Development of hemodynamically relevant acquired arterio-venous fistulae in patients with venous malformations.

BACKGROUND: Venous malformations tend to retain their slow-flow behavior, even in progressive disease or regression following therapy. OBJECTIVE: The aim of this study is to analyze the development of acquired hemodynamic relevant arterio-venous fistulae in patients with slow-flow malformations. METHODS: This study is a retrospective analysis based on a consecutive local registry at a tertiary care Interdisciplinary Center for Vascular Anomalies. Patients with venous malformations and development of secondary arterio-venous fistulae were included. Indications for therapy of the vascular malformation were based on patients' symptoms and complications. The following endpoints were of clinical interest and were assessed: origin of development of arteriovenous fistula, development of secondary comorbidities as a result of the vascular malformation. For analysis we focused on descriptive statistics. RESULTS: Out of 1213 consecutive patients with vascular malformations, in 6 patients perfusion changed from slow flow to arterio-venous fast-flow patterns. Four patients developed the fistula after local trauma in the area of the malformation, the other 2 patients developed the fistula due to progression of the disease and recurrent thrombophlebitis. These 2 patients had no trauma or interventions at the time of arterio-venous fistula development. CONCLUSIONS: Acquired arterio-venous fast-flow fistula in patients with slow flow vascular malformation is very rare and might be a result of local trauma or the progression of the disease with recurrent thrombophlebitis. Specific evidence-based treatment options for these patients do not exist.

[The marginal vein-Still a rare entity: case series of 16 patients]. / Die Marginalvene ­ nach wie vor eine seltene Entität: Fallserie von 16 Patienten.

BACKGROUND: The marginal vein (MV) is a congenital, predominantly venous vascular malformation, which is based on a lack of regression of the embryonic venous system in the lower extremities and is associated with a variety of complications. So far, no uniform treatment regimens have been described in the literature. OBJECTIVE: What are the treatment strategies and outcomes in patients with a MV? MATERIAL AND METHODS: In the period from 1 January 2008 to 31 December 2020, all patients treated at the University Hospital Augsburg with MV were retrospectively reviewed. RESULTS: The median age at the time of diagnosis was 14.8 years (3-42 years). Out of 16 patients 12 had a leg length difference, 75% of patients (12/16) already had chronic venous insufficiency (CVI) at the time of diagnosis of MV. Open surgical removal of MV was performed as first-line treatment in 31.3% (5/16) patients. The MV was primarily closed by endovenous laser therapy (EVLT) in 1/16 patients, 15/16 patients were treated with redo procedures and 2.6 ± 2.4 (mean±SD) redo procedures were performed per patient in follow-up. The mean follow-up was 8.1 years. DISCUSSION: In order to prevent/avoid progression of CVI and thrombosis prophylaxis, the MV should be closed/removed promptly after diagnosis. The use of conventional surgical techniques for the removal of MV seems to have an advantage over treatment with minimally invasive procedures in terms of the number of secondary interventions required.

Vascular malformations of the female and male genitalia: type and distribution patterns revealed by magnetic resonance imaging.

BACKGROUND: Vascular malformations of the genitalia often go undetected in clinical examination. These vascular malformations can cause a variety of clinical symptoms such as swelling, pain and bleeding. AIM: To characterize the distribution patterns of genital vascular malformations using magnetic resonance imaging (MRI) and to correlate these patterns with clinical findings in order to guide diagnostic decisions. METHODS: A retrospective analysis of MRIs of the pelvis and legs in 370 patients with vascular malformation was performed to determine the involvement of the internal and external genitalia. RESULTS: In 71 patients (19%), genital involvement could be identified by MRI. Of these, 11.3% (8 of 71) presented with internal involvement, 36.6% (26 of 71) with external involvement and 52.1% (37 of 71) with both internal and external involvement. Over half (57.1%) of the 49 patients with visible external genital signs detected during a clinical examination had additional internal genital involvement. CONCLUSIONS: Genital involvement is a common finding in patients with vascular malformation of the legs and/or pelvis. Based on our data, we recommend MRI of the legs and pelvic region in patients with externally visible signs of a vascular malformation of the external genitalia in order to exclude additional internal involvement.

Super selective percutaneous transhepatic coil embolization of intrahepatic pseudoaneurysm after pediatric liver transplantation: a case report.

BACKGROUND: Intrahepatic arterial pseudoaneurysms are a rare, life-threatening complication after pediatric liver transplantation. Treatment of choice represents interventional radiological management with endovascular embolization of the segmental artery proximal and distal to the aneurysm. However, this technique results in loss of arterial perfusion distal to the aneurysm with subsegment arterial ischemia. CASE PRESENTATION: We report a case of a 1-year-old girl with a pseudoaneurysm in the split-liver graft. Direct percutaneous, transhepatic access to the pseudoaneurysm was performed followed by super selective coil application into the aneurysm. CONCLUSION: Super selective percutaneous, transhepatic coil application is feasible even in pediatric patients after liver transplantation and results in preservation of the entire course of the liver artery.

Contrast-enhanced ultrasound with perfusion analysis in patients with venous malformations before and after percutaneous treatment with ethanol-gel.

INTRODUCTION: Percutaneous sclerotherapy is a well-established treatment option for venous malformations (VM). A recently established sclerosing agent is ethanol-gel. Aim of this study was to identify, if contrast-enhanced ultrasound (CEUS) with an integrated perfusion analysis allows for differentiation between untreated VM, healthy tissue, and with gelified ethanol treated malformation tissue. MATERIAL AND METHODS: In this institutional review board approved prospective study symptomatic VM patients underwent CEUS at exactly the same position before and after sclerotherapy with ethanol-gel. Two experienced sonographers performed all examinations after the bolus injection of microbubbles using a multi-frequency probe with 6 -9 MHz of a high-end ultrasound machine. An integrated perfusion analysis was applied in the center of the VM and in healthy, surrounding tissue. For both regions peak enhancement (peak), time to peak (TTP), area under the curve (AUC), and mean transit time (MTT) were evaluated. Wilcoxon signed rank test was executed; p-values <0.05 were regarded statistically significant. RESULTS: In 23 patients including children (mean age 25.3 years, 19 females) before treatment all identified parameters were significantly higher in the VM center compared to healthy tissue (peak: p < 0.01; TTP: p < 0.01; AUC: p < 0.01; MTT: p < 0.01). Comparing the VM center before and after treatment, TTP (p < 0.02) and MTT (p < 0.01) reduced significantly after sclerotherapy. In surrounding tissue only peak changed after treatment in comparison to pre-treatment results (p = 0.04). Comparing data in the VM center with surrounding tissue after sclerotherapy, results still differed significantly for peak (p < 0.01), TTP (p < 0.01), and AUC (p < 0.01), but assimilated for MTT (p = 0.07). CONCLUSION: All with CEUS identified parameters seem to be excellent tools for differentiating between VM and healthy tissue. TTP and MTT could distinguish between with ethanol-gel sclerotized VM portions and untreated malformation parts and thereby might assist the monitoring of sclerotherapy with ethanol-gel.

Kidney Failure After Occlusion of Accessory Renal Arteries in Endovascular Abdominal Aneurysm Repair.

PURPOSE: To evaluate the incidence of acute renal failure and chronic kidney disease due to occlusion of accessory renal arteries during endovascular aneurysm repair of infrarenal abdominal aortic aneurysm. MATERIAL AND METHODS: We retrospectively reviewed the course of 181 patients (mean age, 71, SD ± 9  years) who underwent EVAR of infrarenal abdominal aortic aneurysm. The renal vessel anatomy was analyzed in all pre- and postoperative CT scans. Diameter and origin of accessory renal arteries were evaluated. Renal function was determined by pre- and postoperative serum creatinine and eGFR levels. Long-term follow-up (>3 months) of patients was available in 121 cases (66.9%). Acute kidney injury and chronic kidney failure were defined according to guidelines of "Kidney Disease: Improving Global Outcomes" (KDIGO). RESULTS: In 65 of 181 patients (33.9%), 82 accessory renal arteries were identified preoperatively. In 19 of 181 patients (10.5%), one or more accessory renal arteries were covered and subsequently occluded by the implanted stent-graft device. Neither acute kidney injury (10.3% vs 12.5%; p = .785) nor chronic kidney disease (10.7% vs 15.38%; p = .452) was detected significantly more often in patients with covered accessory renal artery. The only significant predictor of acute kidney injury was the preoperative serum creatinine level (1.12 mg/dl vs. 0.98 mg/dl; p = .03). Significant predictors for chronic kidney disease were preoperative serum creatinine, eGFR, and impaired renal function (p < .001). CONCLUSION: Coverage of accessory renal artery due to stent-graft does not lead either to temporary acute kidney injury after endovascular aneurysm repair or to chronic kidney disease. LEVEL OF EVIDENCE: Level II b.

[Diagnosis and management of vascular malformations : Interdisciplinary teamwork in demand]. / Diagnostik und Management von Gefäßmalformationen : Interdisziplinäres Teamwork gefragt.

Vascular anomalies are very rare, but can occur in children and adults in almost every region of the body. Due to the complexity of this disease, the path to a definitive diagnosis is often difficult. It requires interdisciplinary teamwork with close exchange of information between the individual treatment partners to reach the correct diagnosis and then to start the best therapy. This article provides an overview of the main types of vascular malformations from a clinical, imaging, and histological point of view, following the current classification of the International Society for the Study of Vascular Anomalies (ISSVA).

Radiofrequency Ablation of the Marginal Venous System in Patients with Venous Malformations.

PURPOSE: To evaluate radiofrequency ablation (RFA) for closure of marginal veins in pediatric and adult patients with venous malformations. MATERIALS AND METHODS: Medical records, imaging and procedure details were retrospectively reviewed in patients who underwent RFA of the marginal vein in a 17-month period. Additional sclerotherapy (n = 19) and coil embolization of the marginal vein were performed (n = 2). RESULTS: A total of 23 marginal veins were treated in 20 patients. Mean age at treatment was 16 years ± 9.4 (1-37 years). Pre-procedural magnetic resonance imaging revealed thoracoabdominal marginal veins in 3 patients. A type I marginal vein (draining in the great saphenous vein below the popliteal vein) was identified in 1 and type IIa/IIb (draining in a median/lateral accessory saphenous vein) in 2/8 cases. Type III (draining into the profunda femoral vein) was detected in 8, and type IV (draining into gluteal veins) in 1. Mean diameter of the marginal veins was 13.2 mm ± 4 (7-20 mm). Patency was found in 1 during follow-up (22 months ± 9.8). Complete or partial occlusion was achieved in 94.5% of the veins. One patient showed signs of thrombophlebitis after the procedure, and another incomplete paresis of the peroneal nerve. CONCLUSION: RFA is effective as minimally invasive treatment of the marginal venous system. These veins should be treated early in life. Marginal veins with large diameter, residual tributaries and the intrafascial courses usually require adjunct coil embolization and sclerotherapy. LEVEL OF EVIDENCE: Case series, Level IV.

Abdominal lymphatic malformations.

BACKGROUND: Abdominal lymphatic malformations (LM) are relatively rare findings in the differential diagnosis of focal abdominal lesions; however, they represent a challenge especially in younger patients. The aim of this review article is to provide up-to-date information about the different kinds of LM manifestations. In addition, related syndromes and typical imaging features to facilitate the diagnosis are discussed. RESULTS: The clinical presentation of abdominal LM is unspecific, whereby most are asymptomatic and comprise incidental findings of thin-walled cystic masses anywhere in the abdomen. The fluid in the cystic masses may be proteinaceous, contain blood, or be infected. Radiological imaging features overlap with other cystic diseases; hallmark in LM is a lack of a solid component and exclusive enhancement of the walls and septa. CONCLUSION: In cystic abdominal masses in early childhood or young adults, abdominal LM must be taken into account by the radiologist. Newly defined entities in this spectrum of diseases are central conducting lymphatic anomaly (CCLA) and generalized lymphatic anomaly (GLA).

Evaluation of dynamic effects of therapy-induced changes in microcirculation after percutaneous treatment of vascular malformations using contrast-enhanced ultrasound (CEUS) and time intensity curve (TIC) analyses.

The aim of this follow-up study was to demonstrate the effect of percutaneous interventional treatment on local microcirculation of peripheral vascular malformations using CEUS and TIC analysis. MATERIAL AND METHODS: Retrospective analysis of 197 patients (136 female; 61 male; 3-86 years) with 135 venous (VM), 39 arterio-venous (AVM), 8 lymphatic and 15 veno-lymphatic peripheral vascular malformations before and after the first percutaneous treatment.CEUS was performed after i.v. injection of 1-2.4 ml of sulfur hexafluoride microbubbles (SonoVue®) using a 6-9 MHz linear probe. Digitally stored cine loops (starting in the early arterial phase for 60 sec) were read by independent readers in consensus. Regions of interest (ROI) were defined in the center and at the margins of the malformation, as well as in the healthy surrounding tissue. TIC analyses with Time to Peak (TTP) and Area under the Curve (AUC) were calculated using integrated perfusion software. RESULTS: After the treatment there was a significant decrease for median AUC in VM in the center from 297.8 (14.5-2167.6) rU down to 243.3 (0.1-1678.8) rU (p = 0.043) and in the surrounding tissue down to 107.7 (20.2-660.2) rU (p = 0.018). For the other malformations AUC decreased in the center and the margins as well. TTP rose, however these changes did not reach the level of significance. CONCLUSION: Analyzing the capillary microcirculation TICs offer a possibility of monitoring therapy-induced capillary changes of vascular malformations.

[Abdominal lymphatic malformations. German version]. / Abdominelle lymphatische Malformationen.

BACKGROUND: Abdominal lymphatic malformations (LM) are relatively rare findings in the differential diagnosis of focal abdominal lesions; however, they represent a challenge especially in younger patients. The aim of this review article is to provide up-to-date information about the different kinds of LM manifestations. In addition, related syndromes and typical imaging features to facilitate the diagnosis are discussed. RESULTS: The clinical presentation of abdominal LM is unspecific, whereby most are asymptomatic and comprise incidental findings of thin-walled cystic masses anywhere in the abdomen. The fluid in the cystic masses may be proteinaceous, contain blood, or be infected. Radiological imaging features overlap with other cystic diseases; hallmark in LM is a lack of a solid component and exclusive enhancement of the walls and septae. CONCLUSION: In cystic abdominal masses in early childhood or young adults, abdominal LM must be taken into account by the radiologist. Newly defined entities in this spectrum of diseases are central conducting lymphatic anomaly (CCLA) and generalized lymphatic anomaly (GLA).

Value of different ultrasound elastography techniques in patients with venous malformations prior to and after sclerotherapy.

AIM: Comparison of different ultrasound elastography techniques for detection of changes after sclerotherapy within venous malformations. MATERIAL AND METHODS: In patients with venous malformations sonography was executed at exactly the same position prior to and after ethanol-gel sclerotherapy. Both examinations included B-Mode, vascular sonography with Color-Coded Duplex Sonography, and additional sonography with different elastography techniques (strain, qualitative and quantitative Acoustic Radiation Force Impulse (ARFI) elastography) with a linear transducer (6-9 MHz). Qualitative elastograms were read in consensus and scored. Differences of elasticity scores were statistically analyzed, p-values <0.05 were regarded significant. RESULTS: Elasticity scores of strain and qualitative ARFI elastography in 25 patients (21 females, averagely 24.4 years old) were comparable before treatment (p = 0.69). After therapy qualitative ARFI scores changed significantly compared to pre-treatment scores (p = 0.0017), whereas strain elastography scores revealed no significant changes (p = 0.13). Quantitative ARFI values obtained after sclerotherapy within the venous malformations were significantly higher compared to pre-treatment values (p = 0.049), and significantly higher to values obtained in surrounding tissue (p = 0.030). Comparison of pre- and post-treatment ARFI values of the surrounding tissue was not significant (p = 0.67). CONCLUSION: Elasticity scores of qualitative ARFI elastography reliably detect ethanol-gel induced changes in venous malformations. Quantitative ARFI may be a tool for therapy planning, and for monitoring sclerotherapy outcome as well as the effect of sclerosing agents on malformation and surrounding tissue in patients with venous malformations.

The Influence of Preoperative Aneurysmal Thrombus Quantity and Distribution on the Development of Type II Endoleaks with Aneurysm Sac Enlargement After EVAR of AAA.

PURPOSE: To determine the influence of preoperative aneurysmal thrombus quantity and distribution on the development of type II endoleak with aneurysm sac enlargement after endovascular aneurysm repair (EVAR). MATERIALS AND METHODS: We retrospectively analyzed the pre- and postoperatively performed CT scans of 118 patients who had follow-up imaging for at least 1 year after EVAR available. We assessed preoperative thrombus perimeter (T Peri), diameter (T Dia), cross-sectional area (T CSA), and volume (T Vol). The preoperative thrombus distribution was classified into no thrombus, semilunar-shaped (anterior, right side, left side, posterior) thrombus, and circumferential type thrombus. The number of preoperative patent aortic side branches (ASB) was identified. Endpoint was type II endoleak with aneurysm volume (A Vol) increase of ≥5 % during follow-up. RESULTS: During follow-up (2 years, range 1-9 years), 17 patients with type II endoleak had significant A Vol increase. Less preoperative T Peri, T Dia, T CSA, and T Vol were associated with A Vol increase. A circumferential thrombus distribution significantly protected against aneurysm enlargement (p = 0.028). The variables with the strongest significance for A Vol increase were preoperative T Vol/A Vol ratio (OR 0.95; p = 0.037) and number of patent ASB (OR 3.52; p < 0.001). CONCLUSION: A low preoperative T Vol/A Vol ratio and a high number of patent ASB were associated with aneurysm sac enlargement after EVAR.

Perfusion quantification of vascular malformations using contrast-enhanced ultrasound (CEUS) with time intensity curve analysis before and after treatment: First results.

AIM: Aim of this pilot-study was to quantify perfusion changes of vascular malformations before and after the first interventional treatment using contrast-enhanced ultrasound (CEUS). MATERIAL AND METHODS: 29 patients (10 male, 19 female) between 6 and 63 years (mean 28.1 years) with 12 arterio-venous (AVM) and 17 venous malformations (VM) were examined before and after their first percutaneous interventional treatment. CEUS was performed with a 2.4 ml bolus injection of sulfur-hexafluorid microbubbles, and a 6-9 MHz mulitfrequency transducer. A 60 sec cine sequence was recorded and regions of interest (10 mm×30 mm) were defined in the centre, and the margins of the vascular malformation as well as in the surrounding healthy tissue. Time Intensity Curves (TIC) were analysed, and Time to Peak (TTP) as well as Area under the Curve (AUC) were calculated. RESULTS: For VM there was a significant perfusion difference (p < 0.05) in AUC between centre and the surrounding tissue before (323.1 vs. 130.4 rU) and after treatment (331.0 vs. 106.9 rU). There was no significant difference for TTP in ROIs of VM (19.1 vs. 26.5 sec). After the treatment there was a significant decrease in AUC for all three regions in AVMs, and an increase in TTP for AVM. However TTP for AVM in the centre ROI still remained shorter than in the surrounding tissue even after therapy (20.9 vs. 25.4 sec). CONCLUSION: CEUS with TIC analysis is a promising imaging method for the evaluation of perfusion before and after percutaneous treatment of vascular malformations. AUC decrease indicates therapy-induced changes in perfusion of VM whereas an increase in TTP shows therapy-related changes in AVM.

Percutaneous treatment of symptomatic superior mesenteric vein stenosis using self-expanding nitinol stents.

PURPOSE: To evaluate the technical and clinical success of percutaneous superior mesenteric vein (SMV) stenting in symptomatic patients using self-expanding nitinol stents. METHODS: We retrospectively analyzed the technical and clinical success of percutaneous SMV stenting of 6 symptomatic patients (3 men, mean age 67 years, range 48-81 years). Stenosis of the SMV was caused by postoperative stricture (n=3), pancreas carcinoma (n=1) and pancreatitis (n=2). As a result of the stenosis, 3 patients had symptomatic ascites, 2 patients showed signs of mesenteric ischemia and 1 patient had recurrent gastrointestinal bleeding. Stenting was performed by a percutaneous transhepatic approach using self-expanding nitinol stents. RESULTS: Stenting of the SMV was technically and clinically successful in all patients. No peri-interventional complications occurred. The stent diameters ranged from 6 to 14 mm. During the mean follow-up of 6 months (range, 2-10 months) 1 patient presented early stent occlusion 2 weeks after placement. CONCLUSION: Stenting of a symptomatic SMV stenosis using self-expanding nitinol stents is feasible and clinically effective.

Color-coded perfusion analysis of CEUS for pre-interventional diagnosis of microvascularisation in cases of vascular malformations.

AIM: Aim of our pilot study was the application of a contrast-enhanced color-coded ultrasound perfusion analysis in patients with vascular malformations to quantify microcirculatory alterations. MATERIAL AND METHODS: 28 patients (16 female, 12 male, mean age 24.9 years) with high flow (n = 6) or slow-flow (n = 22) malformations were analyzed before intervention. An experienced examiner performed a color-coded Doppler sonography (CCDS) and a Power Doppler as well as a contrast-enhanced ultrasound after intravenous bolus injection of 1 - 2.4 ml of a second-generation ultrasound contrast medium (SonoVue®, Bracco, Milan). The contrast-enhanced examination was documented as a cine sequence over 60 s. The quantitative analysis based on color-coded contrast-enhanced ultrasound (CEUS) images included percentage peak enhancement (%peak), time to peak (TTP), area under the curve (AUC), and mean transit time (MTT). RESULTS: No side effects occurred after intravenous contrast injection. The mean %peak in arteriovenous malformations was almost twice as high as in slow-flow-malformations. The area under the curve was 4 times higher in arteriovenous malformations compared to the mean value of other malformations. The mean transit time was 1.4 times higher in high-flow-malformations compared to slow-flow-malformations. There was no difference regarding the time to peak between the different malformation types. The comparison between all vascular malformation and surrounding tissue showed statistically significant differences for all analyzed data (%peak, TTP, AUC, MTT; p < 0.01). High-flow and slow-flow vascular malformations had statistically significant differences in %peak (p < 0.01), AUC analysis (p < 0.01), and MTT (p < 0.05). CONCLUSION: Color-coded perfusion analysis of CEUS seems to be a promising technique for the dynamic assessment of microvasculature in vascular malformations.