1/8. Hepatic involvement in hereditary hemorrhagic telangiectasia: an unusual indication for liver transplantation.Hereditary hemorrhagic telangiectasia (HHT) is a vascular disease with multiple organ manifestations. Severe hepatic involvement is an unusal indication for liver transplantation. We report on a 39-year-old woman diagnosed with HHT and decompensation of the disease during her second pregnancy. After delivery, hemobilia occurred, followed by severe therapy-resistant cholangitis and progressive liver dysfunction. Because of progressive loss of liver function, orthotopic liver transplantation needed to be performed. The various aspects of liver involvement in Osler's disease, diagnosis, and therapy are discussed.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/8. Early childhood presentation of neurovascular disease in hereditary haemorrhagic telangiectasia.This paper describes an unusual case of hereditary haemorrhagic telangiectasia related cerebrovascular disease with an arteriovenous malformation and aneurysm presenting with intracranial haemorrhage in early childhood.- - - - - - - - - - ranking = 5keywords = vascular disease (Clic here for more details about this article) |
3/8. 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 = vascular disease (Clic here for more details about this article) |
4/8. Multiple cerebral and pulmonary arteriovenous malformations in association with brain and subcutaneous abscesses: a possible variant of hereditary hemorrhagic telangiectasia--case report.The authors present a case in which multiple cerebral and pulmonary arteriovenous malformations (AVMs), a brain abscess, and a recurrent subcutaneous abscess were found concurrently in a 52-year-old male. He was admitted to our hospital for evaluation of a subcutaneous abscess in the right nuchal area and a right occipital AVM that had been detected at another hospital. Of his eight siblings, three had died of cerebrovascular disease (one in childhood and two as young adults), one had died of unknown causes in childhood, one had been hemiplegic since infancy, one had recently undergone removal of a pulmonary AVM, one was killed in world war ii at the age of 24 years, and the remaining sibling was healthy. He had had surgery for a right occipital brain abscess three years prior to this admission. A general examination revealed no abnormalities other than the painful right nuchal mass. Neurological evaluation disclosed left homonymous hemianopsia, which was probably a result of his previous surgery for the right occipital brain abscess. cerebral angiography showed AVMs in the right parietal (2 x 2 cm), right occipital (1.5 x 1.5 cm), and right cerebellar areas (1 x 1 cm). Digital subtraction angiography of the lung revealed multiple bilateral AVMs. The cerebral and pulmonary AVMs were removed in a two-stage operation. Although this case did not correspond precisely to the triad of hereditary hemorrhagic telangiectasia (HHT), the authors consider it to be related to HHT, since that syndrome is often complicated by multiple cerebral and pulmonary AVMs.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
5/8. central nervous system arteriovenous malformations in multiple generations of a family with hereditary hemorrhagic telangiectasia.Hereditary hemorrhagic telangiectasia is described in four generations of a kinship. The family is unique in that three generations manifest central nervous system vascular disease, which was pathologically confirmed in two generations. genetic linkage was not identified for 32 genetic markers studied. The literature is reviewed for patients with central nervous system arteriovenous malformations and hereditary hemorrhagic telangiectasia. The pathogenesis of the disease is discussed, and therapeutic modalities are considered.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
6/8. Focal cerebral ischemia distal to a cerebral aneurysm in hereditary hemorrhagic telangiectasia.Neurologic complications in patients with Hereditary Hemorrhagic Telangiectasia (HHT) occur as a consequence of a variety of vascular malformations. One type of intracranial malformation, saccular aneurysm, has recently been appreciated as a potential source of ischemic cerebrovascular disease on the basis of aneurysm to artery emboli. We report a patient with HHT and an unruptured saccular intracranial aneurysm presenting with ischemic vascular events.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
7/8. liver failure caused by hepatic angiodysplasia in hereditary hemorrhagic telangiectasia.Hereditary hemorrhagic telangiectasia is a systemic vascular disease with autosomal dominant inheritance that results in telangiectasia, arteriovenous malformations, and hemangiomas. The liver is one of the organs commonly affected in hereditary hemorrhagic telangiectasia, and hepatic lesions consist of angiodysplasia and fibrosis. A patient with hereditary hemorrhagic telangiectasia and significant impairment of synthetic liver function is reported. Dynamic computed tomography revealed marked enlargement of the common hepatic and intrahepatic arteries, heterogeneous parenchymography, and early opacification of the hepatic veins consistent with telangiectasias and arteriovenous shunting. overall, the liver was predominantly occupied by vascular structures and scarce residual hepatic parenchyma. Other causes of liver dysfunction, such as viral hepatitis and alcohol abuse, were excluded. In general, hepatic fibrovascular dysplasia seen in hereditary hemorrhagic telangiectasia usually results in only mild liver dysfunction; however, this case shows that hepatic involvement may rarely result in hepatic failure.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
8/8. Osler-Rendu-Weber with liver involvement.Osler-Rendu-Weber disease, or hereditary hemorrhagic telangiectasia, is a vascular disease with autosomal dominant transmission. The liver is involved in 31% of patients with hereditary hemorrhagic telangiectasia (1). It appears that the lesions uncommonly causes serious disease. We report the imaging findings and the therapy in a case with extensive telangiectasis in the liver.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |