1/117. 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.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
2/117. 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.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
3/117. 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.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
4/117. 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.- - - - - - - - - - ranking = 0.2keywords = malformation (Clic here for more details about this article) |
5/117. Recurrent gastrointestinal bleeding and high output cardiac failure caused by hereditary hemorrhagic telangiectasia.Hereditary hemorrhagic telangiectasia, commonly known as Osler-Weber-Rendu disease, is a systemic autosomal dominant inherited disorder, that occurs in Caucasian populations. We report the case of a 56-year-old housewife who was admitted to the gastrointestinal and cardiovascular ward because she had suffered from recurrent gastrointestinal bleeding and heart failure from 1994 to 1997. Panendoscopy showed vascular ectasia scattered over the tongue, larynx, esophagus, and posterior wall of the gastric body. colonoscopy showed clusters of telangiectasia over the cecum and ascending colon. arteriovenous malformations (AVMs) were found in the liver and lungs on computerized tomography. Recurrent gastrointestinal bleeding was controlled by estrogen treatment during the follow-up period. In July 1997, the patient was readmitted to our cardiovascular section due to aggravated dyspnea, orthopnea and bilateral lower leg edema. cardiac catheterization showed a large fistula from the left pulmonary artery to the left atrium and left ventricle, pulmonary arterial pressure of 37/13 mmHg and cardiac output of 9.61/minute. Other studies excluded the possibility of sepsis, and high-output cardiac failure was suspected. The patient was discharged in a stable condition and scheduled for AVM embolization management. Unfortunately, she died of a suspected heart attack at home two weeks following discharge.- - - - - - - - - - ranking = 0.2keywords = malformation (Clic here for more details about this article) |
6/117. arteriovenous malformations of the gastrointestinal tract.During an eight-year period, 17 patients ranging in age from 7 months to 81 years were found to have arteriovenous malformations (AVMs) of the gastrointestinal tract complicated by major hemorrhage. After review of these cases, a clinical classification of AVMs was developed, based on angiographic characteristics, localization, age of the patient, and family history. Type 1 AVMs were solitary, localized lesions within the right side of the colon. They occurred in seven patients 55 years of age or older. None were palpable or visible at operation. Type 2 AVMs occurred in seven patients. They were larger and occasionally visible, most commonly in the small intestine, and probably of congenital origin. Symptoms all began before 50 years of age. Type 3 AVMs were punctate angiomas causing gastrointestinal hemorrhage. They occurred in three patients with the classical findings of hereditary hemorrhagic telangiectasia. The diagnosis of most AVMs can be determined preoperatively only by selective angiographic studies.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
7/117. liver transplantation for hepatic arteriovenous malformation with high-output cardiac failure in hereditary hemorrhagic telangiectasia: hemodynamic study.We describe a case of orthotopic liver transplantation used as a therapeutic method to correct high output cardiac failure related to a liver arteriovenous fistula due to hereditary hemorrhagic telangiectasia. Detailed hemodynamic changes as they occurred during liver transplantation are described.- - - - - - - - - - ranking = 0.8keywords = malformation (Clic here for more details about this article) |
8/117. 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 = 0.2keywords = malformation (Clic here for more details about this article) |
9/117. Hereditary hemorrhagic telangiectasia diagnosed by the ophthalmologist.PURPOSE: To show the clinical features of a case of hereditary hemorrhagic telangiectasia in which the diagnosis was prompted by ophthalmologic examination. methods: A retrospective case review of a 56-year-old East Indian woman whose presentation to the eye clinic with a history of bloody tears and conjunctival vascular malformations prompted a systemic evaluation that resulted in the diagnosis of hereditary hemorrhagic telangiectasia. RESULTS: The diagnosis of hereditary hemorrhagic telangiectasia was eventually confirmed by gastrointestinal endoscopy and otorhinolaryngologic examination. CONCLUSION: Although hereditary hemorrhagic telangiectasia is typically diagnosed on the basis of gastrointestinal and otorhinolaryngologic history and examination, the ophthalmologic features of this case were striking enough to arouse suspicion of this disease.- - - - - - - - - - ranking = 0.2keywords = malformation (Clic here for more details about this article) |
10/117. Splenic vascular malformations and portal hypertension in hereditary hemorrhagic telangiectasia: sonographic findings.Hereditary hemorrhagic telangiectasia, or Osler-Rendu-Weber disease, is an autosomal dominant disorder in which a variety of vascular dysplasias occur throughout the organ systems. We report the gray-scale and color Doppler sonographic findings in a case of hereditary hemorrhagic telangiectasia. Gray-scale sonographic examination revealed massive splenomegaly, multiple dilated intrasplenic vascular structures (some with adjacent punctate calcifications), an aneurysmal dilatation of the splenic vein, dilated intrahepatic portal branches, and marked atrophy of the right hepatic lobe. color Doppler sonography showed dilatation of the truncus coeliacus and high-velocity flow in the splenic artery. There were significant aliasing in the splenic hilum and an abnormal, arterialized flow in intrasplenic branches of the splenic vein. The splenic vein was massively enlarged with increased flow velocity and contained an isolated aneurysmal dilatation in the hilum. There were multiple serpiginous retroperitoneal collateral vessels, and the left gastric vein was dilated with hepatofugal flow. The patient had portal hypertension that developed secondary to the increased portal flow.- - - - - - - - - - ranking = 0.8keywords = malformation (Clic here for more details about this article) |
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