Low-flow Vascular Malformations Research Articles

Role of sclerotherapy in management of low flow vascular malformations

Background: Low flow vascular malformations are most common in the head and neck region. Only symptomatic malformations require treatment. Sclerotherapy followed by surgery was considered the gold standard treatment but in the head and neck region, it may produce cosmetic and physiological defects. In the present study, multiple injections of sclerotherapy with 3% sodium tetradecyl sulphate was used for the treatment of low flow vascular malformations.Methods: Twenty cases of low flow vascular malformations of the oral cavity who presented in the outpatient department of ESIC Model Hospital, Ludhiana from 2014-2016 were selected for the study. Only significantly sized (>4 cm) and easily accessible lesions were included. Staged sequential sclerotherapy with 3% STS under strict fluoroscopy control was used as the sole treatment.Results: A total of 20 patients were taken of which, 25% required three sessions, 65% five to six sessions each and 10% required eight sessions each. All patients showed good results with complete regression and no mucosal ulceration.Conclusions: Staged sequential sclerotherapy with 3% STS should be the treatment of choice in low flow vascular lesions involving mucosal and cutaneous structures of head and neck region especially anterior two-thirds of tongue, palate, gingiva, buccal mucosa and lips. Surgical removal may affect critical neurovascular structures and cause cosmetic deformity. So the removal is advisable in life-threatening conditions, lesions requiring general anaesthesia and single sitting removal.

Low-flow vascular malformations of the hand and forearm: a multidisciplinary experience in a tertiary paediatric centre.

Vascular malformations (VMs) involving the hand and forearm in children provide management challenges due to complex anatomy, indispensable functionality and developmental implications. The institution's Vascular Registry was searched for patients with hand and arm VMs, supplemented by chart review of included patients. Twenty-one patients were identified, 52% male, with mean presenting age 5.2 years. Venous malformations predominated (71%), followed by lymphatic-venous (19%), lymphatic (5%) and glomuvenous (5%). Symptoms included pain (76%), swelling (71%), cosmetic concerns (81%), functional compromise (29%) and stiffness (5%). Imaging modality was ultrasound (100%), and magnetic resonance imaging (71%). Treatment included compression (62%), sclerotherapy (62%) and surgery (24%). Post-sclerotherapy ultrasounds showed complete sclerosis (25%), near complete sclerosis (58%) and partial sclerosis (17%). Post-surgery, patients reported improved cosmesis (80%), size (100%), pain (60%) and function (40%). Complications occurred in 24%, including bleeding, digital ischaemia and thrombosis. Mean follow-up was 3.4 years. Children with low-flow VMs of the hand and forearm experience significant symptoms and functional limitations. A multidisciplinary approach to management ensures optimal outcomes.

Factors influencing quality of life in children with low-flow vascular malformations: a qualitative study using focus groups.

Very few studies have evaluated the quality of life (QoL) of children suffering from low-flow vascular malformations. This is the first study investigating the influencing factors. To identify the factors influencing QoL in children with low-flow vascular malformations. We conducted a qualitative study employing focus group interviews (Clinical Trials Number: NCT03440827). The study was a prospective, interventional, non-comparative, multicentre study performed in four expert centres for vascular anomalies. Qualitative data about personal experiences, feelings, difficulties, needs and various factors influencing behaviours were collected. Theme-based content analysis (manual and specialist textural software guided) were used to analyse the verbatim transcripts of all focus group sessions. Manual qualitative discourse analysis was performed to identify the different themes and categories. Informatics' analyses were subsequently performed for each individual category. Ten focus groups (26 individuals including 10 children aged 11 to 15years) were conducted until saturation. Influencing factors were related to 4 categories: medical care, self-image, social impact on daily activities and challenging social relationships. These factors were responsible for intrafamily upheavals and may lead to future identity-building problems. This study provides an essential framework from which physicians can develop strategies to improve patient care and quality of life. These data may also be useful to develop specific age-sensitive QoL questionnaires.

Efficacy and Safety of Sclerosants in the Treatment of Venous Malformations: A Network Meta-Analysis of Randomized Controlled Trials

Background: Venous malformations (VMs) are the most common low-flow vascular malformations. Various sclerosants have been shown to be effective, but their relative effects are not well established. Methods We performed a Bayesian network meta-analysis (NMA) to assess the efficacy and adverse effects of different sclerosants for VMs. We searched PubMed, Embase, Cochrane Library, Scopus and Web of Science for randomized clinical trials up to April 1, 2021. We estimated odds ratios using pairwise and network meta-analysis with random effects. We also calculated the surface under the cumulative ranking curve for each treatment and carried out the cluster analysis. Findings: A total of 22 randomized clinical trials (1 805 patients) comparing 11 different therapeutic regimens were identified. Polidocanol foam and ethanol showed significant improvement in VMs compared with bleomycin and sodium morrhuate, while polidocanol foam and polidocanol combined with bleomycin were statistically significantly superior to ethanol and sodium morrhuate in safety. Bleomycin foam was ranked high as the most effective treatment, but the evidence stems from one small study and most comparative effect estimates were not statistically significant. Ethanol ranked second only to bleomycin foam in efficacy. SUCRA cluster analysis demonstrated that bleomycin, polidocanol, polidocanol foam, bleomycin combined with dexamethasone and polidocanol combined with bleomycin are the five treatments with higher SUCRA values for both outcomes compared with the second group of sodium morrhuate and sodium tetradecyl sulfate. Last cluster only included ethanol, characterized by the relatively good efficacy and obvious side effects. Interpretation: Polidocanol foam and ethanol are more effective than bleomycin and sodium morrhuate in VMs, while ethanol and sodium morrhuate are associated with severe adverse effects. Funding: None to declare. Declaration of Interest: None to declare.

Role of Foam Sclerotherapy with Injection Ethanolamine Oleate for the Treatment of Venous Malformation in Head-Neck Region

Background: Venous malformations (VMs) are a variety of low flow vascular malformations, which are developmental error of morphogenesis of veins where veins are dysplastic lined by quiescent or normal endothelium. Although surgical extirpation is the standard method for the treatment of vascular malformations, this procedure often leads to significant loss of motor function, nerve damage, or massive bleeding in patients which may endanger the life. Therefore, sclerotherapy has now been accepted as a less invasive alternative and good results have been obtained.
 Objectives: This study was conducted to evaluate the clinical outcomes after Foam sclerotherapy with injection Ethanolamine Oleate (EO) for the treatment of VMs in head-neck region.
 Methods: This quasi-experimental study was conducted in the Department of Otolaryngology- Head & Neck Surgery, Bangabandhu Sheikh Mujib Medical University (BSMMU). Forty-three patients with venous malformation in head neck region that had the inclusion criteria were enrolled as a study sample. The patients were diagnosed mostly clinically & confirmed by demonstrating non-pulsatile blood flow and venous space using Duplex ultrasound. The sclerosing solution 5% Ethanolamine Oleate (EO) was used in this study. Sclerofoam was produced using the Tessari method in 4:1 air to liquid ratio, the foam had been used within 60- 90 seconds. Results of the study were categorized as excellent, good, fair and poor. Ethical clearance was obtained from the Institutional Review Board (IRB) of BSMMU.
 Results: Among 43 patients 34(79.1%) patients underwent single session and 9(20.9%) were two sessions. All the lesions were responded to EO. Response to sclerotherapy categorized as excellent were in two third 29 (67.4%) patients and 14 (32.6%) had good response. No sessions resulted in poor responses. No complications occurred following any procedures. All of the sessions were performed as a day case basis without anesthesia.
 Conclusions: Foam sclerotherapy with injection EO appears to be safe and effective for the treatment of VMs in the head and neck region and should be considered when treating these complex lesions.
 Bangladesh J Otorhinolaryngol; October 2020; 26(2): 79-85

Bleomycin for orbital and peri-orbital veno-lymphatic malformations - A systematic review.

Orbital and peri-orbital venolymphatic malformations (VLM) are low flow vascular malformations. Intralesional bleomycin is now commonly being used to treat such malformations. The purpose of this systematic review is to synthesize evidence on the safety and efficacy of bleomycin/pingyangmycin sclerotherapy for the treatment of orbital and peri-orbital VLM. We searched Medline, Embase, Scopus and Cochrane database for studies reporting outcomes of bleomycin/pingyangmycin sclerotherapy for orbital and peri-orbital VLM between 1974 to April 5th, 2019. Nine retrospective cohort studies enrolling 132 patients were included. Two reviewers independently screened and extracted data and assessed the risk of bias. Predefined outcome measures were subjective and objective reduction of the lesion and associated complications. Subjective reduction of the lesions was seen in 96.2% of the studies. Objective reduction of the lesion and symptomatic improvement were reported in 91.6 and 95% of the studies respectively. Non responders were 9.0%. Minor adverse events were reported in 18.1% of the studies. Major complications like pulmonary toxicity or pulmonary fibrosis was not encountered in any of the included studies. Quality of evidence was generally low. Bleomycin/pingyangmycin sclerotherapy is very effective and relatively safe for the treatment of orbital and periorbital VLM and is not associated with any major side effects including pulmonary fibrosis.Limitations: The systematic review is limited mainly due to low quality of the included studies with retrospective design.

Lymphatic malformations

Lymphatic malformations are low-flow vascular malformations that arise due to errors in vascular development. Lymphatic malformations are benign and usually noted at birth or in the first few years of life. Lymphatic mass lesions are composed of varying size of cysts; this article focuses on discussion of cystic lymphatic malformations. Lymphatic malformations can occur throughout the body especially in lymphatic rich areas such as the cervical and axillary locations as well as the groin, trunk, retroperitoneum, extremities, abdominal or thoracic cavities. Treatment options vary based upon size of cysts and location. A multimodal and interdisciplinary approach is essential to care for patients with lymphatic malformations. Management options include observation, pharmacotherapy, sclerotherapy, and surgical procedures.

The Role of Interventional Radiologists in the Treatment of Congenital Lymphatic Malformations.

Lymphatic malformations are low-flow vascular malformations that are typically apparent in the pediatric population and can cause significant functional limitations and effects on quality of life. While surgical resection has historically been the mainstay of therapy, percutaneous sclerotherapy has garnered increasing popularity due to its efficacy and low complication rates. The role of interventional radiology in the multidisciplinary management of these often complex malformations requires thorough understanding of the disease process. This article will review the pathophysiology, clinical presentation, imaging workup, and management options of lymphatic malformations. Special attention will be devoted to available sclerosants, the mammalian target of rapamycin inhibitor sirolimus, and complex lymphatic anomalies.

Clinicoradiologic predictors of sclerotherapy response in low-flow vascular malformations

ObjectiveTo evaluate the clinical effectiveness of sclerotherapy agents in low-flow vascular malformations (LFVM) and identify clinical/imaging features to predict response. MethodsA retrospective analysis of hospital records of symptomatic LFVM patients who underwent phlebosclerotherapy from January 2015 to April 2018 was done. Patients were subdivided into venous malformations (VM) and lymphatic malformations (LM). Out of 246 cases, 223 patients (132 males, 91 females; age range, 2-52 years) had VM and 23 (13 males, 10 females; age range, 3 months to 45 years) had LM. The clinical response was graded as excellent (>60%), good (30%-60%), and poor (<30%). More than 30% was considered as acceptable response. The χ2 test was performed for correlation between clinical response and clinical, sonographic, magnetic resonance imaging, phlebographic parameters followed by multilinear regression. ResultsCavitary (43%) and spongy (37.7%) were the most common phlebographic patterns seen among VM and a cavitary pattern (87%) was most frequent in LM. Sodium tetradecyl sulphate and bleomycin were most commonly used sclerosants in VM and LM, respectively. The mean number of sessions was 4.35 (range, 1-23) in VM and 2.64 (range, 2-7) in LM. Among VM, 114 patients (51.1%) had excellent response to treatment (>60%) and 75.8% patients had an acceptable response (>30%). All patients with LM had an acceptable response (excellent response in 86.9%). Clinical disfigurement, discoloration, diffuse involvement, dysplastic venous morphology on phlebogram, and late and indirect draining vein correlated with poor response to sclerotherapy in VM (P = .003, P = .036, P = .007, P = .008, P = .003, and P = .035, respectively). Cystic components on ultrasound examination and direct draining vein were seen more often in excellent responders (P = .004 and P = .007) in addition to absence of disfigurement, discoloration, and diffuse involvement (P = .032, P = .003, and P = .002). Mod els comprising clinical disfigurement, dysplastic veins, and late draining vein had the greatest predictive value for poor response (R2 = 0.256). Also, the best model for predicting excellent response comprised presence of direct draining vein and absence of skin discoloration (R2 = 0.109). Eleven instances of minor complications occurred among a total of 1032 sessions, seven with sodium tetradecyl sulphate and four with polidocanol. ConclusionsAcceptable response to sclerotherapy was achieved in majority of LFVM with extremely low complication rates. Clinicoradiologic features, especially phlebographic findings, correlated with response to sclerotherapy.

Incidence of major complication following embolo-sclerotherapy for upper and lower extremity vascular malformations.

The current literature on the major complications of embolo-sclerotherapy of upper and lower extremity vascular malformations is scarce. Evaluating and understanding the rates and types of potential major complications of embolo-sclerotherapy of vascular malformations help treatment planning and informed consent. Therefore, this study reviewed major complications following embolo-sclerotherapy of all upper and lower extremity vascular malformations in a single specialized multidisciplinary vascular malformation center over a 5-year period. All patients with vascular malformations underwent multidisciplinary directed intervention. Demographic, procedural, follow-up, and complication data were collected prospectively in a dedicated database, and reviewed retrospectively. Major complications for upper and lower extremity vascular malformations from 1 January 2013 to 31 December 2017 were analyzed. All embolo-sclerotherapies of high-flow vascular malformations (HFVMs) were performed under selective catheter angiography and direct injection, but low-flow vascular malformations (LFVM) with direct injection only. Major complications were defined as any tissue or functional damage caused by direct injection, distal embolization, or tissue reaction. Seventy patients (median age of 25 years; 44 males and 26 females) had 150 embolo-sclerotherapy procedures for upper extremity vascular malformation. Of these, 28 patients had embolo-sclerotherapy for HFVM and 42 patients for LFVM; total 78 and 72 procedures, respectively. A total of 107 patients (median age of 26 years; 42 males and 65 females) had 160 embolo-sclerotherapy interventions for lower extremity vascular malformations. Of these, 18 patients had embolo-sclerotherapy for HFVM and 89 patients for LFVM; total of 30 and 130 procedures, respectively. The overall major complication rates following embolo-sclerotherapy of upper and lower extremity vascular malformations were 14.3% and 4.7%, respectively (P = 0.030). In the upper extremity HFVM group, major complications from embolo-sclerotherapy occurred in five patients; three ischemic fingers requiring amputation and two skin ulcerations. Meanwhile, in the upper extremity LFVM group, major complications occurred in five patients; one median nerve injury requiring nerve grafting and hand therapy, one hand contracture requiring tendon release, and three skin ulcerations. There was only one major complication, which was cellulitis in the lower extremity HFVM group. In the lower extremity LFVM group, major complications occurred in four patients; two skin ulcerations, one cellulitis, and one deep vein thrombosis. Embolo-sclerotherapy is relatively safe for upper and lower extremity vascular malformations in a high-volume experienced center where our major complication rates were 14.3% and 4.7%, respectively, which compare favorably or similar to those reported in most recent literature. These outcomes will direct treatment strategies to avoid local and systemic toxic complications in the upper and lower extremity, for both HFVM and LFVM, and to improve informed consent.

Utility of Intraprocedural Cross-sectional Imaging in the Treatment of Low-Flow Vascular Malformations

There is a high recurrence rate after sclerotherapy of low-flow vascular malformations. Conventional treatment uses fluoroscopy and ultrasound to assess the diffusion of the sclerosing agent during therapy. These modalities give a limited two-dimensional image. Employing intraprocedural cross-sectional imaging (ICSI) has the potential to give a three-dimensional assessment of the sclerosant’s diffusion. The purpose of this study was to evaluate the impact of ICSI on immediate outcomes of sclerotherapy of low-flow vascular malformations. A retrospective review was performed of all low-flow vascular malformation sclerotherapy procedures since the introduction of ICSI. Procedural metrics collected include type of ICSI used, quality of ICSI, assessment of sclerosing agent diffusion, percentage of lesion infusion, influence of ICSI on subsequent treatment planning, extravasation detection, and recurrence rate. There were 19 sclerotherapy procedures (11 venous malformations, 8 lymphatic malformations) performed in 16 patients using ICSI. All intraprocedural cross-sectional images were successfully acquired with fluoroscopy suite cone beam computed tomography, diagnostic computed tomography scanner, or diagnostic magnetic resonance imaging scanner (52.6% vs 42.1% vs 5.2%, respectively). Image quality was rated excellent or good for 78.9% of studies. ICSI improved assessment of therapeutic agent diffusion in 89.4% of cases compared with the other concurrent imaging modalities (ultrasound, fluoroscopy, and digital subtraction angiography, alone or in combination). ICSI demonstrated percentage of lesion infusion at 67.6% (95% confidence interval, 61%-73%). ICSI detected extravasation in 10.5% of cases. ICSI influenced management decisions in 52.6% of cases including needle repositioning, additional treatment access sites, and planning for future therapy sessions. In comparison to conventional imaging, ICSI provides additional information to aid in intraprocedural management of low-flow vascular malformations. ICSI may help optimize therapy in performing sclerotherapy of these lesions.

Safety and efficacy of foam sclerotherapy for treatment of low-flow vascular malformations in children

ObjectiveCongenital vascular malformations are a heterogeneous group of lesions with the potential to cause significant lifelong morbidity in children. Diagnosis and treatment of these lesions may be complex and require a multidisciplinary approach. Sclerotherapy is widely used for the treatment of low-flow vascular malformations (LFVMs) as an alternative to surgical resection in adults; however, limited data of its use in a pediatric setting are available. The purpose of this study was to evaluate the efficacy and safety of sclerotherapy for pediatric LFVMs. MethodsIn this retrospective study, we reviewed our multidisciplinary vascular malformations team database for all patients younger than 18 years treated for congenital vascular malformations from 2008 to 2017. Of these, patients with LFVM treated with foam sclerotherapy were included. Dynamic contrast-enhanced magnetic resonance imaging was used to select patients for sclerotherapy by the multidisciplinary team. Foam sclerotherapy was performed with either polidocanol or sodium tetradecyl sulfate. Patients' characteristics, including demographics, presenting symptoms, and anatomic location of malformation, were assessed. Outcomes included treatment response, number of procedures, and postprocedural complications. ResultsThe 61 patients with 61 LFVMs included 27 boys (44.3%) and 34 girls (55.7%), with mean age of 10.3 years (standard deviation, ± 5.3 years). The cohort included 32 venous (52.5%), 16 lymphatic (26.2%), and 8 mixed venous and lymphatic (13.1%) malformations along with 5 (8.2%) associated with Klippel-Trénaunay syndrome. Primary indications for intervention included pain and swelling (n = 12 [19.6%]), pain alone (n = 23 [37.7%]), swelling alone (n = 15 [24.6%]), functional impairment (n = 8 [13.1%]), and bleeding (n = 3 [4.9%]). Anatomic distributions varied, with 13 head and neck (21.3%), 5 truncal (8.2%), 10 upper extremity (16.4%), 27 lower extremity (44.3%), and 6 diffuse (9.8%). Among the head and neck lesions, 8 (13.1%) extended to the face; and of the extremity lesions, 5 (8.2%) extended to the hand and 17 (27.9%) to the foot. Overall, sclerotherapy resulted in significant improvement or complete resolution of symptoms in 53 patients (86.9%). Complications were observed in seven patients (11.4%); six cases (9.8%) of superficial skin ulceration resolved without intervention, and one infection (1.6%) required antibiotics. No patients experienced adverse hemodynamic consequences or venous thromboembolism. ConclusionsThis series of pediatric LFVMs, the largest of its kind to date, demonstrates that sclerotherapy with foam-based agents effectively reduces symptoms with an acceptable rate of complications. Further study is needed to determine the optimal sclerosing agents for individual subsets of LFVMs in the pediatric population.

Percutaneous MR-Guided Cryoablation of Low-Flow Vascular Malformation: Technical Feasibility, Safety and Clinical Efficacy.

To retrospectively assess the technical feasibility, safety and clinical efficacy of percutaneous MR-guided cryoablation of low-flow vascular malformations (LFVM). Between July 2013 and May 2019, 9 consecutive patients (5 male; 4 female; mean age 39.4±15.3years, range 15-68) underwent MR-guided cryoablation of LFVM. Patients were treated due to pain in all cases. Procedural data, complications and clinical results were analyzed. Technical success defined as complete coverage of the LFVM by the iceball without involvement of nearby non-target thermal-sensitive structures was achieved in 9/9 (100%) cases. Mean procedure time was 122±20min (range 90-150); 2-6 cryoprobes (mean 3.7±1.2) and 2-4 freezing cycles (mean freezing time 19.8±11.8min; range 4-40) were applied. No complications were noted. Mean time from the first treatment to the last follow-up was 548days (range 30-1776). Persistent/recurring pain was noted in 3/9 cases (33%) 30, 133 and 639days after cryoablation, respectively, and was related in all cases to MR-confirmed local residual/recurring disease. A second cryoablation treatment was performed in these 3 cases with complete pain control at the last available follow-up (153, 25, 91days, respectively). In the whole population, at mean 161 days (range 25-413) after the last treatment, on the numerical pain rate scale, pain significantly dropped from mean 6.4±2.1 (range 3-9/10) before CA to mean 0.3±0.9 (range 0-3/10) after (p = 0.009). Percutaneous MR-guided cryoablation is technically feasible, safe and effective for the treatment of symptomatic LFVM. Level 3b, retrospective cohort study.

The Influence of Physical Activity on Cavernous Malformation Hemorrhage

Background and Aim: Cavernous malformations are low-flow vascular malformations of the central nervous system. Brainstem location and prior hemorrhage increase future hemorrhage risk. We sought to determine the influence of physical activity on hemorrhage risk. Methods: Consecutive patients with radiologically confirmed cavernous malformations participated in a prospective registry with structured, baseline interviews, surveys, and examinations. Patients were asked about unusual events prior to initial clinical presentation. Medical records and MRIs were reviewed. Patients were surveyed about their physical activity after receiving their diagnosis. Annual follow-up surveys to patients ascertained new hemorrhages. Follow-up terminated at last follow-up, hemorrhage, surgery, or death. Univariate analysis assessed the risk of physical activities on prospective hemorrhage. Results: Of 195 patients, 117 (60%) were female. Median diagnosis age was 41 years. After diagnosis, 103 (52.8%) patients returned the physical activity survey (cerebral n = 100; spine n = 3). Over 539.4 patient years, 23 had a prospective hemorrhage. Five patients were removed from analysis because they had less than 6 weeks of physical-activity exposure before censor. The remaining 98 had no difference in prospective hemorrhage risk than those patients participating greater than or equal to 3 times monthly in walking, running, greater than 20-pound or less than 20-pound weight lifting, or noncontact sports. Few (n = 5) reported contact-sport participation, scuba diving (n = 2), or high-altitude climbing (n = 1) greater than 3 times monthly. Conclusions: Aerobic activity and noncontact sports do not increase hemorrhage risk in cerebral cavernous malformation; patients should not be restricted. Less is known about contact sports, high-altitude climbing, scuba diving, and those with spinal-cord cavernous malformation.

Abstract No. 696 Utility of intraprocedural cross-sectional imaging in the treatment of low-flow vascular malformations

Abstract No. 696 Utility of intraprocedural cross-sectional imaging in the treatment of low-flow vascular malformations

Abstract 126: The Influence of Physical Activity on Cavernous Malformation Hemorrhage

Background: Cavernous malformations (CM) are low flow vascular malformations of the central nervous system. Brainstem location and prior hemorrhage increase the risk for future hemorrhage. Little is known about the influence of physical activity on increasing the risk of hemorrhage. Methods: Consecutive patients with radiologically confirmed CM were recruited to participate in a prospective registry beginning in 2015. A structured interview, survey, and examination were performed at baseline. Patients were asked if anything unusual occurred in the days leading to initial clinical presentation. Medical records and magnetic resonance imaging (MRI) studies were reviewed. Patients were surveyed about their involvement in select physical activity after the diagnosis of CM was made. Follow up surveys were sent annually to patients to ascertain new clinical hemorrhages. Follow up was censored at last follow up, hemorrhage, surgery, or death. Univariate analysis was performed to assess whether involvement in select physical activities increased the risk of prospective hemorrhage. Results: Of 195 patients, 117 (60 %) were female and the average age at diagnosis was 41 years (31-56 years). One hundred and three (52.8%) patients returned the survey about physical activity after diagnosis (cerebral n=100; spine n=3). Twenty-three patients had a prospective hemorrhage over 539.4 patient years. Five of these patients were removed from the analysis because they had less than 6 weeks of physical activity exposure before censor. Of 98 remaining patients, there was no difference in prospective hemorrhage risk in those patients participating 3 or more times monthly in walking, running, lifting more than 20 pound weights, lifting less than 20 pound weights, or participating in non-contact sports. Very few patients reported participation in contact sports (n=5), scuba diving (n=2) or high altitude climbing (n=1) more than 3 times monthly. Conclusion: Aerobic activity and non-contact sports do not increase the risk of hemorrhage in patients with cerebral CM and patients should not be restricted. Less information is known about contact sports, high altitude climbing or scuba diving and those with spinal cord CM.

Management of a curious case of a large recurrent truncal vascular anomaly in a young adult

Venous malformations (VMs) as the name implies contain only venous blood which is slow moving and are hence classified under low-flow vascular malformations. Sclerotherapy is the main modality of treatment for small VMs. However, bigger lesions require surgical excision in addition to interventional procedures. Hereby, we present a successfully resected case of a large, diffuse, multiloculated, and multiplanar vascular anomaly of the trunk, which presented to us after incomplete resolution following multiple sittings of interventional therapy. Surgical excision was tricky and challenging because of its large size and complex anatomical involvement. Complete resolution of the lesion was achieved with no neurological deficit. There was full recovery of movements at the shoulder joint on follow-up.

Reduced Serious Complication Rates from Embolo-sclerotherapy of Lower Extremity High and Low Flow Vascular Malformations from a Specialist Single Centre Over 5 Years

Introduction - Therapeutic management of vascular malformations (VMs) often requires multiple interventions due to their recurrent nature. Embolosclerotherapy (EST) is the gold standard treatment for peripheral VMs including those of the lower extremities. Despite being minimally invasive, EST carries significant risk of serious complications when used for lower extremity VMs ischaemia, amputation, nerve injury, ulceration, thromboembolism and even death, with as high as 24% serious complication rates for foot treatment most recently reported (1). This study reviews 5-year serious complications following EST of all lower extremity VMs in our tertiary referral center for vascular anomalies. Methods - All VM patients underwent multidisciplinary review directed intervention, and demographic, procedural, follow-up and complication data collected prospectively in a dedicated database. Treatment outcomes for lower extremity VMs from 1 January 2013 to 31 December 2017 were analysed. All ESTs were performed under fluoroscopic guidance. All ESTs of high-flow vascular malformations (HFVM) were performed under selective catheter angiography and direct injection but low-flow vascular malformations (LFVM) with direct injection only. Serious complications were defined as any tissue or functional damage caused by direct injection, distal embolization or tissue reaction. Results - During the study period, 107 patients had a total of 168 interventional procedures; 160 (95.2%) ESTs, 4 (2.4%) surgical excisions, and 4 (2.4%) endothermal venous ablations and phlebectomies for lower extremity VMs. In this cohort of patients, the median (range) age was 26 (8-70) years; 42 males and 65 females. Of these, 18 patients (17%) had HFVM and 89 patients (83%) LFVM. These included 13 patients with Klippel-Trenaunay Syndrome (1 HFVM, 12 LFVM), one Mafucci’s Syndrome (HFVM) and one Milroy’s Disease (LFVM). Of the 160 ESTs, 86.25 (n=12 HFVM, n=72 LFVM) included the use of foam sclerotherapy, 8.12%(n=5HFVM and n=2 LFVM) alcohol, and 0.63% ( n=1 LFVM) coils. 5% of patients received a combination of sclerosants (e.g alcohol and coil, alcohol and STS). In total, 74.8% (n=80) of patients had clear clinical improvement and have been discharged since. Overall, 93.4% (100) of patients did not suffer from complications. 1.9% (n=2) of patients had non-serious complications characterized by excessive swelling. Meanwhile, 4.7% (n=5) of patients experienced serious complications:HFVM: 1 cellulitisLFVM: 2 skin ulcerations (Figure1), 1 cellulitis and 1 deep vein thrombosis (DVT) All the complications resolved with conservative and medical treatment without significant long-term physical and/or functional disability although the single DVT case required prolonged anticoagulant therapy. Conclusion - Currently, EST remains to be the interventional treatment of choice and is relatively safe and effective for lower extremity VMs. Our serious complication rate of 4.7% compares favorably to 11-24% in recent literature on peripheral VMs EST (2). This is possibly due to selective use of foam sclerotherapy versus alcohol, and improved classification and targeted treatment. Majority of the complications were due to local toxicity after direct injection. These outcomes will direct treatment strategies to avoid local toxic complications in the lower extremities for both HFVM and LFVM, and informing consent.

Transcriptome-wide Profiling of Cerebral Cavernous Malformations Patients Reveal Important Long noncoding RNA molecular signatures

Cerebral cavernous malformations (CCMs) are low-flow vascular malformations in the brain associated with recurrent hemorrhage and seizures. The current treatment of CCMs relies solely on surgical intervention. Henceforth, alternative non-invasive therapies are urgently needed to help prevent subsequent hemorrhagic episodes. Long non-coding RNAs (lncRNAs) belong to the class of non-coding RNAs and are known to regulate gene transcription and involved in chromatin remodeling via various mechanism. Despite accumulating evidence demonstrating the role of lncRNAs in cerebrovascular disorders, their identification in CCMs pathology remains unknown. The objective of the current study was to identify lncRNAs associated with CCMs pathogenesis using patient cohorts having 10 CCM patients and 4 controls from brain. Executing next generation sequencing, we performed whole transcriptome sequencing (RNA-seq) analysis and identified 1,967 lncRNAs and 4,928 protein coding genes (PCGs) to be differentially expressed in CCMs patients. Among these, we selected top 6 differentially expressed lncRNAs each having significant correlative expression with more than 100 differentially expressed PCGs. The differential expression status of the top lncRNAs, SMIM25 and LBX2-AS1 in CCMs was further confirmed by qRT-PCR analysis. Additionally, gene set enrichment analysis of correlated PCGs revealed critical pathways related to vascular signaling and important biological processes relevant to CCMs pathophysiology. Here, by transcriptome-wide approach we demonstrate that lncRNAs are prevalent in CCMs disease and are likely to play critical roles in regulating important signaling pathways involved in the disease progression. We believe, that detailed future investigations on this set of identified lncRNAs can provide useful insights into the biology and, ultimately, contribute in preventing this debilitating disease.

Percutaneous sclerotherapy with gelified ethanol of low-flow vascular malformations of the head and neck region: preliminary results.

We aimed to evaluate the safety and effectiveness of percutaneous sclerotherapy using gelified ethanol in patients with low-flow malformations (LFMs). A retrospective study was performed, analyzing treatment and outcome data of 6 patients that presented with 7 LFMs (3 lymphatic and 3 venous). Median diameter of LFMs was 6 cm (interquartile range [IQR], 4.5-8.5 cm). Data regarding pain, functional and/or cosmetic issues were assessed. Diagnosis was performed clinically and confirmed by Doppler ultrasound, while extension of disease was assessed by magnetic resonance imaging (MRI). Percutaneous puncture was performed with 23G needle directly or with ultrasound guidance. All the LFMs were treated with gelified ethanol injection. The median volume injected per treatment session was 4.4 mL. Technical and clinical success were obtained in all cases. No recurrences were recorded during a median follow up of 17 months (IQR, 12-19 months). Among the 6 patients, 5 had complete relief (83%) and one showed improvement of symptoms. The median VAS score was 7 (IQR, 6-7.5) before and 0 (IQR, 0-0) after treatment. All patients had functional and esthetic improvement (100%). Four patients (66.7%) revealed very good acceptance and two patients (33.3%) good acceptance. No major complications or systemic side effects were observed. Gelified ethanol percutaneous sclerotherapy was easy to handle, well-tolerated, safe and effective in the short-term follow-up. Longer follow-up of efficacy is mandatory for further conclusions.