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Biomedical subjects

K Bailey Freund

Publications and source records attributed to K Bailey Freund.

At least 19 recordsLinked to original sources

Idiopathic macular telangiectasia.

OBJECTIVES: To review the frequency and nature of idiopathic macular telangiectasia and to classify the disorders based on new clinical and imaging observations. METHODS: A combined retrospective and prospective analysis of newly diagnosed patients seen over a period of 3 years. Patients were identified based on the Gass-Blodi classification and were studied with biomicroscopy, fluorescein angiography, and optical coherence tomography. RESULTS: Ten patients associated with aneurysmal telangiectasia (Gass-Blodi group 1) and 26 patients with perifoveal telangiectasia (Gass-Blodi group 2) were recruited. None with occlusive telangiectasia (Gass-Blodi group 3) were identified. New observations based on clinical, fluorescein angiographic, and optical coherence tomographic findings were made. CONCLUSIONS: Our series was similar to that in the Gass-Blodi study in terms of frequency. New observations in groups 1 and 2 have expanded our knowledge of the clinical spectrum of these disorders. A simplified classification termed idiopathic macular telangiectasia with 2 distinct types (type I, or aneurysmal telangiectasia, and type II, or perifoveal telangiectasia) was proposed to produce a better understanding of the entities and to enhance teaching and research. The third type, occlusive telangiectasia, has been omitted from our classification based on its rarity and presence of capillary nonperfusion rather than macular telangiectasia as the primary abnormality.

Adult↗

Sequenced combined intravitreal triamcinolone and indocyanine green angiography-guided photodynamic therapy for retinal angiomatous proliferation.

OBJECTIVE: To study sequenced combined therapy using intravitreal triamcinolone acetonide followed by photodynamic therapy for the treatment of retinal angiomatous proliferation. METHODS: Patients newly diagnosed as having retinal angiomatous proliferation underwent intravitreal triamcinolone injection to reduce intraretinal and subretinal exudation, followed 7 to 14 days later by indocyanine green angiography-guided photodynamic therapy with verteporfin. Complete ocular examination, fluorescein angiography, indocyanine green angiography, and optical coherence tomography were performed at baseline and at standard intervals thereafter. RESULTS: Twenty-seven eyes of 26 patients underwent this sequenced combined treatment and were followed up for 12 months. The triamcinolone injection reduced the cystoid edema before photodynamic therapy. Complete resolution of the angiographic leakage was achieved in 89% of eyes. Visual acuity improved in 37% and was stable in 52% of eyes. Eight eyes developed recurrent leakage after 3 to 11 months. Complete resolution of leakage was observed after subsequent treatment. CONCLUSIONS: This sequenced combined treatment in patients with retinal angiomatous proliferation was effective in reducing or eliminating the edema, achieving rapid regression of neovascularization, and stabilizing or improving visual acuity. To our knowledge, no study to date has achieved such promising results in the management of retinal angiomatous proliferation. A randomized clinical trial is under way to compare sequential and simultaneous combined therapy.

Aged↗

Multiple evanescent white dot syndrome.

OBJECTIVES: To study the clinical and angiographic features of lesions in a case series of multiple evanescent white dot syndrome (MEWDS), to describe a newly recognized clinical variation of the disorder, and to gain insight into its pathophysiological nature. METHODS: Five patients with MEWDS (selected based on angiographic manifestations of the disorder) were examined using slitlamp biomicroscopy and studied using fluorescein angiography and indocyanine green angiography. RESULTS: All 5 patients exhibited the newly recognized angiographic features termed dots and spots, which varied in size and location in the fundus. Small dots were in the inner retina or at the level of the retinal pigment epithelium, and larger spots were more external in the subpigment epithelial area. All patients exhibited other characteristics typical of MEWDS, including field loss and foveal granularity. CONCLUSIONS: In this case series of MEWDS, a clinical variant consisting of dual-layered lesions with specific features on clinical examination, fluorescein angiography, and indocyanine green angiography was identified. On late indocyanine green angiography, these lesions produced highly specific findings of small hypofluorescent lesions overlying larger hypofluorescent lesions. Based on the angiographic findings, it seems as if MEWDS is a chorioretinopathy with varying degrees of retinal and choroidal involvement.

Adult↗

Fundus autofluorescence in patients with pseudoxanthoma elasticum.

PURPOSE: To evaluate the autofluorescence findings of patients with pseudoxanthoma elasticum, a disease resulting from a defect in a reputed transport protein encoded by the gene adenosine triphosphate-binding cassette subtype C number 6. DESIGN: Observational case series. METHODS: Color, red-free monochromatic, and autofluorescence photography and fluorescein angiography of patients with pseudoxanthoma elasticum seen in a referral practice were evaluated. MAIN OUTCOME MEASURES: Cataloging of the abnormalities as detected by autofluorescence photography. RESULTS: The 8 subjects ranged in age from 26 to 60 years (mean, 55+/-12), and their best-corrected visual acuity ranged from 20/20 to 5/400 (mean, 20/50). Of the 16 eyes of the 8 patients, all had abnormalities typical of pseudoxanthoma elasticum, including angioid streaks in 14, peau d'orange in 4, and choroidal neovascularization in 11. Angioid streaks appeared as hypoautofluorescent fissures, sometimes showing expansion of the hypoautofluorescence suggestive of retinal pigment epithelium (RPE) absence or atrophy. Peau d'orange had a stippled appearance of autofluorescence, and drusen of the optic nerve appeared as hyperautofluorescent bodies. In addition to the expansion of RPE atrophy around angioid streaks, 3 additional configurations of RPE atrophy were recognized as RPE rips in 6 eyes, multilobular areas of atrophy in 9 eyes, and broad areas of poorly demarcated atrophy in 5 eyes. Some eyes had more than one manifestation of RPE atrophy, but the latter 3 types of atrophy occurred in eyes with, but not necessary contiguous to, concurrent choroidal neovascularization. CONCLUSIONS: Autofluorescence photography demonstrated that patients with pseudoxanthoma elasticum have more widespread areas of RPE disturbance, particularly atrophy, than what is detectable by other means of ocular imaging, which suggests that the RPE disturbance may play a role in the pathogenesis of visual loss in patients with pseudoxanthoma elasticum.

Adult↗

Vitelliform macular dystrophy.

PURPOSE: To investigate and integrate the photographic, angiographic, and tomographic findings from a group of patients with various stages of vitelliform macular dystrophy type 2 (VMD2; also known as Best's disease) and use this information to propose mechanisms of disease pathogenesis. DESIGN: Retrospective observational case series. PARTICIPANTS: Nine consecutive patients seen in a private practice referral setting by the authors. METHODS: Patients with VMD2 were imaged with conventional fundus and autofluorescence photography, fluorescein angiography, fundus photography, and optical coherence tomography (OCT). MAIN OUTCOME MEASURES: The integrated ocular imaging findings. RESULTS: Early stage lesions were smaller and had accumulation of yellowish material in the central macula. This material was highly autofluorescent and appeared to be located on the outer retinal surface by OCT. Later stages were characterized by larger lesions with central clearing of the yellowish material and deposition of autofluorescent subretinal material at the outer borders of the lesion. Both early and late lesions had a subretinal fluid component with no reflectivity as detected by OCT. Fluorescein angiography showed transmission defects with a suggestion of late leakage, much like that seen in chronic central serous chorioretinopathy (CSC). CONCLUSIONS: Similar to that seen in CSC, patients with VMD2 have an accumulation of material on the outer retina, which may represent shed photoreceptor outer segments in association with subretinal fluid.

Adolescent↗

Intravitreal bevacizumab (Avastin) treatment of macular edema in central retinal vein occlusion: a short-term study.

PURPOSE: To report the short term anatomic and visual acuity response after intravitreal injection of bevacizumab (Avastin, Genentech) in patients with macular edema due to central retinal vein occlusion (CRVO). METHODS: The authors conducted a retrospective study of patients with macular edema due to CRVO who were treated with at least one intravitreal injection of bevacizumab 1.25 mg in 0.05 mL. Patients underwent Snellen visual acuity testing, optical coherence tomography (OCT) imaging, and ophthalmoscopic examination at baseline and follow-up visits. RESULTS: There were 16 eyes of 15 consecutive patients with a mean age of 76.1 years (SD 9.8 years). Intravitreal triamcinolone had been previously administered to 9 patients, but all of these patients either had no improvement or had excessive intraocular pressure caused by the triamcinolone. The patients received a mean of 2.8 injections of bevacizumab per eye. No adverse events were observed, including endophthalmitis, clinically evident inflammation, increased intraocular pressure, retinal tears, retinal detachment, or thromboembolic events in any patient. The mean central macular thickness at baseline was 887 microm and decreased to a mean of 372 microm at month 1 (P < 0.001). The mean baseline acuity was 20/600 (logMAR = 1.48) and the mean acuity at month 1 was 20/200 (logMAR = 1.05), a difference that was highly significant (P = 0.001). At last follow-up, a mean of 3 months after the first injection, the mean visual acuity was 20/138 (logMAR = 0.84), which was significantly better than baseline (P < 0.001). Visual acuity improvement, defined as a halving of the visual angle, was seen in 14 of the 16 eyes. CONCLUSION: Initial treatment results of patients with macular edema secondary to CRVO did not reveal any short-term safety concerns. Intravitreal bevacizumab resulted in a significant decrease in macular edema and improvement in visual acuity. The number of patients in this pilot study was limited and the follow-up is too short to make any specific treatment recommendations, but the favorable short-term results suggest further study is needed.

Aged↗

Anecortave acetate treatment for retinal angiomatous proliferation: a pilot study.

PURPOSE: The purpose of this study was to evaluate anecortave acetate treatment of retinal angiomatous proliferation (RAP), a neovascular form of age-related macular degeneration, with specific regard to inhibition of neovascularization and maintenance of vision. METHODS: Thirty-four patients with RAP with any stage of neovascularization were randomized 1:1:1 for treatment with three different quantities (30 mg, 15 mg, 3 mg) of anecortave acetate sterile suspension for juxtascleral administration. Best-corrected visual acuity (Early Treatment Diabetic Retinopathy Study chart), intraocular pressure measurement, biomicroscopy, funduscopy, digital fluorescein, and indocyanine green angiography were recorded at baseline and at 3 months. A 6-month retreatment interval was established for this study with a follow-up of 12 months. In selected patients optical coherence tomography was performed. The outcomes were mean changes in visual acuity and lesion size at 1 year. RESULTS: The detachment of the neurosensory retina and retinal pigment epithelium improved in all eyes, but all neovascular lesions increased in size. Vision loss occurred in the majority of study eyes (22 out of 34 eyes, 64.7%) independent of the concentration administered. CONCLUSION: The results suggest that a posterior juxtascleral injection of anecortave acetate reduces capillary permeability in patients with RAP. However, in spite of improvement of the exudation there is a progression of neovascularization and a significant loss of vision in all these patients. Like other monotherapeutic methods used to treat this variant of neovascular age-related macular degeneration, anecortave acetate alone does not appear to benefit these patients. Future studies should investigate a combination form of therapy.

Aged↗

Anecortave acetate for the treatment of idiopathic perifoveal telangiectasia: a pilot study.

PURPOSE: To investigate the use of anecortave acetate, a new angiostatic cortisene, for the treatment of the leakage and/or neovascularization associated with idiopathic perifoveal telangiectasia (IPT) in an open label prospective pilot study. METHODS: Seven eyes of six patients were treated with posterior juxtascleral administration of anecortave acetate delivered adjacent to the macula using a specially designed curved cannula. A full clinical examination and fluorescein angiography were performed at baseline and at 3-month intervals. The primary efficacy outcome for this pilot study was the mean change in visual acuity (Early Treatment Diabetic Retinopathy Study) from baseline. RESULTS: The visual acuity remained unchanged in two eyes of two patients with nonproliferative disease after 24 months. The five eyes of four patients presenting with subretinal neovascularization, the proliferative stage of IPT, showed stabilization or improvement of lesion size, resolution of leakage, and stabilization of vision at last follow-up. CONCLUSION: The results of this study suggest that anecortave acetate may inhibit retinal and subretinal permeability as well as neovascular proliferation in patients with IPT. A larger study accordingly should be designed in the future to evaluate the effectiveness and treatment of IPT with this drug.

Adult↗

Oral phosphate binders in the treatment of pseudoxanthoma elasticum.

BACKGROUND: Pseudoxanthoma elasticum (PXE) is a systemic connective tissue disorder involving elastic fiber calcification and fragmentation with major clinical manifestations occurring in the cutaneous, ocular, and cardiovascular systems. Normalization of the serum calcium-phosphate product through hemodialysis in a previous patient with perforating periumbilical PXE and elevated serum phosphate resulted in regression of skin lesions. OBJECTIVE: We sought to study the effect of pharmacologically limiting the intestinal absorption of phosphate in patients with PXE. METHODS: Patients received baseline skin examinations, target skin lesion evaluation, and photography; renal function tests and serum calcium and phosphate levels; urine calcium, phosphate, and creatinine levels; skin biopsy; and eye examinations and indocyanine-green angiography. Patients were treated with aluminium hydroxide tablets or liquid and returned every 2 to 4 months for skin photography and lesion evaluation. Repeated skin biopsies were performed on clinically improved target sites. Ophthalmologic evaluation was obtained at yearly intervals. RESULTS: Of 6 patients, 3 showed significant clinical improvement of skin lesions and all 3 of these patients showed histopathologic regression of disease in their target lesions. No deterioration of eye disease was seen in any of the 6 patients at 1-year follow-up. CONCLUSION: Our results demonstrate that the calcification seen in PXE may be reversible in some patients. This could hold true for eye and vascular lesions and for skin. Further studies supporting these results could reveal the first real treatment option for PXE.

Adult↗

Peripapillary detachment in pathologic myopia.

OBJECTIVE: To describe peripapillary detachment in pathologic myopia (PDPM), a newly recognized fundus lesion. DESIGN: Retrospective medical record review. METHODS: We evaluated a series of myopic eyes that had a yellow-orange elevation of the retina and retinal pigment epithelium at the inferior border of the myopic conus. RESULTS: Twenty eyes of 15 patients were identified during a 17-year period to have characteristic findings of PDPM. The mean age of the patients was 58 years. They were followed up for an average of 6 years. The mean spherical equivalent correction was -11.00 diopters (D) (range, -6.00 to -16.00 D). The mean axial length was 27.4 mm (range, 25.3-28.9 mm). In each case, ophthalmic coherence tomographic examination showed a localized detachment of the retinal pigment epithelium and retina corresponding to the PDPM lesion. During the follow-up period, the lesion remained stable in all cases except for 1. No apparent negative effect on visual function was noted. CONCLUSIONS: Peripapillary detachment in pathologic myopia is an asymptomatic, yellow-orange peripapillary detachment of the retinal pigment epithelium and retina in pathologic myopia. Recognition of this lesion is important to distinguish it from other fundus pathologic conditions, such as tumors or choroidal neovascularization, which require further investigation and treatment.

Adult↗

Verteporfin infusion-associated pain.

PURPOSE: To determine if oral hydration decreases the incidence of verteporfin infusion-associated pain and to find out if other factors play a role in predisposing to this undesired complication. METHODS: Nonrandomized clinical trial. We prospectively examined 250 consecutive patients who have been diagnosed with subfoveal choroidal neovascularization secondary to age-related macular degeneration and received photodynamic therapy using verteporfin. One hundred twenty-five patients were assigned to receive 500 ml of water orally administered 30 minutes before beginning the verteporfin infusion, and the remaining 125 consecutive patients were used as controls. Historical and clinical factors in these patients were evaluated for their association with the presence of verteporfin infusion-associated pain. RESULTS: Out of 125 patients receiving water before treatment 12 (9.6%) experienced verteporfin infusion-associated pain. Among the 125 patients who did not get hydration before therapy 12(9.6%) experienced verteporfin infusion-associated pain. There was no statistical difference between the incidence of pain in the two groups (P = 1.0). No statistically significant association was evidenced between the presence of pain and participant's baseline characteristics, except for pain on previous administration of verteporfin (P < .001). Out of 250 total patients 24 (9.6%) developed verteporfin infusion-associated pain. Back pain was the most common and occurred in 21 (8.4%) patients, but other sites included leg, groin, chest, buttock, arm, and shoulder pain concurrently or independently. All patients had resolution of their pain, including chest pain, on cessation of the infusion. CONCLUSIONS: Verteporfin infusion-associated pain may be more common than has been previously reported and is not limited to the back area. It appears to be an idiosyncratic reaction to the drug. It does not seem to be prevented by oral hydration before infusion of verteporfin, and no baseline characteristics, other than a history of pain on previous infusion, seem to be predictive of verteporfin infusion-associated pain.

Administration, Oral↗

Treatment of idiopathic subfoveal choroidal neovascular lesions using photodynamic therapy with verteporfin.

PURPOSE: To report the effects of photodynamic therapy using verteporfin in the treatment of patients with subfoveal idiopathic choroidal vascularization (CNV). DESIGN: Interventional case series. METHODS: In a retrospective study, eight eyes of eight consecutive patients aged 55 years or younger with subfoveal idiopathic CNV treated with photodynamic therapy using verteporfin were evaluated. Visual acuity was considered to be improved if the visual angle was halved, while acuity was thought to be worse if the visual angle doubled. RESULTS: The eight patients included three men and five women with a mean (+/- SD) age of 34.6 (+/- 9.7) years (range 25-53 years). The mean follow-up time was 13.5 months. At the end of the follow-up period the visual acuity improved in five eyes (62.5%), remained unchanged in one (12.5%), and decreased in two (25%). The mean acuity improvement was 3.6 lines of Snellen acuity by the end of the follow-up period, a change that was statistically significant (P =.027, Wilcoxon signed-rank test). No patient had any complication from the treatment. CONCLUSIONS: There is no widely accepted method for treating subfoveal idiopathic CNV and all previously investigated methods have had a significant number of serious side effects. Although the follow-up time and the number of patients in this pilot study were limited, the encouraging results and lack of complications suggest that further study is indicated.

Adult↗

Corticosteroids and central serous chorioretinopathy.

PURPOSE: The purpose of this study is to investigate the relationship between corticosteroid use and central serous chorioretinopathy (CSC). DESIGN: A prospective, case-controlled study. PARTICIPANTS AND CONTROLS: A consecutive series of patients with acute manifestations of CSC and a control group matched for age, race, and gender were recruited between January 2000 and July 2000. METHODS: A detailed clinical history was taken, and fundus examination with slit-lamp biomicroscopy was performed on all patients. Fluorescein angiography was obtained on the study patients. RESULTS: A total of 50 patients was recruited. Twenty-six patients (52%) had a history of exogenous steroid use, including oral, intravenous, intranasal, and intraarticular administration. Two additional patients had a history of endogenous hypercortisolism (Cushing's syndrome). In a matched control group, eight patients (18%) had a history of steroid use. The difference in corticosteroid exposure between study patients and controls was statistically significant (P < 0.0001). MAIN OUTCOME MEASURES: History of corticosteroid use or Cushing's syndrome. CONCLUSIONS: This study is consistent with previous reports associating steroid use with CSC. It identifies corticosteroids as a significant risk factor for the development of acute, exudative macular manifestation and implicates hypercortisolism as a factor in the pathogenesis of this disorder. Several forms of corticosteroid administration were observed to be a risk factor for CSC. Accordingly, susceptible patients in need of corticosteroids should be advised of the risk of developing acute manifestations of CSC.

Administration, Intranasal↗