Validating a Curvature-Based Marker of Cervical Carotid Tortuosity for Risk Assessment in Heritable Aortopathies.

BACKGROUND: Cervical arterial tortuosity is associated with adverse outcomes in Loeys-Dietz syndrome and other heritable aortopathies. METHODS AND RESULTS: A method to assess tortuosity based on curvature of the vessel centerline in 3-dimensional space was developed. We measured cervical carotid tortuosity in 65 patients with Loeys-Dietz syndrome from baseline computed tomography angiogram/magnetic resonance angiogram and all serial images during follow-up. Relations between baseline carotid tortuosity, age, aortic root diameter, and its change over time were compared. Patients with unoperated aortic roots were assessed for clinical end point (type A aortic dissection or aortic root surgery during 4 years of follow-up). Logistic regression was performed to assess the likelihood of clinical end point according to baseline carotid tortuosity. Total absolute curvature at baseline was 11.13±5.76 and was relatively unchanged at 8 to 10 years (fold change: 0.026±0.298, P=1.00), whereas tortuosity index at baseline was 0.262±0.131, with greater variability at 8 to 10 years (fold change: 0.302±0.656, P=0.818). Baseline total absolute curvature correlated with aortic root diameter (r=0.456, P=0.004) and was independently associated with aortic events during the 4-year follow-up (adjusted odds ratio [OR], 2.64 [95% CI, 1.02-6.85]). Baseline tortuosity index correlated with age (r=0.532, P<0.001) and was not associated with events (adjusted OR, 1.88 [95% CI, 0.79-4.51]). Finally, baseline total absolute curvature had good discrimination of 4-year outcomes (area under the curve=0.724, P=0.014), which may be prognostic or predictive. CONCLUSIONS: Here we introduce cervical carotid tortuosity as a promising quantitative biomarker with validated, standardized characteristics. Specifically, we recommend the adoption of a curvature-based measure, total absolute curvature, for early detection or monitoring of disease progression in Loeys-Dietz syndrome.

Whole-lesion assessment of volume and signal changes after sclerotherapy of extremity venous malformations.

PURPOSE: To investigate quantitative changes in MRI signal intensity (SI) and lesion volume that indicate treatment response and correlate these changes with clinical outcomes after percutaneous sclerotherapy (PS) of extremity venous malformations (VMs). METHODS: VMs were segmented manually on pre- and post-treatment T2-weighted MRI using 3D Slicer to assess changes in lesion volume and SI. Clinical outcomes were scored on a 7-point Likert scale according to patient perception of symptom improvement; treatment response (success or failure) was determined accordingly. RESULTS: Eighty-one patients with VMs underwent 125 PS sessions. Treatment success occurred in 77 patients (95 %). Mean (±SD) changes were -7.9 ± 24 cm3 in lesion volume and -123 ± 162 in SI (both, P <.001). Mean reduction in lesion volume was greater in the success group (-9.4 ± 24 cm3) than in the failure group (21 ± 20 cm3) (P =.006). Overall, lesion volume correlated with treatment response (ρ = -0.3, P =.004). On subgroup analysis, volume change correlated with clinical outcomes in children (ρ = -0.3, P =.03), in sodium tetradecyl sulfate-treated lesions (ρ = -0.5, P =.02), and in foot lesions (ρ = -0.6, P =.04). SI change correlated with clinical outcomes in VMs treated in 1 PS session (ρ = -0.3, P =.01) and in bleomycin-treated lesions (ρ = -0.4, P =.04). CONCLUSIONS: Change in lesion volume is a reliable indicator of treatment response. Lesion volume and SI correlate with clinical outcomes in specific subgroups.

Percutaneous Sclerotherapy of Large Venous Malformations Using Consecutive Polidocanol and Bleomycin Foam: MR Imaging Volumetric and Quality-of-Life Assessment.

PURPOSE: To retrospectively evaluate sclerotherapy using consecutive polidocanol and bleomycin foam (CPBF) for large untreated venous malformations (VMs) and/or those resistant to prior treatment. MATERIALS AND METHODS: This retrospective study included all patients treated with CPBF for untreated VMs larger than 10 mL and/or refractory to treatment between May 2016 and October 2019. Baseline and follow-up VM volumes were measured on fat-suppressed T2-weighted magnetic resonance (MR) imaging. Outcome was evaluated on postprocedural MR imaging volumetry and by a retrospective survey assessing clinical response and adverse events. Imaging response was considered good for volume reduction from 50% to 70% and excellent for volume reduction ≥70%. Symptoms and quality-of-life (QoL) scores were compared before and after CPBF sclerotherapy. RESULTS: Forty-five patients (mean age, 16 years; range, 1-63 years; 25 males) with 57 VMs were analyzed and treated by 80 sclerotherapy. Sixty percent (27 of 45) of patients had undergone prior treatment for VM. Median VM volume was 36.7 mL (interquartile range, 84 mL) on pretherapy MR imaging. Good and excellent results after the last sclerotherapy were achieved in 36% (16 of 45) and 29% (13 of 45) of patients, respectively, corresponding to a decrease of >50% in 60% (34 of 57) of VMs. QoL score increased by at least 3 points, regardless of initial symptoms. Most patients did not desire additional sclerotherapy owing to near complete symptomatic relief, even for patients who did not achieve a good response. Swelling, pain, and motor impairment scores significantly improved after CPBF. Adverse events included fever (44%, 15 of 34) and nausea/vomiting (29%, 10 of 34). CONCLUSIONS: CPBF sclerotherapy represents an effective therapy for large and/or refractory VMs with minimal adverse events.

Poor concordance between definitions of type III arch and implications for risk prediction and assessment for carotid artery stenting.

OBJECTIVE: The type III arch configuration has been inconsistently reported as a stroke risk factor during carotid artery stenting. However, at least three different methods for the definition of type III arch can be identified in the literature, related to the level of the origin of the innominate artery (IA). According to Casserly's definition, a type III arch presents with an origin of the IA below the horizontal plane of the inner curvature. According to Madhwal's definition, a type III arch has a distance greater than twice the diameter of the left common carotid artery between the highest point of the arch and the origin of the IA. According to MacDonald's definition, a type III arch presents with a distance of ≥2 cm between the highest point of the arch and the origin of the IA. Our aim was to assess the level of concordance between these different methods. METHODS: Anonymized thoracic computed tomography scans of 100 healthy patients were reviewed. Two of us independently stratified the selected cases as a type I to III arch, according to the three considered definitions. The interobserver level of concordance for each type III arch classification and level of concordance among the three definitions were assessed. RESULTS: The 100 selected patients (64% male) were 76 ± 7 years old. For each definition, the interobserver repeatability was almost perfect for all three (Madhwal, κ = 0.81; 95% confidence interval [CI], 0.71-0.99; MacDonald, κ = 0.82; 95% CI, 0.72-0.92; Casserly, κ = 0.84; 95% CI, 0.74-0.93). The level of concordance among the different definitions was very low (Madhwal vs MacDonald, 85% [P = .002]; 33% for type III arch; Madhwal vs Casserly, 60% [P < .0001]; 12% for type III arch; MacDonald vs Casserly, 75% [P < .0001]; 12% for type III arch). CONCLUSIONS: The three definitions of the type III arch have a very low level of concordance, which might account for the varying clinical relevance of this configuration. Our findings have relevant implications for risk prediction for carotid artery stenting based on the presence of a type III arch, for comparisons of the results from different studies, and for comparisons of different datasets from multicenter trials.

Value Improvement by Assessing IR Care via Time-Driven Activity-Based Costing.

PURPOSE: To evaluate time-driven activity-based costing (TDABC) in interventional radiology for image-guided vascular malformation treatment as an example. MATERIALS AND METHODS: Retrospective analysis was performed on consecutive vascular malformation treatment cycles [67 venous malformations (VMs) and 11 arteriovenous malformations (AVMs)] in a university hospital in 2018. All activities were integrated with a process map, and spent resources were assigned accordingly. TDABC uses 2 parameters: (i) practical capacity cost rate, calculated as 80% of theoretical capacity, and (ii) time consumption of each resource determined by interviews (23 items). Thereby, the total costs were calculated. Treatment cycles were modified according to identified resource waste and TDABC-guided negotiations with health insurance. RESULTS: Total personnel time required was higher for AVM (1,191 min) than for VM (637 min) treatment. The interventional procedure comprised the major part (46%) of personnel time required in AVM, whereas it comprised 19% in VM treatment. Materials represented the major cost type in AVM (75%) and VM (45%) treatments. TDABC-based treatment process modification led to a decrease in personnel time need of 16% and 30% and a cost reduction of 5.5% and 15.7% for AVM and VM treatments, respectively. TDABC-guided cost reduction and TDABC-informed negotiations improved profit from -56% to +40% and from +41% to +69% for AVM and VM treatments, respectively. CONCLUSIONS: TDABC facilitated the precise costing of interventional radiologic treatment cycles and optimized internal processes, cost reduction, and revenues. Hence, TDABC is a promising tool to determine the denominator of interventional radiology's value.

Radiofrequency Ablation of Facial Venolymphatic Malformations: Assessment of Efficacy and Safety and the Role of Injectable Electrodes.

PURPOSE: To evaluate the efficacy and safety of ultrasound (US)-guided radiofrequency (RF) ablation in the treatment of facial microcystic lymphatic malformations (mLMs) or mixed venolymphatic malformations (VLMs) and to introduce the "induced-paralysis" technique with the use of an injectable electrode to minimize neural complications. MATERIALS AND METHODS: Data from patients with symptomatic facial mLMs or mixed VLMs who underwent US-guided RF ablation in 2014-2017 were retrospectively reviewed. Computed tomography (CT) and/or magnetic resonance (MR) imaging was used to localize any functional structures close to the target lesion. Treatment outcome was assessed by 4-point cosmetic grading score (CGS). Treatment outcome was compared between patients with vs without a functional structure close to the lesion and those treated with a noninjectable vs an injectable electrode. Immediate and late complications were assessed during follow-up. RESULTS: Data from 15 patients were evaluated. Initial CGS and median volume were significantly decreased at the last follow-up in all patients (from 4 to 1 and from 7 mL to 0.7 mL, respectively; P < .05). Treatment outcome differed significantly between those with and without a functional structure on CT or MR imaging (P < .05) but showed no difference between noninjectable (n = 9) and injectable electrodes (n = 6). Two cases of transient cranial nerve palsy were seen in the noninjectable electrode group. CONCLUSIONS: US-guided RF ablation is effective in patients with facial mLMs or mixed VLMs. The induced-paralysis technique with an injectable electrode has the potential to ensure technical safety without compromising efficacy.

Volumetric Assessment of Pediatric Vascular Malformations Using a Rapid, Hand-Held Three-Dimensional Imaging System.

The effect of percutaneous, surgical, and medical therapies for vascular malformations (VMs) is often difficult to quantify volumetrically using cross-sectional imaging. Volumetric measurement is often estimated with serial, expensive MRI examinations which may require sedation or anesthesia. We aim to explore whether a portable 3D scanning device is capable of rapid, accurate volumetric analysis of pediatric VMs. Using an iPad-mounted infrared scanning device, 3D scans of patient faces, arms, and legs were acquired over an 8-month study period. Proprietary software was use to perform subsequent volumetric analysis. Of a total of 30 unilateral VMs involving either the face, arms, or legs, 26 (86.7%) VMs were correctly localized by discerning the larger volume of the affected side compared to the normal contralateral side. For patients with unilateral facial VMs (n = 10), volume discrepancy between normal and affected sides differed compared with normal controls (n = 19). This was true for both absolute (60 cc ± 55 vs 15 cc ± 8, p = 0.03) as well as relative (18.1% ± 13.2 vs 4.0% ± 2.1, p = 0.008) volume discrepancy. Following treatment, two patients experienced change in leg volume discrepancy ranging from - 17.3 to - 0.4%. Using a portable 3D scanning device, we were able to rapidly and noninvasively detect and quantify volume discrepancy resulting from VMs of the face, arms, and legs. Preliminary data suggests this technology can detect volume reduction of VMs in response to therapy.

International Society for the Study of Vascular Anomalies Classification of Soft Tissue Vascular Anomalies: Survey-Based Assessment of Musculoskeletal Radiologists' Use in Clinical Practice.

AIM: There is controversy regarding the diagnosis and classification of vascular anomalies (VA). As torso and extremities are the second most common body part for presentation of VAs, musculoskeletal (MSK) radiologists play a central role in VA classification. The purpose of this study was to evaluate the awareness and clinical use of the International Society for the Study of Vascular Anomalies (ISSVA) classification by MSK radiologists. MATERIALS AND METHODS: A Web-based survey was designed and electronically sent to Society of Skeletal Radiology (SSR) members, with 3 questions on demographics and 7 questions on ISSVA classification use and knowledge. The Z-test for binomial proportions is used to assess for statistical significance between subgroups. RESULTS: The response rate was 12% (130 of 1091), comprised of 64% (83 of 130) academic and 36% (47 of 130) nonacademic MSK radiologists. VAs accounted for only a small (0%-5%) proportion of clinical practice in the majority 92% (119 of 130). Only 17% (22 of 130) of MSK radiologists used the ISSVA classification in practice. Of all respondents, 74% (94 of 127) considered hemangioma a type of vascular malformation (VM). There was no significant difference in the response characteristics between academic and nonacademic radiologists. A greater proportion of MSK radiologists with >5 years' experience provided responses that were discordant with the ISSVA classification compared with less experienced radiologists (0-5 years) in diagnosing hemangiomas in adult patients (P = 0.02), and using the presence of phleboliths to diagnose hemangiomas (P = 0.004). CONCLUSION: Our survey results indicate a lack of familiarity with the ISSVA classification by the MSK radiology community.

Assessment of Congenital Vascular and Organ Anomalies in Subjects With Thalidomide Embryopathy Using Non-Contrast Magnetic Resonance Angiography.

BACKGROUND: To determine the type and frequency of vascular and organ malformations in adults with thalidomide embryopathy (TE) using non-contrast magnetic resonance angiography (MRA) and to assess the effect of the observed malformations on renal function. Methods and Results: The institutional ethics committee approved this prospective study and written informed consent was given by all 78 subjects (50 females) with TE (mean age: 55±1.1 years), who were examined by non-contrast MRA at 3T. ECG-triggered balanced turbo field echo images of the chest, abdomen and pelvis were obtained in coronal and sagittal orientations. Two observers assessed the frequency of vascular and organ malformations. Serum creatinine and estimated glomerular filtration rate (eGFR) were obtained to assess renal function. In 58 subjects, 99 vascular anomalies were observed, including 68 arterial (69%) and 31 venous anomalies (31%); 15 patients had 16 abdominal organ malformations including 12 kidney anomalies and 4 cases of gallbladder agenesis. Most vascular anomalies affected the renal vessels (n=66, 67%) or supraaortic arteries (n=28, 28%). Serum creatinine and eGFR revealed normal renal function in all subjects. CONCLUSIONS: Vascular and organ anomalies occurred in a high number of subjects with TE without evidence of renal dysfunction. Information about the presence of malformations may be important for future surgical interventions in subjects with TE.

[Quality standards for ultrasonographic assessment of peripheral vascular malformations and vascular tumors. Report of the french society for vascular medicine. 2018 Update]. / Standards de qualité pour la pratique de l'examen écho-Doppler dans l'étude des malformations et tumeurs vasculaires. Rapport de la Société française de médecine vasculaire (SFMV) : actualisation 2018.

The quality standards of the French Society of Vascular Medicine for the ultrasonographic assessment of vascular malformations are based on the two following requirements: (1) technical know-how: mastering the use of ultrasound devices and the method of examination; (2) medical know-how: ability to adapt the methods and scope of the examination to its clinical indication and purpose, and to rationally analyze and interpret its results. AIMS OF THE QUALITY STANDARDS: To describe an optimal method of examination in relation to the clinical question and hypothesis. To homogenize practice, methods, glossary, and reporting. To provide good practice reference points, and promote a quality process. ITEMS OF THE QUALITY STANDARDS: The 3 levels of examination; their clinical indications and goals. The reference standard examination (level 2), its variants according to clinical needs. The minimal content of the examination report; the letter to the referring physician (synthesis, conclusion and proposal for further investigation and/or therapeutic management). Commented glossary (anatomy, hemodynamics, semiology). Technical bases. Setting and use of ultrasound devices. Here, we discuss ultrasonography methods of using of ultrasonography for the assessment of peripheral vascular malformations and tumors (limbs, face, trunk).

Assessment of tetralogy of Fallot-associated congenital extracardiac vascular anomalies in pediatric patients using low-dose dual-source computed tomography.

BACKGROUND: To investigate the diagnostic value of dual-source computed tomography (DSCT) in the evaluation of tetralogy of Fallot (TOF)-associated extracardiac vascular abnormalities in pediatric patients compared with transthoracic echocardiography (TTE). METHODS: One hundred and twenty-three pediatric patients diagnosed with TOF were included in this retrospective study. All patients underwent DSCT and TTE preoperatively. All associated extracardiac vascular abnormalities and their percentages were recorded. The diagnostic performances of DSCT and TTE were compared based on the surgical results. The image quality of DSCT was rated, and the effective radiation dose (ED) was calculated. RESULTS: A total of 159 associated extracardiac vascular deformities were confirmed by surgery. Patent ductus arteriosus (36, 22.64%), right-sided aortic arch (29, 18.24%), and pulmonary valve stenosis (23, 14.47%) were the most common associated extracardiac vascular abnormalities. DSCT was superior to TTE in demonstrating associated extracardiac anomalies (diagnostic accuracy: 99.13% vs. 97.39%; sensitivity: 92.45% vs. 77.07%; specificity: 99.81% vs. 99.42%). The agreement on grading the image quality of DSCT was excellent (κ = 0.80), and the mean score of the image quality was 3.39 ± 0.50. The mean ED of DSCT was 0.86 ± 0.47 mSv. CONCLUSIONS: Compared to TTE, low-dose DSCT has high diagnostic accuracy in the depiction of associated extracardiac vascular anomalies in pediatric patients with TOF, and could provide more morphological details for surgeons.

Imaging assessment of portal venous system: pictorial essay of normal anatomy, anatomic variants and congenital anomalies.

The purpose of this pictorial essay is to describe anatomic variants and congenital anomalies of portal venous system and related liver parenchymal alterations. The imaging findings of some of these entities have been previously described in other articles, however this work encompasses all congenital anomalies of portal venous system with attention to their features on various imaging modalities; in particular we illustrated with detailed pictures all the main portal vein variants, congenital extra- and intra-hepatic porto-systemic venous shunts and portal vein aneurysm. Variants of portal branches and intrahepatic portosystemic shunts are quite uncommon, however, when present, they should be recognized before performing surgery or interventional procedures. Congenital absence of the portal vein is an important finding as the complete loss of portal perfusion predisposes the liver to focal or diffuse hyperplastic or dysplastic changes. Portal vein aneurysm is a rare clinical entity that can affect intra- and extra-hepatic portal branches; although usually asymptomatic, thrombosis can occur. Awareness of congenital variants of portal venous system among radiologists should allow a more confident diagnosis and permit an accurate planning of surgical procedures and percutaneous interventions; identification of portal system anomalies also suggest an accurate evaluation of associated hepatic parenchymal anomalies such as nodular regenerative hyperplasia, focal nodular hyperplasia (FNH), and adenomas with high risk of malignant transformation.

Accessory (multiple) renal arteries - Differences in frequency according to population, visualizing techniques and stage of morphological development.

The aim of this paper is to comprehensively analyze the literature focused on frequency of the presence of the accessory renal arteries in the human body. A systematic analysis of papers has been made. With regard to ethnicity, the incidence of accessory renal arteries fluctuates from 4% in a Malaysian population to 61.5% in a Brazilian population. The frequency is lowest in eastern and southern Asia (from 4% to 18.4%). In some, not ethnically homogenous populations, wide span of occurrence of accessory renal arteries is described (e.g. American - averaging from 18% to 28.8%). A higher frequency of accessory renal arteries was observed in fetuses compared to adults. Moreover, differences in the presence and number of accessory renal arteries reported in different papers are a consequence of type of visualizing technique used in research - especially when computed tomography and anatomical dissection were compared. The increasing number of surgical interventions, especially where laparoscopic methods are concerned, underlines the importance of such knowledge especially to surgeons, interventional radiologists, nephrologists, and vascular surgeons.

[Quality standards for ultrasonographic assessment of peripheral vascular malformations and vascular tumors. Report of the French Society for Vascular Medicine]. / Standards de qualité pour la pratique de l'examen écho-doppler dans l'étude des malformations et tumeurs vasculaires. Rapport de la Société française de médecine vasculaire (SFMV).

THE QUALITY STANDARDS OF THE FRENCH SOCIETY OF VASCULAR MEDICINE FOR THE ULTRASONOGRAPHIC ASSESSMENT OF VASCULAR MALFORMATIONS ARE BASED ON THE TWO FOLLOWING REQUIREMENTS: Technical know-how: mastering the use of ultrasound devices and the method of examination. Medical know-how: ability to adapt the methods and scope of the examination to its clinical indication and purpose, and to rationally analyze and interpret its results. AIMS OF THE QUALITY STANDARDS: To describe an optimal method of examination in relation to the clinical question and hypothesis. To achieve consistent practice, methods, glossary, and reporting. To provide good practice reference points, and promote a high-quality process. ITEMS OF THE QUALITY STANDARDS: The three levels of examination; their clinical indications and goals. The reference standard examination (level 2), its variants according to clinical needs. The minimal content of the examination report; the letter to the referring physician (synthesis, conclusion and proposal for further investigation and/or therapeutic management). Commented glossary (anatomy, hemodynamics, semiology). Technical bases. Settings and use of ultrasound devices. Here, we discuss the methods of using ultrasonography for the assessment of peripheral vascular malformations and tumors.

[Angiographic assessment of the incidence rate of atypical variants of the development of crural and plantar arteries].

OBJECTIVE: assessing the incidence rate of atypical anatomical variants of the development of the popliteal artery and plantar arteries according to the findings of digital subtraction angiography. MATERIAL: Retrospective analysis of angiograms of 2,456 lower limbs in 1,305 patients. The character of the arterial lesion and the quality of the angiograms allowed of evaluating the anatomy of the popliteal artery and plantar arteries on a total of 1,609 extremities (65%). RESULTS: The incidence of atypical ramification of the popliteal artery amounted to 9%. Altered blood supply of the foot was observed in 7.6% of cases. The total incidence rate of atypical anatomical variants in men and women did not differ significantly. In the presence of a typical anatomical variant on one extremity, the prevalence of atypical blood supply of the other limb amounted to 16.3%. In the presence of an atypical anatomical variant on one limb, the incidence of atypical blood supply of the other amounted to 42%. In case of atypical blood supply of the foot on one limb, the incidence rate of the same-type abnormality on the other equalled 48%. CONCLUSION: In a typical variant of blood supply of one extremity, the probability of typical blood supply of the other amounted to 83 .7%, in an atypical variant decreasing to 58%. In case of atypical blood supply of one foot, one should suppose an analogous anatomical variant on the other foot in half of the cases.