1/41. 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.- - - - - - - - - - ranking = 1keywords = vein (Clic here for more details about this article) |
2/41. 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.- - - - - - - - - - ranking = 3.5keywords = vein (Clic here for more details about this article) |
3/41. Bilateral juxtafoveolar telangiectasis in monozygotic twins.PURPOSE: To report the clinical and angiographic features of two monozygotic twins affected by bilateral group 2 idiopathic juxtafoveolar telangiectasis. METHOD: case reports. RESULTS: Two 64-year-old women, who were identical twins, were suffering from visual loss. One twin had suffered from visual loss for 1 year and had a visual acuity of 20/25 in both eyes, and the other twin had suffered for 2 years and had a visual acuity of 20/30 in both eyes. fluorescein angiography disclosed similar fundus features. An analogous area of capillary telangiectasis and leakage was observed in the right macula, where no intraretinal pigment was seen; the left macula showed a similar amount of intraretinal pigment associated with tiny right-angle venules and minimal leakage. CONCLUSION: This observation raises the issue of genetic influences in the pathogenesis of this disease.- - - - - - - - - - ranking = 2527756.4726966keywords = telangiectasis (Clic here for more details about this article) |
4/41. 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 = 421292.74544943keywords = telangiectasis (Clic here for more details about this article) |
5/41. Hereditary haemorrhagic telangiectasia simulating biliary tree obstruction secondary to malignant porta hepatis lymphadenopathy.Hereditary telangiectasis is an uncommon condition and is thus not usually considered in the differential diagnosis of porta hepatis lymphadenopathy causing biliary tree obstruction. The present case illustrates a patient in whom tortuous vessels in the porta hepatis simulated masses on delayed contrast-enhanced CT with subsequent referral for biopsy.- - - - - - - - - - ranking = 421292.74544943keywords = telangiectasis (Clic here for more details about this article) |
6/41. 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 = 2keywords = vein (Clic here for more details about this article) |
7/41. Three-dimensional organization of the hepatic microvasculature in hereditary hemorrhagic telangiectasia.Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant systemic fibrovascular dysplasia. Although hepatic vascular shunts are often observed in HHT, the responsible pathological mechanism is unknown. This issue was addressed by performing a 3-dimensional reconstruction study of the hepatic microvasculature of an HHT-involved liver in a 79-year-old woman. Clinical observation revealed high-output congestive heart failure and hepatic encephalopathy due to arteriovenous and portovenous shunts, respectively. Angiography revealed tortuous dilation of hepatic arterial branches and intrahepatic arteriovenous shunts. The 3-dimensional analysis of the autopsy liver revealed focal sinusoidal ectasia, arteriovenous shunts through abnormal direct communications between arterioles and ectatic sinusoids, and portovenous shunts due to frequent and large communications between portal veins and ectatic sinusoids. Type 1 HHT was suggested by the lack of endoglin immunoreactivity in the liver. The 3-dimensional reconstruction study of hepatic microvasculature was successful in identifying the pathological changes responsible for the intrahepatic shunts in HHT.- - - - - - - - - - ranking = 0.5keywords = vein (Clic here for more details about this article) |
8/41. Hereditary hemorrhagic telangiectasia: demonstration of portosystemic venous shunts using power Doppler sonography and sonographically guided percutaneous transhepatic portovenography.Hereditary hemorrhagic telangiectasia is a rare autosomal dominant inherited disease associated with vascular abnormalities, which may occur in any organ. Cases of hereditary hemorrhagic telangiectasia accompanied by intrahepatic portosystemic venous shunts, however, have rarely been described. We report a case of hereditary hemorrhagic telangiectasia in which intrahepatic portosystemic shunts were detected using power Doppler sonography and portovenography with percutaneous transhepatic contrast agent injection. On gray-scale sonography, the common hepatic artery was dilated, and dilated tubular structures mimicking dilated biliary tracts were found. Power Doppler sonography demonstrated the continuity of tortuous vascular channels connecting a branch of the right portal vein to a branch of the right hepatic vein. The dilated vascular channels and tributaries of the right hepatic vein showed a monophasic waveform pattern on spectral analysis. Portovenography showed a tangle of vascular structures connecting with a branch of the right hepatic vein.- - - - - - - - - - ranking = 2keywords = vein (Clic here for more details about this article) |
9/41. Pulmonary arteriovenous fistula in the newborn: a case report of Rendu-Osler-Weber syndrome and a review of the literature.In most instances, congenital arteriovenous fistula is only one manifestation of a more widespread abnormality; 60% of patients also have hereditary hemorrhagic telangiectasis (Rendu-Osler-Weber syndrome). Among those with congenital pulmonary arteriovenous fistula, the diagnosis is made during infancy in only 15% of patients. We present a case of pulmonary arteriovenous fistula in a newborn and review the literature. This rare condition of newborns can be treated with different surgical procedures. Only 17 cases of newborn pulmonary arteriovenous fistula/have been reported, and only two of those had associated Rendu-Osler-Weber syndrome. The results of surgical procedures were good in most of these cases. We treated our case with lobectomy successfully.- - - - - - - - - - ranking = 421292.74544943keywords = telangiectasis (Clic here for more details about this article) |
10/41. Three-dimensional gadolinium-enhanced magnetic resonance angiography of pulmonary arteriovenous malformation.Pulmonary arteriovenous malformations (AVMs) are rare vascular malformations of the lung. Although most patients are asymptomatic, AVMs can bleed and result in haemoptysis and haemothorax. We describes a case of pulmonary AVMs in a patient with Osler-Weber-Rendu syndrome with 3-D contrast-enhanced MR angiography. magnetic resonance angiography provided accurately and non-invasively the number and size of the feeding arteries and draining veins.- - - - - - - - - - ranking = 0.5keywords = vein (Clic here for more details about this article) |
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