The pathogenesis of telangiectasis in the bovine liver. I. The incidence of Telangiectasis.
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BACKGROUND: Idiopathic juxtafoveolar retinal telangiectasis may cause visual loss. The treatment of this disease is controversial. METHODS: The authors reviewed the records of 140 patients with idiopathic juxtafoveolar telangiectasis. A classification scheme based on biomicroscopic and fluorescein angiographic findings is presented. In addition, the effect of photocoagulation on the natural history of the disorder is evaluated. FINDINGS: Patients are categorized into three groups. Group 1 comprises 39 male patients with nonfamilial, easily visible telangiectasis and intraretinal exudation. The telangiectasis is unilateral in 94% of patients. The telangiectasia in this group is probably of developmental origin (Coats syndrome). Group 2 comprises 94 patients with occult juxtafoveolar telangiectasis, minimal exudation, superficial retinal crystalline deposits, and right-angle venules. Late in the course of the disease, foveolar atrophy, intraretinal pigment plaques, and subretinal neovascularization develop. The telangiectasis is acquired during middle age and is bilateral in 98% of patients. Group 3 comprises seven patients with bilateral easily visible telangiectasis, minimal exudation, and capillary occlusion. All of these patients had systemic disease, which was probably related to their eye disease. CONCLUSION: Slow visual loss beginning in adulthood characterizes most of these patients. The telangiectasis appears to be caused primarily by retinal capillary leakage in group 1, capillary diffusion abnormalities in group 2, and capillary occlusion in group 3. Photocoagulation is probably beneficial for patients in group 1 and not for patients in group 2, at least before their development of subretinal neovascularization.
The association between retinitis pigmentosa and retinal telangiectasis has been rarely reported. The case of a young woman affected with retinitis pigmentosa and telangiectasis involving bilaterally and symmetrically the inferior retinal periphery is described. Moreover, the patient showed a high degree edema of the retina produced by the permeabilization of the retinal capillaries (especially foveal capillaries) and by the passage of fluid from the choroid through the damaged retinal pigment epithelium. The telangiectasis were successfully treated with cryotherapy. The telangiectasis found in patients with retinitis pigmentosa have to be distinguished from other forms of telangiectasis, as those found in young males or those of the temporal retinal periphery of adults, in which respect they have particular characteristics. The cause of the telangiectasis and of the breakdown of the blood-retinal barrier in retinitis pigmentosa is not know. It is probable that they are produced by a toxic or inflammatory action on the vessels mediated by retinal antigens.
Five patients with mild nonproliferative diabetic retinopathy had visual loss associated with parafoveal telangiectasis. Minimal macular edema with characteristic parafoveal plaques of subretinal pigment epithelial hyperplasia was seen in all patients. Fluorescein angiography revealed the presence of ectatic, dilated, leaking perifoveal capillaries. The occurrence of parafoveal telangiectasis in patients with diabetic retinopathy has, to our knowledge, not been previously described. One previous clinicopathologic report described the histologic appearance of parafoveal telangiectasis in a nondiabetic patient to be similar to that seen in diabetic patients. The association of parafoveal telangiectasis and diabetic retinopathy raises interesting speculations into the pathogenesis of the entity of parafoveal telangiectasis. In addition, the importance of obtaining fluorescein angiography prior to therapy of diabetic macular edema is emphasized.
PURPOSE: An increasing variety of eponymous terms have been used to describe atypical cases of Coats' disease. A group of typical cases of Coats' disease and other cases of differing severity were classified as one of four subtypes of idiopathic retinal telangiectasis with exudation and compared with regard to clinical outcome. METHODS: In a retrospective clinical review patients with typical and atypical Coats' disease were classified as severe, focal, juxtafoveal or associated (with another disease) forms of idiopathic retinal telangiectasis with exudation. RESULTS: 53 eyes in 50 patients were examined of which 62% (n=31) were male. 12 eyes were classified as severe (group 1), 22 focal (group 2), 12 juxtafoveal (group 3) and 7 associated (group 4). The mean age at diagnosis was lowest in group 1 eyes (6.8 years). The best visual acuity at presentation was 6/60 in group 1 whereas high proportions of eyes in the other groups had initial visual acuities of 6/24 or better. In group 1 only one eye was treated, the majority of eyes were blind or had been enucleated whereas 34 (79%) of eyes in the other groups were suitable for treatment and 29 eyes (67%) retained pre-treatment visual acuity or better at last follow-up. CONCLUSIONS: Idiopathic retinal telangiectasis with exudation is a spectrum of disease, which is synonymous with Coats' disease. In this retrospective study eyes with severe idiopathic retinal telangiectasis with exudation corresponding to typical Coats' disease, have poorer vision at presentation, are less suitable for treatment and have worse outcomes than eyes with other subtypes. The spectrum of disease severity seen in idiopathic retinal telangiectasis with exudation may be due to second somatic mutations in genes with an existing germline mutation (the two hit theory) and a mosaic phenotype.
Forty six patients with paramacular retinal telangiectasis were reviewed; unilateral telangiectasis was present in 26 and bilateral disease was present in 20. Patients with unilateral macular telangiectasis had the more extensive vascular involvement associated with exudation and were more likely to have peripheral telangiectasis. Bilateral macular telangiectasis was associated with pigment epithelial changes and sub-retinal scars. Visual acuity was mildly reduced in the majority of patients; severe loss of vision was most frequent in patients with bilateral macular disease. The evolution of vascular changes was very slow and only 5 patients lost vision of two lines or more during an average period of 40 months. The high incidence of peripheral changes in unilaterally involved patients implies the possibility of a primary defect of the retinal vessels in these patients. There is evidence to suggest that there may be a primary defect of the retinal pigment epithelium in some patients with bilateral involvement.
Twenty-seven healthy adult patients had visual loss in one or both eyes because of exudation from juxtafoveolar retinal capillary telangiectasis of uncertain cause. These patients were subdivided as follows: group 1, men with uniocular involvement, intraretinal lipid exudation, and telangiectasis largely confined to the temporal half of the juxtafoveolar area; group 2, mostly men with symmetric areas of telangiectasis affecting the temporal half of the juxtafoveolar areas and minimal intraretinal exudation; group 3, both sexes with symmetric involvement of all of the parafoveolar capillary bed an minimal exudation; and group 4, one case of telangiectasis with occlusive perifoveolar capillary changes and familial optic disc pallor. The visual acuity prognosis in groups 1 through 3 is relatively good. Photocoagulation may be of some value in the treatment of patients in group 1.
PURPOSE: To report the outcome of a patient with a subretinal neovascular membrane secondary to idiopathic juxtafoveolar retinal telangiectasis treated with photodynamic female therapy. METHODS: Interventional case report. A 43-year-old female with type 2A idiopathic juxtafoveolar retinal telangiectasis presented with a 2-week history of decreased vision and a subretinal neovascular membrane in the left eye. RESULTS: Visual improvement from 20/70-2 to 20/50-2 in the left eye was observed after two photodynamic therapy treatments of a juxtafoveal subretinal neovascular membrane with verteporfin, and was maintained through 7 months of follow-up. Fluorescein angiography showed no leakage specific to the subretinal neovascular membrane, but leakage from the juxtafoveal telangiectasis continued. CONCLUSION: A substantial improvement was seen in this patient after treatment of the juxtafoveal subretinal neovascular membrane by photodynamic therapy. Given the poor prognosis of these lesions, photodynamic therapy may therefore be a useful treatment for patients with subretinal neovascular membranes associated with idiopathic juxtafoveolar retinal telangiectasis.
Forty-five livers from conventionally slaughtered Holstein-Friesian steers with telangiectasis were studied by histochemical methods, immunolabelling for fibronectin, laminin and type IV collagen, and transmission electron microscopy. None of the previously described changes in telangiectasis (necrosis, hepatitis, thromboembolism, dilatation of the space of Disse by glycogen extruded from hepatocytes and reduced density of the perisinusoidal reticulin framework) were evident. Pretelangiectasis (sinusoidal dilatation) and telangiectasis (blood-filled cavities) were characterized by sinusoidal barrier alterations, leading to sinusoidal capillarization; and there was progressive formation of a true basement membrane and perisinusoidal fibrosis. Comparison of bovine liver telangiectasis and human peliosis hepatis suggests that they have a similar pathogenesis. It is suggested that a primary alteration of the sinusoidal barrier is responsible for an increased deposition of basement membrane components (fibronectin, laminin, type IV collagen) in the perisinusoidal region, and fibrosis. These are likely to render the exchange of oxygen and substrates between blood and hepatocytes more difficult and to produce haemodynamic imbalances, leading to hepatocyte atrophy and eventually to sinusoidal disruption.
PURPOSE: To study the different clinical presentation of the idiopathic juxtafoveolar retinal telangiectasis and its treatment with argon laser. MATERIAL AND METHOD: We studied retrospectively nine eyes of eight patients, seen from 1990 to 1997, with diagnosis of idiopathic juxtafoveolar retinal telangiectasis. They have been studied with fluorescein angiography and treated with argon laser. RESULT: The most frequent clinical presentation was large areas of retinal telangiectasis affecting the horizontal rafe temporal to the macula. The diagnosis was stabilised as regards clinical manifestation and after ruling out other causes. Six out of eight patient with visual disfunction for macular edema and exudation improved considerably after laser photocoagulation. In 3 patients the vascular abnormalities were progressive after the treatment. CONCLUSIONS: The idiopathic juxtafoveolar retinal telangiectasis with exudation is an uncommon disease, that causes visual disturbance, but may be treated with laser photocoagulation.
The surgical treatment of telangiectasis synchronous to sclerotherapy is presented in this paper. Two female patients with extensive telangiectasis of the lower limbs comprise our report. They were considered typical cases for application of the proposed technique: abolishing venous reflux. The adjacent veins were resected through skin incisions of about 2 mm with crochet hooks, under local anesthesia, immobilized with adhesive tape. Sclerotherapy was performed simultaneously. The cosmetic result was excellent in both cases, with complete removal of the telangiectasis. This procedure should be considered the method of choice in the treatment of telangiectasis resulting from obvious venous reflux.
The beneficial effect of laser therapy on idiopathic juxtafoveolar telangiectasis is questionable. We therefore studied retrospectively the spontaneous course of the disease and tried to show a correlation between the initial angiographic findings and visual outcome. In addition, the results after laser therapy were compared with untreated patients. Thirteen patients (24 eyes) with idiopathic juxtafoveolar telangiectasis diagnosed by fluorescein angiography were reexamined after a mean time interval of 38 months. Argon laser coagulation had been performed in 6 patients. The initial extension of fluorescein leakage was divided angiographically into three different stages. The existence of secondary macular changes and peripheral involvement was noted. The mean visual acuity decreased from 0.71 to 0.62 over the follow-up period. No correlation could be found between the final visual outcome and initial extension of telangiectasis or existence of secondary changes. After laser coagulation the patients did not show a better visual outcome. Due to the relatively benign spontaneous course of idiopathic juxtafoveolar telangiectasis laser coagulation cannot be recommended in general. Only in exceptional cases (e.g., development of subretinal neovascularization) can laser coagulation be of benefit.
OBJECTIVE: To study the natural history and retinal findings associated with the intraretinal and subretinal fibrovascular tissues that develop in the late phases of bilateral juxtafoveal telangiectasis. METHODS: The records of 10 patients (11 eyes) with bilateral juxtafoveal telangiectasis who developed these fibrovascular tissues were examined. RESULTS: Throughout the follow-up period (average 44 months), only 2 eyes (18%) lost 2 or more lines of vision; the final visual acuities were similar for the eyes both with and without fibrovascular tissues. Sixty-four percent of fibrovascular tissues showed little to no growth. Eyes with fibrovascular tissue commonly had retinal pigment epithelial hyperplasia (72%), draining retinal venules (82%), and retinal vascular distortion (64%). CONCLUSIONS: Fibrovascular tissues of bilateral juxtafoveal telangiectasis have little proliferative potential and minimal effects on visual acuity. Nevertheless, these fibrovascular tissues do remodel over time, leading to retinal vascular distortion. Given these benign findings, the role of laser photocoagulation treatment of these tissues is questionable.
Bilateral parafoveal telangiectasis typically includes the presence of fluorescein staining of outer retinal capillaries, no macular ischemia, right-angled venules, and migration of pigment along these venules. In the bilateral form, atypical parafoveal telangiectasis with macular ischemia but without right-angled venules or migration of pigment along these venules has been reported in only five patients previously. An atypical case is presented of bilateral parafoveal telangiectasis complicated, not only by areas of macular nonperfusion, but also by peripheral ischemia with subsequent anterior and posterior segment neovascularization in the absence of any systemic abnormalities. As in previously reported cases, this patient had no evidence of right-angled venules or pigment migration. Pan-retinal photocoagulation resolved the rubeosis and caused regression of the retinal neovascularization.