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Venous malformation (VM) is a prevalent congenital vascular anomaly characterized by abnormal blood vessel growth, leading to disfigurement and dysfunction. Sclerotherapy, a minimally invasive approach, has become a primary therapeutic modality for VM, but its efficacy is hampered by the rapid dilution and potential adverse effects. In this study, we introduced a series of cationic amphiphilic molecules, fatty alcohol esters (TA6, TA8, and TA9) of tranexamic acid (TA), which self-assembled into low-molecular-weight gels (LMWGs) in water. The TA9, in particular, is released slowly when hydrogel is injected into the vein locally. Then, it damages the venous wall by destroying cell membranes and precipitating proteins, causing inflammation and thrombosis, thickening of the venous wall, effectively inducing irreversible vein fibrosis. Additionally, TA9 can be rapidly degraded into TA in plasma to reduce toxicity caused by diffusion. Overall, this study suggests that the cationic amphiphilic molecule TA9 is a promising sclerosant for VM treatment, offering a novel, effective, and safe therapeutic option with potential for clinical translation., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objective: To compare and analyze the efficacy and safety of different methods for injecting polidocanol in the treatment of venous malformations., Methods: The medical records of patients with venous malformations in our hospital from April 2021 to April 2023 were analyzed retrospectively, and they were divided into control group ( n = 38) and observation group ( n = 20) according to different treatment methods. Control group was injected with polidocanol under ultrasound guidance, while observation group was injected with polidocanol under digital subtraction angiography (DSA) monitoring. Therapeutic effects of observation group and control group after 3-6 months of treatment were compared and analyzed, and complications that can be used to reflect the safety of treatment in two groups were collected and recorded., Results: After treatment, the total effective rates of observation group and control group were 97.37% (37/38) and 75.00% (15/20) respectively, with significant differences (both p < .05). After 3-6 months of treatment, 13 complications occurred in observation group, while 16 occurred in control group. The number of complications in observation group was 6, with an incidence rate of 15.78%; while that in control group was 9, with an incidence rate of 45.00%, with a significant difference ( p < .05). There were no significant differences among other baseline data, age and complications between two groups (all p > .05)., Conclusion: Injection of polidocanol in patients with venous malformations under DSA monitoring was more effective and safer., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Venous malformations (VMs) are slow-flow vascular anomalies that pose significant health challenges. Bleomycin (BLM) is frequently used in Sclerotherapy for VMs, but its mechanism, particularly through pyroptosis, remains poorly understood. This study explores the role of BLM-induced endothelial cell pyroptosis in VMs sclerotherapy and its regulatory effects on fibrosis via the NLRP3/caspase-1/GSDMD pathway. Using a combination of TUNEL staining, Western blotting, and immunohistochemistry, we investigated the effects of BLM on VMs and endothelial cells in vitro. Pyroptosis and fibrosis were quantified, and the involvement of the NLRP3/caspase-1/GSDMD pathway was assessed. BLM treatment significantly increased pyroptosis and fibrosis in VMs tissues and cultured endothelial cells. Activation of the NLRP3/caspase-1/GSDMD pathway was crucial for these effects, which could be mitigated by pathway inhibition. BLM regulates fibrosis and induces pyroptosis through the NLRP3/caspase-1/GSDMD pathway in VMs. Understanding this mechanism could enhance the effectiveness and safety of Sclerotherapy in clinical settings., Competing Interests: Declarations Conflict of interest The authors have no conflicts of interest related to this article., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

Introduction: Vascular malformations (VMs) typically appear at birth and grow commensurately with patients. They can vary broadly in vessel type and tissue involvement, and upper extremity (UE) VMs can pose unique functional and aesthetic challenges in children. Given the advent of operative and nonoperative technologies like sclerotherapy and medications, a contemporary review of the surgical management of UE VMs is warranted., Methods: We performed a retrospective review of all patients who had surgical management of VMs from 2010 to 2021 at The Children's Hospital of Philadelphia. Demographics, lesion characteristics, treatment (including preceding nonsurgical therapies), complications, and final outcomes were recorded. Operative notes were reviewed for date of operation, depth of excision, type of closure, and current procedural terminology code., Results: Sixty-seven patients with 88 procedures were studied. Average patient age was 5.8 years, with 64% White and 67% male. Venous (34%) and lymphatic (19%) malformations were most common, and anatomic locations were most frequently on the hand (33%) and forearm (25%). The average lesion diameter was 4.2 cm, although this varied by location (eg, 2.9 cm, hand; 11.1 cm, chest wall). Fifty-eight patients (87%) underwent surgical excision as their index procedure, and 9 had sclerotherapy before surgery. Thirty-nine patients (60%) had subcutaneous excisions, and the remainder required subfascial or intramuscular excisions. Nearly all excisions were closed primarily (97%). Of the 53 patients with documented follow-up, 32 patients (60%) had complete resolution of their lesion as of their final visit. Thirty of these 32 patients with no clinical evidence of residual VM had only 1 surgery for excision., Conclusion: Upper extremity VMs were composed of diverse conditions with varying vessel types, size, depth, and anatomic sites. Surgical excision of VMs of the UE was safe and effective. A majority of VMs were fully excised after 1 procedure and frequently closed primarily with relatively low complication rates. Future work should investigate decision-making and outcomes of all treatment options of VMs of the UE for optimal functionality and aesthetics., Competing Interests: Conflicts of interest and source of funding: The authors have no conflicts of interest to declare. This research received no specific grant from any funding agency., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: The objective of this study was to retrospectively evaluate the effectiveness of polidocanol in managing pain, swelling, functional limiting and cosmetic disorders in patients with venous malformations (VMs)., Methods: This retrospective study included patients who underwent sclerotherapy with polidocanol for VMs between 2020 and 2022. Patient records, imaging findings, and evaluation questionnaires used in the preprocedure and follow-up phases were reviewed. After sclerotherapy, patients were followed up at 1, 2, 3, and 6 months. During these visits, the previously used 11-point verbal numerical rating scale (from 0 [no pain] to 10 [worst pain thinkable]) was used to evaluate the severity of symptoms such as pain, swelling, cosmetic discomfort, and functional limitation, and patients were asked to report the number of days per week they experienced these symptoms owing to the VM., Results: A total of 194 sclerotherapy procedures (mean, 1.6 ± 0.3 procedures) in 84 patients (55 female and 29 male patients; mean age, 22.45 ± 11.83 years) were conducted. The majority of these malformations (81%, or 68 patients) were located in the extremities. We found a significant decrease in pain, swelling, functional limitation, cosmetic appearance, and number of painful days between all time points, except for the comparison between months 3 and 6 (P < .001) CONCLUSIONS: Polidocanol sclerotherapy is a safe and effective treatment for VMs that significantly decreases patient complaints and has a very low complication rate. Particularly, following patients at short intervals and administering additional sclerotherapy sessions when necessary will significantly increase patient satisfaction., Competing Interests: Disclosures None., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

Objective: This study aims to investigate the difference in safety and efficacy between two treatments for venous malformations (VMs), electrochemotherapy combined with polidocanol foam (ECP) and bleomycin polidocanol foam (BPF), providing alternative therapies for VMs., Methods: We conducted a retrospective review of 152 patients with VMs treated with ECP and BPF. Pre- and post-treatment magnetic resonance images (MRIs) were collected, and clinical follow-up assessments were performed. Imaging results were used to calculate lesion volume changes. Clinical outcomes included changes in pain and improvements in perceived swelling. Patients were followed up at 1 week and 6 months after surgery. All emerging complications were documented in detail., Results: Of the 152 patients, 87 (57.2%) received BPF treatment, and 65 (42.8%) received ECP treatment. The most common location of VMs was the lower extremities (92/152; 60.2%), and the most common symptom was pain (108/152; 71.1%). Forty-three patients had previously undergone therapy in the BPF group (43/87; 49.4%), whereas 30 patients had received prior treatment in the ECP group (30/65; 46.2%). The study found that the percentage of lesion volume reduction in the BPF group was not significantly different from that in the ECP group (75.00% ± 17.85% vs 74.69% ± 8.48%; P = .899). ECP was more effective when the initial lesion volume was greater than 30 mL (67.66% ± 12.34% vs 73.47% ± 8.00%; P = .048). Patients treated with BPF had significantly less posttreatment pain than those treated with ECP, in different baseline lesion size. In the overall sample, pain relief was significantly higher in the BPF group than in the ECP group (4.21 ± 1.19 vs 3.57 ± 0.76; P = .002). However, there was no difference in pain relief between the two groups for the treatment of initially large VMs (4.20 ± 0.94 vs 3.70 ± 0.87; P = .113). The ECP group was significantly more likely to develop hyperpigmentation (5/87; 5.75% vs 11/65; 16.92%; P = .026) and swelling (9/87; 10.34% vs 16/65; 24.62%; P = .019) 1 week after surgery than the BPF group., Conclusions: Our study demonstrates that both BPF and ECP are effective treatments for VMs, with BPF being a safer option. ECP is a better choice for patients with the initial lesion volume greater than 30 mL, but it is more likely to lead to early swelling and hyperpigmentation., Competing Interests: Disclosures None., (Copyright © 2024. Published by Elsevier Inc.)

Fibro-adipose vascular anomaly (FAVA) is a rare and complex vascular malformation associated with persistent pain, limb contracture, and even restriction of activity. However, the pathophysiology of FAVA remains unclear. Although FAVA is a benign vascular malformation, it is highly misdiagnosed and often thus undergoing repeated surgical resection and interventional sclerotherapy, resulting in worsening of symptoms and irreversible dysfunction. Therefore, aggressive diagnosis and treatment are essential. There are several different treatment options for FAVA, including surgical resection, sclerotherapy, cryoablation, drug therapy, and physical therapy. This article reviews the clinical manifestations, pathological features, pathogenesis, and treatment methods of FAVA., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Intramuscular vascular malformations (IVMs) are rare developmental congenital structural abnormalities. Their clinical diagnosis is difficult, and imaging studies are essential to determine the type and extent of vessels involved. Treatment can be challenging and must be managed by a multidisciplinary team., Methods: A descriptive, observational, retrospective, longitudinal study of clinical records of patients diagnosed with IVMs who were evaluated at the vascular anomalies clinic from January 2011 to December 2021 was performed. Demographic, clinical, imaging, diagnosis, treatment, and response data were collected., Results: Seven patients (five females and two males) with a mean age of 13.66 years (standard deviation 5.82 years) were included in the study. In all cases, the clinical diagnosis was venous and lymphatic malformation. The radiological findings were dilated and tortuous vascular structures or multilobulated lesions with septa inside, with or without vascular flow; these findings allowed diagnosis in all cases. Treatment modalities included sclerotherapy in five patients, surgical resection in two, medical treatment with sirolimus in three, and surveillance in one. Subsequent clinical evolution was favorable in all patients, with decreased pain in six (partial in four and total in two) and size reduction in one patient., Conclusion: IVMs in our pediatric population most frequently affect the lower extremities. The main symptoms and signs were pain on exertion and volume increase. Treatment can be challenging given the extension and depth of the malformations, so a combination of therapeutic modalities may be necessary to obtain the best outcome., (Copyright: © 2024 Permanyer.)

Background: Vascular malformations (VaMs) are caused by errors in vascular morphogenesis. Diagnosis and treatment can be complex. Few specialized centers care for these patients, and limited literature exists regarding their characteristics and clinical course. The vascular anomalies clinic (VAC) at the Instituto Nacional de Pediatría (National Institute for Pediatrics) is a multidisciplinary team and has been a reference center for patients with VaMs since 2012. We sought to describe the characteristics of patients cared for at the VAC, types of VaMs, treatments used, and clinical course., Methods: This was a descriptive, observational, retrospective, and cross-sectional study conducted from 2012 to 2022., Results: We included 435 patients with VaMs; the median age of presentation was 1 month. The most frequent signs and symptoms were increased volume (97.2%), superficial color change (65.5%), and pain (43.3%). The most common VaMs were lymphatic (36.7%) and venolymphatic (18.3%). Sclerotherapy was the most frequent treatment (73.4%), followed by medical treatment with sirolimus (18.5%); response to both was excellent/good in > 85% of cases., Conclusion: In this retrospective study of children with VaMs, we found that low-flow malformations were the most common, and sclerotherapy and sirolimus were the most frequently used treatments. The therapeutic response was excellent/good in most cases., (Copyright: © 2024 Permanyer.)

Aim: To evaluate the usefulness of handheld ultrasound in comparison with high-end ultrasound for lesion evaluation before and after sclerotherapy in pediatric patients with venous malformations (VMs)., Material and Methods: 10 pediatric patients prior to and after sclerotherapy were scanned by an experienced examiner using handheld ultrasound (Vscan AirTM) and high-end ultrasound (LOGIQ E9/E10) as reference. Patients with associated venous thromboses and intralesional aneurysms had been excluded. Results were interpreted independently by two readers in consensus., Results: 10 patients (4-17 years; 10.0±4.32 years; female n = 6, male n = 4) with 10 VMs (4 of the head and neck region, 4 of the upper and 2 of the lower extremities) were examined. 7 phleboliths were detected. The average rating score achieved by the high-end device never was less than 4, by Vscan AirTM never less than 3. An exception was the assessment of AV fistulas. In comparison with the evaluation of variables examined, we found a significant difference between the high-end scanner and the handheld device regarding the achieved image quality., Conclusion: Vscan AirTM ultrasound device allows new possibilities for procedure planning and post-procedural control of pediatric patients with VMs.

Purpose: To retrospectively evaluate sclerotherapy using consecutive polidocanol and bleomycin foam (CPBF) for large untreated venous malformations (VMs) and/or those resistant to prior treatment., Materials and Methods: This retrospective study included all patients treated with CPBF for untreated VMs larger than 10 mL and/or refractory to treatment between May 2016 and October 2019. Baseline and follow-up VM volumes were measured on fat-suppressed T2-weighted magnetic resonance (MR) imaging. Outcome was evaluated on postprocedural MR imaging volumetry and by a retrospective survey assessing clinical response and adverse events. Imaging response was considered good for volume reduction from 50% to 70% and excellent for volume reduction ≥70%. Symptoms and quality-of-life (QoL) scores were compared before and after CPBF sclerotherapy., Results: Forty-five patients (mean age, 16 years; range, 1-63 years; 25 males) with 57 VMs were analyzed and treated by 80 sclerotherapy. Sixty percent (27 of 45) of patients had undergone prior treatment for VM. Median VM volume was 36.7 mL (interquartile range, 84 mL) on pretherapy MR imaging. Good and excellent results after the last sclerotherapy were achieved in 36% (16 of 45) and 29% (13 of 45) of patients, respectively, corresponding to a decrease of >50% in 60% (34 of 57) of VMs. QoL score increased by at least 3 points, regardless of initial symptoms. Most patients did not desire additional sclerotherapy owing to near complete symptomatic relief, even for patients who did not achieve a good response. Swelling, pain, and motor impairment scores significantly improved after CPBF. Adverse events included fever (44%, 15 of 34) and nausea/vomiting (29%, 10 of 34)., Conclusions: CPBF sclerotherapy represents an effective therapy for large and/or refractory VMs with minimal adverse events., (Copyright © 2023. Published by Elsevier Inc.)

Background: Percutaneous sclerotherapy is an effective treatment for lymphatic malformations (LM) of the head and neck in adults. The purpose of this study was to examine the indications and efficacy of sclerotherapy for head/neck LM in the neonate and infant population., Methods: We retrospectively reviewed patients treated with percutaneous sclerotherapy for LM of the head/neck at age ≤12 months at a single vascular anomalies clinic. The clinical, anatomic, and technical aspects of each treatment, complications, and post-treatment clinical and imaging outcomes were analyzed., Results: 22 patients underwent 36 treatments during the first year of life. Median age at first treatment was 6.2 months (range 2-320 days). Severe airway compromise was the most frequent indication for treatment (31.8%). Sclerosants included doxycycline (80.5%), sodium tetradecyl sulfate (55.5%), bleomycin (11.1%) and ethanol (2.8%). There were no immediate procedure-related complications; sclerosant-related laboratory complications included transient metabolic acidosis (8.3%) and hemolytic anemia (5.5%). Median follow-up was 3.7 years (IQR 0.6-4.8). 47.6% of patients showed >75% lesion size reduction and 19.0% showed minimal response (<25% improvement). At last follow-up, 71.4% of children were developmentally normal and asymptomatic, 23.8% had recurring symptoms, and 4.8% required permanent tracheostomy. Patients with ongoing symptoms or limited response to percutaneous sclerotherapy (33.3%) were treated with long-term sirolimus., Conclusions: Percutaneous sclerotherapy is a safe and effective treatment for symptomatic LM of the head and neck in neonates and infants. Treatment strategy and management of recurrent symptoms requires consensus from an experienced, multidisciplinary team., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Venous malformations (VMs) are histopathologically benign but can greatly impair patients' quality of life. Screlothprapy is known to be effective in improving symptoms without a scar, but surgical resection of residual lesions is sometimes necessary due to inadequate reduction. However, there is no consensus on what criteria should be used to consider switching to surgical treatment, and individualized decisions must be made for each case. To investigate the factors that contribute to the lack of efficacy of sclerotherapy in reducing lesions and how to predict this, the authors performed a retrospective clinical imaging and histopathological study of 6 cases of labial vein malformations treated with sclerotherapy and 3 cases without sclerotherapy. Clinical image investigations are based on magnetic resonance imaging before and after sclerotherapy. The authors found a significant decrease in the percentage of cystic components in the total lesion of VMs after sclerotherapy. Histopathological investigations are based on resected VMs with or without sclerotherapy. Elastica van Gieson stains suggested a significant increase in fibrotic tissue inside VMs treated with sclerotherapy compared with those without. In conclusion, magnetic resonance imaging signal changes inside the VMs after sclerotherapy was observed, and it may reflect fibrosis of the tissue. These changes in the VMs after sclerotherapy may reduce the effect of sclerotherapy on tissue reduction should be considered., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Purpose: This study aimed to evaluate the value of fat-suppressed T2-weighted imaging (FS-T2WI) for predicting short-term pain relief after polidocanol sclerotherapy for painful venous malformations (VMs) in the extremities., Materials and Methods: This retrospective study included patients with painful VMs in the extremities between October 2014 and September 2021, had their first sclerotherapy without history of surgical therapy, and underwent magnetic resonance imaging before sclerotherapy. Pain relief was assessed 2 months after 3% polidocanol sclerotherapy and was categorized as follows: progression, no change, partial relief, or free of pain. The associations between pain relief and imaging features on FS-T2WI were analyzed., Results: The study included 51 patients. The no change, partial relief, and free of pain groups included 6 (11.8%), 25 (49.0%), and 20 (39.2%) patients, respectively. No patient experienced progressive pain. The lesion diameter was ≤ 50 mm in 13 (65.0%) patients in the free of pain group, whereas it was > 50 mm in all patients in the no change group (p = 0.019). The lesions showed well-defined margin in 15 (75.0%) patients in the free of pain group, whereas they showed ill-defined margin in 5 (83.3%) patients in the no change group (p = 0.034). The most common morphological type was cavitary in the free of pain group (14 [70.0%] patients), whereas there was no patient with cavitary type lesion in the no change group (p = 0.003). Drainage vein was demonstrated in 6 (100%), 22 (88.0%), and 11 (55.0%) patients in the no change, partial relief, and free of pain group, respectively (p = 0.011)., Conclusion: A lesion size of 50 mm or less, a well-defined margin, a cavitary type, and no drainage vein on FS-T2WI were significant features for predicting short-term pain relief after polidocanol sclerotherapy for painful VMs in the extremities., (© 2023. The Author(s).)

Background: Venous malformations (VMs) involving the vulva are rare but often cause debilitating pain, aesthetic concern, and functional impairment. Treatment with medical therapy, sclerotherapy, operative resection, or a combination thereof may be considered. The optimal therapeutic approach remains unclear. We report our experience resecting labial VMs in a large cohort of patients., Methods: A retrospective review of patients who underwent partial or complete resection of a labial VM was conducted., Results: Thirty-one patients underwent forty-three resections of vulvar VMs between 1998 and 2022. Physical examination and imaging demonstrated: 16% of patients had focal labial lesions, 6% had multifocal labial lesions, and 77% had extensive labial lesions. Indications for intervention included pain (83%), appearance (21%), functional impairment (17%), bleeding (10%), and cellulitis (7%). Sixty-one percent of patients underwent a single resection, 13% multiple partial resections, and 26% a combination of sclerotherapy and operative resection(s). Median age was 16.3 years at first operation. All patients requiring multiple operations had extensive VMs. Median blood loss was 200 mL. Postoperative complications included wound infection/dehiscence (14%), hematoma (2%), and urinary tract infection (2%). The median follow-up assessment was 14 months: 88% of patients had no complaints and 3 patients were experiencing recurrent discomfort., Conclusions: Surgical resection is a safe and effective approach to treating vulvar labial VMs. Patients with focal or multifocal VMs can be successfully treated with a single resection, whereas patients with an extensive VM may require multiple partial resections or combined sclerotherapy and resection(s) to achieve long-term control., Type of Study: Retrospective Study., Level of Evidence: IV., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: To report an innovative endoscopic surgery for subcutaneous vascular malformations and intramuscular fibro-adipose vascular anomaly (FAVA) at our center., Background: Historically, open surgical resection has been the treatment of choice. Recent advances in minimally invasive surgery have led to the successful application of endoscopic resection techniques for the surgical management of diseases of soft tissue., Methods: Patients who underwent endoscopic resection of vascular anomalies were included in this retrospective review. Data were extracted from our Vascular Anomalies Center database between September 2019 and October 2022, including sex, age, symptoms, diagnosis, sites of surgery, previous treatment, surgery, and follow-up., Results: There were 13 females and 15 males in the current study, with ages ranging from 1 to 17 years. The diagnoses included microcystic lymphatic malformation (LM) (n = 8), Klippel-Trénaunay syndrome (n = 7), venous malformation (n = 6), FAVA (n = 6), and mixed cystic LM (n = 1). Surgical sites included the lower extremity (n = 24), abdominal wall (n = 2), upper extremity (n = 1), and thoracic wall (n = 1). Five patients had an intramuscular lesion (FAVA). The endoscopic technique used 2 or 3 small ports in a gas inflation manner. Surgery included thrombectomy, radical resection, and debulking of vascular anomalies. Postoperative sclerotherapy with bleomycin was performed through a drainage tube in 6 patients with microcystic LM. Technical success was obtained in 27 patients. The conversion to open surgery was performed in one patient owing to the deep location of the lesion. No wound-related complication was observed., Conclusions: Endoscopic surgery is a minimally invasive, effective, and safe treatment for subcutaneous vascular malformations and intramuscular FAVA. This approach can set a new standard that minimizes wound complications and reduces recovery time in patients undergoing resection for benign soft-tissue lesions., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc.)

Percutaneous sclerotherapy is an effective technique for treating lymphatic malformations of the head and neck, with clinical success rates exceeding 84%.1 Sodium tetradecyl, which damages lipid membranes and stimulates free radical-induced local damage, and doxycycline, which inhibits angiogenesis, have emerged as the safest and most effective of several available sclerosants.2-4 Although severe periprocedural morbidity is rare, temporary local complications are reported in 14% and skin necrosis or scarring in up to 0.8-5.8% of sclerotherapy procedures.5 As these lesions are frequently located in the face and/or neck, even minor complications can be disfiguring and must be avoided. This technical video describes a 'dual-agent' approach for percutaneous sclerotherapy of macrocystic lymphatic malformations using sodium tetradecyl as a 'primer' followed by doxycycline as a definitive sclerosant (video 1). This technique emphasizes meticulous backtable preparation and effective use of ultrasound and fluoroscopy to minimize complications. neurintsurg;15/9/931/V1F1V1Video 1 ., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Sclerotherapy has achieved great success in treating most venous malformation (VM) lesions., Objective: To compare the effects of foam sclerotherapy on infantile hemangioma and pyogenic granuloma (PG). In addition, we analyzed the data and outcomes of foam sclerotherapy for the VM., Materials and Methods: Thirty-nine patients with hemangiomas and 83 patients with VMs were treated, and clinical outcomes, resolution, and complication rates were compared. Sclerotherapy data from the VM group were also analyzed., Results: The average age of the patients and the distribution and tissue involvement of lesions among the 3 groups were significantly different ( p < .001). The average amount of sclerosing foam administered per session in VMs was significantly higher than that in the other 2 groups ( p < .0001) (whereas that in the PG group was lower than that in the infantile hemangioma group [ p < .0001]). However, the overall therapeutic efficacy and side effects in the 3 groups were not significantly different. For VMs, the frequency of ultrasound guided foam sclerotherapy and use of 3% polidocanol increased from superficial to deep lesions, whereas the use of 1% POL decreased ( p < .0001)., Conclusion: Infantile hemangioma and PG treatments presented good results and minor adverse reactions comparable with those of VMs., (Copyright © 2023 by the American Society for Dermatologic Surgery, Inc. Published by Wolters Kluwer Health, Inc. All rights reserved.)

Percutaneous sclerotherapy is a well-recognized treatment for management of low-flow vascular malformations. It is regarded as a safe and effective procedure, and there are very few reports of sight-threatening complications in the medical literature. We describe a case of a 12-year-old girl who developed severe orbital compartment syndrome due to a combination of orbital inflammation and hemorrhage, resulting in massive proptosis, severe chemosis, optic nerve compression, severe lagophthalmos, exposure keratitis and visual loss following bleomycin sclerotherapy for a right orbital and periorbital lymphaticovenous malformation. Despite presenting to the emergency eye clinic 5 days after the onset of her symptoms, when her vision was thought to be irretrievably lost, she had a surprisingly good visual outcome after medical and surgical intervention. Although sclerotherapy for low-flow vascular malformations of the orbit is generally safe, it can be complicated by severe sight-threatening orbital inflammation and hemorrhage for which urgent intervention is usually required. However, with adequate management, visual recovery may occur even after late presentation.

Objective: To retrospectively correlate imaging findings post-sclerotherapy of low-flow vascular malformations with clinical outcome. Materials and Methods: We retrospectively evaluated 81 pediatric patients who had sclerotherapy in our department over a 14-year period. Patients with a diagnosis of low-flow vascular malformation, pre and post-treatment ultrasound (US) and clinical follow-up evaluation were included in the study. Exclusion criteria were coexisting high-flow vascular malformations, history of additional surgical or medical treatment to their malformation and large infiltrative lesions difficult to measure on US. Pre and post-treatment sonographic volumes of the malformation were assessed. Changes in volume were categorized into 6- increased volume, stable and volume decrease of 1-25%/26-50%/51-75%/75-100%. Clinical outcomes were categorized into 4 - worse, no change, improved and symptom free. In cases where pre-treatment MRI was available, the estimated malformation volumes in both modalities were correlated using Spearman's rank correlation. The change in sonographic volume was correlated with clinical outcome using Spearman's rank correlation. P -values < .05 were considered significant. Results: Twenty-nine patients were included in the study; 13 with venous malformation (VM), and 16 with lymphatic malformation (LM). Nineteen patients had both pre-treatment US and MRI, showing correlation in volume between the 2 modalities ( P < .001). Post-treatment change in volume correlated with clinical outcome for combined venous and LMs (rho = .44, P = .02). No correlation was found when venous (rho = .48, P = .09) and lymphatic (rho = .33, P = .21) malformations were considered separately. Conclusion: Ultrasound can potentially be used as an objective tool in evaluating sclerotherapy treatment response of low-flow vascular malformations in the pediatric population.

Management of tongue venous malformations can be challenging in the pediatric population due to their heterogeneity in presentation, extent of involvement and functional compromise. It is important to recognize the value of various treatment options in order to guide management of each patient in an individualized fashion. Here we describe a series of patients with tongue venous malformations that are managed using diverse modalities to illustrate the relative benefits and risks of each technique. The challenges of venous malformation treatment can be mitigated by tailoring the approach to each individual patient and malformation. This case series also emphasizes the need and importance of working in the setting of a multidisciplinary vascular anomalies team., Competing Interests: Potential conflicts of interest The authors have no conflicts of interest relevant to this article to disclose., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Purpose: Here, we report our experience treating a patient with Maffucci syndrome and evaluate the outcomes resulting from surgical management combined with sclerotherapy in the treatment of head and neck venous malformations (VMs). A 19-years-old woman with multiple enchondromas and heterauxesis complained of masses in the oral cavity that had gradually increased in size and eventually affected her daily life. A tracheotomy was performed followed by traditional sclerotherapy to treat the oropharyngeal VMs. Next, we surgically excised the VMs of the oral cavity and maxillofacial skin., Results: Magnetic resonance imaging indicated that the oral VMs were nearly eradicated and the oropharyngeal VMs had stabilized. The patient's appearance and normal maxillofacial region function were restored., Conclusion: In summary, local resection combined with sclerotherapy facilitated timely and efficient VMs removal from the head and neck region of a patient with Maffucci syndrome.

Background: Venous malformations (VMs) are congenital vascular lesions caused by enlarged and ectatic venous channels. Current methods of treatment for VMs involve a combination of sclerotherapy, laser therapy, and surgical resection. While sclerotherapy remains the most commonly used treatment for small VMs, surgery remains an important tool for isolated VMs or larger VMs with higher flow due to potential local and systemic side effects associated with the use of certain sclerosing agents., Methods/results: Here we present a case of a patient with a naso- and oropharyngeal venous malformation which was successfully resected with endoscopic-assisted transoral surgery., Conclusions: This is a low-fingerprint technique to tumors of the oropharynx with excellent visualization and maneuverability in cases where TORS is not an option. This technique does not require palate splitting or excessive retraction, allows multiple surgeons to work simultaneously, and is associated with significantly lower morbidity than transcervical techniques., Level of Evidence: N/A., Competing Interests: Conflicts of interest None relevant., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: This study evaluated the effectiveness and safety of percutaneous sclerotherapy for maxillofacial venous malformations., Study Design: Patients who had venous malformations involving the masticatory muscles and who underwent sclerotherapy were enrolled in this retrospective study., Results: Twenty-four patients (13 female, 11 male; mean age 21 years) were analyzed. Major clinical symptoms were swelling (100%) and intralesional pain (54%). Intramuscular lesions involved the masseter muscle only in 38% of cases, both the masseter and temporalis muscles in 33%, all masticatory muscles in 21%, and the temporalis muscle only in 8%. Extramuscular involvement was observed in 58% of patients. Absolute ethanol and polidocanol were used as sclerosants. The mean number of sclerotherapy sessions per patient was 6.6 (range, 1-32). The mean follow-up duration after the first sclerotherapy session was 64.8 months (range, 6-178). The complications included paralysis of the facial nerve (25%), intraoral ulceration (8%), and hemoglobinuria (8%). The effectiveness of treatment was rated as excellent in 33% of cases, good in 46%, and fair in 21%. Better results were obtained in patients without extramuscular involvement., Conclusion: Percutaneous sclerotherapy can be effective and safe for maxillofacial intramuscular venous malformations, especially for localized lesions of the masseter muscle., (Copyright © 2022. Published by Elsevier Inc.)

Background: Vascular malformations are rare diseases and treatment remains controversial. Sclerotherapy is accepted as a minimally invasive treatment with good results; however, some patients do not report good response to sclerotherapy, and it is not clear which variables influence treatment outcome, preventing optimal patient selection. We hypothesized that large diameter lesions have reduced efficacy and satisfaction with sclerotherapy. Therefore, we prospectively evaluated the clinical response of low-flow vascular malformations treated with 1% polidocanol foam sclerotherapy and determined the correlation of symptom improvement with clinical variables., Methods: Prospective cohort study of patients with symptomatic low flow vascular malformations treated at the Hospital das Clínicas between December 2016 and November 2018. Lesions were classified according to location, extension, diameter, type of vessels, margins, and symptoms. Sclerotherapy was performed by direct injection of 1% polidocanol foam, with a maximum volume of 10 ml per session. Symptom improvement was evaluated using the visual analog scale and patient-reported quality of life was assessed by the Short-form 6 dimensions (SF-6D) questionnaire. Follow-up was performed every 3 months after treatment., Results: Forty lesions were treated in 38 patients, with 19 women and 19 men; median age was 20.5 years (range 4 months to 63 years). Most lesions (80%) were venous, and most commonly in the lower limbs (47.5%); 27.5% were superficial, 35% were deep, and 37.5% affected both compartments. The most common clinical presentations were deformity (95%), pain (78%), and functional limitation (58%); deep compartment lesions were more associated with functional limitation (69% vs. 27%; P = 0.030). Fifty-three per cent of lesions were >10 cm and were associated with increased numbers of psychosocial symptoms (P = 0.038). Two-hundred and forty-two sessions of sclerotherapy were performed, with 6 mean treatments per lesion (range 1-15). The mean follow-up time was 23 months (range 2-23 months). The most common complications were pain and local edema, without any major complications; some patients reported symptom recurrence (15% pain, 10% deformity, and 12% functional limitation). Sclerotherapy was associated with significant improvement of all symptoms (P < 0.001). However, lesions >10 cm were associated with poor improvement in deformity (P = 0.003). Quality of life improved for most patients (60%) and treatment satisfaction was high (82%)., Conclusions: Treatment of low-flow vascular malformations with 1% polidocanol foam is safe and effective; sclerotherapy improves symptoms and quality of life. Large diameter lesions (>10 cm) are associated with increased treatment failure. A small percentage of patients reported recurrent symptoms and require other modalities for successful treatment., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: To evaluate the efficacy and safety of transarterial bleomycin sclerotherapy of early-stage facial arteriovenous malformation (AVM)., Methods: A retrospective review was performed of patients who underwent bleomycin sclerotherapy for early-stage AVM (Schobinger stage I or II) in a single-referral vascular anomalies center. Bleomycin was slowly infused transarterially with flow control techniques to prolong the effects of bleomycin. Procedure details, AVM characteristics, and previous treatments were reviewed. Initial therapeutic outcomes were determined by 5 categories using both radiological and clinical findings in a 6-month follow-up. Further follow-up outcomes were reviewed to evaluate the long-term efficacy and safety of the treatment. Procedure-related complications were also analyzed., Results: Nineteen patients (mean age 22.4 ± 14.0 years, 14 females) with 31 sessions of sclerotherapies were enrolled. All AVMs were Cho-Do classification type III (type IIIa [n = 13], type IIIb [n = 2], and type IIIa+b [n = 4]). Patients received a mean of 1.6 (range, 1-4) sessions of treatment. The mean cumulative bleomycin dose was 23,600 IU ± 14,500 (range, 8000 - 60,000 IU). The results showed that 14 patients (74%) were responsive to transarterial bleomycin sclerotherapy, including complete response (n = 3), marked improvement (n = 1), and partial improvement (n = 10). The remaining 5 (26%) showed no response. During a mean follow-up of 32.6 months, 5 (26%) showed slight progression compared with 6-month outcomes and 14 (74%) were stable. There were only 2 minor complications [hyperpigmentation (n = 1) and cellulitis (n = 1)]., Conclusions: Transarterial bleomycin sclerotherapy using flow control techniques can be a safe and feasible alternative treatment option for facial early-stage AVM., Competing Interests: Declaration of Competing Interest None., (Copyright © 2022 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

Purpose: This study evaluated the results of polidocanol sclerotherapy in the treatment of venous malformations (VM) including patient satisfaction, perceived improvement, and predictors of satisfaction., Material and Method: Patients with VM that underwent polidocanol foam sclerotherapy between June 2013 and July 2021 in a single center were retrospectively evaluated. Patient demographics, VM, and treatment characteristics were analyzed. Patient-reported outcomes and satisfaction were analyzed with a questionnaire., Results: This study included 232 (136, 58.6%, female) patients. The mean age was 24.49 ± 12.45 years (range 3-72). The clinical response rate was 82.3%. The rate of satisfaction was 82.3%, and 116 (50%) patients were significantly satisfied. There were no major complications. Clinical response and VM margin were related to satisfaction ( p < 0.01, p = 0.012, respectively). Clinical response to pretreatment swelling was related to significant satisfaction ( p = 0.02)., Conclusion: Polidocanol sclerotherapy was safe and effective in VM treatment with high satisfaction and low complication rates.

Introduction: Venous malformations affect around 2 per 10,000 newborn and are the most common type of congenital vascular malformation. They are always present at birth and are often misdiagnosed with hemangiomas. Accurate diagnosis and adequate targeted therapy through a multidisciplinary approach is advocated for a successful treatment, considering a combination of modalities in complex cases. We present here the workup and treatment of a venous malformation in a 14 month old child by combining a preoperative sclerotherapy with sodium tetradecyl sulfate (STS) followed by complete surgical excision respecting the calvarium., Case Presentation: A large right extracranial fronto-parietal venous malformation and scalloping of the underlying calvarium, attached to the pericranium, surgically excised after preoperative sclerotherapy with STS in a 14-month-old child., Results: The patient had an uneventful postoperative course with complete resection of the lesion, satisfying cosmetic appearance and no complications. Histopathological examination confirmed a venous malformation., Conclusion: We demonstrated the workup and the rationale of the combined sclerotherapy followed by surgical resection of a large growing extracranial venous malformation. Preoperative direct contrast injection and sclerotherapy allowed to rule out significative extracranial to intracranial venous inflow and reduce blood loss for the surgical procedure, respectively. Complete removal of the malformation minimized the impact on future growth of the calvarium., (© 2022. The Author(s).)

Objective: To evaluate the efficacy and safety of lauromacrogol foam sclerotherapy in the treatment of children with lip venous malformation., Methods: Fifty-two children (27 males and 25 females) aged from 6 months to 17 years with lip VM who underwent lauromacrogol foam injection with ultrasonic guidance from July 2018 to December 2020 in our hospital were retrospectively recruited for this study. All the children were examined by MRI, ultrasound, blood routine and coagulation before operation. We were guided by ultrasound to locate the blood flow area (nests), injecting lauromacrogol foam to fill the venous malformation. The follow-up time was 14.31 ± 5.96 (6-24) months. Follow-up items include clinical manifestations, imaging data, efficacy and complications., Results: This group of children was treated 3-5 times, an average of 4 times/case. The total effective rate was 90.38%. Pain in 4 cases, fever in 4 cases, infection in 2 cases, ulcer in 1 case. There were no serious complications such as cardiopulmonary accident., Conclusion: Ultrasound guiding foam sclerotherapy with lauromacrogol is effective and safe for children with lip venous malformation., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Orbital vascular malformation, one of the common orbital diseases, is confronted with problems like complex clinical manifestations, difficulties in treatment, and poor efficacy. In recent years, the therapeutic methods have been continuously explored and expanded, including sclerotherapy, laser coagulation, intra-arterial embolization, surgical resection, and targeted therapy. And the technology has been constantly innovated and improved to overcome difficulties in treatment and enable more favorable outcomes of orbital vascular malformation. By summarizing the existing innovations, this article proposes to study the pathogenesis, widely explore novel therapeutic targets, and integrate advanced auxiliary technologies, so as to develop more accurate and minimally invasive therapies for orbital vascular malformation.

Objective: The objective of this research was to retrospectively investigate the difference of safety and efficacy between polidocanol foam and bleomycin polidocanol foam (BPF) in the treatment of venous malformations (VMs), and provide clinical evidence for the application of BPF for VMs., Methods: Patients with VMs treated with polidocanol foam and BPF were included between July 2018 and July 2020. The VM tissue involvements and symptoms were collected. The treatment outcomes were evaluated by the clinical improvement of symptoms and the degree of devascularization on ultrasound examination or magnetic resonance imaging. Patients were followed up for 1, 3, and 6 months after the sclerotherapy. Immediate and delayed complications were closely followed and recorded., Results: A total of 51 patients were included, including 34 females and 17 males with a mean age of 26.8 years (range, 5-65 years). The most commonly involved sites were lower extremities (31/60 [51.7%]) and the most common symptom was pain (33/51 [64.7%]). Fifty-four sclerotherapies were performed with a mean of 1.06 ± 0.24 sessions (range, 1-2 sessions) per patient. The reduction percentage of lesion volume in the BPF group was significantly higher than the polidocanol foam group (79.4 ± 1.6% vs 55.7 ± 6.1%; P < .001). Patient satisfaction scores in the BPF group were significantly higher than the polidocanol foam group (7.2 ± 1.1 vs 5.7 ± 0.8; P < .001). No major complication was observed in either group. Cardiovascular and Interventional Radiological Society of Europe (CIRSE) grade 1 complications occurred in 5 of 21 patients in the BPF group and 7 of 30 patients in the polidocanol foam group, CIRSE grade 2 complications occurred in 5 of 21 patients in the BPF group and 4 of 30 patients in the polidocanol foam group; there were no significant differences between the two groups., Conclusions: BPF is a safe and effective sclerosant for VMs, showing better efficacy and similar safety as commonly used mild sclerosants. It could be a promising agent to treat VMs or other slow-flow vascular malformations., (Copyright © 2022 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Sclerotherapy for venous malformation has been widely used; however, no guidelines are available to assess the effectiveness of different sclerotherapy agents. We conducted a systematic review and network meta-analysis to investigate the effectiveness of sclerotherapy agents for venous malformations., Methods: Three electronic databases were searched from their inception (1950) to April 29, 2021. Studies comparing the effectiveness of different sclerotherapy agents were included. The risk of bias within and across studies was assessed. Pairwise meta-analyses were conducted, followed by a network meta-analysis. We also assessed inconsistency and publishing bias using various approaches., Results: Seven studies with 547 patients in six arms were included in the present study. We defined the response and complete response as two separate outcomes. Significant differences were observed in four comparisons with respect to the response (ethanol vs bleomycin, ethanol vs polidocanol, ethanol vs sodium tetradecyl sulfate, polidocanol vs sodium tetradecyl sulfate). No statistically significant differences were found in the other comparisons. The evidence network revealed that for the response outcome, ethanol ranked first, followed by pingyangmycin, polidocanol, sodium morrhuate, bleomycin, and, finally, sodium tetradecyl sulfate. For the complete response outcome, pingyangmycin had the best results, followed by sodium morrhuate, polidocanol, ethanol, bleomycin, and, finally, sodium tetradecyl sulfate. Major complications, such as facial nerve palsy, serious local swelling, and necrosis, had occurred mostly in the ethanol group and rarely in the other groups. Because of the limited data, no further analysis of major complications was conducted. Our confidence in the comparisons and rankings was low. We found no verified inconsistency or publishing bias in the present study using the existing approaches., Conclusions: Ethanol showed a significantly better response statistically compared with the other agents. However, ethanol had also resulted in the highest incidence of complications. Pingyangmycin showed the second-best response, best complete response, and a low rate of complications, respectively. Overall, pingyangmycin achieved excellent performance and balance in terms of the different outcomes. However, they could not be adequately recommended from the available data. More superior trials, especially randomized controlled trials, are needed in the future., (Copyright © 2022 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: To report the clinical effectiveness and the safety of cryoablation in first or second-line therapy in symptomatic soft tissues vascular malformation, a mini-invasive therapeutic alternative to sclerotherapy or surgery., Materials and Methods: This retrospective and monocentric study included patients with symptomatic low-flow vascular malformation. The interventions were carried out under computed tomography (CT) scan, Cone-beam CT (CBCT) and/or USA guidance. Clinical response was evaluated by collecting the Numerical Rating Scale (NRS) for symptoms before and after the ablation. Safety was assessed based on criteria proposed by the Cardiovascular and Interventional Radiological Society of Europe. Imaging response was evaluated with post-ablation Magnetic Resonance Imaging., Results: Twenty-one patients were included. Cryoablation was the first operative treatment for 12 patients (12/21, 57%). The remaining patients had already undergone surgery (2/21, 9%) or one or more sclerotherapy procedures (7/21, 33%).Symptoms assessed by Numerical Rating Scale dropped from a median of 7 [IQR 6-8] before the procedure to a median of 1 [IQR 0-2] after cryoablation (p < 0.001). Half of the patients declared a full effectiveness of cryoablation on their symptoms (11/21). No major complications and four minor adverse events (two skin lesions, two patients with neuropathic pain) were reported (19%). Lesional volume significantly decreased after cryoablation (median from 3.7 cm 3 [1-10.4] to 0.25 cm 3 [0-2.0], p < 0.001)., Conclusion: Cryoablation seems to be a safe and effective first- or second-line therapy for soft tissue vascular malformations., (© 2022. Springer Science+Business Media, LLC, part of Springer Nature and the Cardiovascular and Interventional Radiological Society of Europe (CIRSE).)

Purpose: To evaluate factors that contribute to adverse events (AEs) after embolization of venous malformations (VMs) using sclerosing agents., Materials and Methods: A retrospective review of patients who underwent direct puncture embolization of VMs with a sclerosing agent and adjunctive techniques, also termed as sclerotherapy, from 2016 to 2021 was performed. Three-hundred one embolizations in 137 patients (median age, 19.0 years; interquartile range, 12.8-31.5 years) were evaluated. The VM characteristics and embolization details were analyzed. Anticoagulation was also evaluated based on D-dimer levels. Cutaneous and noncutaneous AEs were analyzed. The AEs were categorized using Society of Interventional Radiology guidelines., Results: VMs involving tissue planes were subcutaneous (n = 104), intramuscular (n = 84), and combined (n = 113). Overlying skin involvement was observed in 56% of the patients. Sodium tetradecyl sulfate was mainly used (83%). Periprocedural anticoagulation was used in 9% of the patients. Most AEs were minor (30/301, 9.7%), and only 1.7% (5/301) were major (21 cutaneous and 14 noncutaneous AEs). Age below 18 years (odds ratio, 4.4502; 95% confidence interval, 1.5607-12.6890; P = .0052) and overlying skin involvement (odds ratio, 7.1794; 95% confidence interval, 1.6226-31.7656; P = .0094) were factors associated with cutaneous AEs. All noncutaneous AEs developed in patients with intramuscular VMs; however, this association was not statistically significant. There was no severe hematologic AE or permanent consequence., Conclusions: The overall AE rate after VM embolization was 11.6%; however, major AEs were rare (1.7%). Cutaneous AEs were associated with young age and overlying skin involvement. All noncutaneous AEs developed in patients with intramuscular VMs., (Copyright © 2022 SIR. Published by Elsevier Inc. All rights reserved.)

This single-center, single-arm, prospective open-label trial is being conducted to evaluate the short-term efficacy and safety of percutaneous sclerotherapy with polidocanol foam for painful venous malformations. This study will include patients who were clinically diagnosed with venous malformation by using ultrasound and/or magnetic resonance imaging, and whose pain persisted even after treatment with medications. Written informed consent for sclerotherapy will be obtained from all patients. The institutional review board approved this prospective study protocol. The primary endpoint is pain relief at three months after sclerotherapy. Local pain related to venous malformation will be evaluated using a numeric rating scale. Patient recruitment commenced in December of 2020. Enrolment of 13 patients is planned over a 3-year recruitment period. Herein, we describe the details of the clinical trial protocol., Competing Interests: The authors have no commercial, financial, or other conflicts of interest to declare for this research. This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Background: Venous malformations (VMs) and sclerotherapy may disrupt the normal systemic coagulation profile in individuals. This study investigated a correlation between the clinical efficacy of sclerotherapy in the treatment of VMs and the changes in coagulation indexes to provide data that will inform the future application of this therapy., Methods: From September 2019 to September 2020, 61 patients were enrolled in this study to receive sclerotherapy with absolute alcohol. The clinical outcomes and the coagulation profile were assessed., Results: Sclerotherapy induced increasing fibrin (original) degradation products (FDP) and D-dimer (D-D) levels. The changes in FDP and D-D level pretreatment and posttreatment were positively correlated with treatment outcomes. Moreover, a repeated treatment with absolute alcohol may restore normal levels of FDP and D-D., Conclusions: Upregulation of FDP and D-D levels after sclerotherapy results in good therapeutic outcomes. Therefore, monitoring changes in FDP and D-D levels in patients with VMs undergoing sclerotherapy may reflect the effects of sclerotherapy., (Copyright © 2022 Elsevier Inc. All rights reserved.)

This study aimed to review systematically the efficacy, safety, and technical aspects of cryoablation in the treatment of venous malformations (VMs) and to provide the groundwork for future studies. A literature search for clinical studies utilizing percutaneous cryoablation of VMs was performed. All clinical studies related to primary or secondary treatment of VMs with percutaneous cryoablation were included in this review. These selected studies were evaluated for patient characteristics, cryoablation technique, technical success, lesion size and pain scores before and after cryoablation, and adverse outcomes. Random effects analysis of postprocedural changes in lesion volume and pain scores was performed. There were 54 patients with 55 cases of cryoablation of VMs. Of these cases, 27 recorded changes in lesion volume and 31 recorded changes in pain scores. The weighted mean postprocedural decrease in lesion size was 92.0% (raw average, 71.7%). The weighted mean reduction in pain score was 77% (raw average, 78.2%). Considering all treated cases (55), complete resolution of symptoms was seen in 35 cases (63.6%) and overall (complete or partial) improvement in 52 cases (94.5%). Common postprocedural symptoms included pain, bruising, swelling and numbness lasting less than 2 weeks. There were two major adverse events (3.7%), with both cases due to persistent dysesthesia. Patients with a history of prior sclerotherapy demonstrated lower preprocedural and postprocedural pain scores (4.7 and 1.3) than patients without prior treatments (5.8 and 2.8). Cryoablation of VMs appears to be potentially safe and effective on limited short-interval follow-up., (Copyright © 2022 SIR. Published by Elsevier Inc. All rights reserved.)

Background: Intralesional injection of a sclerosing agent is a standard treatment for vascular malformations, but many repeat treatments are often required to achieve a response. The temporary application of an electrical field to the treated area increases cellular membrane permeability and therefore augments drug delivery., Objective: To report a case series of patients with vascular malformations who received bleomycin electrosclerotherapy (EST)., Materials and Methods: A prospective observational case series of patients with vascular malformations treated with bleomycin EST. Only patients who did not respond or had limited response to standard sclerotherapy were included. The response to the treatment was assessed using a global assessment score and patient-reported outcome measures., Results: Thirty patients were treated with EST. Most of the patients had venous malformations (57%). Complete response or significant improvement was observed in 17 (57%) and 7 (23%) patients, respectively. Most of the patients were satisfied or very much satisfied with the outcome of the treatment. Most commonly reported complications were swelling, pain, and bleeding., Conclusion: Electrosclerotherapy is a promising method of augmenting the efficacy of intralesional bleomycin injections when treating vascular malformations. It can potentially lead to a reduction in the administered dose and number of treatment sessions needed., (Copyright © 2021 by the American Society for Dermatologic Surgery, Inc. Published by Wolters Kluwer Health, Inc. All rights reserved.)

Background: Vascular malformations of the head and neck are common. The management of these lesions is complex and challenging due to the high complication rate and recurrence following treatment. Palatal vascular malformations (PVMs) are infrequent and present as slow growing lesions in the palate with recurrent bleeding and pain. These lesions are best managed by sclerotherapy due to their posterior location and risk of bleeding if surgery is attempted. Many sclerosants have been used for treating PVMs but the use of intralesional bleomycin for these lesions has not been reported at length. This paper describes the use of intralesional bleomycin injections for the treatment of deep-seated palatal vascular malformations., Methods: Intralesional bleomycin injections were given directly into the lesion with the patients under short general anaesthesia. The total dose of bleomycin ranged between 8 and 15 IU, which depends upon the body weight and was repeated every four weeks till the resolution of lesion was observed., Results: All the lesions in 12 patients regressed significantly with serial bleomycin injections. Clinically, the involved palatal mucosa became normal and magnetic resonance imaging demonstrated the significant regression of the lesion in all the cases. No complications were encountered with the use of intralesional bleomycin., Conclusions: Intralesional bleomycin injections have proved to be an emerging modality in the management of remotely situated palatal vascular malformations. Their rapid regressive effect on the lesion coupled with a high safety margin makes bleomycin sclerotherapy the first choice of treatment for palatal vascular malformations., Competing Interests: Conflict of interest disclosure The authors do not have any financial and personal relationships with other people or organisations that could inappropriately influence this work. We do not have any conflict of interest in areas like employment, consultancies, stock ownership, honoraria, paid expert testimony, patent applications/registrations or any grants or any other funding., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Purpose: To investigate if the detergent sclerosant sodium tetradecyl sulfate (STS) is deactivated by the lipid-based contrast agent ethiodised oil., Method: 3% STS was mixed with ethiodised oil and room air in a 2:1:4 ratio in two luer lock syringes and a three way connector and agitated to make foam (the Tessari technique) to replicate the clinical use of the products. The assay of STS in the mixture was assessed using the British Pharmacopoeia method. Briefly this is a manual titration method where the solution of STS is mixed with an indicator solution and titrated with hyamine solution of known concentration; the concentration of the STS can then be calculated with the titration results. To further mimic the clinical environment with the presence of blood, the effect of adding increasing amounts of albumin to the STS-ethiodised oil mixture was assessed., Results: The assay of STS in the solution after mixing with ethiodised oil was 3% indicating that the ethiodised oil did not deactivate the STS. The addition of albumin to the STS-contrast mixture resulted in near linear neutralisation of the STS with increasing concentrations in the same quantities as with STS alone., Conclusions: The mixture of the lipid-based contrast agent ethiodised oil with the detergent sclerosant STS did not affect the availability of the sclerosant. The continued use of STS-ethiodised oil in the management of vascular malformations can be supported.

Objective: (1) Review a multidisciplinary vascular anomalies center's practice regarding periprocedural anticoagulation for venous malformations (VM) and the associated risk of thromboembolic and disseminated intravascular coagulation (DIC) events. (2) Compare the risk of thromboembolic events and DIC post-procedure between head and neck (H&N) and extremity VM patients., Methods: An Institutional Review Board (IRB)-approved, retrospective chart review was performed on 120 VM patients. A thromboembolic event was defined as a thrombus formation post-sclerotherapy or post-surgery within 2 months in a distant or local venous structure not directly addressed by the procedure., Results: There were 39 cases involving the H&N and 81 cases based at the extremities. There were eight cases of post-procedure thrombus formation within the extremity VM group (8/71; 11.3%) as opposed to 0 cases in the H&N group (OR: 0, 95% CI .00-.09), p = .049. There was no difference in incidence of post-procedure thromboembolic events between those with elevated D-dimer (H&N: 0%, extremity: 22.7%, 5/22) and normal D-dimer values (H&N: 0%, extremity: 6.3% [1/16], P = .370). There was no difference in incidence of post-procedure thromboembolic events between those who received periprocedural anticoagulation (H&N: 0%, extremity: 21%, 4/19) and those who did not (H&N: 0%, extremity: 8.2%, 4/49), (Extremity: OR: 3.00, .67-13.50, P = .206)., Conclusion: Post-procedure thromboembolism is rare in the treatment of venous malformations, especially in the head and neck subsite. Regardless of anticoagulation use, there were no thromboembolic events for H&N VM patients. Such events are rare, and the odds may approach zero, especially with small sample size., Level of Evidence: 4 Laryngoscope, 131:1163-1167, 2021., (© 2020 American Laryngological, Rhinological and Otological Society Inc, "The Triological Society" and American Laryngological Association (ALA).)

Background/objectives: Slow-flow vascular malformations are abnormal vessels that can lead to activation and consumption of coagulation factors and thrombosis, known as localized intravascular coagulopathy (LIC). Most clinical and research evidence of vascular malformation hemostasis relies on conventional coagulation studies, which may not provide a complete picture. Thromboelastograpy (TEG) is a tool that can provide real-time assessment of a patient's coagulation dynamics, and may allow for a more individualized treatment approach. We hypothesized that patients with slow-flow vascular malformations will have changes in TEG parameters peri-procedure that will help predict blood product or medication administration., Design/methods: Institutional Review Board approved prospective study of patients with slow-flow vascular malformations undergoing a sedated, minor procedure. TEG and conventional coagulation studies were obtained preprocedure, 15 min, and when possible, at 30 min after the start of the procedure., Results: Twenty-five patients were enrolled. Median age was 15 years (range 3-47 years). Procedures included laser and/or sclerotherapy. There were no changes in TEG parameters from baseline to 15 min or 30 min. The following decreased from baseline to 15 min: fibrinogen 313 to 287 mg/dL (P = .001), D-dimer 1.3 to 1.1 mg/L (P = .02), hemoglobin 12.8 to 11.8 g/dL (P = .001), and platelet count 272 000 to 256 000 (P = .006). No patient had a bleeding/thrombotic complication during or within 1 week postprocedure., Conclusion: We saw no change in TEG parameters or bleeding or clotting complications despite significant numerical changes in conventional coagulation profiles, suggesting that conventional studies may not be as useful in determining risks of bleeding or thrombotic complications peri-procedure for minor procedures., (© 2020 Wiley Periodicals LLC.)

Background: Intramuscular venous malformations, often erroneously called "intramuscular hemangiomas," present to pediatric orthopaedic surgeons either as a differential diagnosis of tumor or as a cause of muscle pain. Treatment options include injection sclerotherapy or surgery. There is some literature to indicate that sclerotherapy can reduce pain, but little evidence on the effectiveness of surgery. The primary aim of this study was to evaluate the efficacy of surgical resection for intramuscular venous malformations, with a secondary aim to evaluate the natural history and presentation of intramuscular venous malformations to improve clinician understanding of this condition., Methods: A retrospective chart analysis was performed of cases identified from a vascular anomalies database from January 2004 and December 2018. Primary outcome was change in preoperative and postoperative pain. Natural history of the lesion was assessed, including age when the lesion was first noticed, when it became painful, and when it required treatment., Results: Fifty-four cases were identified in the study period of which 40 underwent surgery. Pain improved in 36 of 39 patients (92.3%) who had pain before surgery and 29 (74.4%) were pain free after surgery. All 13 patients who required whole muscle excision to resect the lesion experienced an improvement in pain and 10 (76.9%) were pain free. A history of previous intervention, with surgery or sclerotherapy showed a trend towards less successful surgical outcomes. Patients presented across a wide age range from infancy to adulthood, but the most common presentation was pain with exercise between 9 and 16 years of age, with presence of a mass in about half of cases., Conclusions: Surgery, when performed by a surgeon with appropriate experience, is an effective first-line treatment for painful intramuscular venous malformations, offering pain relief in the majority of cases. Magnetic resonance imaging and ultrasound are diagnostic in most cases. The majority of lesions are resectable, meaning they can be removed with a margin leaving a functional limb. Sometimes resection of a whole muscle is required., Level of Evidence: Level IV-case series.

We report the case of a 35-year-old woman who presented with recurrent macroscopic haematuria and known diagnosis of Klippel-Trenaunay syndrome. Imaging and cystoscopy identified an extensive venous malformation involving a large area of the bladder wall. Holmium laser therapy was ineffective at obtaining symptom control. Following a multidisciplinary team meeting, transvenous sclerotherapy with sodium tetradecyl sulphate was performed under image guidance. A reduction in venous density was observed on cystoscopy and the patient has had complete resolution of symptoms within 6 weeks and continued to be asymptomatic up to 24-month follow-up. We propose that transvenous sclerotherapy is considered first-line treatment in this clinical setting., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Diffuse venous malformations that involve all tissues in the upper limb and ipsilateral chest wall are known as "phlebectasia of Bockenheimer." The authors describe their experience with management of this uncommon vascular anomaly., Methods: The authors' Vascular Anomalies Center registry comprised 18,766 patients over a 40-year period. This review identified 2036 patients with venous malformations of the extremities (10.8 percent), of whom only 80 (0.43 percent) had Bockenheimer disease. The authors retrospectively analyzed patient characteristics, diagnostics, treatments, and complications., Results: The venous malformation was first noted at birth or within the first few years of life with slow and gradual progression. Pain was related to engorgement of the limb. Thromboses and phleboliths were common, but diffuse intravascular coagulopathy occurred in only 12 patients (15 percent). Skeletal involvement was demonstrated as lytic lesions, cortical scalloping, osteopenia, and pathologic fractures. Management included compression garments (100 percent), sclerotherapy (27.5 percent), and resection of symptomatic areas in 35 percent of patients. Adjunctive pharmacologic medication was given in 7.5 percent. Following resection, 17 patients (60 percent) had one or more complications: hematoma, wound dehiscence, flap loss, contracture, and psychosis. There were no deaths. Symptoms improved in all patients with useful functional outcomes., Conclusions: The decision to pursue compression, sclerotherapy, pharmacologic treatment, or resection alone or in combination was made by an interdisciplinary team. Although extensive venous malformations cannot be completely ablated, debulking of symptomatic regions, resection of neuromas, and noninvasive treatments improve the quality of life. Despite the bulk and weight of the arm, forearm, and hand, and the ominous appearance on magnetic resonance imaging, these patients remain functional., Clinical Question/level of Evidence: Therapeutic, V.

Background: Foam sclerotherapy is an effective treatment strategy for venous malformations. Both polidocanol (POL) and bleomycin are effective sclerosants; however, no studies have reported POL-bleomycin foam., Objective: To introduce a method for producing POL-bleomycin foam and evaluate the stability of POL-bleomycin foam with bleomycin concentrations., Materials and Methods: Group A: 2 mL of 1% POL + 8 mL of air; Group B: 2 mL of 1% POL + 3 U bleomycin + 8 mL of air; Group C: 2 mL of 1% POL + 6 U bleomycin + 8 mL of air; Group D: 2 mL of 1% POL + 12 U bleomycin + 8 mL of air. Tessari method was used for foam generation. The foam half-life time (FHT) was used to evaluate foam stability. Five recordings were made for each group., Results: The FHT was 148.6 ± 2.9 seconds in Group A, 148.8 ± 4.0 seconds in Group B, 148.4 ± 2.6 seconds in Group C, and 148.8 ± 1.6 seconds in Group D. The FHT in different groups showed no significant differences., Conclusion: The POL-bleomycin foam was prepared successfully and its FHT was as long as the POL foam.

Background: Vascular anomalies comprise highly variable pathophysiology and commonly pose diagnostic and management dilemmas. Consequently, patients often benefit from input from multiple specialists. This study describes the inception of a multidisciplinary team (MDT) Vascular Anomaly Clinic (VAC) in a tertiary paediatric centre, and the subsequent experience managing this complex patient group., Methods: This was a retrospective study of paediatric patients (< 18 years old) attending an MDT VAC from its inception in October 2012 until November 2019. Patient demographics, presentation, diagnosis and management were reviewed., Results: One hundred and thirty-three paediatric patients were seen over 7 years with a median age of 9.8 years. Vascular malformations were the most common diagnosis (88%), with venous malformations predominating (27%). The most common symptoms were pain (46%) and swelling (34%). Patients often required ≥ 2 investigations, with Doppler ultrasound (86%) and magnetic-resonance imaging (61%) being most common. Management included surgery (27%), sclerotherapy (26%), compression garments (23%), analgesia (12%), laser (15%), embolisation (5%) and sirolimus (3%)., Conclusions: The complex nature of vascular anomalies and high proportion of patients requiring multi-specialty management justified the establishment of an MDT VAC in our centre. Our experience demonstrates the success of an efficient one-stop MDT environment in the management of these challenging conditions., Level of Evidence: IV.

Background: Foam sclerotherapy is an effective treatment strategy for vascular malformations, and its sclerosing power depends on foam stability. Twenty quick passages have been widely used as an indicator of the most stable state of sclerosants, but the universality of their effectiveness has not been proven yet., Objective: We aimed to identify simple and objective indicators of the most stable state of commonly used sclerosants and provide practitioners with suggestions to judge when foam producing is completed in sclerotherapy., Materials and Methods: The universality of the effectiveness of 20 passages was tested by producing bleomycin foam with different passages. Further study was performed by testing modified bleomycin, polidocanol, and sodium tetradecylsulfate foam., Results: The bleomycin foam became denser as passages were added, and the sound of each passage became almost silent after 40 passages. The almost silent sound can be an indicator of foam stability for most sclerosants. It has a different application range compared with 20 quick passages., Conclusion: We suggest that practitioners choose a different indicator depending on the foam used.

Background: Bleomycin (BLM) foam sclerotherapy is effective in the treatment of venous malformations (VMs). Foam stability is influenced by factors such as sclerosant concentration, viscosity, and liquid-gas ratio., Objective: To investigate whether hyaluronic acid (HA) could increase the stability of BLM foam and to evaluate the safety and efficacy of HA-BLM foam., Materials and Methods: Experiment: BLM 6.0 IU + human serum albumin (HSA, 2, 1.95, 1.90, and 1.85 mL, respectively) + 1% HA (0, 0.05, 0.10, and 0.15 mL, respectively) + air 6 mL to create foam using the Tessari method. The foam half-life (FHL) was used to evaluate foam stability. Clinical study: Twenty-eight patients with head and neck VMs were enrolled between June 2018 and August 2019 treated by HA-BLM foam to evaluate the safety and efficacy., Results: The FHL of the BLM foam was 8.46, 8.95, 10.45, and 14.51 minutes, respectively. All patients achieved significant efficacy, and no obvious side effects were observed., Conclusion: Addition of HA could improve the stability of BLM foam.