Arterial Spin-Labeling MR Imaging in the Detection of Intracranial Arteriovenous Malformations in Patients with Hereditary Hemorrhagic Telangiectasia.

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. We now present data regarding the incremental benefit of including arterial spin-labeling (ASL) perfusion sequences as part of MR imaging/MRA screening in patients with this condition. MATERIALS AND METHODS: We retrospectively analyzed 831 patients at the UCSF Hereditary Hemorrhagic Telangiectasia Center of Excellence. Of these, 42 patients had complete MR imaging/MRA, ASL perfusion scans, and criterion-standard DSA data. Two neuroradiologists reviewed imaging studies and a third provided adjudication when needed. RESULTS: Eight patients had no brain AVMs detected on DSA. The remaining 34 patients had 57 brain AVMs on DSA. Of the 57 identified AVMs, 51 (89.5%) were detected on ASL and 43 (75.4%) were detected on conventional MR imaging/MRA sequences (P = .049), with 8 lesions detected on ASL perfusion but not on conventional MR imaging. CONCLUSIONS: ASL provides increased sensitivity for brain AVMs in patients with HHT. Inclusion of ASL should be considered as part of comprehensive MR imaging/MRA screening protocols for institutions taking care of patients with HHT.

Identifying racial disparities in hereditary hemorrhagic telangiectasia.

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. The primary outcomes of this study were the Epistaxis Severity Score (ESS) and the presence of pulmonary, cerebral, gastrointestinal, spinal, and hepatic arteriovenous malformations (AVMs). We analyzed racial differences using t-tests and analysis of variance (ANOVA) for continuous variables, and chi-squared tests for categorical variables. We then performed multivariable linear and logistic regressions on outcomes. RESULTS: Our review identified 35 Asian, 6 Black or African American, 72 Hispanic or Latino, and 244 White or Caucasian patients who met the inclusion criteria. Through an analysis of variance model, race/ethnicity was not significantly associated with ESS. Two univariable logistic regression models between race and both pulmonary and brain AVMs showed that race was associated with the incidence of pulmonary AVMs (p<0.01), with Asian patients at a 2.3-fold increased risk of pulmonary AVMs compared with White patients (p=0.03). Race was also associated with the incidence of cerebral AVMs (p<0.01) with Hispanic or Latino patients at a 4.8-fold increased risk compared with White patients (p<0.01). CONCLUSION: Patients who identified as Asian may have higher rates of pulmonary AVMs while patients identifying as Hispanic or Latino may have more cerebral AVMs. The correlations may be important for identifying risk factors in certain patient populations.

Synergy in IR-Hybrid CT/C-arm in the setting of critical trauma.

Access 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. Hybrid CT/fluoroscopy suite provides rapid, reliable MDCT assessment of trauma patients before and after emergent surgery, as well as access to the entire spectrum of emergent image-guided interventions in the same suite.

Hereditary Hemorrhagic Telangiectasia: The Convergence of Genotype, Phenotype, and Imaging in Modern Diagnosis and Management of a Multisystem Disease.

Hereditary 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. International guidelines were recently updated and form the basis for a detailed discussion of the role of imaging and image-guided therapy in HHT. © RSNA, 2021 Online supplemental material is available for this article.

The Role of Liver Imaging in Hereditary Hemorrhagic Telangiectasia.

Hereditary 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. Radiologic imaging, including ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI), is an important tool for assessing liver involvement. Doppler ultrasonography is the first-line screening modality for HHT-related liver disease, and it has a standardized scale. Imaging can determine whether shunting is principally to the hepatic vein or the portal vein, which can be a key determinant of patients' symptoms. Liver-related complications can be detected, including manifestations of portal hypertension, focal liver masses as well as ischemic cholangiopathy. Ultrasound and MRI also have the ability to quantify blood flow through the liver, which in the future may be used to determine prognosis and direct antiangiogenic therapy.

Angiopoietin-2 predicts morbidity in adults with Fontan physiology.

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. Plasma was collected from the hepatic vein and superior vena cava and underwent protein profiling for a panel of 20 analytes involved in angiogenesis and endothelial dysfunction. Ten (40%) of Fontan patients had evidence of PAVM, eighteen (72%) had a history of arrhythmia, and five (20%) were actively in arrhythmia or had a recent arrhythmia. Angiopoietin-2 (Ang-2) was higher in Fontan patients (8,875.4 ± 3,336.9 pg/mL) versus the ASD group (1,663.6 ± 587.3 pg/mL, p < 0.0001). Ang-2 was higher in Fontan patients with active or recent arrhythmia (11,396.0 ± 3,457.7 vs 8,118.2 ± 2,795.1 pg/mL, p < 0.05). A threshold of 8,500 pg/mL gives Ang-2 a negative predictive value of 100% and positive predictive value of 42% in diagnosing recent arrhythmia. Ang-2 is elevated among adults with Fontan physiology. Ang-2 level is associated with active or recent arrhythmia, but was not found to be associated with PAVM.

Assessment of cell yield among different devices for endovascular biopsy to harvest vascular endothelial cells.

Endovascular 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. Assessment of samples in 38 rabbits and 32 humans found no differences for species, location or pathology. Phenox clot retriever devices and retrievable stents yielded more cells (LR 64.2; p < 0.001) than other devices. Phenox clot retrievers and retrievable stents yield more cells than other device types. Further study of these devices for endovascular sampling is warranted to refine its use for this purpose.

Use of angioembolization in urology: a review.

This 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.

Percutaneous embolization of post traumatic splenic pseudoaneurysm.

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. Percutaneous embolization of splenic pseudoaneurysms can be considered a viable technique in patients who fail endovascular embolization or have lesions inaccessible to endovascular repair.

Gallbladder Cryoablation: Proof of Concept in a Swine Model for a Percutaneous Alternative to Cholecystectomy.

PURPOSE: To investigate the feasibility of percutaneous gallbladder cryoablation (GBC) under CT guidance in a swine model with histopathologic correlation. MATERIALS AND METHODS: Institutional Animal Care and Use Committee approval was obtained for this study protocol. Five pigs underwent GBC. Under CT guidance, 3-4 cryoprobes were positioned percutaneously at the gallbladder margins. Thermocouple probes were placed percutaneously at the gallbladder fundus, neck, free wall, and gallbladder fossa. Two freeze-thaw cycles ranging from 10 to 26 min were performed. The subjects were sacrificed 5 h after cryoablation. The gallbladder and bile ducts were resected, stained, and examined microscopically. RESULTS: GBC was completed in all subjects. A 10-mm ablation margin was achieved beyond all gallbladder walls. Thermocouple probes reached at least -20 °C. Intra-procedural body temperature decreased to a minimum of 35 °C but recovered after the procedure. Intra- and post-procedural vital signs otherwise remained within physiologic parameters. Non-target ablation occurred in the stomach and colon of the first two subjects. Histology demonstrated complete denudation of the gallbladder epithelium, hemorrhage, and edema within the muscularis layer, and preservation of the microscopic architecture of the common bile duct in all cases. CONCLUSION: Percutaneous gallbladder cryoablation is feasible, with adequate ablation margins obtained and histologic changes demonstrating transmural necrosis. Adjacent structures included in the ablation may require conservative ablation zones, hydrodissection, or continuous saline lavage.

CT-detected traumatic small artery extremity injuries: surgery, embolize, or watch? A 10-year experience.

Advances in computed tomography (CT) angiography have increased the sensitivity and specificity of detecting small branch arterial injuries in the extremities of trauma patients. However, it is unclear whether these patients should undergo surgery, angioembolization, or conservative watchful waiting. We hypothesized that uncomplicated small arterial branch injuries can be managed successfully with watchful waiting. A 10-year retrospective review of extremity CT angiograms with search findings of arterial "active extravasation" or "pseudoaneurysm" was performed at a level 1 county trauma center. Subgroup analysis was performed on those with isolated extremity injury and those with concurrent injuries. A total of 31 patients had CT-detected active extravasation (84 %) or pseudoaneurysm (16 %), 71 % of which were isolated vascular injuries. Of the patients evaluated, 71 % (n = 22) were managed with watchful waiting, 19 % (n = 6) with angioembolization, and 10 % (n = 3) with surgery. Watchful waiting complications included progression to alternative treatment (n = 1) and blood transfusions (n = 2). Complications of surgery included the inability to find active bleeding (n = 1) and postoperative psychosis (n = 1). Complications of angioembolization were limited to a postprocedure blood transfusion (n = 1). Patients with isolated vascular injuries had an average length of stay of 2.9 days, with management averages of the following: 2.7 days with watchful waiting (n = 16), 3.3 days with angioembolization (n = 3), and 3.7 days with surgery (n = 3). CT angiography has greatly increased the reported incidence of traumatic arterial injury in the extremity. We propose that small branch arterial injuries in the extremities can be managed successfully with watchful waiting and do not often require immediate embolization.

Intraprocedural Safety and Technical Success of the MVP Micro Vascular Plug for Embolization of Pulmonary Arteriovenous Malformations.

This case series describes early experience, intraprocedural safety, and technical success of the MVP Micro Vascular Plug (MVP; Covidien, Irvine, California) for embolization of 20 pulmonary arteriovenous malformations (PAVMs) using 23 plugs in seven patients with hereditary hemorrhagic telangiectasia. There was no device migration, and all devices were successfully detached electrolytically. Immediate cessation of flow through the feeding artery was achieved in 21 of 23 (91%) deployments. There was one minor complication. This series demonstrates the MVP to be safe and technically successful in the treatment of PAVMs.

Bartonella quintana Aortitis in a Man with AIDS, Diagnosed by Needle Biopsy and 16S rRNA Gene Amplification.

A man with newly diagnosed AIDS presented with months of back pain and fever. Computed tomography (CT) results demonstrated aortitis with periaortic tissue thickening. DNA amplification of biopsy tissue revealed Bartonella quintana, and Bartonella serologies were subsequently noted to be positive. The patient improved with prolonged doxycycline and rifabutin treatment. This case illustrates how molecular techniques are increasingly important in diagnosing Bartonella infections.