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Massive subhyaloidal hemorrhage associated with severe PAI-1 deficiency.

PURPOSE: To report an association between spontaneous subhyaloidal hemorrhage and severe plasminogen activator inhibitor-1 (PAI-1) deficiency. METHODS: Case report. RESULTS: A 29-year-old woman presented with sudden, painless visual loss to hand motion in her right eye. Ophthalmoscopy showed a massive subhyaloidal hemorrhage. The patients' medical history was negative for cardiovascular risk factors, trauma, infections or bleeding complications. Further investigation into possible causes revealed hyperfibrinolysis secondary to severe PAI-1 deficiency. The non-clearing subhyaloidal hemorrhage was successfully treated by pars plana vitrectomy, and her visual acuity improved to 20/20. CONCLUSION: When ordering laboratory tests in patients with spontaneous subhyaloidal hemorrhage to rule out fibrinolytic disorders, severe PAI-1 deficiency should be considered in the differential diagnosis. Selective screening may be helpful in identifying ophthalmologic patients with hyperfibrinolysis, especially in young individuals with subhyaloidal hemorrhages in the absence of other recognized risk factors.

Adult↗

Digital imaging in differential diagnosis of small choroidal melanoma.

BACKGROUND: To assess the role of digital imaging and a new subtraction method for differential diagnosis of choroidal nevus and small choroidal melanoma. METHODS: Of 241 consecutive patients referred to a tertiary referral center for suspected choroidal melanoma, 110 who underwent digital imaging of the ocular fundus were eligible for this study. Digital color, red-free and red light retinal images were evaluated in a randomized and masked manner and by the subtraction method for diagnosis of the fundus lesion. The reference standard was based on the combined results of ophthalmological examination, including mydriatic ophthalmoscopy, B scan ultrasonography, digital imaging and fluorescein angiography of the ocular fundus. RESULTS: Comparative use of digital color, red-free and red light imaging had 85.7% (95%CI 42.1-99.6) sensitivity, 99.0% (95%CI 94.7-99.9) specificity and 98.2% (95%CI 93.6-99.8) exact agreement versus reference standard in differentiation of small choroidal melanoma from pseudomelanoma. Direct comparison between use of digital images and the reference standard showed excellent agreement in detecting small choroidal melanoma from suspected choroidal lesions (K 0.847; 95%CI 0.639-1.0). The subtraction method was useful to show growth in four of 94 melanocytic choroidal tumors. The mean annual incidence of choroidal melanoma in Southwest Finland was 0.80 per 100.000 population. The most frequent choroidal pseudomelanomas were choroidal melanotic and amelanotic nevi, disciform lesions, congenital hypertrophy of the retinal pigment epithelium, and circumscribed choroidal hemangioma. CONCLUSIONS: Combined use of digital color, red-free and red light imaging was a suitable adjunct in differentiation of small choroidal melanoma from different pseudomelanomas. The subtraction method may reveal early growth of the melanotic choroidal tumors.

Adolescent↗

Massive bilateral vitreoretinal hemorrhage in patient with chronic refractory idiopathic thrombocytopenic purpura.

BACKGROUND: We describe a patient with chronic, refractory idiopathic thrombocytopenic purpura (ITP) who developed massive vitreoretinal hemorrhages in both eyes. METHODS: A 49-year-old woman complained of an acute decrease of vision, and ophthalmoscopy revealed bilateral preretinal and subretinal hemorrhages around the optic disc. Her platelet counts were less than 5000/mul in spite of repetitive administration of corticosteroids and immunoglobulins with splenectomy for the chronic refractory ITP. One month later, she developed massive vitreoretinal hemorrhages bilaterally, and vision decreased to hand-motion. RESULTS: Vitrectomy was performed on both eyes after medical treatment to increase platelet counts. The intraretinal hemorrhages were absorbed from the area around the disc and vision recovered to 20/100 in the right eye and 20/2000 in the left after one month. CONCLUSION: Massive vitreoretinal hemorrhages can develop in patients with chronic refractory ITP, and vitrectomy can be beneficial for this condition.

Chronic Disease↗

Quantification and anatomic distribution of choroidal abnormalities in patients with type I neurofibromatosis.

BACKGROUND: Choroidal abnormality manifesting as a bright patchy lesion under infrared monochromatic light has previously been described in neurofibromatosis type I patients in whom the choroid appears normal under conventional ophthalmoscopic examination or on the fluorescein angiogram. We investigated the correlation between patient age and the number of choroidal abnormalities, as well as the anatomic distribution of choroidal abnormalities in the fundus. METHODS: We examined the fundus of 28 eyes in 14 patients with neurofibromatosis type I. Patients ranged in age from 2 to 38 years and were examined between April 2001 and April 2002 by confocal scanning laser ophthalmoscopy with infrared monochromatic light (780 nm wavelength). We divided the fundus into five regions (one within the retinal vascular arcade and those supero-temporal, infero-temporal, supero-nasal, and infero-nasal to it), and lesions on the border between regions were assigned to the region containing the greater part of the lesion. We studied the total number of choroidal abnormalities and the correlation between the total number and age. RESULTS: A positive correlation was found between the total number of choroidal abnormalities and age (Spearman rank correlation coefficient, r=0.6209, P=0.0178). There was a significantly greater number of choroidal abnormalities in the arcade region than in the other four regions (ANOVA, P<0.001). CONCLUSIONS: Choroidal abnormalities tend to increase with age and are most often observed within the vascular arcade.

Adolescent↗

Behavior of retinal epithelium to bleb detachment versus retinectomy.

PURPOSE: To compare the behavior of the retinal pigment epithelium to a bleb detachment versus removal of a segment of neural retina. METHODS: A bleb detachment was performed on 14 adult pigmented rabbits. In seven rabbits, the neural retina was removed within the bleb detachment. The rabbits were followed for months after surgery by scanning laser ophthalmoscopy and post mortem histology using light and electron microscopy. RESULTS: Bleb detachment produces a transformation of the retinal epithelial layer that results in migration, enlargement of cell size and accumulation of large amounts of lysosomal material, resembling that found in Chediak-Higashi syndrome. Retinectomy causes migration of the epithelial cells, development of multiple layers with vacuoles but no accumulation of lysosomal material. CONCLUSION: The neural retina appears to exert a strong influence on the behavior of the retinal epithelial layer. Brief separation of the neural retina from the epithelium provokes a rapid transformation of this cell layer leading to migration of the cells and apparent faulty digestion of phagosomes, causing an enormous buildup of lysosomal debris. Removal of the neural retina also provokes retinal epithelial cell migration but no buildup of lysosomal debris occurs, presumably due to the absence of photoreceptor outer segments and consequently phagosomes. This migratory tendency, which appears to alter lysosomal degradation, could lead to apoptosis of these epithelial cells.

Animals↗

Light-induced damage of the retina through slit-lamp photography.

BACKGROUND: I report on two patients who suffered severe loss of visual acuity after slit-lamp photography of the anterior eye segment. METHODS: Both patients were pseudophakic and pictures were taken to document the degree of posterior capsule opacification via retroillumination with dilated pupils. Both patients had excellent visual acuity at the time of photography was taken and experienced reduction of to hand movement afterwards. A Zeiss 75 SL device was used for the slit-lamp photography. In cooperation with Carl Zeiss Jena the slit lamp was investigated. RESULTS: Ophthalmoscopy and angiography after photography discovered a central macular defect, similar to a photocoagulation. The visual acuity did not recover. CONCLUSION: Under certain circumstances slit-lamp photography of the anterior eye segment can lead to severe light-induced damage of the retina.

Aged↗

Photoreceptor allografts in a feline model of retinal degeneration.

BACKGROUND: Photoreceptor transplants provide a potential means to restore function in a degenerate retina and/or rescue degenerating host photoreceptors by trophic influences. We have examined photoreceptor allografts in the Abyssinian cat model of hereditary photoreceptor degeneration to determine the viability and influence of such transplants on the host retina. METHODS: Small pieces of 3- to 5-day-old normal kitten retina containing undifferentiated photoreceptors were injected into the subretinal space of adult Abyssinian cats at an early stage of retinal degeneration using standard vitreo-retinal surgical techniques. The retinas were examined by ophthalmoscopy and fundus photography, then by light and electron microscopy at different times after surgery. RESULTS: Such allografts survive for at least 6 months after surgery. The photoreceptors develop outer segments, invariably in rosettes. The transplants gradually integrate with the host retina but detach the host photoreceptor layer from the retinal pigment epithelium (RPE), which tends to reduce the number of host photoreceptors over the transplant. There is no slowing of the photoreceptor degeneration in neighboring non-detached retina. Inflammation or rejection was not detected. CONCLUSION: Undifferentiated, neonatal photoreceptor allografts survive and develop outer segments in the subretinal space of the Abyssinian mutant feline retina. The allografts gradually integrate with the host neural retina without inducing rejection. In the vicinity of the transplant there is increased loss of host photoreceptors, considered to be due to their detachment from the RPE layer. There is no evidence of any rescue of host photoreceptors elsewhere in this mutant retina.

Animals↗

A model for xenogenic immune response.

PURPOSE: To develop a model for analyzing the immune response after xenogenic human fetal retinal pigment epithelium (HFRPE) transplantation. MATERIALS AND METHODS: Pure sheets of HFRPE cells were isolated and attached to poly (DL-lactide-co-glycolide) polymer films and HFRPE spheroids were formed. The spheroids were transplanted into the subretinal space of New Zealand albino rabbits and were observed for 5 months. Bare polymer films were transplanted into the subretinal space of Dutch Belted pigmented rabbits, serving as control. RESULTS: The polymer film was biodegraded within 3 weeks in the subretinal space. No signs of inflammation in the retina or choroid were observed. The HFRPE spheroids were easily transplanted into the subretinal space. The immune response was followed with ophthalmoscopy. Light microscopy indicated a localized immune response in the transplanted area in which the retina and the choroid were infiltrated with immune cells. This infiltration was denser in the choroid. CONCLUSIONS: HFRPE spheroid transplantation may be utilized as a model for studying the xenogenic immune response after HFRPE transplantation. This model may also have applications in evaluating the role of immune suppressive agents in preventing rejection after HFRPE transplantation.

Absorbable Implants↗

Spectrum of color gene deletions and phenotype in patients with blue cone monochromacy.

Blue cone monochromacy (BCM) is an X-linked ocular disease characterized by poor visual acuity, nystagmus, and photodysphoria in males with severely reduced color discrimination. Deletions, rearrangements and point mutations in the red and green pigment genes have been implicated in causing BCM. We assessed the spectrum of genetic alterations in ten families with BCM by Southern blot, polymerase chain reaction, and sequencing analysis, and the phenotype was characterized by ophthalmoscopy, fluorescein angiography, and a battery of tests to assess color vision in addition to routine ophthalmological examination. All families showed clinical features associated with BCM. Acuities were reduced in all affected males, and photopic b-wave was reduced by more than 90% in seven families. In three families, however, the photopic b-wave response showed uncharacteristic relative preservation of 30-80% (of the clinical low-normal value). The color vision was unusually preserved in two affected males, but this was not correlated with photopic electroretinography retention. Progressive macular atrophy was observed in affected members of two BCM families while the rest of the families presented with normal fundus. In nine families deletions were identified in the gene encoding the red-sensitive photopigment and/or in the region up to 17.8 kb upstream of the red gene which contains the locus control region and other regulatory sequences. In the same nine families the red pigment gene showed a range of deletions from the loss of a single exon to loss of the complete red gene. In one family no mutation was found in the exons of the red gene or the locus control region but showed loss of the complete green gene. No association was observed between the phenotypes and genotypes in these families.

Adult↗

Application of revised diagnostic criteria for vogt-koyanagi-harada disease in Japanese patients.

PURPOSE: To determine whether the International Revised Diagnostic Criteria for Vogt-Koyanagi-Harada (VKH) disease is applicable to Japanese patients at the early stage of VKH disease. METHODS: The medical records of 49 patients with VKH disease were studied. The patients had been examined at the Akita University School of Medicine Hospital between 1989 and 2001, and their mean age was 47.6 years with a range of 15 to 69 years. In our study, the patients were divided into two groups; an Early Group, which consisted of those who were examined within 1 month of the onset of signs or symptoms, and a Late Group, which consisted of those who were examined more than 1 month after the onset of signs or symptoms. The initial diagnosis was based on findings by ophthalmoscopy, fluorescein angiography, pleocytosis of cerebrospinal fluid, and genotyping of human leukocyte antigen (HLA). The final diagnosis was based on the presence of these findings in addition to skin and internal ear manifestations of VKH disease. RESULTS: In our retrospective study, 41 of the 49 patients were placed in the Early Group, and 8 were placed in the Late Group. When classified by the Revised Diagnostic Criteria, 39 of the 41 in the Early Group would have been diagnosed with incomplete VKH, and the other 2 patients would have been diagnosed as not having VKH disease. None of the patients would have been diagnosed as having complete VKH disease, even at 2 weeks after the onset of symptoms or signs. At the final examination, 35 patients would still have been classified as having incomplete VKH disease, and only 6 patients would have been classified as having complete VKH disease according to the Revised Diagnostic Criteria. In the Late Group, all of the patients would have been diagnosed as having incomplete VKH at 2 weeks after the onset of any signs or symptoms. At the final examination, two of eight patients would still have been diagnosed as having incomplete VKH, and the other six would have been diagnosed as having complete VKH. The skin manifestations always appeared later than the other alterations, with an average of 143.5 days from disease onset to detection. CONCLUSIONS: Although the Revised Diagnostic Criteria are effective for making the final diagnosis of VKH disease, they are not an effective tool for diagnosis at the very early stage of VKH disease in Japanese patients.

Acute Disease↗

Radiation retinopathy caused by low dose irradiation and antithyroid drug-induced systemic vasculitis.

BACKGROUND: We report on a patient with Graves' disease with radiation retinopathy caused by low-dose irradiation and antithyroid drug-induced antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis. CASE: A 38-year-old woman with Graves' disease presented with bilateral blurred vision, microaneurysms, telangiectasia, and macular edema. The patient was examined by ophthalmoscopy and fluorescein angiography, and radiation retinopathy was diagnosed. OBSERVATIONS: The patient had been treated with low-dose irradiation for her Graves' ophthalmopathy a few years earlier. She also had ANCA-positive vasculitis induced by the antithyroid drug (propylthiouracil, PTU) that had been prescribed for her at that time. Because of multiple avascular areas on both retinas, she was treated by intensive retinal photocoagulation to control progressive retinopathy. CONCLUSIONS: The radiation doses used to treat Graves' disease ophthalmopathy are low. Nevertheless, there is still a risk of radiation retinopathy developing in patients with PTU-induced ANCA-positive vasculitis.

Adult↗

Population-based study of nonproliferative diabetic retinopathy among type 2 diabetic patients in Kinmen, Taiwan.

PURPOSE: This study was conducted to assess the prevalence and associated factors of nonproliferative diabetic retinopathy among type 2 diabetic patients in Kinmen, Taiwan. METHODS: From 1991 to 1993, 971 type 2 diabetic patients in Kinmen underwent diabetic retinopathy screening performed by a panel of ophthalmologists using indirect ophthalmoscopy and 45 degrees color fundus retinal photographs. RESULTS: Of the 971 patients screened in 1991-1993, 578 (59.5%) were examined for this study. Diabetic retinopathy was diagnosed in 127 patients (22.0%), including nonproliferative diabetic retinopathy in 13.3%, proliferative diabetic retinopathy in 1.4%, legal blindness in 1.4%, and ungradable diabetic retinopathy in 5.9%. Significant associated factors of nonproliferative diabetic retinopathy based on multiple logistic regression analysis were fasting plasma glucose (FPG) at baseline [> or =126 mg/dl vs. <126 mg/dl; odds ratio (OR) = 2.89; 95% confidence interval (CI), 1.01-9.09], 2-h postload at baseline (> or =200 vs. <200 mg/dl; OR = 1.48; 95% CI, 1.09-2.07); HbA1c at follow-up (> or =7% vs. <7%; OR = 6.54; 95% CI, 3.01-14.20), duration of diabetes (> or =15 years vs. <10 years; OR = 6.72; 95% CI, 2.13-21.18), and incremental systolic blood pressure between baseline and follow-up (OR = 1.02; 95% CI, 1.00-1.04). CONCLUSIONS: In addition to the longer duration of type 2 diabetes, FPG at baseline, poorly controlled glucose concentration, and altered blood pressure may increase the risk of nonproliferative diabetic retinopathy in type 2 diabetic patients.

Adult↗

Dilute triamcinolone-assisted peeling of the internal limiting membrane in cases of diffuse diabetic macular oedema.

PURPOSE: In this study we evaluated the efficiency of diluted triamcinolone particles in peeling of the internal limiting membrane (ILM) in cases of diffuse diabetic macular oedema. METHODS: A prospective observational study of thirteen consecutive patients with diffuse diabetic macular oedema who had undergone ILM peeling aided by use of a diluted (1 mg mL(-1)) suspension of triamcinolone particles (0.05 mg). The results were assessed by measurement of visual acuity and intraocular pressure, by ophthalmoscopy, and by fluorescein angiography. RESULTS: ILM peeling in all eyes could be performed easily and completely with the aid of triamcinolone particles homogeneously dispersed over the macular ILM. During the peeling procedure, triamcinolone particles resulted in clear contrast between the peeled and unpeeled ILM thus enabling us to easily find the edge of the ILM and to be able to continue peeling at further attempts. In twelve of the thirteen eyes the macular oedema was completely resolved or reduced. Visual acuity improved by at least two lines in nine eyes (70%). The intraocular pressure increase did not exceed 21 mmHg in any of the patients. CONCLUSIONS: The diluted triamcinolone suspension enables good visualisation of the ILM during the ILM peeling procedure in cases of diffuse diabetic macular oedema. Use of a diluted suspension may reduce the risk of intraocular pressure increase and the potential toxicity of triamcinolone.

Aged↗

A portable monocular indirect ophthalmoscopic technique.

A simple, inexpensive, and portable method of monocular indirect ophthalmoscopy is used as the physician's examining eye is aligned in the axis of the patient's eye, a flashlight is held against the examiner's malar area beneath the dominant eye, and the beam of the flashlight is directed to the patient's eye. A plus 30 lens is then interposed 1 or 2 inches in front of the patient's eye and the fundus viewed.

Humans↗

Clinical features of idiopathic preretinal macular fibrosis. Schoenberg Lecture.

Of 150 consecutive cases of preretinal macular fibrosis that occurred predominantly in older subjects, 118 cases involved only one eye while 32 occurred binocularly. The ratio of women to men was 3:2. Visual acuity was usually 20/50 or better, and only four of 178 eyes had acuity below 20/200. The vast majority of lesions involved the macula and few extended outside the macular zone. The characteristic clinical finding on ophthalmoscopy was a glinting reflex probably due to a layer of connective tissue cells lying on the internal limiting membrane. Small white exudates, venous abnormalities, and occasional hemorrhages may also be part of prepretinal macular fibrosis. Amsler grid testing usually demonstrated irregularity and distortion of some part of the grid. Fluorescein angiography revealed a characteristic tortuosity of the small macular vessels. Most eyes had previous posterior vitreous detachments but, except for one possible case, there were no cases where the posterior vitreous body was adherent to either the retinal membrane or the macular region. There was no significant systemic disease related to preretinal macular fibrosis. In most instances, preretinal macular fibrosis either was stationary or slowly progressive. The etiology and treatment of the condition remains obscure.

Adult↗

Rentrolental fibroplasia in a full-term infant.

A 3,800-g infant, born after 40 weeks' gestation, developed retrolental fibroplasia, documented by indirect ophthalmoscopy. Elevated arterial oxygen tensions may have occurred during brief treatment for two episodes of cyanosis.

Adult↗

Experimental intravitreal gentamicin.

Rabbits received intravitreal injections of gentamicin and indirect ophthalmoscopy, electroretinography, and histopathology determined relative toxicity. An intravitreal dose of 0.1 to 0.2 mg of commercially available gentamicin was well tolerated in the rabbit eye. A dosage of 0.4 mg or higher consistently produced significant ocular toxicity as evidenced by extinction of the electroretinogram, gross fundus changes, and histopathologic alterations.

Animals↗

Early diagnosis and evolution of dominant retinitis pigmentosa.

A 5-month-old girl with retinitis pigmentosa was the healthy offspring of a family with progressive autosomal dominant retinitis pigmentosa. Serial evaluation by both electroretinograph and ophthalmoscopy over a four-year period confirmed the diagnosis and indicated that as early as 5 months of age, electroretinographic abnormalities were present in the patient. These consisted of a reduction in the dark-adapted b-wave amplitude, prominent X-wave, and no suppression of the second b-wave response to paired light stimuli at 75 msec. Progressive reduction in the scotopic electroretinography amplitude has occurred over the ensuing four years, although light-adapted (cone) function still appears normal. Clinical evidence of retinitis pigmentosa became apparent only late in the patient's third year when fine peripheral pigment clumping and vessel attentuation could be seen.

Adolescent↗