Peripheral Vascular Malformations Research Articles

A Comprehensive Review of Sonographic Assessment of Peripheral Slow-Flow Vascular Malformations.

This comprehensive review explores the role of sonographic assessment in diagnosing and characterizing peripheral slow-flow vascular malformations (PSFVM). The review begins with an introduction providing the background and significance of PSFVM, defining these vascular anomalies, and emphasizing the importance of sonography in their diagnosis. The objectives focus on a thorough examination of existing literature, assessing the effectiveness of sonography in delineating morphological and hemodynamic features crucial for accurate classification. The summary of key findings highlights the diagnostic accuracy of sonography while acknowledging its limitations. Implications for clinical practice emphasize the practical utility of sonography in early diagnosis and preoperative planning, suggesting integration into multimodal approaches. The conclusion underscores the need for standardized criteria, ongoing education, and future research, positioning sonography as a valuable tool in the comprehensive management of PSFVM.

Deep learning-based differentiation of peripheral high-flow and low-flow vascular malformations in T2-weighted short tau inversion recovery MRI.

Differentiation of high-flow from low-flow vascular malformations (VMs) is crucial for therapeutic management of this orphan disease. A convolutional neural network (CNN) was evaluated for differentiation of peripheral vascular malformations (VMs) on T2-weighted short tau inversion recovery (STIR) MRI. 527 MRIs (386 low-flow and 141 high-flow VMs) were randomly divided into training, validation and test set for this single-center study. 1) Results of the CNN's diagnostic performance were compared with that of two expert and four junior radiologists. 2) The influence of CNN's prediction on the radiologists' performance and diagnostic certainty was evaluated. 3) Junior radiologists' performance after self-training was compared with that of the CNN. Compared with the expert radiologists the CNN achieved similar accuracy (92% vs. 97%, p = 0.11), sensitivity (80% vs. 93%, p = 0.16) and specificity (97% vs. 100%, p = 0.50). In comparison to the junior radiologists, the CNN had a higher specificity and accuracy (97% vs. 80%, p < 0.001; 92% vs. 77%, p < 0.001). CNN assistance had no significant influence on their diagnostic performance and certainty. After self-training, the junior radiologists' specificity and accuracy improved and were comparable to that of the CNN. Diagnostic performance of the CNN for differentiating high-flow from low-flow VM was comparable to that of expert radiologists. CNN did not significantly improve the simulated daily practice of junior radiologists, self-training was more effective.

Incidence of Peripheral Vascular Malformations of Limbs in SMS Hospital Jaipur

Peripheral vascular malformations (PVMs) include a wide spectrum of diseases that can produce potentially life- or limb-threatening complications. The International Society for the Study of Vascular Anomalies (ISSVA) Classification System categorized vascular anomalies into two basic types, the vaso proliferative or vascular neoplasms and developmental vascular abnormalities.&#x0D; Method: A retrospective, cross-sectional study was conducted for the period of 2 year. All patients under surgery OPD with a Peripheral limb vascular anomaly excluding upper limbs and lower limbs were taken into the study and investigated and followed up for at least 6 months.&#x0D; Result: In this study, out of 38 patients 26 patients (12 (44%) males and 14 (56%) females) were diagnosed withvascular malformation of upper and lower limbs. It is observed that low flow lesions are more common in both upper and lower limbs. Male patients with these lesions tend to present much later in life than female patients.&#x0D; Conclusion: AVMs show variance in site and type based on gender. Male patients were more likely present with high-flow AVMs in lower limbs whereas female patients typically presented with low-flow AVMs on the upper extremities.&#x0D; Keywords: Peripheral limb vascular malformations, varicose veins, AVM.

Clinical phenotype of adolescent and adult patients with extracranial vascular malformation

ObjectiveIn recent years, genotypic characterization of congenital vascular malformations (CVMs) has gained attention; however, the spectrum of clinical phenotype remains difficult to attribute to a genetic cause and is rarely described in the adult population. The aim of this study is to describe a consecutive series of adolescent and adult patients in a tertiary center, where a multimodal phenotypic approach was used for diagnosis. MethodsWe analyzed clinical findings, imaging, and laboratory results at initial presentation, and set a diagnosis according to the International Society for the Study of Vascular Anomalies (ISSVA) classification for all consecutively registered patients older than 14 years of age who were referred to the Center for Vascular Malformations at the University Hospital of Bern between 2008 and 2021. ResultsA total of 457 patients were included for analysis (mean age, 35 years; females, 56%). Simple CVMs were the most common (n = 361; 79%), followed by CVMs associated with other anomalies (n = 70; 15%), and combined CVMs (n = 26; 6%). Venous malformations (n = 238) were the most common CVMs overall (52%), and the most common simple CVMs (66%). Pain was the most frequently reported symptom in all patients (simple, combined, and vascular malformation with other anomalies). Pain intensity was more pronounced in simple venous and arteriovenous malformations. Clinical problems were related to the type of CVM diagnosed, with bleeding and skin ulceration in arteriovenous malformations, localized intravascular coagulopathy in venous malformations, and infectious complications in lymphatic malformations. Limb length difference occurred more often in patients with CVMs associated with other anomalies as compared with simple or combined CVM (22.9 vs 2.3%; P < .001). Soft tissue overgrowth was seen in one-quarter of all patients independent of the ISSVA group. ConclusionsIn our adult and adolescent population with peripheral vascular malformations, simple venous malformations predominated, with pain as the most common clinical symptom. In one-quarter of cases, patients with vascular malformations presented with associated anomalies on tissue growth. The differentiation of clinical presentation with or without accompanying growth abnormalities need to be added to the ISSVA classification. Phenotypic characterization considering vascular and non-vascular features remains the cornerstone of diagnosis in adult as well as pediatric patients.

Prospective Observational Study of Pain Severity and Pain Interference Outcomes Following Percutaneous MRI-guided Laser Ablation or Cryoablation for Focal Painful Peripheral, Soft Tissue Vascular Malformations: 12-month Outcomes

Background: Percutaneous image-guided thermal ablation has emerged as a treatment option for vascular malformations (VM). However, prospective data on intermediate to long-term effectiveness with uniform follow-up are lacking. Purpose: To prospectively evaluate the 12-month pain severity and pain interference outcomes following percutaneous MRI-guided laser ablation and cryoablation of focal painful, peripheral soft tissue VM. Materials and Methods: Participants undergoing clinically indicated MRI-guided laser ablation and cryoablation for focal painful (worst pain ≥4 out of 10 on Visual Analog Scale), peripheral soft tissue vascular malformations (VM) were enrolled in an IRB-approved prospective, observational study after giving informed consent. Participants completed the Brief Pain Inventory questionnaire prior to ablation and 1, 3, 6, and 12-month postablation. Differences in pain severity and interference outcomes from baseline on a 0 to 10 scale were compared using a paired t-test. Results: Seventeen participants (15 female; mean age 28.3 ± 9.3 years) underwent 24 MRI-guided and monitored ablation sessions including laser (n = 21), cryoablation (n = 2), or both (n = 1) for treatment of painful slow-flow (n = 16) or high-flow (n = 1) VM. Median maximal VM diameter was 5.7 cm (range 1.1 to 31.0 cm). Mean (±SD) preablation worst pain score was 7.9 ± 1.4. There was a significant decrease in worst pain at 1-month postablation (–3.5 ± 2.9; P = .0007) that was sustained at 3 (–3.4 ± 3.2; P = .0007), 6 (–4.2 ± 3.6; P = .0003), and 12 months (–3.6 ± 3.9; P = .002), with similar results for average, current, and least pain scores. There was a significant improvement in pain interference outcomes at 12-month postablation: general activity (P = .018), walking ability (P = .008), work (P = .003), sleep (P = .007), and enjoyment of life (P = .033). Conclusions: These data suggest that MRI-guided and monitored thermal ablation is safe and provides early and sustained significant improvements in pain severity and pain interference outcomes during the first year of follow-up in participants with focal painful peripheral soft tissue VM.

Embolization in Pediatric Patients: A Comprehensive Review of Indications, Procedures, and Clinical Outcomes.

Embolization in pediatric patients encompasses a large spectrum of indications, ranging from the elective treatment of congenital diseases of the cardiovascular system to the urgent management of acute hemorrhagic conditions. In particular, the endovascular treatment of central and peripheral vascular malformations and hypervascular tumors represents a wide chapter for both congenital and acquired situations. Thanks to the progressive availability of low-profile endovascular devices and new embolic materials, the mini-invasive approach has gradually overtaken surgery. In this review, the main embolization procedures will be illustrated and discussed, with a focus on clinical indications and expected outcomes. The most recent mini-invasive techniques will be described, with hints on the cutting-edge devices and embolic materials.

Appearance-related concerns and their impact on health-related quality of life in patients with peripheral vascular malformations.

Peripheral vascular malformations (VMs) may lead to disfigurement of the body and face, potentially impairing aesthetic appearance. Yet, data on appearance in this population is limited. This study aimed to examine appearance-related concerns and their impact on health-related quality of life (HR-QoL) in patients with VMs. In this cross-sectional study, 384 adults and 194 children with VMs were invited to complete the Outcome Measures for VAscular MAlformations (OVAMA) questionnaire to evaluate potential appearance-related concerns on a five-point verbal-rating scale, with higher scores indicating more appearance-related concerns (e.g., colour-difference, facial-distortion, reduced self-esteem, and dissatisfaction with appearance). HR-QoL was evaluated using Patient-Reported Outcome Measurement Information System (PROMIS). Subgroups of patients reporting more appearance-related concerns were identified using univariate analysis. Associations between appearance-related concerns and various HR-QoL domains (e.g., anxiety and social participation) were assessed. A total of 184 patients (32%) completed the questionnaires; 121 patients (66%) reported that one or more appearance outcome was severely affected (i.e., 4-5 out of 5). The following factors statistically significant associated with more appearance-related concerns: capillary/combined origin, facial localization, subcutaneous tissue involvement, larger size, overgrowth, and diagnosis of a syndrome. In adults, dissatisfaction with appearance and reduced self-esteem due to the appearance of the VM correlated with more anxiety and depression symptoms. Reduced self-esteem correlated with less social participation. In children, bodily distortion and being stared at were correlated with less peer relationships. Severe appearance-related concerns were present in two-thirds of patients with VMs, impairing their mental HR-QoL. Clinicians should acknowledge appearance-related aspects, monitor psychological well-being, and offer intervention aimed at improving satisfaction with appearance.

Long-term changes of health-related quality of life in patients with peripheral vascular malformations – a prospective observational study

The aim of this observational study was to assess health-related quality of life (HRQOL) changes in patients with vascular malformations, over a period of almost eight years, and to assess clinical and demographic characteristics possibly associated with HRQOL changes. Eighty out of 111 patients who were included in a previously published comparative HRQOL study accepted inclusion in this follow-up study. HRQOL at baseline and follow-up was assessed with the Short-Form 36-item questionnaire (SF-36). Median observation time was 7.9 years. Linear mixed models and linear regression models were applied to assess HRQOL change and possible associations with demographic and clinical variables. The median age of the patient cohort at baseline evaluation (n=111) was 27.0 years. Ninety-six out of 111 (86.5%) patients were diagnosed with venous malformations. Significantly higher SF-36 scores at follow-up were found for the physical domains Role limitations due to (RLDT) physical problems (difference=13.5; 95% CI [1.6, 25.3]) and Bodily pain (difference=11.3; 95% CI [3.8, 18.8]). No deterioration of HRQOL was found in any domain. In multivariate analyses, female gender, muscle/bone involvement, and higher age were associated with a positive relative change in SF-36 in the domains Physical functioning, RLDT physical problems, and RLDT emotional problems, respectively. Invasive treatment was not associated with long-term HRQOL change. Over a period of almost eight years, significant improvement of SF-36 scores was observed in the physical domains RLDT physical problems and bodily pain. Female gender, muscle/bone involvement, and higher age were associated with HRQOL improvement in certain domains.

A study of duplex ultrasound examination in peripheral vascular malformations

Vascular malformations belong to wide spectrum of disease involving all vascular segments- arteries, veins as well as lymphatics. Being Ultrasound as the primary diagnostic modality, all peripheral vascular malformation diagnosis can be made by clinical examination and duplex ultrasound examination.A study of 50 patients was carried over a period of 1 year from 1 May 2021 to 1 May 2022 on the patient who came with complain of slow growing, superficial swelling which is painless in nature. The machine used is Affinity Philips 70 G.With help of duplex ultrasound, various types of vascular malformations were diagnosed with incidence such as Slow Flow Venous malformation (60%- 30 patients), High flow Arterio-venous malformations (16%- 8 patients), Capillary malformations (12%- 6 patients) and Lymphatic malformations (12%- 6 patients).Vascular malformations have been classified into venous, arterial, capillary, combined and lymphatic malformations types. Besides age presentations, detailed history and clinical examination, ultra-sound imaging plays an important role in correct diagnosis and classification of vascular malformations. It assesses exact flow dynamics in order to differentiate between Slow flow v/s High flow vascular malformations. MRI is most valuable diagnostic tool for comprehensive assessment of vascular malformationsUltrasound are first imaging modality in peripheral vascular malformations. It has plays crucial role in proper treatment planning of the lesion as it describes the extent, type, flow pattern within the lesion.

Long-Term Outcome of Single-Session, Ultrasound-Guided, Radiofrequency Ablation for Symptomatic Small, Lower Limb, Venous Malformations.

Objectives:To analyze the long-term clinical outcome of percutaneous, ultrasound-guided radiofrequency ablation (RFA) of peripheral low-flow vascular malformations (VM).Materials & Methods:Adolescent patients presenting with symptomatic VMs and referred for percutaneous management using RFA between January 2010 and January 2015 were identified by a search in the institutional interventional radiology database. Clinical and radiological follow-up up to April 2021 was based on retrospective analysis of patients’ electronic medical records and imaging files. This retrospective study was approved by the institutional ethics committee.Results:Four female patients (median age 16 years) presented with lower extremity pain, swelling and functional disability related to VM as confirmed by magnetic resonance imaging. Two patients underwent percutaneous sclerotherapy previously. Clinical follow-up (mean of 8,5 years) showed complete and sustained resolution of the symptoms in all patients; in one patient a persistent decrease in muscular strength of the treated limb was noted.Conclusion:Percutaneous, ultrasound-guided RFA is relatively safe and efficient with durable symptom relief in the management of small, lower limb VMs in adolescent patients.

MANAGEMENT OF PERIPHERAL VASCULAR MALFORMATIONS BY ABSOLUTE ALCOHOL

Objective: According to ISSVA 2014 classification, peripheral vascular malformations are divided to fast and slow flow. In spite of clear diagnosis, management is still challenging because of low rate of getting rid of lesions and high risk of complications. Endovascular management is first choice in daily practice, but cured rate is maximum about 30%. Combined treatment increases rate of success to get rid of malformations, especially surgical remvoving after endovascular management. There are many embolized materials, but absolute alcohol seem to be effective, but highly complications. Study and method: A prospective intervened study without case control. 89 patients satisfied standard are collected from 06/2016 to 06/2020. We evaluated clinical response, imaging improvement and effectiness of absolute alcoholic management. Results: Of 89 patients, female is majority with 62.9%, older than 18 is about 42.9%, mean age is 23.1. Sessions, method of approaching, volume of alcohol, combined treatment are significantly remarkable different between fast and slow flow malformations (p value = 0.021). Treatment are seem to be more effective in slow flow (p = 0.023),but fast flow malformations are curable in clinical and imaging studies. Major complications is indifferent between two groups. Conclusion: Venous malformations are majority amongst vascular malformations in our study. Because of complex scenario, difficulty of management in fast flow lesions, follow up is prolonged, volume of alcohol is increased, therapeutic sessions is repeated many times compared to slow flow. Successful treatment is higher in slow flow malformations than fast flow, but latter one is able to be cured. There are no difference between two group in field of complications. However, glycemia is negative correlation with minor complications, natremia is also reverse correlation to postoperational pain. Pain scale, symptom’s onset and APTT are positive correlations with complications. Key words: fast flow malformations, slow flow malformations, absolut alcohol, major complications, minor complications.

Clinical characteristics associated with pain in patients with peripheral vascular malformations

ObjectiveVascular malformations (VM) can negatively impact the patient's quality of life (QoL). Pain is a common problem in these patients. The aim of this study was to investigate risk factors associated with pain and to assess how pain affects QoL. MethodsThis prospective cross-sectional study was conducted in a tertiary vascular anomaly expertise center. Between June and December 2020, all patients from our local database (334 adults and 189 children) with peripheral VMs were invited to complete the Outcome Measures for VAscular MAlformations questionnaire to evaluate the presence, frequency, and intensity of pain. Additionally, patients were asked to complete several Patient-Reported Outcome Measurement Information System scales to evaluate their QoL. Risk factors associated with pain were identified in bivariate analysis and multivariable logistic regression. QoL domains were compared between patients who experienced pain and patients who did not. ResultsA total of 164 patients completed the questionnaire about pain and 133 patients completed all QoL questionnaires. Approximately one-half of the patients (52%) reported pain in the past four weeks and 57% of these patients reported pain daily or several times a week. Female sex (P = .009), lesions located in the upper extremity (P < .001) or lower extremity (P < .001), and intramuscular/intraosseous lesions (P = .004) were independently associated with the presence of pain. The following QoL domains were diminished in patients who experienced pain in comparison with patients who did not: pain interference (P < .001), physical functioning (P < .001), and social participation (P < .001) in adults, and pain interference (P = .001), mobility (P = .001), and anxiety (P = .024) in children. ConclusionsPain is a frequently reported complaint in patients with VMs and is present in approximately one-half of the patients. Patients with lesions located in the upper or lower extremity, intramuscular/intraosseous lesions, and female patients are more likely to experience pain. The presence of pain negatively impacted patients' QoL. Although VM are a benign condition and expectative management is frequently applied, our study shows that pain is a serious concern and needs to be actively assessed. Pain is a sign of various etiologies and should be examined to properly treat the pain.

Getting to the core of peripheral vascular malformations: measuring what matters.

Linked Article: Lokhorst et al. Br J Dermatol 2021; 185:797–803.

Discussion: Surgical Treatment of Peripheral Vascular Malformations: A Systematic Review and Meta-Analysis.

Discussion: Surgical Treatment of Peripheral Vascular Malformations: A Systematic Review and Meta-Analysis.

Surgical Treatment of Peripheral Vascular Malformations: A Systematic Review and Meta-Analysis.

Surgical treatment of peripheral vascular malformations is widely performed as primary and secondary treatments. Excellent results have been reported; however, it is thought that complications are likely to occur because of damage to adjacent structures. This systematic review aimed to elucidate the indications and outcomes of surgical treatment of vascular malformations. PubMed, EMBASE, and the Cochrane Central Register of Controlled Trials were searched for studies reporting outcomes of surgery in at least 15 patients with a single type of peripheral soft-tissue vascular malformation. The authors extracted data on patient and lesion characteristics, treatment characteristics, and outcomes (including complications). Meta-analysis was conducted on recurrence and complication rates. A total of 3042 articles were found, of which 24 were included: nine studies on arteriovenous malformations, seven on venous malformations, and eight on lymphatic malformations, totaling 980 patients. Meta-analyses showed pooled proportions for recurrences of 11 percent in arteriovenous malformations, 5 percent in venous malformations, and 9 percent in lymphatic malformations. Pooled proportions of major complications were 9 percent for arteriovenous malformations, 3 percent for venous malformations, and 1 percent for lymphatic malformations. The authors found a 5 percent pooled recurrence proportion in total resections, compared with 28 percent in subtotal resections. The pooled odds ratio for recurrence in total and subtotal resections showed a significant lower recurrence rate after total resection (odds ratio, 0.14, p = 0.02). Surgical treatment of vascular malformations appears to be effective and safe in many cases. However, it seems that surgery is performed predominantly in small lesions, and subtotal resection has a higher risk of recurrence than total resection.

Responsiveness of quality of life measures in children with peripheral vascular malformations: The OVAMA project

SummaryBackgroundThe OVAMA (Outcome Measures for VAscular MAlformations) project determined quality of life (QoL) as a core outcome domain for evaluating treatment effect in vascular malformations. To correctly evaluate treatment effect on QoL, patient-reported outcome measures (PROMs) are needed that are responsive to changes. In children with vascular malformations, we explored if two widely used PROMs were responsive to changes: the Pediatric Quality of Life Inventory (PedsQL) and the Children's Dermatology Life Quality Index (CDLQI).MethodsIn an international multicenter prospective study, conservatively and invasively treated children completed the PedsQL and CDLQI at baseline and after follow-up of 6–8 weeks. At follow-up, change in health was assessed by a global rating of change (GRC) scale. Responsiveness was assessed by testing hypotheses on expected correlation strength between change scores of the PROMs and the GRC scale, and by calculating the area under the receiver operating characteristics curve (AUC). The PROMs were considered responsive if ≥75% of the hypotheses were confirmed or if the AUC was ≥0.7.ResultsTwenty-nine children were recruited in three centers in the Netherlands and United States, of which 25 completed all baseline and follow-up measurements. For both the PedsQL and CDLQI, less than 75% of the hypotheses were confirmed and the AUC was <0.7.DiscussionThe results suggest that these PROMs are not sufficiently responsive for evaluating treatment effect in peripheral vascular malformations. Our study emphasizes the need for assessing responsiveness before using a PROM in evaluating treatment effect.

Patient radiation exposure from embolo-sclerotherapy of peripheral vascular malformations

ObjectiveEmbolo-sclerotherapy (EST) is the mainstay therapy for peripheral vascular malformations that involves the exposure of patients to ionizing radiation. We analyzed the radiation exposure to patients from EST of peripheral vascular malformations during a 5-year period in a single specialist center. MethodsAll patients who had undergone EST at a single specialist center for peripheral vascular malformations from January 1, 2013 to January 8, 2018 were identified from a prospectively collected database. Data collection included basic demographics, procedure date, anatomic site, type of vascular malformations, and procedural details. Radiation exposure, measured as the dose–area product (DAP) and fluoroscopy time, of all patients who had undergone EST during the study period were retrospectively reviewed. Statistical analysis was performed using the Mann-Whitney U and Kruskal-Wallis tests for comparison between subgroups. P < .05 was considered statistically significant. ResultsA total of 237 patients (median age, 30 years; range, 1-73 years) had undergone 419 EST sessions during the study period. Of the 237 patients, 61 (25.7%) had had arteriovenous malformations (AVMs) and had undergone 140 EST sessions (33.4%) and 176 (74.3%) had had venous and lymphatic malformations and had undergone 279 EST sessions (66.6%). Patients with AVMs had undergone a median of 2 procedures (range, 1-13) compared with a median of 1 (range, 1-6) for venous and lymphatic malformations within the study period. The median DAP for the single and cumulative EST for peripheral vascular malformations was 1.26 Gycm2 (range, 0.00-698.36 Gycm2) and 1.91 Gycm2 (range, 0.00-1300.24 Gycm2), respectively. The median fluoroscopy time for single and cumulative EST was 19 seconds (range, 1-3846 seconds) and 30 seconds (range, 1-5843 seconds), respectively. Significantly greater patient radiation exposure, in DAP and fluoroscopy time, was measured for single and cumulative EST for AVMs compared with venous and lymphatic malformations (P < .01 for both; Mann-Whitney U test). A significant difference in DAP but not fluoroscopy time was found when the anatomic areas of vascular malformations were compared. ConclusionsPatient radiation exposure for EST for peripheral vascular malformations, measured in DAP and fluoroscopy time, appeared to be generally less than that reported for endovascular arterial and deep venous interventions. However, some patients with peripheral vascular malformations received relatively high radiation doses. Further studies to investigate the risk factors and long-term side effects of radiation exposure in these patients and strategies to reduce these are required.

3T MRI of Peripheral Vascular Malformations: Characteristics andComparison of Two Fat-Saturated sequences: Short Tau Inversion Recovery Versus Three-Dimensional High-Resolution Volume Interpolated Gradient Recalled Echo.

To assess morphological and hemodynamic characteristics of peripheral vascular malformations on 3 T magnetic resonance imaging (MRI) including qualitative comparison of two fat-saturated sequences: short tau inversion recovery (STIR) and three-dimensional high-resolution volume interpolated gradient recalled echo (GRE). During 9 months, 100 patients with suspected or known vascular malformations were prospectively assessed on a 3 T scanner using T2-weighted STIR and turbo spin echo (TSE), T1-weighted TSE, time-resolved contrast-enhanced magnetic resonance angiography (MRA) with interleaved stochastic trajectories (TWIST) and T1-weighted volume interpolated breath-hold examination (VIBE) after contrast enhancement. The analysis included signal behavior and morphologic and hemodynamic characteristics. Additionally, the image quality of the fat-saturated sequences was evaluated by 2radiologists. 86 patients (14 dropouts; 57 female, 29 male; mean age 26.8 years, age range 1-56) were analyzed. 22 had high-flow and 64 low-flow malformations, including 14 with a lymphatic component. In 21 of 22 patients with high-flow malformations, typical characteristics (flow voids, hyperdynamic arteriovenous fistula, dilated main/feeder-arteries and draining veins) were documented. Patients with low-flow malformations had phleboliths in 35 cases, fluid-fluid levels in 47and dilated draining veins in 23. Lymphatic malformations showed peripheral contrast enhancement of cyst walls in the volume interpolated GRE. The comparison of fat-saturated sequences showed significantly better results of the volume interpolated GRE in all categories except the presence of artifacts which were significantly reduced in the STIR (p < 0.05). 3 T MRI with MRA provides detailed morphological and hemodynamic information of different types of peripheral vascular malformations. Contrast-enhanced high-resolution volume interpolated GRE proved superior to STIR in differentiating morphologic features and to be diagnostic in the differentiation of lymphatic parts and joint involvement. · 3 T MRI with MRA offers detailed information about vascular malformations.. · Fat-saturated MRI provides especially information about morphological characteristics, extent and tissue involvement.. · Volume interpolated GRE proved superior in almost all categories compared to STIR.. · Volume interpolated GRE showed more artifacts.. · Volume interpolated GRE additionally allows differentiation of lymphatic parts and evaluation of joint involvement.. · Höhn F, Hammer S, Fellner C et al. 3T MRI of Peripheral Vascular Malformations: Characteristics and Comparison of Two Fat-Saturated sequences: Short Tau Inversion Recovery Versus Three-Dimensional High-Resolution Volume Interpolated Gradient Recalled Echo. Fortschr Röntgenstr 2021; 193: 446 - 458.

Detecting changes in quality‐of‐life over time in patients with peripheral vascular malformations: the OVAMA project

Vascular malformations are a group of rare congenital (present at birth) vessel anomalies. Mostly visible as a bumpy mass different in colour from normal skin, they can cause disfigurement of the face or body. Patients often experience pain, physical impairment, bleeding, and psycho‐social problems, leading to an overall poor quality of life. Thus, a main goal of treatment is improving their quality of life. Quality of life is measured with questionnaires, which are filled in by patients. To determine if a patient's quality of life improved or worsened after treatment, it is crucial that the questionnaire can detect changes over time. Never has a quality of life questionnaire been studied if it can detect changes in patients with vascular malformations. Consequently, it is impossible to evaluate if current treatments actually improve these patients’ quality of life. This study, from the Netherlands and U.S.A., aimed to find out if two widely used quality of life questionnaires, the ‘SF‐36’ and ‘Skindex‐29’, were able to detect changes in patients with vascular malformations. Sixty‐seven patients filled in the two questionnaires at two points in time. One group received treatment in between, and another group did not. When filling in the two questionnaires for the second time, patients were also asked if they experienced changes in aspects of quality of life. To deem the questionnaires as able to detect changes, a certain correlation was expected between the experienced change, and change in the scores of the questionnaires. For both questionnaires, the expected correlation was not found. The authors concluded that other questionnaires must be identified or developed to correctly detect changes in quality of life in patients with vascular malformations. This is a summary of the study: Responsiveness of quality‐of‐life measures in patients with peripheral vascular malformations: the OVAMA project

Responsiveness of quality-of-life measures in patients with peripheral vascular malformations: the OVAMA project.

SummaryBackgroundThe OVAMA (Outcome Measures for Vascular Malformations) project determined quality of life (QoL) as a core outcome domain for patients with vascular malformations. In order to measure how current therapeutic strategies alter QoL in these patients, a patient‐reported outcome measurement (PROM) responsive to changes in QoL is required.ObjectivesTo assess the responsiveness of two widely used generic QoL PROMs, the Medical Outcomes Study Short Form 36 (SF‐36) and Skindex‐29, in adult patients with vascular malformations.MethodsIn an international multicentre prospective study, treated and untreated patients completed the SF‐36 and Skindex‐29 at baseline and after a follow‐up period of 6–8 weeks. Global rating of change (GRC) scales assessing various QoL‐related outcome domains were additionally completed. Per subscale, responsiveness was assessed using two methods: by testing hypotheses on expected correlation strength between change scores of the questionnaires and the GRC scales, and by calculating the area under the receiver operating characteristics curve (AUC). The questionnaires were considered responsive if ≥ 75% of the hypotheses were confirmed or if the AUC was ≥ 0·7.ResultsEighty‐nine participants were recruited in three centres in the Netherlands and the U.S.A., of whom 67 completed all baseline and follow‐up questionnaires. For all subscales of the SF‐36 and Skindex‐29, < 75% of the hypotheses were confirmed and the AUC was < 0·7.ConclusionsOur findings suggest that the SF‐36 and Skindex‐29 seemed unresponsive to change in QoL. This suggests that alternative PROMs are needed to measure – and ultimately improve – QoL in patients with vascular malformations. What's already known about this topic? Quality of life is often impaired in patients with vascular malformations.Quality of life is considered a core outcome domain for evaluating treatment of vascular malformations.To measure the effect of treatment on quality of life, a patient‐reported outcome measure is required that is responsive to changes in quality of life. What does this study add? This is the first study assessing the responsiveness of quality‐of‐life measures in patients with vascular malformations.The results seem to indicate that the Medical Outcomes Study Short Form 36 (SF‐36) and Skindex‐29 are not responsive to changes in quality of life in patients with vascular malformations. What are the clinical implications of this work? Medical Outcomes Study Short Form 36 (SF‐36) and Skindex‐29 are not ideal to assess the effect on quality of life over time, of treatment strategies for peripheral vascular malformations.