1/5. brain abscess and hereditary haemorrhagic telangiectasia: a report of three cases.Hereditary haemorrhagic telangiectasis (HHT) has long been viewed as a rare disease which is not usually associated with high morbidity and mortality. We report three patients with HHT who developed cerebral abscesses secondary to pulmonary arteriovenous malformations (PAVM).- - - - - - - - - - ranking = 1keywords = telangiectasis (Clic here for more details about this article) |
2/5. 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.- - - - - - - - - - ranking = 2keywords = telangiectasis (Clic here for more details about this article) |
3/5. Intrahepatic Osler's disease: report of two cases and review of the literature.Osler-Weber-Rendu disease is a hereditary vascular disease with multiple manifestations. The liver is involved rarely. Vascular abnormalities include telangiectasis and arteriovenous fistulas, sometimes associated with fibrosis and cirrhosis. Hepatic arteriovenous shunting may include secondary portal hypertension, reduced liver function and high cardiac output. Two cases of Osler-Weber-Rendu disease with extensive hepatic arteriovenous fistulation were described in detail and we report on their clinical features. In the first patient, treatment was symptomatic since liver transplantation is not indicated because the patient was asymptomatic. Embolization treatment of hepatic arteries was indicated in the second patient because he had biliary disease and recurrent cholangitis secondary to vasculo-biliary shunts. Therapy with arterial embolization, banding, or ligature of hepatic arteries is still limited and provides unsatisfactory long-term results. liver transplantation offers now another therapeutic option for patients with intrahepatic high shunting and secondary pulmonary hypertension.- - - - - - - - - - ranking = 1keywords = telangiectasis (Clic here for more details about this article) |
4/5. Control of severe vesical haemorrhage by therapeutic embolisation.Therapeutic embolisation is now recognised as having a role in the management of bladder haemorrhage and is particularly valuable in severe cases where the patient's life is at risk. This paper describes the procedures which were used to manage two patients with massive haematuria, from a tumour in one case and from an arterial malformation in the other. Previous reports indicate that haemorrhage from a bladder tumour can usually be controlled by embolisation which is limited to the tumour itself but haemorrhage due to post-radiation telangiectasis requires more extensive arterial occlusion and this was taken into account in planning the embolisation procedure in the first case. Neither patient developed gluteal pain after embolisation which was probably due to the fact that the superior gluteal arteries were not completely occluded. The left axillary approach was used as a catheter passing down the aorta can usually be manipulated into the internal iliac arteries and their smaller branches on both sides without difficulty.- - - - - - - - - - ranking = 1keywords = telangiectasis (Clic here for more details about this article) |
5/5. Haemodynamics and gas exchange before and after coil embolization of pulmonary arteriovenous malformations.A complete description of haemodynamics and gas exchange before and after percutaneous coil embolization of multiple pulmonary arteriovenous malformations is reported in a 45 year old woman with hereditary haemorrhagic telangiectasis (HHT). Before treatment, whilst the patient complained of severe dyspnoea during daily activities, an intrapulmonary shunt of 31% was measured (inert gas elimination technique), together with a cardiac output (thermodilution technique) of 12.4 L.min-1, resulting in a resting arterial oxygen tension (PaO2) of 8.53 kPa. Effective occlusion of all visible pulmonary malformations resulted in a rapid and major improvement in exercise tolerance, whilst resting PaO2 remained almost unchanged. A second investigation performed 4 months after treatment revealed a persistent intrapulmonary shunt of 19%, a cardiac output of 7.35 L.min-1, and a resting PaO2 of 10.53 kPa. We conclude that major increases in cardiac output largely contribute to the maintenance of PaO2 in patients with multiple pulmonary arteriovenous malformations and intrapulmonary shunt. The benefit of coil embolization is due both to an improvement in arterial oxygenation and a normalization of cardiac output.- - - - - - - - - - ranking = 1keywords = telangiectasis (Clic here for more details about this article) |