , Persona de Mediana Edad , FotoquimioterapiaRESUMEN
BACKGROUND: Several lines of evidence suggest that macular pigment may play a protective role against age-related macular degeneration, but the influence of age on macular pigment density levels remains unclear. This study was designed to investigate the relationship between age and the normal distribution of macular pigment optical density (MPOD) values surrounding the fovea. METHODS: Consecutive healthy subjects with no evidence of ocular disease were enrolled in this study. After inclusion, MPOD values were measured at specific eccentricities (0.5, 1, and 2 degrees) from the foveal center using a dual-wavelength autofluorescence method employing a modified confocal scanning laser ophthalmoscope. Whenever both eyes were eligible, one was randomly selected for analysis. The correlation between age and MPOD values was investigated using regression analysis. RESULTS: Thirty subjects (30 eyes) were included (mean age 48.6 ± 16.4 [range 23-77] years). Significant differences were found between MPOD values measured at 0.5, 1, and 2 degrees from the center of the fovea (0.49 ± 0.12 density units, 0.37 ± 0.11 density units, and 0.13 ± 0.05 density units, respectively, P < 0.05). Significant correlations between age and MPOD values at 0.5 and 1 degree were found (P ≤ 0.02). Values measured at 2 degrees did not correlate significantly with age (P = 0.06). CONCLUSION: In healthy subjects, MPOD values were highest near the foveal center. These values appeared to increase during adulthood (peak at 45-50 years), followed by a gradual reduction after 60 years of age.
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BACKGROUND/PURPOSE: Light-chain deposition disease (LCDD) is a rare condition characterised by deposition of monoclonal immunoglobulin light chains (LCs) in tissues, resulting in varying degrees of organ dysfunction. This study reports the characteristic clinical ocular findings seen in advanced LCDD upon development of ocular fundus changes. This is the first report to describe this entity in vivo in a series of patients. METHODS: A case series of ocular fundus changes in three patients with kidney biopsy-proven LCDD. All patients underwent best corrected visual acuity (BCVA) exam, perimetry, colour fundus photography and fluorescein angiography; two patients underwent indocyanine green angiography, optical coherence tomography, ultrasound and electroretinography; and one patient underwent fundus autofluorescence. RESULTS: Three patients, 53-60 years old at initial presentation, were studied. All three presented with night blindness, poor dark adaptation, metamorphopsia and visual loss. Examination revealed serous and serohaemorrhagic detachments, multiple retinal pigment epithelial (RPE) tears, diffuse RPE degeneration and progressive fibrotic changes. Neither choroidal neovascularisation nor other vascular abnormalities were present. Final best corrected visual acuity (BCVA) ranged from 20/40 to 20/300. CONCLUSIONS: Progressive LC deposition in the fundus seems to damage RPE pump function with flow disturbance between choroid and retina. This pathogenesis can explain the evolution to RPE detachments and subsequent rips and progressive retinal malfunction.
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Cadenas Ligeras de Inmunoglobulina/metabolismo , Paraproteinemias/complicaciones , Degeneración Retiniana/etiología , Desprendimiento de Retina/etiología , Perforaciones de la Retina/etiología , Colorantes , Progresión de la Enfermedad , Femenino , Angiografía con Fluoresceína , Estudios de Seguimiento , Humanos , Verde de Indocianina , Masculino , Persona de Mediana Edad , Paraproteinemias/metabolismo , Degeneración Retiniana/diagnóstico , Desprendimiento de Retina/diagnóstico , Perforaciones de la Retina/diagnóstico , Epitelio Pigmentado de la Retina/patología , Tomografía de Coherencia Óptica , Agudeza Visual/fisiología , Pruebas del Campo Visual , Campos Visuales/fisiologíaRESUMEN
PURPOSE: The purpose of this study was to report diving-related visual loss in the setting of angioid streaks. METHODS: Observational case reports of two patients with angioid streaks suffering sudden visual loss immediately after diving. RESULTS: Two young adult male patients presented with visual loss after diving headfirst. Funduscopy revealed angioid streaks, peau d'orange, subretinal hemorrhages, and ruptures of Bruch membrane. Choroidal neovascularization developed during follow-up. Both patients had an otherwise uneventful personal and familial medical history. CONCLUSION: In patients with angioid streaks, diving headfirst can lead to subretinal hemorrhages and traumatic ruptures in Bruch membrane and increase the risk of maculopathy. Ophthalmologists should caution patients with angioid streaks against diving headfirst.
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PURPOSE: The purpose of this study was to report vertical transmission of macular telangiectasia type 2 and type 2 diabetes mellitus in 3 families. METHODS: In this retrospective interventional case series, the charts of patients with inherited macular telangiectasia type 2 were reviewed. A large spectrum of presentations of macular telangiectasia type 2 was observed and has been studied with different techniques including best-corrected visual acuity, microperimetry, confocal blue reflectance fundus autofluorescence, fluorescein angiography, and time domain and spectral domain optical coherence tomography. RESULTS: Vertical transmission of macular telangiectasia type 2 and associated type 2 diabetes mellitus is described in 3 families. Symptomatic as well as asymptomatic eyes with macular telangiectasia type 2 were identified. In 2 families, a mother and son experienced visual loss and were diagnosed with macular telangiectasia type 2. All 4 patients had type 2 diabetes. Diabetic retinopathy was observed in one mother and her son. In the third family, the index patient was diagnosed macular telangiectasia type 2 after complaints of metamorphopsia. She and her family members had type 2 diabetes mellitus, and further screening of her family revealed familial macular telangiectasia type 2. None of the patients were treated for macular telangiectasia type 2. CONCLUSION: Macular telangiectasia type 2 may be more common than previously assumed, as vision can remain preserved and patients may go undiagnosed. Screening of family members is indicated, and detection of mild anomalies is possible using fundus autofluorescence and spectral domain optical coherence tomography.
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BACKGROUND AND OBJECTIVE: To determine the normal ultrasonographic thickness of the iris and ciliary body. PATIENTS AND METHODS: This prospective 35-MHz ultrasonographic study included 80 normal eyes of 40 healthy volunteers. The images were obtained at the 12-, 3-, 6-, and 9-o'clock radial meridians, measured at three locations along the radial length of the iris and at the thickest section of the ciliary body. Mixed model was used to estimate eye site-adjusted means and standard errors and to test the statistical difference of adjusted results. Parameters included mean thickness, standard deviation, and range. RESULTS: Mean thicknesses at the iris root, midway along the radial length of the iris, and at the juxtapupillary margin were 0.4 ± 0.1, 0.5 ± 0.1, and 0.6 ± 0.1 mm, respectively. Those of the ciliary body, ciliary processes, and ciliary body + ciliary processes were 0.7 ± 0.1, 0.6 ± 0.1, and 1.3 ± 0.2 mm, respectively. CONCLUSION: This study provides standard, normative thickness data for the iris and ciliary body in healthy adults using ultrasonographic imaging.
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Cuerpo Ciliar/diagnóstico por imagen , Iris/diagnóstico por imagen , Microscopía Acústica/métodos , Adulto , Pesos y Medidas Corporales , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Valores de Referencia , Adulto JovenRESUMEN
PURPOSE: To investigate the benefit of preoperative treatment with either topical nonsteroidal anti-inflammatory drug (NSAID) or steroid in terms of clinical outcomes following trabeculectomy. DESIGN: Prospective, randomized placebo-controlled trial. PARTICIPANTS: Sixty-one patients. METHODS: Between July 2005 and October 2007, 61 consecutive medically uncontrolled glaucoma patients scheduled for first-time trabeculectomy were randomized to 1 of 3 study topical medication groups: nonsteroidal anti-inflammatory drugs (ketorolac), steroids (fluorometholone), or placebo (artificial tears). Patients instilled 1 drop 4 times daily for 1 month before the procedure and were examined on days 1 and 2, at weeks 1, 2, and 4, and at months 3, 6, 12, 18, and 24 after trabeculectomy. MAIN OUTCOME MEASURES: Incidence of postoperative surgical or medical interventions (needling, laser suture lysis, needling revision, and intraocular pressure [IOP]-lowering medication). RESULTS: Fifty-four patients (54 eyes) were entered for analysis. The mean number of preoperative medications was 2.3+/-0.9. The mean baseline IOP was 21.0+/-6.0 mmHg. The mean postoperative target IOP was 16.5+/-1.8 mmHg. The mean follow-up was 23.6+/-4.0 months. The percentage of patients requiring needling within the first year was 41% in the placebo group, 6% in the NSAID, and 5% in the steroid group (P = 0.006). The percentage of patients requiring IOP-lowering medication to reach the target IOP at 1 year was 24% in the placebo group, 18% in the NSAID group, and 0% in the steroid group (P = 0.054 overall; P = 0.038 for steroids vs. others). The log-rank test showed a significant (P = 0.019) difference in medication-free survival curves between the different groups. More specifically, patients in the steroid group needed significantly less medication over the total follow-up (P = 0.007). CONCLUSIONS: Topical ketorolac or fluorometholone for 1 month before surgery was associated with improved trabeculectomy outcomes in terms of likelihood of postoperative needling. In the steroid group, there was a significantly reduced need for additional postoperative IOP-lowering medication compared with the other groups. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
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Antiinflamatorios no Esteroideos/administración & dosificación , Glaucoma/cirugía , Glucocorticoides/administración & dosificación , Trabeculectomía , Administración Tópica , Anciano , Anciano de 80 o más Años , Antihipertensivos/administración & dosificación , Conjuntiva/efectos de los fármacos , Conjuntiva/patología , Método Doble Ciego , Femenino , Fibrosis/prevención & control , Fluorometolona/administración & dosificación , Glaucoma/tratamiento farmacológico , Humanos , Presión Intraocular , Ketorolaco/administración & dosificación , Masculino , Persona de Mediana Edad , Mitomicina/administración & dosificación , Complicaciones Posoperatorias/prevención & control , Cuidados Preoperatorios , Estudios Prospectivos , Resultado del TratamientoRESUMEN
BACKGROUND: The combination of verteporfin photodynamic therapy (PDT) and anti-angiogenics has been shown to be safe and efficacious in the treatment of choroidal neovascularization (CNV) secondary to age-related macular degeneration (AMD). The purpose of this study is to demonstrate long-term prevention of vision loss and improvement in best-corrected visual acuity (BCVA) after treatment with one-time reduced-fluence-rate PDT followed by administration of ranibizumab on a variable dosing regimen over 24 months in patients with neovascular AMD. Secondary outcome measures included the change in central macular thickness (CMT), reinjection frequency, and safety. METHODS: This prospective, nonrandomized, open-label, single-center study enrolled 27 consecutive patients (27 eyes) presenting at the Leuven University Eye Hospital with previously untreated, active neovascular AMD between September 2006 and January 2007. All patients were treated with one-time, reduced-fluence-rate verteporfin PDT, followed by intravitreal ranibizumab 0.5 mg on the same day. A second and third ranibizumab injection were given at weeks 4 and 8, respectively, after which patients were followed up monthly for 24 months. Additional treatment with ranibizumab was administered to eyes with active neovascularization as indicated clinically and on imaging studies. Retreatment was based on the following criteria: (1) presence of subretinal fluid (SRF), intraretinal edema or sub-retinal pigment epithelial fluid, as seen on OCT; (2) increase of CMT by >100 mm on OCT; (3) signs of active CNV leakage on fluorescein angiography; (4) new sub- or intraretinal hemorrhage; and (5) BCVA decreased of > or =5 letters on the Early Treatment of Diabetic Retinopathy Study (ETDRS) chart. If any single criterion for reinjection was fulfilled, retreatment with ranibizumab was administered. RESULTS: Twenty-five patients completed the 2-year study. Occult CNV was present in 64% and retinal angiomatous proliferative (RAP) lesions were present in 24% of the study eyes. The remaining three eyes had lesions classified as classic (one eye) or predominantly classic (two eyes) CNV. Month 24 data are available for 25 eyes (25 patients; age 55-86 years; mean 77; standard deviation (SD) = 7.2). Mean baseline VA was 58.6 letters (range: 35-70; SD = 8.4); 24-month VA was 66.2 letters (35-82; 12.7), not including one warfarin-treated patient who suffered vitreous hemorrhage. The mean visual acuity improved by 7.2 letters (p < 0.05) and the mean CMT decreased by 146 mum. VA improved >3 lines (15 letters) in 16%; improved 1-3 lines in 20%; remained within one line of baseline in 32%, decreased 1-3 lines in 16%, and decreased >3 lines in 16%. Losses of >3 lines were due to vitreous hemorrhage, geographic atrophy, fibrosis, and growth of an initially small CNV lesion. An average of 5.1 injections (range: 3-9) were administered during the first 12 months, and 7.1 injections (3-13) over 24 months. A total of 178 injections were performed; no systemic side-effects, uveitis, or choroidal collateral vascular damage were observed. Two patients were lost to follow-up. CONCLUSION: Combined PDT and ranibizumab injection the same day was well tolerated in all patients. Eighty-four percent of patients had stable or improved vision at month 24.