1/23. 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 = 1keywords = vessel (Clic here for more details about this article) |
2/23. 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 = 1keywords = vessel (Clic here for more details about this article) |
3/23. 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 = 1keywords = vessel (Clic here for more details about this article) |
4/23. External beam radiotherapy for severe epistaxis from Osler-Weber-Rendu disease.Hereditary Hemorrhagic Telangiectasia (HHT) is a genetic disorder of blood vessels, which affects about 1 in 10,000 people. The disorder is also sometimes referred to as Osler-Weber-Rendu disease after the doctors who studied HHT. The disorder affects males and females from all racial and ethnic groups, and the most commonly affected organs are the nose, lungs, gastrointestinal tract, and brain--in that order. radiation therapy should be considered for intractable bleeding from the upper airway or alimentary tract.- - - - - - - - - - ranking = 9.2034322431695keywords = blood vessel, vessel (Clic here for more details about this article) |
5/23. Huge pulmonary arteriovenous fistula: diagnosis and treatment and an unusual complication of embolization.We report the case of a 42-year-old woman with Rendu-Osler-Weber syndrome (hereditary hemorrhagic telangiectasia) and a huge pulmonary arteriovenous fistula that involved the entire right middle lobe. She had a history of dyspnea and intermittent atrial flutter for 6 months. A chest radiograph showed a discrete shadowing of the middle part of the right lung. arteriovenous fistula was suspected based on the echocardiographic finding of immediate bubble detection in the left atrium on introducing echocardiographic contrast medium into the venous system. Atrial shunt was excluded. angiography revealed a huge pulmonary arteriovenous fistula in the entire middle lobe of the right lung. A trial of interventional embolization was performed, but the size of the fistula made it impossible to achieve complete closure of all segmental fistulas. Furthermore, the patient complained of unusual, severe chest pain after implantation of the first coil, so that the coil had to be removed. Therefore the patient underwent surgical resection of the entire right middle lobe and the upper part of the right lower lobe. anatomy was clearly delineated and all connecting vessels were suture-closed, but both lungs showed diffuse microscopic superficial pulmonary arteriovenous fistulas, which were too small to be detected by angiography and which were also partly closed. The postoperative course was uneventful: the patient recovered completely, she no longer had shortness of breath, and blood gas analysis showed normal P(aO)(2). It is not clear whether the patient's improved physical performance will last, because the development of diffuse microscopic arteriovenous fistulas bilaterally in the lungs is not predictable. Therefore close follow-up is necessary and in the case of recurrence (ie, enlargement of the existing small fistulas), early interventional embolization should be performed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/23. A novel treatment for patients with hereditary haemorrhagic telangiectasia.Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by dermal, mucosal, and visceral telangiectases as well as pulmonary and cerebral arteriovenous malformations. Recurrent epistaxis occurs in the majority of patients, and by the very nature of the thin walled vessels involved it is often refractory to conventional forms of treatment. We present the case of an 82-year-old lady with intractable epistaxis secondary to HHT, that was successfully controlled by the application of fibrin glue.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/23. Hereditary hemorrhagic telangiectasis treated by the harmonic scalpel.BACKGROUND: Hereditary hemorrhagic telangiectasis (HTT) is a familial autosomal dominant genetic disorder that causes abnormalities of the wall of peripheral blood vessels. Severe nosebleed often is the dominant symptom. A variety of therapies have been proposed for epistaxis control in HHT but with limited success. methods: We report two cases of HHT in which recurrent nasal bleeding was successfully controlled using the Harmonic Scalpel. RESULTS: Use of the Harmonic Scalpel avoids the carbonization and incrustation of the nasal mucosa that commonly results from electrocautery and laser irradiation. Even during active bleeding, hemostasis can be achieved by repeated applications of the scalpel blade. CONCLUSION: This method can be performed quick and reliably on an outpatient basis. The Harmonic Scalpel may be the treatment of choice for recurrent epistaxis in HHT.- - - - - - - - - - ranking = 9.2034322431695keywords = blood vessel, vessel (Clic here for more details about this article) |
8/23. Infra red coagulation for bleeding mucosal telangiectasia.The technique of infra red coagulation is well suited to the destruction of superficial blood vessels in the skin and/or mucosal surfaces. A method is described here for the destruction of resistant bleeding telangiectasia of the palate and lip in Osler-Weber-Rendu syndrome.- - - - - - - - - - ranking = 9.2034322431695keywords = blood vessel, vessel (Clic here for more details about this article) |
9/23. Interventional embolization of a giant pulmonary arteriovenous malformation with right-left-shunt associated with hereditary hemorrhagic telangiectasia.A 46-year old woman experienced an episode of arterial desaturation despite administration of 100% oxygen during anesthetization for an elective laparoscopy. Further evaluation revealed a giant pulmonary arteriovenous malformation (PAVM) with right-left shunt associated with previously undiagnosed hereditary hemorrhagic telangiectasia (HHT, Morbus Osler- Weber-Rendu). The PAVM was treated interventionally with an Amplatzer duct occluder. Transcatheter embolization of the PAVM was well tolerated with symptomatic and hemodynamic improvement. CT scan after six months demonstrated correct position of the duct occluder in the left pulmonary artery with nearly complete occlusion of the feeding vessel.PAVMs are rare direct communications between pulmonary arteries and veins, associated with HHT in the majority of cases and often presenting with dyspnea or major neurological complications due to paradoxic embolism. In this case report, we present a rational and stepwise diagnostic workup for this rare medical condition and show that transcatheter embolization is an appropriate treatment for larger malformations.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/23. Microvascular free flap in hereditary hemorrhagic telangiectasia.A 69-year-old male patient with hereditary hemorrhagic telangiectasia presented with severe epistaxis requiring repeated transfusions. Both nasal passages were densely populated with these vascular malformations that involved the entire nasal mucosa. A total rhinotomy was performed and all the nasal mucosa and turbinates were excised. Both nasal passages were completely resurfaced with a free radial forearm skin flap. The vascular pedicle was delivered from the nasal passage via the maxillary antrum to anastomose with the facial vessels in the cheek. Following surgery the patient had no further significant epistaxis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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