Background And Purpose: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease that causes vascular malformations in a variety of organs and tissues, including brain AVMs. Because brain AVMs have the potential to cause disabling or fatal intracranial hemorrhage, detection of these lesions before rupture is the goal of screening MR imaging/MRA examinations in patients with HHT. Prior studies have demonstrated superior sensitivity for HHT-related brain AVMs by using postcontrast MR imaging sequences as compared with MRA alone.
View Article and Find Full Text PDFInt Forum Allergy Rhinol
December 2023
Background: Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder characterized by recurrent epistaxis, telangiectasias, and visceral arteriovenous malformations. Individuals with HHT often identify low humidity and temperature as detrimental to epistaxis severity. We set out to assess the relationship between humidity and temperature on epistaxis severity in patients with HHT.
View Article and Find Full Text PDFJ Neurointerv Surg
October 2023
Background: Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder characterized by recurrent epistaxis (nose bleeds), mucosal telangiectasias (spider veins), and arteriovenous malformations. Although HHT affects all racial groups, few studies have explored racial disparities among patients with HHT.
Methods: We performed a retrospective chart review of HHT patients who were seen at a single academic center between July 1, 2014 and January 1, 2022.
Background: Aneurysms are rare anomalies of the portomesenteric venous system. Thrombotic complications of these lesions can lead to mesenteric venous ischemia and bowel infarction, potentially requiring surgical intervention. Herein we describe a case of mesenteric ischemia due to a large thrombosed portomesenteric aneurysm treated with endovascular techniques.
View Article and Find Full Text PDFAccess to multi-detector computed tomography (MDCT) scanning for interventional procedures can prove to be logistically challenging as resources are often in different areas within the hospital. At some institutions, interventional radiology suites have moved to the operating room, separate from the diagnostic radiology department. At these institutions, complex interventional procedures requiring both fluoroscopy and MDCT may pose logistical challenges, especially as they pertain to timely patient transfers.
View Article and Find Full Text PDFHereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease that manifests as vascular malformations in the brain, lung, liver, gastrointestinal tract, nasal mucosa, and skin. Diagnosis and management of HHT is guided in large part by imaging studies, making it a condition with which the radiology community needs familiarity. Proper screening and care lead to improved morbidity and mortality in patients with HHT.
View Article and Find Full Text PDFInsulinomas are rare, and even rarer in patients with end-stage renal disease (ESRD). Clear criteria for the biochemical diagnosis of insulinomas in patients with renal failure have not been established, and hypoglycemia is often attributed to the renal disease itself, frequently leading to a delay in diagnosis. We describe a case of a patient who presented with asymptomatic recurrent hypoglycemia during hemodialysis.
View Article and Find Full Text PDFHereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder characterized by spontaneous epistaxis, telangiectasia, and visceral vascular malformations. Hepatic vascular malformations are common, though a minority are symptomatic. Symptoms are dependent on the severity and exact type of shunting caused by the hepatic malformation: Arteriosystemic shunting leads to manifestations of high output cardiac failure, and arterioportal shunting leads to portal hypertension.
View Article and Find Full Text PDFDescription: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease with an estimated prevalence of 1 in 5000 that is characterized by the presence of vascular malformations (VMs). These result in chronic bleeding, acute hemorrhage, and complications from shunting through VMs. The goal of the Second International HHT Guidelines process was to develop evidence-based consensus guidelines for the management and prevention of HHT-related symptoms and complications.
View Article and Find Full Text PDFHereditary hemorrhagic telangiectasia (HHT, Osler-Weber-Rendu disease) is a rare multisystem vascular disorder causing chronic gastrointestinal bleeding, epistaxis, and severe anemia. Bevacizumab, an anti-vascular endothelial growth factor antibody, may be effective to treat bleeding in HHT. This international, multicenter, retrospective study evaluated the use of systemic bevacizumab to treat HHT-associated bleeding and anemia at 12 HHT treatment centers.
View Article and Find Full Text PDFPurpose: To assess the short-term safety and efficacy of gallbladder cryoablation in high-risk patients.
Materials And Methods: A single-center, retrospective review of clinical and imaging follow-up from patients who were referred for gallbladder cryoablation between August 2018 and July 2019 was performed. All patients had serious pre-procedural comorbidities and were unacceptable surgical candidates (mean age, 52.
Morbidity in patients with single-ventricle Fontan circulation is common and includes arrhythmias, edema, and pulmonary arteriovenous malformations (PAVM) among others. We sought to identify biomarkers that may predict such complications. Twenty-five patients with Fontan physiology and 12 control patients with atrial septal defects (ASD) that underwent cardiac catheterization were included.
View Article and Find Full Text PDFA 71-year-old poor surgical candidate with acute calculous cholecystitis was initially managed with cholecystostomy tube drainage for 28 days. He subsequently underwent gallbladder cryoablation under moderate sedation with 3 cryoprobes and 2 separate 10-8-10 freeze-thaw cycles targeting the gallbladder neck/body and fundus followed by cholecystostomy tube removal. He was discharged 1 day after ablation.
View Article and Find Full Text PDFEndovascular biopsy can increase understanding of vascular disease by granting access to epigenetic data that are not normally attainable. This study compares biopsy yields among multiple devices used, examining differences in cell counts according to species, device type, sampling location and disease state. Chi-square analysis compared means of cells harvested with respect to these variables.
View Article and Find Full Text PDFThis review discusses current and developing indications for angioembolization (AE) techniques in urology cases, including trauma and non-trauma uses for kidney, prostate, and bladder conditions. AE methods, complications and technical and clinical outcomes are outlined for each indication for the purpose of aiding urologists in selecting ideal candidates for this procedure.
View Article and Find Full Text PDFPurpose: To demonstrate feasibility of endothelial cell (EC) biopsy from dialysis arteriovenous fistulas (AVFs) with the use of guidewires and to characterize gene expression differences between ECs from stenotic and nonstenotic outflow vein segments.
Materials And Methods: Nine consecutive patients undergoing fistulography for AVF dysfunction from June to August 2016 were enrolled. ECs were biopsied with the use of guidewires from venous outflow stenoses and control outflow veins central to the stenoses.
Management of splenic pseudoaneurysms in hemodynamically stable patients has shifted toward nonoperative management, including watchful waiting and endovascular embolization. Standard of treatment does not include percutaneous embolization for splenic pseudoaneurysm repair. In this case report, we document a successful percutaneous embolization of a post traumatic splenic pseudoaneurysm with thrombin.
View Article and Find Full Text PDFPurpose: To assess the effectiveness of disposable radiation-absorbing surgical drapes on operator radiation dose during transjugular liver biopsy (TJLB).
Materials And Methods: This dual-arm prospective, randomized study was conducted between May 2017 and January 2018 at a single institution. TJLB procedures (N = 62; patient age range, 19-80 y) were assigned at a 1:1 ratio to the use of radiation-absorbing surgical drapes or standard surgical draping.