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1/276. The liver in hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease).

    The livers of four patients with hereditary hemorrhagic telangiectasia--including the original case of Osler--were examined at autopsy. Characteristic random focal fibrovascular lesions were found in all. The importance of recognizing the apparently common and seemingly benign hepatic involvement in this disease is emphasized in view of its possible confusion with more serious types of liver disease that may complicate the condition. ( info)

2/276. spinal cord arteriovenous malformations in two patients with hereditary hemorrhagic telangiectasia.

    We report two cases, in first cousins, of spinal arteriovenous malformations (AVMs) of the perimedullary fistula type and hereditary hemorrhagic telangiectasia (HHT). Spinal AVMs are a rare clinical presentation of HHT, but can be the first manifestation in a child with this disorder. The importance of considering a coexisting disorder of vascular dysplasia, such as HHT, when a child presents with a spinal AVM is discussed. ( info)

3/276. Transcatheter embolization of hepatic arteriovenous fistulas in Rendu-Osler-Weber disease: a case report and review of the literature.

    A patient with hereditary hemorrhagic telangiectasia and diffuse intrahepatic arteriovenous fistulas developed secondary high-output ventricular failure and pulmonary hypertension. A serial staged hepatic arterial coil embolization was performed with long-term resultant haemodynamic and clinical improvement. The methods of this procedure and related complications are discussed. ( info)

4/276. The spectrum of arteriographic findings in Osler-Weber-Rendu disease.

    In Osler-Weber-Rendu syndrome, visceral organs are frequently involved in addition to cutaneous and mucous membranous lesions. Detailed arteriographic investigation of 5 patients with this abnormality disclosed characteristic angiodysplastic lesions involving the lungs, liver, spleen, pancreas, gastrointestinal tract and mesentery. Arterial and aortic aneurysms appear to be an integral part of this abnormality. Common to angiodysplastic lesions is a variable degree of arteriovenous shunting. The arteriographic characteristics and the hemodynamic significance of major visceral arteriovenous shunts in hereditary hemorrhagic telangiectasia are discussed. ( info)

5/276. Sequential Doppler sonographic studies of embolization in a patient with hepatic involvement in hereditary hemorrhagic telangiectasia: correlation with angiographic findings.

    A 41-year-old man was admitted for symptoms of progressive congestive heart failure. His family history and the results of a physical examination were highly suggestive of Osler-Weber-Rendu disease (hereditary hemorrhagic telangiectasia, HHT). cardiac catheterization and hepatic angiography demonstrated HHT with left-to-right shunting from the liver. The patient underwent transcatheter arterial embolization (TAE) of the right hepatic artery. We performed both Doppler sonography and angiography before and after TAE. The treatment improved the clinical manifestations of congestive heart failure, including the edema of the leg and dyspnea. Doppler sonographic studies also showed an increased resistive index in the right hepatic artery and decreased flow volumes and velocities in the right and middle hepatic veins, respectively, after treatment. Corresponding changes on angiography after TAE showed decreased right hepatic arterial flow and nonopacified branches distal to the coils, disappearance of the mottled hepatogram in the right lobe, reduction of contrast agent staining, and enhanced calibers in the right and middle hepatic veins. This case illustrates that qualitative and quantitative studies with duplex and color Doppler ultrasound can be used to detect or define the extent of hepatic involvement in HHT patients before TAE, monitor hemodynamic changes of the intrahepatic vasculature after TAE, evaluate the efficacy of treatment, and possibly obviate the need for repeated angiography for diagnosis only. ( info)

6/276. brain abscesses associated with pulmonary arteriovenous malformations.

    We present three patients with brain abscesses who also had pulmonary arteriovenous malformations (AVMs). All patients had hereditary haemorrhagic telangiectasia (Osler's disease). It is probable that the lung AVMs contributed to the development of the brain abscesses by allowing bacteria easier access to the systemic circulation through a right to left pulmonary vascular shunt, bypassing the filtering action of the pulmonary capillaries. In addition, one patient required a period of postoperative ventilation using PEEP, which may have exacerbated the shunt through the lung AVM and led to difficulty in weaning her from the ventilator. ( info)

7/276. Lupoid hepatitis, Rendu-Osler-Weber syndrome, clubbing cyanosis and hypertrophic osteoarthropathy.

    Chronic liver disease typical of chronic active 'lupoid' hepatitis together with cyanosis, clubbing and hypertrophic osteoarthropathy in a 42-year-old female is described. In addition she had severe nose bleeds, gastro-intestinal haemorrhages, syncopal attacks with generalised convulsive seizures, pulmonary arterio-venous fistulae as manifestations of Rendu-Osler-Weber syndrome. A study of the literature revealed that similar associations are far more frequent than can be attribtued to chance. Possible mechanisms of the cyanosis, clubbing and osteoarthropathy and possible common pathogenesis for these seemingly unrelated disorders are discussed. ( info)

8/276. portal vein aneurysm in hereditary hemorrhagic telangiectasia.

    Both hereditary hemorrhagic telangiectasia (HHT) and portal vein aneurysm are rare disorders. We described the first documented case of HHT associated with portal vein aneurysm. As the portal vein aneurysm in this patient associated with HHT, the pathogenesis in this patient seems to have been a congenital anomaly of the vasculature. ( info)

9/276. Peripheral arterial coil embolization for hepatic arteriovenous malformation in Osler-Weber-Rendu disease; useful for controlling high output heart failure, but harmful to the liver.

    A 55-year-old Japanese housewife, who had Osler-Weber-Rendu disease, was admitted to our hospital because of frequent epistaxis and worsening exertional dyspnea. The computed tomography and hepatic arteriography revealed large hepatic arteriovenous malformation, which was considered to be the leading cause of her high output heart failure. Two series of hepatic arterial coil embolization procedures were performed to reduce hepatic shunt flow. They temporarily improved her cardiac condition, but gradually induced progressive hepatic failure due to intrahepatic cholangitis. Hepatic dysfunction restricted her quality of life and lead to a fatal clinical course one year after the second coil embolization. ( info)

10/276. The liver in hereditary hemorrhagic telangiectasia (Weber-Rendu-Osler disease).

    BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT, Osler disease) is an inborn error in the structure of different vessels. This leads to vascular malformations in multiple organ systems. In the liver vascular abnormalities are associated with a marked fibrosis and/or cirrhosis. methods AND RESULTS: We found hepatic manifestation of Osler disease in four women and one man (51-63 years old) presenting initially with slight disturbances of liver function. In three patients progressive liver insufficiency developed. The characteristic histologic and sonographic findings are described and discussed. CONCLUSION: ultrasonography with color and Doppler analysis is diagnostic, replacing more extensive procedures like angiography, computer tomography, or magnetic resonance tomography. ( info)
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