Outcome prediction for symptomatic patients with fibroids who underwent uterine artery embolization.

PURPOSE: To predict the clinical outcome of symptomatic patients with uterine leiomyomas who underwent uterine artery embolization (UAE), based on clinical and radiological features. METHODS: Patients with symptomatic uterine leiomyomas who underwent UAE from March 2010 to September 2019 were consecutively included in this retrospective study. Patients with persistent or recurrent symptoms and those who needed repeated UAE, myomectomy, or hysterectomy following the initial treatment were considered to have a poor outcome after UAE. The total and enhancing volume of the dominant leiomyoma in each location and uterine volume were obtained before and after UAE. Univariate and multivariate Cox proportional hazard analyses were used to evaluate the parameters that could predict poor outcome. RESULTS: A total of 116 patients (mean age, 45 ± 5 years) were included. Forty-six patients (46/116, 39.7%) showed poor outcome. Cox regression analysis showed higher hazard of poor outcome for younger patients vs. older patients (HR: 0.92, p-value: 0.01), patients with adenomyosis vs. patients without adenomyosis (HR: 2.47, p-value < 0.01), patients with adenomyosis thickness > 2.5 cm before UAE vs. patients without adenomyosis (HR: 4.2, p-value < 0.01) and for patients with intramural fibroid enhancement volume > 440 cm3 compared to patients with no intramural fibroids (p-value: 0.06). Multivariate Cox regression analysis including age, the thickness of adenomyosis, and intramural leiomyoma volume of enhancement before UAE was chosen as the best model to predict the outcome. CONCLUSIONS: Pretreatment clinical and MRI features could identify patients with a higher risk for poor outcome after UAE.

Risk factors and clinical features associated with basal ganglia manganese deposition in patients with hereditary hemorrhagic telangiectasia.

BACKGROUND: T1-hyperintensity of the basal ganglia (BG) due to manganese deposition is a known radiologic finding in patients with hereditary hemorrhagic telangiectasia (HHT), but risk factors and associated clinical manifestations are unclear. This study conducted a quantitative analysis of the association of T1-hyperintensity in HHT patients with specific risk factors, signs, and symptoms. METHODS: Patients seen at our center between 2005 and 2020 with a definitive diagnosis of HHT who had an available non-contrast T1-weighted brain MRI were included. Hyperintensity was evaluated using oval regions of interest measurements. The BG: thalamus intensity ratio was used to quantitatively evaluate T1-hyperintensity. Patient laboratory values and clinical findings were collected from electronic medical records. Hyperintensity was analyzed for its association with laboratory values, and clinical findings. Variables were analyzed through regression analysis. RESULTS: A total of 239 patients were included in this study. On 1.5 T scanners, values that were significant on multivariable regression analysis were age (p < .001), hepatic AVMs (p < .001), iron deficiency anemia (p = .0021), and cirrhosis (p = .016). On 3 T scanners, values that were significant on multivariable analysis were hepatic AVMs (p = .0024) and cirrhosis (p = .0056). On 3 T scanners, hyperintensity was significantly associated with tremor (OR = 1.17, p = .033), restless leg syndrome (OR = 1.22, p = .0086), and memory problems (OR = 1.17, p = .046). CONCLUSIONS: BG hyperintensity due to manganese deposition is significantly associated with hepatic risk factors on 1.5 T and 3 T scanners and iron deficiency anemia on 1.5 T scanners. On 3 T scanners, T1-hyperintensity is associated with neuropsychiatric signs and symptoms, such as tremor, restless leg syndrome, and memory problems.

Comparison between Gradient-Echo and Spin-Echo EPI MR Elastography at 3 T in quantifying liver stiffness of patients with and without iron overload; a prospective study.

OBJECTIVES: To compare the maximum axial area of the confidence mask and the calculated liver stiffness (LS) on gradient-echo (GRE) and spin-echo echo planar imaging (SE-EPI) MR elastography (MRE) in patients with and without iron deposition. METHODS: 104 patients underwent MRE by GRE and SE-EPI sequences at 3 T. R2* values >88 Hz in the liver were categorized in the iron overload group. The maximum axial area and the corresponding LS values were measured by manually contouring the whole area on one slice with the largest confidence mask at both GRE and SE-EPI sequences. RESULTS: In patients with iron overload, SE-EPI provided larger maximum axial confidence area in unfailed images (57.6 ± 41.7 cm2) compared to GRE (45.7 ± 29.1 cm2) (p-value = 0.007). In five patients with iron overload, imaging failed at GRE sequence, whereas at the SE-EPI sequence the maximum area of the confidence mask had a mean value of 33.5 ± 54.9 cm2. In livers without iron overload (R2*: 50.7 ± 13.1 Hz), the maximum area on the confidence mask was larger at SE-EPI (118.3 ± 41.2 cm2) than on GRE (105.1 ± 31.7 cm2) (P-value = 0.003). There was no significant difference in mean LS between SE-EPI (2.0 ± 0.3 kPa) and GRE (2.1 ± 0.5 kPa) in livers with iron overload (P value = 0.24). Similarly, in the group without iron overload, mean LS was 2.3 ± 0.7 kPa at SE-EPI and 2.4 ± 0.8 kPa at GRE sequences (P-value = 0.11). CONCLUSIONS: SE-EPI MRE can successfully provide similar LS measurements as GRE MRE. Furthermore, it provides a larger measurable area on the confidence mask in both groups with and without iron overload.

Responsiveness of the Patient-Reported Outcome Measure for Vascular Malformation Questionnaire in Patients with Low-Flow Vascular Malformations.

PURPOSE: To assess the responsiveness, defined as the ability to detect change in a patient's health or function, of the Patient-Reported Outcome Measure for Vascular Malformation (PROVAM) questionnaire in a cohort of patients with low-flow vascular malformations (VMs). MATERIALS AND METHODS: PROVAM was previously developed to assess symptoms, functional limitations, and social/emotional effects experienced by patients with VMs. This is a prospective cohort study of 56 patients with venous and lymphatic VMs who completed at least 2 PROVAM questionnaires, of whom 43 had undergone treatment with sclerotherapy in the interim between questionnaires. External responsiveness was assessed using a receiver operating characteristic (ROC) curve to ascertain whether a change in the total PROVAM score predicts whether patients reported symptom improvement and by correlating the change in the total PROVAM score and change in symptoms reported during clinic visit. Internal responsiveness was evaluated using Wilcoxon signed rank test, Cohen d effect size (ESp), and standard response mean difference (SRM). RESULTS: The total PROVAM score demonstrated excellent discrimination for symptom improvement with an area under the ROC curve of 0.856. There was a statistically significant, moderate positive correlation between the change in the total PROVAM score and the change in patient symptoms as determined from clinical visits (Spearman correlation coefficient [rs] = 0.67, P < .001). The total PROVAM score and all subdomain scores improved significantly after treatment (all P < .05). ESp and SRM were 0.80 and 0.83, respectively. CONCLUSIONS: PROVAM is responsive to improvement after treatment and may be useful to assess health-related quality of life in patients treated for VMs.

Eye-tracking differences between free text and template radiology reports: a pilot study.

Purpose: One possible limitation of structured template radiology reports is that radiologists look back and forth between viewing and dictation monitors, thereby impacting the length of time spent reviewing images and generating a report. We hypothesize that the total time spent viewing case images is diminished and/or the total time spent creating a report is prolonged when the report is generated using a structured template compared with free text format. Approach: Three neuroradiologists and three senior residents viewed five brain magnetic resonance imaging cases with unique findings while eye position was recorded. Participants generated reports for each case utilizing both structured templates and free text dictation. The time spent viewing images was compared with the time spent looking at the dictation screen. Results: The two main hypotheses were confirmed: the total time viewing images diminished with templates versus free text dictation and the total time to create a report was prolonged with templates. The mean time (s) spent on the "image" region of interest approached statistical significance as a function of the report type [free: attendings = 236.79 (154.43), residents = 223.55 (77.79); template: attendings = 163.40 (73.42), residents = 182.48 (77.47)] and was overall lower with the template reporting for both attendings and residents ( F = 3.77 , p = 0.0623 ), but it did not differ as a function of seniority ( F = 0.017 , p = 0.8977 ). Conclusions: Template-based radiology reports have significant potential to alter the way radiologists view images and report on them, spending more time viewing the report monitor rather than diagnostic images compared with free text dictation. Many radiologists prefer templates for reporting as the structured format may aid in conducting a more systematic or thorough search for findings, although prior work on this assumption is mixed. Future eye-tracking studies could further elucidate whether and how templates and free reports impact the detection and classification of radiographic findings.

Postembolization Persistence of Pulmonary Arteriovenous Malformations: A Retrospective Comparison of Coils and Amplatzer and Micro Vascular Plugs Using Propensity Score Weighting.

BACKGROUND. Endovascular embolization of pulmonary arteriovenous malformations (PAVMs) was historically performed with embolic coils. The Amplatzer Vascular Plug device (AVP) was introduced for this purpose in 2007 and the Micro Vascular Plug device (MVP) in 2013. OBJECTIVE. The purpose of this study was to compare coils, AVPs, and MVPs in terms of risk of persistence after PAVM embolization by use of propensity score weighting to account for biases in device selection. METHODS. This retrospective study included 112 patients (78 women and girls, 34 men and boys; mean age, 45 years) who underwent embolization of 393 PAVMs with a single device type (coil, MVP, or AVP) from January 2003 to January 2020. Persistence was defined as less than 70% reduction in PAVM sac size or contrast enhancement of the sac on follow-up pulmonary CTA. A Cox proportional hazards regression model was used to assess associations between embolic device selection and PAVM persistence. Inverse propensity score weighting was used to account for differences in embolic device selection based on patient and PAVM characteristics. RESULTS. The median postembolization follow-up period was 1.5 years (IQR, 0.3-5.6 years). Persistence was found in 10% (41/393) of PAVMs, including 16% (34/207) of those treated with coils, 8% (7/88) of those treated with AVPs, and 0% (0/98) of those treated with MVPs. Variables associated with embolization device (p < .25) were age, sex, pediatric versus adult status, smoking status, PAVM complexity, PAVM laterality, number of feeding arteries, and feeding artery diameter. The Cox regression model incorporated inverse propensity score weighting to account for the differences between treatment groups in these variables and incorporated feeding artery diameter because of imbalance remaining after weighting. With coils as the referent, MVPs had a hazard ratio for persistence of less than 0.01 (95% CI, < 0.01 to < 0.01; p < .001), and AVPs had a hazard ratio of 0.37 (95% CI, 0.16-0.90; p = .03). CONCLUSION. The risk of persistence after PAVM embolization was significantly lower for MVPs alone than for coils or AVPs alone. In addition, the risk of persistence was lower for AVPs than for coils. CLINICAL IMPACT. The findings support the clinical use of MVPs as the preferred device for PAVM embolization over coils and polytetrafluoroethylene-covered plugs.

Late Surges in COVID-19 Cases and Varying Transmission Potential Partially Due to Public Health Policy Changes in 5 Western States, March 10, 2020, to January 10, 2021.

OBJECTIVE: This study investigates the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) transmission potential in North Dakota, South Dakota, Montana, Wyoming, and Idaho from March 2020 through January 2021. METHODS: Time-varying reproduction numbers, R t , of a 7-d-sliding-window and of non-overlapping-windows between policy changes were estimated using the instantaneous reproduction number method. Linear regression was performed to evaluate if per-capita cumulative case-count varied across counties with different population size or density. RESULTS: The median 7-d-sliding-window R t estimates across the studied region varied between 1 and 1.25 during September through November 2020. Between November 13 and 18, R t was reduced by 14.71% (95% credible interval, CrI, [14.41%, 14.99%]) in North Dakota following a mask mandate; Idaho saw a 1.93% (95% CrI [1.87%, 1.99%]) reduction and Montana saw a 9.63% (95% CrI [9.26%, 9.98%]) reduction following the tightening of restrictions. High-population and high-density counties had higher per-capita cumulative case-count in North Dakota on June 30, August 31, October 31, and December 31, 2020. In Idaho, North Dakota, South Dakota, and Wyoming, there were positive correlations between population size and per-capita weekly incident case-count, adjusted for calendar time and social vulnerability index variables. CONCLUSIONS: R t decreased after mask mandate during the region's case-count spike suggested reduction in SARS-CoV-2 transmission.

Magnetic Resonance Imaging Predictors of Hepatocellular Carcinoma Progression and Dropout in Patients in Liver Transplantation Waiting List.

With the rising incidence of hepatocellular carcinoma (HCC), more patients are now eligible for liver transplantation. Consequently, HCC progression and dropout from the waiting list are also anticipated to rise. We developed a predictive model based on radiographic features and alpha-fetoprotein to identify high-risk patients. Methods: This is a case-cohort retrospective study of 76 patients with HCC who were listed for liver transplantation with subsequent liver transplantation or delisting due to HCC progression. We analyzed imaging-based predictive variables including tumor margin (well- versus ill-defined), capsule bulging lesions, volumetric analysis and distance to portal vein, tumor numbers, and tumor diameter. Volumetric analysis of the index lesions was used to quantify index tumor total volume and volumetric enhancement, whereas logistic regression and receiver operating characteristic curve (ROC) analyses were used to predict the main outcome of disease progression. Results: In univariate analyses, the following baseline variables were significantly associated with disease progression: size and number of lesions, sum of lesion diameters, lesions bulging the capsule, and total and venous-enhancing (viable) tumor volumes. Based on multivariable analyses, a risk model including lesion numbers and diameter, capsule bulging, tumor margin (infiltrative versus well-defined), and alpha-fetoprotein was developed to predict HCC progression and dropout. The model has an area under the ROC of 82%, which was significantly higher than Milan criteria that has an area under the ROC of 67%. Conclusions: Our model has a high predictive test for patient dropout due to HCC progression. This model can identify high-risk patients who may benefit from more aggressive HCC treatment early after diagnosis to prevent dropout due to such disease progression.

Impact of Single Nucleotide Polymorphism in the ANKRD55 Gene on Occurrence and Clinical Characteristics of Rheumatoid Arthritis.

Background: Rheumatoid Arthritis (RA) has multifactorial etiology and numerous genetic and environmental factors have been related to an increased risk of RA. Recently, Genome-Wide Association Studies (GWAS) suggested a large number of Single Nucleotide Polymorphisms (SNPs) loci affecting the susceptibility to RA. One of these loci is rs6859219 (C>A), a functional polymorphism in the ANKRD55 gene which was associated with the expression of ANKRD55 and IL6ST. In the current study, we evaluated the possible association between rs6859219 (intronic variant) in the ANKRD55 gene with RA risk in the Iranian population. Methods: A case-control study using 118 RA patients and 115 healthy counterparts was undertaken in order to determine rs6859219 genotypes using real-time polymerase chain reaction High-Resolution Melting (HRM) method. Results: There was a significant difference in the genotype and allele frequencies of rs6859219 between patients and controls (p<0.001). Logistic regression analysis demonstrates that CC genotype and C allele increased the risk of RA (OR for CC genotype= 7.12; 95%CI [3.51-15.05]/OR for C allele=4.16; 95%CI [2.78-6.28]). Furthermore, regarding the dominant and recessive model of inheritance, RA patients indicated obvious association of the rs6859219 variant compared to healthy controls (p<0.001). Moreover, in the patient group, there was a significant correlation between C-Reactive Protein (CRP) concentration with rs6859219 polymorphism (p<0.001). Conclusion: Our findings propose a substantial correlation between rs6859219 polymorphism and RA risk and clinical characteristics of this disease in the Iranian population.

Assessing long-term locoregional control of spinal osseous metastases after microwave ablation.

Osseous metastases to the spine result in significant pain and decreased quality of life. The purpose of this study was to evaluate the long-term efficacy of microwave ablation (MWA) for the treatment of spinal metastases regarding pain reduction and local control of disease progression. In this single center retrospective study, patients with osseous metastases to the spine undergoing MWA with vertebroplasty from 2013 to 2020 were included. Locoregional control of metabolic activity at the treated level was assessed using PET/CT scan both pre- and post-procedure. Pain reduction was measured using change in visual analog scale (VAS) pain score. Forty-eight spinal levels were treated with MWA in 28 patients (57 % male, mean age 68 ± 9 years). Median ablation time, energy, and temperature were 4 min and 13 s, 3.6 kJ, and 80 °C, respectively. Median pre-procedure maximum standard uptake value (SUVmax) was significantly reduced following ablation, from 4.55 (IQR 3.65-6.1) to 0 (IQR 0-1.8; p < 0.001), over an average of 29 ± 14.1 month follow up period. Pre-procedure VAS pain score was reduced from median (IQR) of 8 (6.5-9) to 1(1-2), 2(1-3) and 1(0.5-3) at 24 h, four weeks, and six months post-procedure, respectively (all p < 0.001 with respect to pre-procedure scores). In conclusion, this study supports microwave ablation as an effective technique for pain palliation and long-term locoregional tumor control of oligometastatic spinal disease as assessed by metabolic response.

Can Posttransarterial Chemoembolization Volumetric Oil Deposition on Computed Tomography Predict Treatment Response on Magnetic Resonance Imaging in Leiomyosarcoma Metastases to the Liver?

OBJECTIVE: To predict early tumor response to transarterial chemoembolization (TACE) based on volumetric oil deposition on posttreatment computed tomography (CT) in patients with leiomyosarcoma liver metastases. METHODS: This retrospective lesion-by-lesion based study included 32 lesions. The volumetric percent enhancing tumor on pre-TACE and 1-month post-TACE venous phase magnetic resonance imaging (MRI), and the percent oil deposition on CT 1 day after TACE were calculated. The predicted post-TACE enhanced percentage was computed by subtracting percent oil deposition from baseline percent enhanced. RESULTS: Mean percentage of viable tumor on pre-TACE MRI was 90.6% ± 9.3%. Mean oil deposition was calculated as 51.4% ± 26.2%. Mean percentage of measured residual tumor enhancement 1 month after TACE was 58.3% ± 27%, which correlates with predicted enhancement percentage of 43.9% ± 25.1% (r = 0.72, P < 0.001). A threshold of 35.5% for enhancement reduction was determined to predict tumor response with an accuracy of 78.1%. CONCLUSION: Volumetric oil deposition on CT can predict residual enhancement on post-TACE MRI.

Vision impairment and cognitive decline among older adults: a systematic review.

OBJECTIVES: There has been increasing epidemiological research examining the association between vision impairment (VI) and cognitive impairment and how poor vision may be a modifiable risk factor for cognitive decline. The objective of this systematic review is to synthesise the published literature on the association of VI with cognitive decline, cognitive impairment or dementia, to aid the development of interventions and guide public policies pertaining to the relationship between vision and cognition. METHODS: A literature search was performed with Embase, Medline and Cochrane library databases from inception to March 2020, and included abstracts and articles published in peer-reviewed journals in English. Our inclusion criteria included publications that contained subjective/objective measures of vision and cognition, or a diagnosis of VI, cognitive impairment or dementia. Longitudinal or cross-sectional studies with ≥100 participants aged >50 years were included. The search identified 11 805 articles whose abstracts underwent screening by three teams of study authors. Data abstraction and quality assessment using the Effective Public Health Practice Project Quality Assessment Tool were performed by one author (NN). 10% of the articles underwent abstraction and appraisal by a second author (LA/VV), results were compared between both and were in agreement. RESULTS: 110 full-text articles were selected for data extraction, of which 53 were cross-sectional, 43 longitudinal and 14 were case-control studies. The mean age of participants was 73.0 years (range 50-93.1). Ninety-one (83%) of these studies reported that VI was associated with cognitive impairment. CONCLUSION: Our systematic review indicates that a majority of studies examining the vision-cognition relationship report that VI is associated with more cognitive decline, cognitive impairment or dementia among older adults. This synthesis supports the need for additional research to understand the mechanisms underlying the association between VI and cognitive impairment and to test interventions that mitigate the cognitive consequences of VI.

Imaging of Colorectal Liver Metastasis.

Colorectal cancer (CRC) is one of the most common cancers in the world. The most important determinant of survival and prognosis is the stage and presence of metastasis. The liver is the most common location for CRC metastasis. The only curative treatment for CRC liver metastasis (CRLM) is resection; however, many patients are ineligible for surgical resection of CRLM. Locoregional treatments such as ablation and intra-arterial therapy are also available for patients with CRLM. Assessment of response after chemotherapy is challenging due to anatomical and functional changes. Antiangiogenic agents such as bevacizumab that are used in the treatment of CRLM may show atypical patterns of response on imaging. It is vital to distinguish patterns of response in addition to toxicities to various treatments. Imaging plays a critical role in evaluating the characteristics of CRLM and the approach to treatment. CT is the modality of choice in the diagnosis and management of CRLM. MRI is best used for indeterminate lesions and to assess response to intra-arterial therapy. PET-CT is often utilized to detect extrahepatic metastasis. State-of-the-art imaging is critical to characterize patterns of response to various treatments. We herein review the imaging characteristics of CRLM with an emphasis on imaging changes following the most common CRLM treatments.

Brain MRI in Autism Spectrum Disorder: Narrative Review and Recent Advances.

Autism spectrum disorder (ASD) is neuropsychiatric continuum of disorders characterized by persistent deficits in social communication and restricted repetitive patterns of behavior which impede optimal functioning. Early detection and intervention in ASD children can mitigate the deficits in social interaction and result in a better outcome. Various non-invasive imaging methods and molecular techniques have been developed for the early identification of ASD characteristics. There is no general consensus on specific neuroimaging features of autism; however, quantitative magnetic resonance techniques have provided valuable structural and functional information in understanding the neuropathophysiology of ASD and how the autistic brain changes during childhood, adolescence, and adulthood. In this review of decades of ASD neuroimaging research, we identify the structural, functional, and molecular imaging clues that most accurately point to the diagnosis of ASD vs. typically developing children. These studies highlight the 1) exaggerated synaptic pruning, 2) anomalous gyrification, 3) interhemispheric under- and overconnectivity, and 4) excitatory glutamate and inhibitory GABA imbalance theories of ASD. The application of these various theories to the analysis of a patient with ASD is mitigated often by superimposed comorbid neuropsychological disorders, evolving brain maturation processes, and pharmacologic and behavioral interventions that may affect the structure and function of the brain. LEVEL OF EVIDENCE: 4 TECHNICAL EFFICACY: Stage 3.

Predicting early necrosis of colorectal liver metastases using volumetric enhancement on baseline MRI and oil deposition on post-cTACE unenhanced CT.

PURPOSE: To predict tumor necrosis after conventional TACE (cTACE) in patients with colorectal liver metastasis (CRLM) based on volumetric oil deposition on CT one day after treatment. METHODS: Thirty-four lesions in 20 men and 6 women were included in this IRB-approved HIPAA-compliant, retrospective lesion-by-lesion-based study. Semiautomatic volumetric segmentation of target lesions was performed on baseline MRI and post-treatment CT. Predicted percentage of tumor necrosis was defined as 100%-(%baseline MRI enhancement-%CT oil deposition). Necrosis on post-TACE MRI was measured after volumetric segmentation to assess the accuracy of predicting tumor necrosis. The relationship between predicted necrosis percent and post-cTACE measured necrosis percent on MRI was compared using Pearson correlation analysis. Inter-reader agreement was calculated by intraclass correlation coefficient (ICC) after using the same method. RESULTS: Patients in this cohort had a mean age of 64 ± 14 years. Mean percentage of the viable tumor on pre-cTACE venous phase MRI was 58.5% ± 23.9%. Mean oil deposition was 19.8% ± 14.6%. Mean percentage of calculated necrosis one month after cTACE was 59.2% ± 22.7% on venous phase MRI, which had a significant correlation with predicted necrotic percentage of 61.3% ± 19.3% (r = 0.89, p < 0.0001). ICC for enhancement percentage on pre-cTACE and post-cTACE venous phase MRIs were 0.93 (95% CI 0.83, 0.97) and 0.86 (95% CI 0.66, 0.94), respectively. ICC for oil deposition was 0.92 (95% CI 0.81, 0.96). CONCLUSION: Measuring oil deposition of the whole tumor on CT one day after cTACE can assist to predict post-cTACE tumor necrosis.

Clinical and molecular characterization of patients with hereditary hemorrhagic telangiectasia: Experience from an HHT Center of Excellence.

In this retrospective single-center study, we evaluated whether/how pathogenic/likely pathogenic variants of three hereditary hemorrhagic telangiectasia (HHT)-associated genes (ENG, ACVRL1, and SMAD4) are associated with specific clinical presentations of HHT. We also characterized the morphological features of pulmonary arteriovenous malformations (AVMs) in patients with these variants. Pathogenic or likely pathogenic variants were detected in 64 patients. Using nonparametric statistical tests, we compared the type and prevalence of specific HHT diagnostic features associated with these three variants. Pathogenic variants in these genes resulted in gene-specific HHT clinical presentations. Epistaxis was present in 93%, 94%, and 100% of patients with ENG, ACVRL1, and SMAD4 variants, respectively (p = 0.79). Pulmonary AVMs were more common in patients with the ENG variant (p = 0.034) compared with other subgroups. ACVRL1 variant was associated with the lowest frequency of pulmonary AVMs (p = 0.034) but the highest frequency of hepatic AVMs (p = 0.015). Patients with the ACVRL1 variant did not have significantly more pancreatic AVMs compared with the other groups (p = 0.72). ENG, ACVRL1, and SMAD4 pathogenic or likely pathogenic variants are associated with gene-specific HHT presentations, which is consistent with results from other HHT centers.

Development and Preliminary Validation of the Patient-Reported Outcome Measure for Vascular Malformation Questionnaire: A Prospective Cohort Study.

PURPOSE: To develop and validate the Patient-Reported Outcome Measure for Vascular Malformation (PROVAM) questionnaire to assess the health-related quality of life in patients with vascular malformations. MATERIALS AND METHODS: We developed and validated PROVAM using a mixed methods design during a prospective clinical trial at a vascular anomalies clinic. From July 2019 to February 2020, 108 consecutive patients completed 130 questionnaires. The 30-item instrument assessed the domains of pain, emotional/social well-being, functional impact, and treatment satisfaction. Two additional items assessed ease of understanding and relevance. The primary outcomes of instrument reliability and validity were evaluated across several indices. The secondary outcome of responsiveness evaluated total score changes for patients who completed questionnaires both before and after treatment. RESULTS: Instrument reliability, as measured by Cronbach alpha, was ≥0.79 for pain, emotional/social well-being, and functional impact domains. Primary domain structure was confirmed by factor analysis (P <. 001) and convergent construct validity for all but 1 Likert scale item. In the subgroup analysis of 13 participants who completed PROVAM before and after treatment, instrument responsiveness, as measured by the total score, showed a significant decrease (median, -10 points; interquartile range [IQR], -3 to -16; P = .04). Participants found the questions easy to understand (median, 5 points; IQR, 4-5 on a 5-point scale) and relevant (median score, 4; IQR, 3-5). CONCLUSIONS: Preliminary data support the reliability and validity of PROVAM in measuring the health-related quality of life in patients with vascular malformations.

Imaging neuroendocrine tumors: Characterizing the spectrum of radiographic findings.

Neuroendocrine tumors (NET) are a group of neoplasms with neuroendocrine differentiation affecting a wide range of organs. Functional NETs present with symptoms due to the particular hormone produced. Functional NETs are usually small at diagnosis and therefore can be challenging to diagnose. In contrast, non-functioning NETs are generally larger and present with mass effect. Imaging plays an indispensable role in diagnosis, staging and management of patients with NETs. The optimal modality and technique for imaging of NETs depend on the location of primary and metastatic lesions. Regardless of the imaging modality, dynamic contrast-enhanced imaging is essential for evaluation of NETs. In general, CT scan is typically the primary imaging modality for evaluating NETs. MRI is used as a complementary modality, being superior to other modalities to assess liver metastasis. Nuclear medicine imaging is also widely used in NET assessment.

Safety of diagnostic spinal angiography in children.

BACKGROUND: Spinal angiography (SA) is associated with low complications in adults but its safety in children has not been properly analyzed. The goal of our study is to assess the safety of pediatric SA. METHODS: This study is the retrospective analysis of a series of 36 consecutive SA procedures performed in 27 children over a 5-year period. Parameters including neurological complications, non-neurological complications requiring additional management, contrast volume, and radiation exposure were analyzed via univariate and bivariate methods. RESULTS: Our cohort included 24 diagnostic and 12 combined therapeutic cases in children with an average age of 11.1 years. No neurological or non-neurological complication requiring additional management was recorded. The average volume of contrast administered was 1.6 mL/kg in the diagnostic group and 0.9 mL/kg in the combined group. The average air kerma was 186.9mGy for an average of 36.8 exposures in the diagnostic group, and 264.5mGy for an average of 21 exposures in the combined group. Patients in the combined group had lower contrast load (45% lower on average) and higher air kerma (1.6 times higher on average). The difference in air kerma was due to a higher live fluoroscopy-related exposure. CONCLUSIONS: This study reports the largest pediatric SA cohort analyzed to date and the only one including radiation dose and contrast load. It confirms that pediatric SA is a safe imaging modality with low risk of complications, and demonstrates that SA can be performed in children with low radiation exposure and contrast load.