Idiopathic polypoidal choroidal vasculopathy of the macula

OBJECTIVE: The authors evaluated the clinical, fluorescein, and indocyanine green (ICG) angiographic characteristics of the macular variant of idiopathic polypoidal choroidal vasculopathy (IPCV). DESIGN: Observational case series. PARTICIPANTS: The records, photographs, and fluorescein and ICG angiograms of eight eyes of seven patients with IPCV lesions confined to the macula were reviewed. MAIN OUTCOME MEASURES: The visual acuity, fundus examination, fluorescein and ICG angiographic characteristics, and clinical course were compared. RESULTS: All patients demonstrated polypoidal lesions arising from macular choroidal vessels on ICG angiography. One patient had bilateral lesions. These lesions appeared hyperfluorescent in the early phases of both fluorescein and ICG angiography. Late-phase leakage was seen in cases associated with subretinal fluid or exudate. None of these patients demonstrated polypoidal lesions arising from the peripapillary choroidal circulation or peripapillary choroidal neovascularization. Three eyes with polypoidal lesions that were associated with subretinal fluid and exudates were treated with photocoagulation. Five eyes were not treated. Final visual acuity ranged from 20/20 to hand motions. Severe visual loss was associated with vitreous and subretinal hemorrhage, but this resolved without permanent severe visual loss in several cases. CONCLUSIONS: In the macular variant of IPCV, ICG and fluorescein angiography demonstrate characteristic macular polypoidal lesions without evidence of peripapillary lesions. The vascular origin of these polypoidal lesions appears to be the macular choroidal circulation. This is distinguished from classic IPCV, in which lesions appear to arise from the peripapillary choroidal circulation. Visual prognosis appears to be good, with most patients retaining visual acuity of 20/80 or better. If subretinal fluid or exudates reduce visual acuity, photocoagulation should be considered.     

Bad ethics, good ethics and the genetic engineering of animals in agriculture

PURPOSE: The purpose of this retrospective study is to compare the measurements of intrapapillary and peripapillary parameters between two observers and test the usefulness of measuring different types of crescents. METHODS: Optic disc photographs of 23 eyes of 23 patients with glaucoma and 23 age-matched normal eyes were measured in Oulu and in Erlangen using manual planimetric techniques. The authors measured the following magnification corrected intrapapillary and peripapillary areas: optic disc, neuroretinal rim, cup: disc area ratio, scleral ring, central (zone beta), and peripheral peripapillary atrophy (zone alpha). Twenty-one patients with glaucoma had a follow-up of 3.2 years (range, 1.1-4.7 years), and follow-up for 19 control eyes was 3.7 years (range, 2.5-5.9 years). The measurements were performed in a masked fashion for the diagnosis and temporal sequence of the photographs. RESULTS: Central peripapillary atrophy (zone beta) was statistically significantly largest in primary open-angle glaucoma in both centers (Oulu, P=0.003; Erlangen, P=0.004), whereas normal and exfoliative eyes did not differ significantly from each other. The results for peripheral peripapillary atrophy (zone alpha) and scleral ring were less consistent. Despite statistically significant interobserver correlations ranging from r=0.30 (scleral ring area; P=0.0472) to r=0.97 (optic disc area; P=0.0001), the means of all parameters, except for zone alpha and beta, differed statistically significantly between the two observers. CONCLUSIONS: The central peripapillary atrophy, or zone beta, is the most reproducible parameter when measuring peripapillary atrophy in glaucoma. Nonetheless, its measurement is of limited usefulness in the recognition of glaucoma or progression of glaucomatous nerve damage.     

Survey of complications of indocyanine green angiography in Japan

PURPOSE: We evaluated the safety of indocyanine green for use in fundus angiography. METHODS: We sent a questionnaire concerning complications of indocyanine green to 32 institutions in Japan, which were selected on the basis of the client list from the Topcon Company, which manufactures the indocyanine green fundus camera. RESULTS: Ophthalmologists at 15 institutions responded, reporting a total of 3,774 indocyanine green angiograms performed on 2,820 patients between June 1984 and September 1992. Before angiography, intradermal or intravenous indocyanine green testing, or both was performed at 13 of 15 institutions. For three patients, the decision was made not to proceed with angiography after positive preangiographic testing. The dosage of indocyanine green used for angiography varied from 25 to 75 mg, depending upon the institution. There were 13 cases of adverse reactions (0.34%), ten of which were mild reactions such as nausea, exanthema, urtication, itchiness, and urgency to defecate, and did not require treatment. Also recorded were one case of pain of the vein, which required treatment, and two cases of hypotension. The two hypotensive patients required treatment for shock. CONCLUSIONS: A comparison of frequency of adverse reactions to indocyanine green with the previously reported frequency of such reactions to fluorescein sodium indicated that indocyanine green is a safe as fluorescein for use in angiography.     

Clinical picture and therapy of hypertrophic cardiomyopathy

Although central vision in Stargardt's disease is impaired relatively early, peripheral function is usually little affected and patients do not lose all vision. We report 4 patients with bull's-eye macular dystrophy that is indistinguishable from Stargardt's disease/fundus flavimaculatus, but with very depressed peripheral retinal function. One patient had bull's-eye maculopathy with a dark choroid on the fluorescein angiogram with a normal peripheral fundus and good retinal function initially. Sixteen years later, however, peripheral bone corpuscle pigmentation and optic disc atrophy developed and the electroretinogram became nonrecordable. Three patients from another family also had peripheral pigment degeneration besides macular degeneration. These cases may represent a rare combination of Stargardt's disease and retinitis pigmentosa. However, there was no clinical sign or even suggestion of retinitis pigmentosa at the initial examination in 1 patient. The mode of inheritance in 3 of our patients is probably autosomal recessive.     

A co-relation of the eye and kidney-in diabetes mellitus and hypertension

The study was undertaken to observe the co-relation between microangiopathic changes in diabetic retinopathy and microvascular changes in diabetic nephropathy. Included in the study were 64 patients with chronic renal failure who were on maintenance dialysis, 40 had hypertension alone, 21 hypertension and diabetes and 3 had diabetes alone. On examination of retina, of 40 hypertensive patients, 14 had positive findings, while in the hypertensive and diabetic group, 20 patients out of 21, had positive findings. Nine patients in the hypertensive group had delayed choroidal filling on fluorescein angiography which was not very accurately reflected on Funduscopy. In the diabetic and hypertensive group, 13 patients having proteinuria of more than 1 gm, also had exudates and haemorrhages in the fundus. It was concluded that a correlation exists between the arterial changes in the fundus of the eye and the glomeruli of the kidney.     

Hepatic lobar atrophy: association with ipsilateral portal vein obstruction

OBJECTIVE: This study was performed to evaluate the association between hepatic lobar atrophy, bile duct obstruction, and portal vein obstruction. MATERIALS AND METHODS: Thirty cases of hepatic lobar atrophy identified on angiography with CT during arterial portography from August 1992 to March 1995 were retrospectively reviewed by two independent observers. Cases were evaluated for vascular patency and bile duct obstruction. Malignant diagnoses were present in 28 of 30 patients. RESULTS: Twenty-two patients (73%) had atrophy in the left lobe and eight patients (27%) had right lobar atrophy. Portal vein obstruction was unilateral and confined to the atrophic lobe in 26 patients (87%). In contrast, bile duct obstruction was bilateral in 23 patients (77%) and in only four patients (13%) was it isolated to the atrophic lobe. The correlation between atrophy and portal vein obstruction was significant, with 90% sensitivity, 97% specificity, and 96% positive predictive value (p < .00001). For the correlation between atrophy and biliary obstruction, the sensitivity of angiography with CT during arterial portography was 90%, specificity was 23%, and positive predictive value was 54% (p = .17). CONCLUSION: Hepatic lobar atrophy usually occurs in the setting of combined biliary and portal vein obstruction. A significant correlation exists between hepatic lobar atrophy and ipsilateral portal vein obstruction.     

Indocyanine green angiography in choroidal osteoma

BACKGROUND: Choroidal osteoma is a rare choroidal tumor; knowledge of its indocyanine green characteristics is limited. METHODS: The fundus photographs and the fluorescein and indocyanine green angiograms of three patients were reviewed. Each patient was examined at least twice with a follow-up varying from 10 to 60 months. RESULTS: Late-phase fluorescein angiograms allow assessment of the extension of the osteoma as it is variably hyperfluorescent due to tumor staining combined with a variable degree of overlying retinal pigment epithelial changes. The hypofluorescent area observed in the early phase of the indocyanine green angiogram corresponds with the extent of the osteoma but the borders may be difficult to demarcate. In the late phase of the indocyanine green angiogram, hypofluorescence due to choriocapillaris loss and hyperfluorescence due to leakage from abnormal choroidal vessels are combined. Infrared angiography high-lights abnormal choroidal vessels and vascular spiders present on the tumor surface. It is difficult to differentiate these choroidal vascular anomalies from subretinal neovascularization. CONCLUSIONS: We find no homogeneous pattern either on fluorescein or on infrared angiography. The findings may change with follow-up, indicating changes within the tumor or the surrounding tissue that are still poorly understood.     

Can vomiting after squint surgery be prevented?

We prospectively evaluated the safety of the washout period from topical beta-adrenergic blockers prior to clinical drug trials. In 30 consecutive patients optic disc parameters (neural rim, optic disc, peripapillary halo and atrophy areas, and venous/arterial diameters) did not change following washout (p > 0.05). Likewise, the mean defect and pattern standard deviation (Humphrey 30-2) did not change following washout (p > 0.05). This study suggests, generally, the safety of the washout period prior to clinical drug trials.     

White fibrous papulosis of the neck

Since its introduction, fluorescein angiography has been widely used to investigate diseases of the ocular fundus. A case of fatal acute myocardial infarction after intravenous fluorescein angiography is presented. This appears to be the first case documented by autopsy in which the findings are compatible with myocardial infarction as the cause of death. Although there are no known contraindications for fluorescein angiography in patients with a history of cardiovascular disease, the indications for this elective procedure should be carefully reviewed in such patients. Adequate emergency resuscitation equipment should be available in the fluorescein angiography suite.     

Indocyanine green angiography in enlarged blind spot syndrome

PURPOSE: To report the indocyanine green angiography findings in a case of acute idiopathic blind spot enlargement syndrome. METHOD: The patient underwent ophthalmologic examination with fluorescein angiography and indocyanine green angiography. RESULTS: A monocular enlarged blind spot was found on automated perimetry; fluorescein angiography showed a hypofluorescent peripapillary atrophic area and indocyanine green angiography highlighted diffuse, small hypofluorescent spots scattered throughout the posterior pole. Visual field defects and indocyanine green angiography abnormalities resolved over 4 weeks. CONCLUSION: Indocyanine green angiography in acute idiopathic blind spot enlargement syndrome showed many lesions not visible with fluorescein angiography, indicating a choroidal involvement reaching not only the peripapillary area but the entire posterior pole.     

Recombinant GM2-activator protein stimulates in vivo degradation of GA2 in GM2 gangliosidosis AB variant fibroblasts but exhibits no detectable binding of GA2 in an in vitro assay

OBJECTIVE: To examine choroidopathy in patients with Beh?et disease. DESIGN: Prospective clinical study. PARTICIPANTS: Thirty-three patients (63 eyes) with Beh?et disease. INTERVENTION: Patients underwent simultaneous indocyanine green (ICG) and fluorescein angiography with a double detector of scanning laser ophthalmoscopy. MAIN OUTCOME MEASURES: Angiographic findings recorded on videotapes were evaluated. The relation of angiographic findings with systemic activity and aqueous inflammation was also analyzed. RESULTS: Fluorescein angiography showed leakage in varying degrees from retinal vessels in 30 patients (53 eyes, 84%). The ICG angiographic findings were choroidal vascular wall staining in 16 eyes (25%), hyperfluorescent spots in 42 eyes (66%) and hypofluorescent plaques in 22 eyes (35%), both of which were not evident with fluorescein, leakage from choroidal vessels in 3 eyes (5%), and irregular filling of choriocapillaris in 11 eyes (17%). These findings did not have a statistically significant correlation with the presence or absence of aqueous inflammation or oral aphthous ulcerations. CONCLUSIONS: The patients with Beh?et disease showed choroidal abnormalities, which could be revealed only by ICG angiography, but not with funduscopy or fluorescein angiography. Simultaneous ICG and fluorescein angiography would be useful for examining choroidal lesions in Beh?et disease.     

Synchronous bilateral fluorescein angiography. A new technique

Simultaneous angiography of both eyes allows the synchronous measurement of the arm-retina-time and the retinal circulation time in each eye. The dye-filling pattern of retinal arteries, choroid, disc, and cilioretinal vessels can be compared. A new method of simultaneous fluorescein angiography of both eyes, using two fundus cameras, is demonstrated. Each camera is operated by a separate power unit, thereby providing sufficient light energy for one picture every 0.6 second. The fluorescein is injected by means of an automatic injector in order to create a compact dye bolus. Simultaneous angiograms of a healthy patient and of a patient with unilateral carotid occlusion are shown.     

Incident light microscopic characterization of vascular patterns in skin tumors

The goal of the study was to determine, using a nonhuman primate (NHP) model, the minimum duration of elevated intraocular pressure (IOP) (induced by an inflated catheter) necessary to produce significant visual system deficits. In Old World monkeys (Macaca mulatta), a catheter was placed retrobulbarly in one orbit and inflated with saline for either 180 min (10 monkeys) or 240 min (six monkeys subjects). Baseline color fundus photographs, monochromatic photography, fluorescein angiography, and IOP measurements were performed preoperatively and at either 2, 4, or 6 weeks postoperatively on both eyes of each monkey prior to killing and histological analysis. Optic neuropathy was demonstrated in eight of these NHPs. In the two most severe cases (240 min inflation condition), complete nerve fiber atrophy with central retinal artery occlusion was observed. Sector nerve fiber atrophy, extending from the temporal disc to beyond the macula, was noted in the other six monkeys, five of which were in the 180 min inflation condition. Thus, optic neuropathy, sufficient to produce visual loss, was noted following increased IOP (> or = 50 mm Hg) for 180 min or 240 min. These data emphasize the need for timely intervention to mitigate the potential detrimental effects of retrobulbar hemorrhage when it occurs in humans.     

Case-control study of the risk factors for age related macular degeneration. France-DMLA Study Group

AIM: A case-control study was initiated to determine the risk factors for the development of age related macular degeneration (AMD). METHODS: Study participants, who were all white, aged 50-85 years, and were recruited from private ophthalmology practices. Each practitioner enrolled patients with bilateral AMD, who were then matched with controls for sex and age. Environmental factors and systemic and ocular histories were screened. All patients had bilateral red-free fundus photographs and fluorescein angiography. Photographs were classified into pigment epithelium alterations, drusen, geographic atrophy, and exudative AMD. Statistical analysis included the identification of risk factors for AMD. A multivariate analysis was performed at the end of the study. Analysis included the entire study population and was carried out for each stage of AMD. RESULTS: 1844 controls were compared with 1844 patients with AMD. Mean age was 71 years for controls and 72 for cases. Logistic regression identified six major risk factors for AMD (whole population): arterial hypertension (odds ratio (OR) = 1.28), coronary disease (OR = 1.31), hyperopia (OR = 1.33), light coloured irises (OR = 1.22), and lens opacities or previous cataract surgery (OR = 1.55). The significance of vascular risk factors was increased for late stages of AMD, especially the atrophic forms (coronary disease, OR = 3.19). CONCLUSIONS: This large case-control study confirms some of the risk factors previously identified and may contribute to the determination of methods for prevention of AMD.     

A case of nonarteritic anterior ischemic optic neuropathy with cilioretinal artery occlusion

We report a case of nonarteritic anterior ischemic optic neuropathy (AION) with cilioretinal artery occlusion. The patient was a 61-year-old man with sudden visual loss in his right eye. Funduscopy showed pale swelling of the entire optic disc with retinal ischemic edema of the upper half of the retina, and fluorescein angiography revealed faint filling of the dye in the optic disc in the retinal arterial phase, and dye staining of the optic disc in the late phase. We initially diagnosed the disease as AION with branch retinal artery occlusion, but systemic administration of a corticosteroid and urokinase were ineffective and the optic disc became atrophic. As the optic disc swelling decreased and the course of arteries in the optic disc became clear, we repeated fluorescein angiography which proved that the involved upper retinal artery was a cilioretinal artery having earlier dye appearance than the lower retinal artery. Thus, we finally diagnosed the disease as AION with cilioretinal artery occlusion. We believe that Hayreh's view that AION may result from occlusion of the posterior ciliary artery is supported by the intraocular findings in this case.     

Atypical aortic coarctations in type I neurofibromatosis

AIM: Quantification of haemodynamics of the peripapillary choroid in and the assessment of possible differences between normal subjects (N), ocular hypertensive (OHT), primary open angle (POAG), and normal pressure glaucoma (NPG) patients. METHODS: Video fluorescein angiograms (Rodenstock SLO 101) were made in 22 N subjects, 12 OHT, 48 POAG, and 46 NPG patients. The angiographically derived dye build up curves were described by means of an exponential model. One of the model parameters is the time constant tau theoretically reflecting local blood refreshment time; the blood refreshment time tau is the time needed to replace the blood volume in the choriocapillaris, inversely proportional to the local choroidal blood flow. Other variables are maximal fluorescence (Fdt) and time of first fluorescence (t0). Mean variable values were calculated for disc area and circular areas around the disc. RESULTS: Fdt of the disc was significantly lower in the POAG and NPG patients. There was no statistical difference in t0 between the study groups. The choroidal blood refreshment time was significantly longer in NPG patients and to a lesser extent in the POAG patients compared with the normal controls. The slowest choroidal blood refreshment can be found in the NPG group. The median choroidal blood refreshment times (25th-75th percentile) in the controls, OHT, POAG, and NPG patients were 4.1 (3.7-4.5), 4.4 (3.7-6.4), 5.8 (4.3-6.8), and 7.1 (5.5-9.3) seconds respectively. CONCLUSIONS: With the help of parametrisation of dye curves, using a one compartmental model, choroidal haemodynamics can be quantified. The blood refreshment time of the peripapillary choriocapillaris was found to be significantly prolonged especially in NPG patients; this may indicate slower choroidal haemodynamics in NPG patients.     

Cerebral aneurysmal arteriopathy in childhood AIDS

OBJECTIVE: To identify cerebral aneurysmal arteriopathy in children with longstanding AIDS. BACKGROUND: Five cases are described from the authors' experience, and eight additional cases are reviewed from the literature. Details are presented in regard to the clinical picture as well as brain imaging with cerebral angiography and magnetic resonance angiography in some cases. Autopsy information is available in four cases, including one of the authors' patients. RESULTS: Seven patients acquired HIV infection perinatally, five patients were infected by blood transfusions, and one patient had both risk factors. In the four postmortem patients, the vascular pathology was similar, showing ectasia and aneurysmal dilatation confined to the large arteries of the circle of Willis. Medial fibrosis and loss of muscularis with destruction of the internal elastic lamina and intimal hyperplasia was common. The latency period following infection varied from 2 to 11 years. Once a diagnosis of aneurysmal arteriopathy was made, the patients deteriorated rapidly, with death occurring in less than 6 months. CONCLUSIONS: The development of cerebral aneurysmal arteriopathy in childhood AIDS tends to occur after a prolonged delay and is usually followed by death in a short period of time. The etiology for the vasculitis is unknown. Varicella zoster virus may be the etiology in some of the cases because of its potential to cause this pathology and the striking unilateral arterial involvement found in Case 5. HIV vasculitis is also a possibility, as suggested by the detection of HIV protein or genomic material in two of the four autopsy cases.     

The intramitochondrial ATP/ADP-ratio controls cytochrome c oxidase activity allosterically

BACKGROUND: This study was carried out to evaluate intraocular or systemic factors associated with the visual field damage progression in eyes with normal-tension glaucoma (NTG). PATIENTS AND METHODS: Forty-seven NTG eyes with a minimum follow-up of 5 years were enrolled into the retrospective study. A stepwise regression analysis was performed to correlate the visual field damage progression, expressed as the mean deviation (MD) change per year, with several independent clinical factors including age, history of disc hemorrhage, initial MD, mean intraocular pressure (IOP), peak IOP, diurnal fluctuation of IOP, presence of a beta zone of peripapillary atrophy, and use of Ca(2+)-channel blockers. RESULTS: Statistical analysis revealed that non-use of Ca(2+)-channel blockers (P = 0.01), peripapillary atrophy (P = 0.03) and disc hemorrhage (P = 0.04) were associated with visual loss progression. CONCLUSIONS: Risk factors unrelated to IOP were suggested to be associated with progression of visual field loss. Systemic use of Ca(2+)-channel blockers has a favorable effect on visual field prognosis in NTG eyes.     

Role of indocyanine green fluorescence videoangiography in evaluation of subretinal disease

BACKGROUND: Indocyanine green (ICG) is a sterile, water-soluble, tricarbocyanine dye that can be used in fundus angiography as an adjunct to sodium fluorescein. It has a peak spectral absorption of 805 nm in blood plasma or blood, as compared with fluorescein, which has a peak spectral absorption of 465 nm. Because the absorption and emission of ICG lies around 835 nm, transmission of energy by the retinal pigment epithelium (RPE) and serosanguinated material is more efficient in this region than in the region of visible light energy. ICG has the property of being approximately 98% bound to blood protein, disallowing extravasation of excessive dye in the highly fenestrated choroidal vasculature. METHODS: The characteristics of ICG are discussed, including administration and dosage, adverse reactions and use of infrared filters for fundus photography. In addition, two cases are presented to illustrate the clinical application of ICG for diagnosis and treatment of choroidal neovascular membranes. RESULTS: ICG videoangiography can be used to reveal subfoveal choroidal neovascular membranes not previously identified with fluorescein; angiograms can also be used to dramatically highlight retinal and choroidal changes. CONCLUSIONS: The use of ICG for fundus videoangiography provides a more accurate and complete evaluation in certain cases of subretinal and choroidal disease.     

Intravitreous injections of vascular endothelial growth factor produce retinal ischemia and microangiopathy in an adult primate

PURPOSE: The purpose of the study is to determine the effect of exogenous vascular endothelial growth factor (VEGF) on the primate retina and its vasculature. METHODS: Ten eyes of five animals were studied. Physiologically relevant amounts of the 165 amino acid isoform of human recombinant VEGF were injected into the vitreous of six healthy cynomolgus monkey eyes. Inactivated human recombinant VEGF or vehicle was injected into four contralateral control subject eyes. Eyes were assessed by slit-lamp biomicroscopy, tonometry, fundus color photography, fundus fluorescein angiography, light microscopy, and immunostaining with antibodies against proliferating cell nuclear antigen and factor VIII antigen. RESULTS: All six bioactive VEGF-injected eyes developed dilated, tortuous retinal vessels that leaked fluorescein. Eyes receiving multiple injections of VEGF developed progressively dilated and tortuous vessels, venous beading, edema, microaneurysms, intraretinal hemorrhages and capillary closure with ischemia. The severity of the retinopathy correlated with the number of VEGF injections. None of the four control eyes exhibited any abnormal retinal vascular changes. The endothelial cells of retinal blood vessels were proliferating cell nuclear antigen positive only in the bioactive VEGF-injected eyes. CONCLUSION: Vascular endothelial growth factor is sufficient to produce many of the vascular abnormalities common to diabetic retinopathy and other ischemic retinopathies, such as hemorrhage, edema, venous beading, capillary occlusion with ischemia, microaneurysm formation, and intraretinal vascular proliferation.