1/4. 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 = physical examination (Clic here for more details about this article) |
2/4. central nervous system lesions associated with hereditary hemorrhagic telangiectasia--three case reports.Hereditary hemorrhagic telangiectasia (HHT) is easily overlooked in patients with central nervous system (CNS) lesions. Our clinical experience of three patients with CNS lesions associated with HHT stresses the importance of considering HHT. A 23-year-old male presented with consciousness disturbance and right hemiparesis. Emergency cerebral digital subtraction angiography revealed occlusion of the left middle cerebral artery and the left anterior cerebral artery. Pulmonary arteriography showed three pulmonary arteriovenous malformations (AVMs). A 62-year-old male presented with consciousness disturbance and sensory aphasia. magnetic resonance imaging revealed a ring-enhanced lesion in the left temporal lobe which was removed by left frontotemporal craniotomy. The diagnosis was brain abscess. Chest computed tomography (CT) revealed two pulmonary AVMs. A 32-year-old female presented with progressive mild weakness in her left hand. Initial CT showed subcortical hemorrhage in the right frontal lobe. cerebral angiography revealed no vascular malformations, but chest CT disclosed five pulmonary AVMs. All three patients had a family history of HHT. The possibility of HHT is important to consider in patients with cerebrovascular disease (CVD) or brain abscess to prevent complications, not only in the patients but also their blood relatives. Therefore, the medical and family history of patients with CVD or brain abscess should be investigated and HHT should be considered during the physical examination.- - - - - - - - - - ranking = 1keywords = physical examination (Clic here for more details about this article) |
3/4. Osler-Weber-Rendu syndrome as a cause of poor growth during adolescence.We report an adolescent boy with poor growth and delayed skeletal age who was found to have pulmonary arteriovenous malformations (PAVMs) consistent with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome). While a host of chronic diseases, including a number of cardiopulmonary diseases, have previously been associated with poor growth or short stature, to our knowledge this is the first documented case of PAVM presenting with poor growth, manifesting as weight loss and delayed adolescence. This case highlights the importance of a thorough physical examination for identifying rare, occult, chronic diseases in patients who present with poor growth.- - - - - - - - - - ranking = 1keywords = physical examination (Clic here for more details about this article) |
4/4. A case of adenocarcinoma of the colon in a patient with hereditary hemorrhagic telangiectasis.A 70-year-old man with a history of hereditary hemorrhagic telangiectasis was found to have occult blood in his stool after a routine physical examination. Endoscopic evaluation revealed a small, sessile tumor that was consistent with well-differentiated adenocarcinoma of the sigmoid colon on histologic examination.- - - - - - - - - - ranking = 1keywords = physical examination (Clic here for more details about this article) |