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PubMedDate not supplied

[Microphlebography. A useful technique in the therapy of microvarices].

The difficulties encountered in compiling preoperative mapping of telangiectasis led the authors to consider possible investigations other than the usual clinical examinations. Of the methods available, only phlebographic examination was found to be of any practical use in telangiectasis. The procedure developed by the authors, known as microphlebography has demonstrated its effectiveness in final mapping and clinico-radiological transfer. Microphlebography is an inoffensive examination which is easy to perform and useful in treating telangiectasia using this personal technique of micro-coagulo-surgery.

Humans↗
PubMed1982

Endothelial cell renewal in skin of patients with progressive systemic sclerosis (PSS): an in vitro autoradiographic study.

The labelling index of endothelial cells of the dermal microvessels was studied by in vitro autoradiography with [3H] thymidine. 14 patients with PSS were compared with 7 control subjects, by sampling the upper-third of the forearm skin. One patient had a second biopsy in this area, on a telangiectasis. Microvessels from patients showed a significant increase in endothelial cell labelling. Within the PSS group, no difference was found between the indurated and the non-indurated skin. There was no correlation between the labelling index and the duration of the disease. A very high uptake was found in the telangiectasis. Such an increased labelling index in PSS is consistent with a response to the endothelial cell destruction as described in electron microscopy and in cultures with patient's serum.

Adult↗
PubMedDate not supplied

[Congenital or acquired unilateral nevoid stellar telangiectasias. About 5 cases (author's transl)].

Based on five personal cases (one neo-natal case, one in childhood, and three cases in pregnant women) and sixteen published cases, this study remind us of the essential caracteristics of unilateral nevoid telangiectasis. Prevalence in women is noticeable appearance during pregnancy, with complete regression during post-partum, is quasi constant. If their nosological place is not clear among the essential telangiectasias, they seem to be quite different from Hutchinson's serpiginious angioma (verrucous telangiectasis), from Osler-Rendu's disease, from Brocq's telangiectasias and from generalizated telangiectasias. The true signification of all these diseases is still completely unknown.

Adult↗
PubMed1982

The prevalence and economic significance of liver disorders and contamination in grain-fed and grass-fed cattle.

Livers were examined from 5,647 grain-fed and 621 grass-fed cattle of similar breed, age and weight, killed at a Queensland abattoir in 1980-81. The prevalences of disorders found in grain-fed cattle were liver abscesses 5.6% and 11.1% (after 80 and 120 days in the feedlot), telangiectasis 8.4%, Echinococcus granulosis 3.3%, Fasciola hepatica 0.4%, unclassified lesions 7.4%, and contamination 3.2%. In grass-fed cattle liver abscesses were 0.2%, telangiectasis 1.0%, E. granulosis 18.9%, F. hepatica 5.4%, unclassified lesions 6.5%, and contamination 1.0%. Liver abscess disease was the main cause of loss in grain-fed cattle and the mean carcase weight of affected steers was 298kg +/- 42kg, compared with 308kg +/- 40kg in non-affected (p less than 0.01). Liver weights of grain-fed and grass-fed cattle were highly significantly correlated with carcase weights (r = 0.78 and 0.49 respectively). Total losses were $2.25 per head in grain-fed steers and $0.90 in grass-fed steers.

Abattoirs↗
PubMed2003

Hereditary haemorrhagic telangiectasia: a questionnaire based study to delineate the different phenotypes caused by endoglin and ALK1 mutations.

BACKGROUND: Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant vascular dysplasia characterised by mucocutaneous telangiectasis, epistaxis, gastrointestinal haemorrhage, and arteriovenous malformations in the lung and brain. Causative mutations for HHT have been identified in two genes, endoglin and ALK1, which encode proteins involved in serine-threonine kinase signalling in the endothelial cell. METHODS: A number of people affected with HHT had completed a postal questionnaire as part of an international study to delineate the HHT phenotype. We identified questionnaires completed by subjects in whom we had identified a mutation in endoglin or ALK1. Further questionnaires were sent to families with known mutations. Data were only included from questionnaires returned by people known to carry disease causing mutations. RESULTS: Questionnaires were completed by 83 subjects with known mutations. Of these, 49 had endoglin mutations (HHT1) and 34 had ALK1 mutations (HHT2). Subjects with HHT1 reported an earlier onset of epistaxis (p=0.01) and telangiectasis (p=0.0001) than those with HHT2. Pulmonary arteriovenous malformations were only reported in the endoglin mutation group in our study (p<0.001). CONCLUSIONS: Our questionnaire based study provides evidence that the HHT phenotype caused by mutations in endoglin (HHT1) is distinct from, and more severe than, HHT caused by mutations in ALK1 (HHT2). This has significant implications for diagnosis, screening, and treatment in the two different forms of HHT, as well as for understanding the pathogenesis of the disease.

Activin Receptors, Type I↗
PubMed2003

Idiopathic and radiation-induced ocular telangiectasia: the involvement of the ATM gene.

PURPOSE: To investigate whether individuals, with no family history of ataxia telangiectasia (AT), in whom idiopathic or radiation-induced ocular telangiectasia developed are carriers of ATM gene mutations. METHODS: The ATM cDNA from lymphoblastoid cell lines established from 16 patients with idiopathic retinal or choroidal telangiectasia and 14 patients with radiation-induced telangiectasia after radiotherapy for age-related macular degeneration (AMD) was screened using the restriction endonuclease fingerprinting technique. The frequency of each detected variant was determined in the French population by either a mass spectrometry-based technique or variant-specific endonuclease digestion. RESULTS: Twenty-one ATM missense alterations, at 10 different sites, 8 of which would result in an amino acid substitution at a conserved position in the ATM protein were found. Four were novel changes, three of which were not detected in the 128 French control subjects screened. Eleven of 16 of the individuals with either idiopathic polypoidal choroidal vasculopathy or juxtafoveolar retinal telangiectasis and 6 of 14 individuals that had choroidal telangiectasis after radiotherapy for AMD carried ATM sequence variants. These latter six individuals had a significantly shorter delay time before the presentation of this vasculopathy compared with those individuals who had a wild-type ATM (11.8 +/- 3.4 months vs. 17.5 +/- 4.5 months, P = 0.024). They had also received a lower average dose of X-rays, although this difference did not reach statistical significance (18.7 +/- 3.9 Gy vs. 23.7 +/- 5.6 Gy, P = 0.09). CONCLUSIONS: ATM missense variants could confer an AT-like phenotype and influence the formation of retinal and choroidal vascular abnormalities.

Adult↗
PubMed1999

[Weber-Rendu-Osler disease: pulmonary arterio-venous malformation with shunt disclosed after 5 occurrences of purulent meningococcal encephalitis].

Hereditary hemorrhagic telangectasis or Weber-Rendu-Osler disease is associated with the presence of capillary malformations with pulmonary visceral shunts. These shunts are the cause of recurrent infections of the nervous system by loss of the anti-infectious lung filter. Hereditary hemorrhagic telangiectasis was diagnosed in a 68-year-old woman with a history of epistaxis, cutaneous telangectasis, purulent and pyogenic brain abscesses and meningitis. Outcome was favorable with antibiotic therapy. Ventilation as well as chest x-ray, brain scan and liver ultrasongraphy were normal. Blood gases showed a PO2 at 63 mmHg in ambient air and 62 mmHg with FiO2 = 1. There was no dyspnea or cyanosis nor any apparent polycythemia. Pulmonary angiography showed and arteriovenous malformation in the lower right lobe. Endovascular embolization was achieved with coils and N-butyl-cyano-acrylate glue which enabled angiographic occlusion and normalization of gas exchange: on ambient air the PaO2 = 71 mmHg and on FiO2 = 1, PaO2 was 359 mmHg. A true shunt was suspected on account of the association of hereditary hemorrhagic telangiectasis and recurrent meningitis. The diagnosis was suspected on blood gases alone.

Aged↗
PubMedDate not supplied

Astrocytoma with angiomatoid vascular proliferation ("angiomatous astrocytoma").

We report a surgical case of unusual anaplastic astrocytoma which was accompanied by an exuberant proliferation of abnormal blood vessels with features resembling those of capillary telangiectasis or cavernous angioma. The patient was a 39-year-old man, who presented with a generalized convulsive seizure, and neuroradiological examination revealed a tumor in the left frontal lobe. The resected tumor showed the features of an anaplastic astrocytoma, grade 3. The proliferation of abnormal blood vessels with dilated lumina and thin walls was seen throughout the tumor, and in the central area these vessels were densely packed and almost replaced the neoplastic astrocytic tissue. Although these dense vascular aggregates in the central area closely simulated capillary telangiectasis or cavernous angioma, they were considered to be of a reactive nature. The term "angiomatous astrocytoma", which is analogous to angiomatous meningioma, seemed to be the most appropriate for the present tumor.

Adult↗
PubMed2001

[Coats disease].

In spite of an unclear definition of Coats disease, this angiomatosis can be defined by the presence of retinal telangiectasis of nondetectable etiology, complicated by severe exudative phenomena. The diagnosis of this serious affection remains difficult because of its numerous clinical forms, which vary according to the age at which the disease appears and the progressive nature of the exudative phenomena. The consequences on the young child are all the more dangerous because it is too often discovered late. It seems necessary to attempt to stop the progression of Coats disease by destroying the telangiectasis responsible for intra- and subretinal exudation. No coagulation technique has been shown to be more effective than others and the predominance of heterogeneous series in the literature makes it difficult to evaluate the therapeutic results. In cases of severe retinal detachment, these coagulations can only be done after the often difficult drainage of the subretinal fluids.

Adolescent↗
PubMed2002

Hereditary hemorrhagic telangiectasia.

Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease, is a rare disorder characterized by arteriovenous communications in visceral organs. The diagnosis of HHT consists of recurrent epistaxis, mucocutaneous telangiectasis, visceral vascular lesion and familial occurrence. HHT can be definitely diagnosed with the presence of all these three criteria. The prevalence of liver involvement of HHT was reported to range from 8 to 31%. Herein, we present a 75-year-old male who was diagnosed as having HHT with liver involvement, based on the findings of recurrent epistaxis, mucosal telangiectasis on the lower lip and hepatic arteriovenous malformation. The clinical presentations of this patient are discussed, and the literature is reviewed.

Aged↗
PubMedDate not supplied

Left-sided leg edema of the elderly: a common variant of the iliac compression syndrome.

BACKGROUND: Anatomically, the right common iliac artery crosses the left iliac vein and its accompanying lymphatics. We hypothesized that this situation could lead to a predominance of edema, telangiectasis, and venous varicosities on the left lower extremities of older persons. METHODS: To test this hypothesis, a research assistant who was blinded to the study goals examined 215 predominantly elderly residents of North Carolina homes for the aged and disabled. RESULTS: Among these subjects, 17.7 percent had predominantly left-sided edema, and 5.7 percent had predominantly right-sided edema (P < 0.001). When the 88 subjects with pitting edema greater than 3 mm were studied, 34.5 percent showed a left-sided predominance, and 6.9 percent showed a right-sided predominance (P < 0.001). In contrast, no significant difference was found in the lateralization of venous varicosities or of telangiectasis. CONCLUSIONS: Asymmetric edema is common and is usually left-sided in older persons. Compression of the left common iliac vein and its accompanying lymphatics by the right iliac artery, rather than overt clinical disease, might explain the majority of asymmetric edema seen in clinical practice.

Adult↗
PubMed1995

[Acoustic neurinomas with large cystic components: a clinical and pathological study of 3 cases].

Acoustic neurinomas containing cystic parts are fairly common, but it is extremely rare for acoustic neurinomas to be mainly composed of cysts. So far, we have encountered three such cases. We studied these cases retrospectively to correlate their characteristic clinical symptoms and their respective pathological findings. Referring to reviews of the relevant literature and our three cases, we would like to discuss the possible mechanism of cyst formation and clinical symptoms. Compared with the patients with ordinary acoustic neurinomas, the patients in our three cases and in some cases in the literature have some distinct and possibly common clinical features. First of all, in spite of the cysts being large, hearing loss was less severe. Secondly, sudden onset of headache and hearing loss were frequently experienced, probably due to intratumoral hemorrhages. Thirdly, intratumoral hemorrhages with niveau formations were frequently able to be identified on CT or MRI scannings. Histologically, our three cases revealed lots of abnormal sinusoid or telangiectasis vessels. Small hemorrhages, hemosiderin deposits and hemosiderin-containing phagocytes were also more abundant than in solid-type neurinomas. The exact mechanism of the formation of these cysts is still controversial. Our hypotheses concerning the probable causes of cyst formation are presented in this study. In our three cases. the most distinct finding was the presence of abundant abnormal sinusoid or telangiectasis-like vessels. We suggest therefore that, in these cases, repeated small hemorrhages from these abnormal vessels, along with subsequent intratumoral changes, are the most probable cause of the formation of these cysts.

Adult↗
PubMed1980

The clinical and pathological spectrum of brain-stem vascular malformations. Long-term course stimulates multiple sclerosis.

Three cases of brain-stem vascular malformation with progressive and fluctuating clinical courses of longer than one year were studied. One patient with a rare brain-stem arteriovenous malformation lived for 20 years with symptoms notably similar to those of multiple sclerosis (MS). One case each of cavernous hemangioma and capillary telangiectasis are also described. The clinical, roentgenographic, and pathologic features of these three cases plus 18 other cases of brain-stem vascular malformations from the literature, with progressive or intermittent clinical courses and with survival of longer than one year from the onset of symptoms, are reviewed. Since nearly all of these cases were once mistakenly diagnosed as MS, a diagnostic approach is proposed to help distinguish brain-stem vascular malformations from MS prior to an obvious subarachnoid hemorrhage.

Adult↗
PubMed1986

Hemangiomas of the central nervous system in Japan: an epidemiological and clinicopathological study with special reference to venous and cavernous malformations.

Our experiences with cerebrovascular malformations in Japanese patients have been reviewed, presenting 29 cases of venous malformation, 30 cases of cavernous angiomas and one case of capillary telangiectasis. We also present the data from the 763 cases of AVM obtained in the cooperative studies which were supported by the Ministry of Health and Welfare of Japan, and discussed the epidemiological and clinicopathological aspects in relation to those reported in the literature. The cooperative studies on these subjects were primarily done in conjunction with the investigation of Moya-Moya disease which is by far frequently found in Japan. Regarding the vascular malformations of the C.N.S., there seem to be no noticeable differences in the epidemiological aspects as seen in Japan and in other countries. It should be stressed also from our experiences that all kinds of vascular malformation, either intracranial or intraspinal, bleed probably more often than we expect. Neurosurgeons should pay more careful attention to these vascular malformations with appropriate concepts of the disease.

Adolescent↗
PubMedDate not supplied

Multiple intrapulmonary arteriovenous fistulas in childhood.

Pulmonary arteriovenous fistulas (AVFs) are a rare but recognized cause of cyanosis in childhood. Lesions may be acquired as in hepatopulmonary syndrome or they may be congenital, particularly in association with certain multisystem disorders. Large fistulas are more common than multiple small connections. Two cases. both boys, presenting in the first decade of life are described. "Bubble" echocardiography was the most telling investigation and strongly suggested the presence of AVFs in both cases. Each patient then underwent cardiac catheterization, which demonstrated normal pulmonary artery pressure and diffuse pulmonary telangiectasis. Both patients were treated effectively with nifedipine and continue with this mode of therapy.

Abnormalities, Multiple↗
PubMed1975

Leber's miliary aneurysms associated with telangiectasia of the nasal mucosa.

A 20-year-old woman, treated with cryopexy and xenon- and argon-laser photocoagulation for Leber's miliary aneurysms in her right eye, suffered from repeated epistaxis. She underwent an endoscopy that showed an unusual number of telangiectatic veins. Laboratory results and routine physical and neurologic examinations showed normal values. Dematologic examination revealed no signs of hereditary hemorrhagic telangiectasis (Osler's disease). Nevertheless, it seems reasonable to assume that the combination of microvascular disturbances in one eye and in the nasal mucous membrane is not a coincidental finding.

Adult↗
PubMed1979

Radiation retinopathy after irradiation of intracranial lesions.

Six patients developed a mild retinopathy after radiation therapy for intracranial lesions. The retinopathy, consisting of capillary microaneurysms, cotton-wool patches, and telangiectasis, developed after a latent period that varied between nine months and three years. The visual acuity remained within normal limits. The severity of the retinopathy is related to the retinal or optic nerve dose of radiation.

Adult↗
PubMed2005

Coats' disease diagnosed in adulthood.

PURPOSE: To investigate the manifestations and clinical course of Coats' disease diagnosed in adulthood and to describe the characteristics of patients who develop it. DESIGN: Retrospective observational case series. PARTICIPANTS: Thirteen eyes of 13 patients with Coats' disease diagnosed after age 35 years. METHODS: All patients examined in the authors' referral practices for Coats' disease diagnosed after the age of 35 years were identified. Patients with a history of radiation exposure, intraocular inflammation, retinal vascular occlusion, or Coats' response were excluded. A review of their historical, clinical, and fluorescein angiographic features and demographics was performed. RESULTS: There were 13 patients with a mean age at diagnosis of 50 years (range, 36-79) and a mean follow-up of 5.8 years (range, 0-17). These patients manifested many findings typical of Coats' disease, including the unilateral nature of the disease (13/13 patients), male predominance (12/13), vascular telangiectasis, lipid exudation, macular edema, and areas of capillary nonperfusion with adjacent webs of filigreelike capillaries. Disease was limited to < or =6 clock hours in 10 of 13 patients (76.9%), did not generally involve the retinal vasculature anterior to the equator, and showed limited potential to expand during the follow-up period. A localized hemorrhage was noted in 10 of 13 patients (76.9%), almost always near larger aneurysmal dilatations. Limited exudative detachment of the retina was seen in 4 eyes, 3 of which responded to localized laser photocoagulation. One patient had subretinal fluid seen on ocular coherence tomography that did not require treatment. On average, patients lost 2.1 lines of visual acuity during the follow-up period; 6 patients had a decline in vision, 2 patients had improved vision, and 3 patients had stable vision. No patient had end-stage findings of Coats' disease, such as iris neovascularization or total exudative detachment. CONCLUSIONS: Coats' disease can first be diagnosed in adulthood with retinal vascular abnormalities similar to those seen in younger patients. There are a number of important differences in disease manifestation in adults, including limited area of involvement, slower apparent progression of disease, and hemorrhage near larger vascular dilatations.

Adult↗