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Background: The marginal vein (MV) is a persisting embryonic vein located at the lateral aspect of the lower limb. The Weber-classification, which was developed on the basis of phlebography in the 1990s, is the only existing classification system for this rare disease. Aim of this study was the structured characterization of the lateral marginal vein (MV) using magnetic resonance imaging (MRI) and evaluation of the applicability of the Weber-classification. Patients and methods: Institutional Review Board approval was obtained for this retrospective, single-center study. All patients who underwent contrast-enhanced MRI (using a prospectively determined protocol) of the untreated MV were included. MV anatomy and associated findings were characterized in a structured way taking into account the criteria of the Weber-classification for MV: inflow, outflow and extension. If three criteria of the Weber-classification were fulfilled the MV was categorized as "classifiable according to Weber". The MV was categorized as "partially classifiable according to Weber", if two criteria were met and as "not classifiable according to Weber" if less than two criteria were applicable. Results: 56 imaging studies of 58 MV (7 thoracoabdominal, 51 lower extremities) were reviewed. 18/51 MV of the lower extremities were "classifiable" according to the Weber-classification. 33/51 lower extremity MV were not definitely categorized according to the Weber-classification: 19/51 MV were "partially classifiable" and 14/51 MV were "not classifiable". 30/51 MV presented with hypoplastic, 1/51 with aplastic deep venous system. 34/51 lower extremity and 6/7 thoracoabdominal MV were associated with an additional vascular malformation (VM). Conclusions: MRI is suitable for detailed anatomic characterization of the MV and reveals additional therapy relevant findings like associated VM. The Weber-classification was not applicable in most cases, reflecting its limits and the heterogeneity of this rare disease. Structured reports rather than an obsolete classification system should be preferred for MRI of the MV.

Objective: To analyze and compare the efficacy and safety of pingyangmycin fibrin glue composite (PFG) and pingyangmycin dexamethasone composite (PD) in the treatment of pharyngolaryngeal venous malformation (VM). Methods: The clinical data of 98 patients with pharyngolaryngeal VM who underwent sclerotherapy with pingyangmycin composite in the First Affiliated Hospital of Sun Yat-sen University from June 2013 to November 2022 were retrospectively analyzed. According to their treatment, patients were divided into PFG group ( n =34) and PD group (n=64), among those patients there were 54 males and 44 females, aged 1-77(37.06±18.86)years. The lesion size, total treatment times and adverse events were recorded before and after treatment. And the efficacy was divided into three grades: recovery, effective and invalid. According to the length of VM, all patients were divided into three subgroups, to compare the differences in efficacy and treatment times between each two groups.And finally the adverse events and their treatments were analyzed. SPSS 25.0 software was used for statistical analysis. Results: The efficacy of PFG group was 94.11%(32/34), the recovery rate was 85.29%(29/34).And the efficacy of PD group was 93.75%(60/64), the recovery rate was 64.06%(41/64). No serious adverse eventst occurred in subgroup comparison, there was no statistical difference between the two groups in efficacy and the times of treatments when the length was≤3 cm ( Z efficacy =1.04, t treatment times =2.18, P >0.05); when the length was 3-5 cm, there was no significant efficacy difference between the two groups( Z efficacy =1.17, P >0.05), but the treatment times of PFG were less ( t treatment times =4.87, P <0.01); when the length≥5 cm, efficacy of PFG was significantly better than PD ( Z efficacy =2.94, P <0.01), and had fewer treatments times ( t treatment times =2.16, P <0.01). There were no serious adverse events in either group during treatment and follow-up. Conclusion: Both PFG and PD are safe and effective composite sclerotherapy agent for the treatment of laryngeal VM, but PFG has a higher cure rate and fewer treatment times for massive lesions.

Objective:  To determine the efficacy of Uterine Artery Embolization in patients with bleeding acquired uterine arteriovenous malformations (AVMs)., Methods:  A prospective review of all patients who underwent Uterine Artery Embolization at our institution between July 2015 and April 2022 was performed. 225 patients were diagnosed with a uterine vascular malformation on doppler and corresponding MRI imaging. All patients underwent transcatheter embolization of the uterine arteries. Embolic agents in the 375 procedures included Histoacryl glue only (n = 326), polyvinyl alcohol (PVA) particles and Histoacryl glue (n = 29), PVA particles (n = 5), Gelfoam (n = 5), coils (n = 4), PVA particles and coils (n = 3), Histoacryl glue and Gelfoam (n = 2), and Histoacryl glue and coils (n = 1)., Results:  A total of 375 embolization procedures were performed in 225 patients. 90 patients required repeat embolization for recurrence of bleeding. The technical success rate of embolization was 100%. The clinical success rate was 92%: bleeding was controlled in 222 of 225 patients and three patients underwent a hysterectomy. 60 of the 225 patients had uneventful intrauterine pregnancies carried to term. The 210 patients who underwent successful embolization had no recurrence of bleeding at a median follow-up of 53 months (range, 5-122 months) after treatment. 15 patients were eventually lost to follow-up. One minor complication (0.4%) of non-flow-limiting dissection of the internal iliac artery occurred., Conclusion:  Uterine Artery Embolization is a safe, effective, minimally invasive method to treat uterine AVMs with long-term efficacy, which can provide the preservation of fertility., Competing Interests: The authors have no conflict of interest to declare., (Federação Brasileira de Ginecologia e Obstetrícia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/).)

Vascular anomalies are malformations or tumors of the blood or lymphatic vasculature and can be life-threatening. Although molecularly targeted therapies can be life-saving, identification of the molecular etiology is often impeded by lack of accessibility to affected tissue samples, mosaicism or insufficient sequencing depth. In a cohort of 356 participants with vascular anomalies, including 104 with primary complex lymphatic anomalies (pCLAs), DNA from CD31+ cells isolated from lymphatic fluid or cell-free DNA from lymphatic fluid or plasma underwent ultra-deep sequencing thereby uncovering pathogenic somatic variants down to a variant allele fraction of 0.15%. A molecular diagnosis, including previously undescribed genetic causes, was obtained in 41% of participants with pCLAs and 72% of participants with other vascular malformations, leading to a new medical therapy for 63% (43/69) of participants and resulting in improvement in 63% (35/55) of participants on therapy. Taken together, these data support the development of liquid biopsy-based diagnostic techniques to identify previously undescribed genotype-phenotype associations and guide medical therapy in individuals with vascular anomalies., (© 2023. The Author(s), under exclusive licence to Springer Nature America, Inc.)

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.

Purpose: To assess the responsiveness, defined as the ability to detect change in a patient's health or function, of the Patient-Reported Outcome Measure for Vascular Malformation (PROVAM) questionnaire in a cohort of patients with low-flow vascular malformations (VMs)., Materials and Methods: PROVAM was previously developed to assess symptoms, functional limitations, and social/emotional effects experienced by patients with VMs. This is a prospective cohort study of 56 patients with venous and lymphatic VMs who completed at least 2 PROVAM questionnaires, of whom 43 had undergone treatment with sclerotherapy in the interim between questionnaires. External responsiveness was assessed using a receiver operating characteristic (ROC) curve to ascertain whether a change in the total PROVAM score predicts whether patients reported symptom improvement and by correlating the change in the total PROVAM score and change in symptoms reported during clinic visit. Internal responsiveness was evaluated using Wilcoxon signed rank test, Cohen d effect size (ESp), and standard response mean difference (SRM)., Results: The total PROVAM score demonstrated excellent discrimination for symptom improvement with an area under the ROC curve of 0.856. There was a statistically significant, moderate positive correlation between the change in the total PROVAM score and the change in patient symptoms as determined from clinical visits (Spearman correlation coefficient [r s ] = 0.67, P < .001). The total PROVAM score and all subdomain scores improved significantly after treatment (all P < .05). ESp and SRM were 0.80 and 0.83, respectively., Conclusions: PROVAM is responsive to improvement after treatment and may be useful to assess health-related quality of life in patients treated for VMs., (Copyright © 2023 SIR. Published by Elsevier Inc. All rights reserved.)

Abstract: Hyperactivation of the PI3K/AKT/mTOR signaling pathway caused by PIK3CA mutations is associated with a category of overgrowth syndromes that are defined as PIK3CA -related overgrowth spectrum (PROS). The clinical features of PROS are highly heterogeneous and usually present as vascular malformations, bone and soft tissue overgrowth, and neurological and visceral abnormalities. Detection of PIK3CA variants is necessary for diagnosis and provides the basis for targeted therapy for PROS. Drugs that inhibit the PI3K pathway offer alternatives to conventional therapies. This article reviews the current knowledge of PROS and summarizes the latest progress in precise treatment, providing new insights into future therapies and research goals., Competing Interests: Conflicts of interest and sources of funding: The authors have no conflict of any interest relevant to this article to disclose., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Vascular malformations are common but complicated types of disease in infants, with unclear causes and lack of effective prevention. The symptoms usually do not disappear and tend to progress without medical intervention. It is extremely necessary to choose correct treatment options for different types of vascular malformations. A large number of studies have confirmed that sclerotherapy has a tendency to become the first-line treatment in near future, but it is also associated with mild or severe complications. Furthermore, to our knowledge, the serious adverse event of progressive limb necrosis has not been systematically analyzed and reported in the literature., Case Presentation: Three cases (two females and one male) were presented who were all diagnosed as vascular malformations and were treated by several sessions of interventional sclerotherapy. Their previous medical records showed the use of several sclerosants in different sessions including Polidocanol and Bleomycin. The sign of limb necrosis did not occur during the first sclerotherapy, but after the second and third sessions. Furthermore, the short-term symptomatic treatment could improve the necrosis syndrome, but could not change the outcome of amputation., Conclusion: Sclerotherapy undoubtedly tends to be the first-line treatment in near future, but the adverse reactions still remain major challenges. Awareness of progressive limb necrosis after sclerotherapy and timely management by experts in centers of experience of this complication can avoid amputation., (© 2023. The Author(s).)

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.)

Orbital vascular anomalies (OVAs) are a heterogeneous group of disorders frequently found in the orbital cone, the periorbital region, or within the orbit itself. OVAs are divided into tumors and malformations. The most frequent clinical presentation is exophthalmos, associated or not with an alteration of the visual axis. They may also cause acute complications, being intralesional bleeding or cellulitis the most frequent, and chronic complications, such as amblyopia and long-term visual acuity impairment. The development of imaging techniques, the use of new drugs, and the implementation of innovative procedures in interventional radiology have resulted in a significant improvement in the diagnostic and therapeutic approaches to these patients, essential to an accurate diagnosis and management., (Sociedad Argentina de Pediatría.)

Background: Vascular malformations are rare diseases that are best managed in multidisciplinary vascular anomaly centers (VAC). So far, there are few published data on the logistic structure, patient allotment, and internal workflow in an interdisciplinary VAC., Purpose: To demonstrate the referral structure and patient allotment in a European VAC., Material and Methods: A retrospective cohort study was conducted. All patients treated in the VAC since its establishment in April 2014 until June 2020 were identified. A total of 461 patients were included in this study., Results: Most often, a venous malformation was diagnosed (49.9%), followed by arteriovenous malformations (8.7%) and syndromal vascular malformations (8.2%). Only 45.1% of referral diagnoses were correct. Of referrals, 35.1% were internal on-campus references, 28.0% were external references through physicians in private practice, and 19.7% came via external cooperating hospitals. Of the patients, 17.1% were self-admissions without a referral., Conclusion: The most important referring clinics are internal medicine, pediatric, and vascular surgery. A substantial proportion of referrals came from private practices. Recruiting these cooperation partners should be considered a high priority when establishing a new VAC. The rate of misdiagnosis is high, and more education of fellow clinicians is urgently needed. Therefore, patients with vascular malformations should be referred to and treated in dedicated centers for vascular anomalies.

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.

Intramuscular capillary-type haemangiomas (ICTH) are rare vascular anomalies that can easily be misdiagnosed as other entities. A systematic review was performed of all cases of ICTH in the literature since its first description in 1972. An adjudication committee reviewed cases to include only ICTHs. Among 1,143 reports screened, 43 were included, involving 75 patients. The most frequent differential diagnosis was intramuscular venous malformations. The mean age of patients at diagnosis was 21.2 years. ICTH was mainly described as a gradually increasing mass (81.8%), painless (73.9%), that could occur anywhere in the body but most frequently on the head and neck (44.0%). Magnetic resonance imaging (MRI) was mainly used for diagnosis (69.1%) and displayed specific features. The most frequent treatment was complete surgical removal (73.9%), which could be preceded by embolization, and led to complete remission without recurrence in all but 1 case.

In the field of vascular anomalies, distinguishing between vascular malformations and tumors has become crucial for a correct therapeutic approach. However, the differential diagnosis between these two groups is not always well explained in classical texts, mainly because many vascular malformations are still known with old names that suggest a tumoral nature. Also, genetic and pathogenic knowledge of these entities has greatly increased in recent decades, so researchers and clinicians now have a better understanding of vascular malformations. In this paper, we present the main histopathological tips to recognize and identify a vascular malformation as such. We also contextualize such information in the clinical and pathogenic knowledge for a better understanding of these entities., (Copyright © 2023. Publicado por Elsevier España, S.L.U.)

In the field of vascular anomalies, distinguishing between vascular malformations and tumors has become crucial for a correct therapeutic approach. However, the differential diagnosis between these two groups is not always well explained in classical texts, mainly because many vascular malformations are still known with old names that suggest a tumoral nature. Also, genetic and pathogenic knowledge of these entities has greatly increased in recent decades, so researchers and clinicians now have a better understanding of vascular malformations. In this paper, we present the main histopathological tips to recognize and identify a vascular malformation as such. We also contextualize such information in the clinical and pathogenic knowledge for a better understanding of these entities., (Copyright © 2022. Publicado por Elsevier España, S.L.U.)

Objective: We have assessed the efficacy and safety of interventional therapy for venous malformations (VMs), with foam sclerotherapy as the treatment of choice according to our experience at a single specialist center., Methods: All the patients with VMs who had undergone interventional therapy (ie, embolo-sclerotherapy and/or open surgery) from January 1, 2015 to December 31, 2019 were identified through a prospective database. The VM types were classified according to the Puig classification. The outcome measures assessed included the efficacy and complications. The former was divided into four groups: no response, mild response, moderate response, and complete response. The complications were defined as any tissue or functional damage, distal embolization, or tissue reaction. The continuous variables were compared using the analysis of variance F test, and discrete variables were analyzed using the χ 2 tests. P values < .05 were considered statistically significant., Results: A total of 207 patients were included. Puig type I lesions were significantly less likely to have received foam sclerotherapy using sodium tetradecyl sulfate (STS) 3% (P ≤ .001) and more likely to have been surgically excised (P ≤ .001). At the patient's first procedure during the study period, the volumes of foam STS 3% were significantly different across all types of VM (P ≤ .001). The patients with type I VMs had received a lower volume of STS 3% compared with those with type II and III VMs. The efficacy outcome categories were significantly different across all types of VMs (P ≤ .001). Overall, only 14 patients (6.8%) had reported no improvement in efficacy, and 38 patients (18%) had not attended follow-up. Therefore, 154 patients (74.8%) had experienced some form of efficacious outcome. Ten patients (4.8%) had developed complications such as hematoma, thrombophlebitis, and ulceration. The incidence of complications differed significantly across the categories (P = .030), with more complications reported for those with type I VMs., Conclusions: We found that intervention with foam sclerotherapy using STS 3% is clinically effective and safe for patients with VMs and was most successful for those with Puig type I and II VMs., (Copyright © 2022 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Minimally invasive percutaneous and endovascular strategies performed by interventional radiologists have become the mainstays of treatment for vascular anomalies, with improved outcomes, decreased complication rates, and reduced morbidity. The aim of this article is to introduce physicians who care for patients with vascular anomalies to state-of-the-art advancements in interventional radiology (IR) for diagnosis and treatment. Part 1 of this review will focus on sclerotherapy and cryoablation. Part 2 will discuss embolization, endovenous laser ablation, and image-guided percutaneous biopsy. Select vascular anomalies will be discussed as examples to highlight IR diagnostic and/or treatment techniques., (© 2023 Wiley Periodicals LLC.)

Objective: The study aimed to evaluate the early follow-up quality of life (QoL), pain and mental health of patients with congenital vascular malformation (CVM) from a variety of treatment options., Methods: All patients with CVM who received care and had follow-up between February 1st 2018 and January 31st 2020 were included. The health-related QoL, pain, and mental health were assessed with RAND Health Care 36-Item Short Form Survey (SF-36), visual analogue score for pain (VAS-P) and Hospital Anxiety and Depression Scale (HADS). Paired t-test was used for all analyses. p < .05 were considered significant., Results: In total, 110 patients with a mean age of 36.9 years were included in this study. In all patients following care, significant improvement was found in the bodily pain domain of SF-36 and VAS-P (both p = .01). This was largely driven by high-flow vascular malformation patients who responded better to embolo-sclerotherapy, which revealed significant improvement in the bodily pain domain of SF-36 ( p = .002) and VAS-P ( p = .02). Patients who received supportive treatment only reported significant improvement in mental health ( p = .004) and social functioning ( p = .03) domains of SF-36. Meanwhile, patients treated with embolo-sclerotherapy reported significant improvement only in VAS-P ( p = .02)., Conclusions: This study concluded that the effects of care on early follow-up QoL, pain and mental health of patients with CVM were heterogenous. Future research should therefore, include larger sample size and longer term follow-up to understand the various factors that affect the QoL and mental health of these patients, as well as the holistic approaches to manage them.

Objective: To analyse the image characteristics of fibro-adipose vascular anomaly (FAVA) and the value of ultrasound and magnetic resonance imaging (MRI) for its diagnosis., Methods: The clinical and imaging data characteristics of 10 patients with FAVA admitted to our hospital between January 2018 and December 2020 who underwent ultrasound and MRI diagnosis were retrospectively analysed., Results: A total of 10 patients (six males and four females) with pathologically confirmed FAVA, aged from 3 to 27 years (median: 13 years), underwent ultrasound and MRI; of these, two patients underwent MRI-enhanced examinations. All lesions involved mainly muscle, with a few disruptions and involvements of subcutaneous fat. Five cases were located subcutaneously near the fascia; one case was adjacent to the periosteum. Ultrasound showed fascial tail in seven cases, and MRI showed fascial tail in six cases. Both ultrasound and MRI showed the lesions to be oval-shaped masses with blurred borders and a mean maximum diameter of 99.8 ± 48.7 mm. Ultrasound showed a mass with a mixture of high and low echoic areas. Magnetic resonance imaging imaging showed a heterogeneous, mixed-signal intensity on T1-weighted images, probably influenced by the fibrous and fatty components of the lesion. T2-weighted images showed a mixed-high signal. Enhanced computed tomography scans showed significant heterogeneous enhancement of the lesions., Conclusion: Both ultrasound and MRI showed that fascial tail sign is an important imaging feature for FAVA disease; this provides a reliable basis for its diagnosis and can be used to distinguish it from venous malformations, which have no fascial tail signs. Therefore, fascial tail signs can be used as imaging features and require special attention in the diagnosis of FAVA disease.

Objectives: This study aimed to retrospectively review imaging findings and the outcomes of uterine artery embolisation (UAE) in symptomatic uterine vascular anomalies (UVA)., Methods: This study included a total of 15 patients with acquired UVA admitted to the Aga Khan University Hospital in Karachi, Pakistan, from 2010 to 2020. These patients were evaluated using ultrasound, computed tomography and magnetic resonance imaging, either alone or in combination. All patients had a history of dilatation and curettage or uterine instrumentation and underwent angiography and embolisation of the uterine arteries. The primary outcome post embolisation was assessed clinically and/or in combination with ultrasound. Post-procedure pregnancies were also recorded., Results: Non-invasive imaging was abnormal in all patients; however, this pre-intervention imaging was unable to accurately classify the type of vascular anomaly, except in the case of a pseudoaneurysm. Conventional angiography showed uterine artery hyperaemia in six patients, arteriovenous malformation in seven patients and pseudoaneurysm in two patients. The technical success rate was 100% and no repeat embolisation was needed. The follow-up ultrasound in 12 patients revealed a resolution of the abnormal findings, while the remaining three were found to be normal on clinical follow-up. Seven patients (46.7%) had a normal pregnancy 15.7 months after the procedure (range: 4-28 months)., Conclusions: UAE is a safe and effective management option for intractable severe bleeding in patients with UVA post instrumentation and it was found that the procedure does not impair future pregnancy., Competing Interests: CONFLICT OF INTEREST The authors declare no conflict of interest., (© Copyright 2023, Sultan Qaboos University Medical Journal, All Rights Reserved.)

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.

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.)

Objective: To evaluate the clinical efficacy of bleomycin lavage combined with N-butyl-2-cyanoacrylate glue embolization and resection in the treatment of orbital vascular malformations. Methods: It was a retrospective case series study. Patients with orbital vascular malformations diagnosed at the Ophthalmology Division of Chinese PLA General Hospital from January 2018 to October 2021 were included and divided into exophthalmos group and non-exophthalmos group based on whether the patients had postural exophthalmos. Intralesional bleomycin injection and N-butyl-2-cyanoacrylate glue embolization were performed. The preoperative and postoperative visual acuity, the dosages of bleomycin and isobutyl cyanoacrylate glue, pathological results, imaging findings and remission rate were recorded and analyzed. The Chi-square test, Wilcoxon signed rank analysis and Mann-Whitney U test were used for statistical analysis. Results: A total of 58 patients (58 eyes)were included, and there were 22 males (37.9%)　and 36 females (62.1%). Nineteen (32.8%) patients had postural exophthalmos, and 39 (67.2%) patients did not suffer postural exophthalmos. The patient ' s age of the two groups was 39.0 (28.0, 54.5) years vs. 14.0 (5.7, 26.5) years, with a statistically significant difference ( Z =-3.96, P <0.001). There was no significant difference in gender, eye laterality, follow-up time and the disease course between the two groups (all P >0.05). During the operation, the dosage of bleomycin was 15 000 (13 500, 15 000) U in the exophthalmos group, and 15 000 (9 000, 16 500) U in the non-exophthalmos group ( Z =-0.70, P =0.944). The dosages of N-butyl-2-cyanoacrylate glue were 2.8 (1.0, 3.0) ml and 1.7 (1.0, 2.2) ml, respectively, in the two groups, with no significant difference ( Z =-1.11, P =0.268). There was no visual impairment in both groups, while the visual acuity in 5 patients without postural exophthalmos was improved postoperatively. The imaging examination results showed no difference in the malformed vascular area before and after the treatment in the exophthalmos group [384.0 (329.0, 458.0) mm 2 vs. 330.5 (271.6, 356.7) mm 2 ; Z =-1.26, P =0.208], but a significantly decreased area after the treatment in the non-exophthalmos group [960.8 (822.1, 1058.3) mm 2 vs. 311.6 (164.6, 361.6) mm 2 ; Z =-2.67, P =0.008]. All patients had no obvious local or systemic adverse reactions during the follow-up. The pathology reports showed vascular malformations in all 15 specimens obtained from the exophthalmos group, as well as vascular malformations in 41.0% (16/39) of specimens and venous lymphatic malformations in 59.0% (23/39) of specimens from the non-exophthalmos group. Thirty-nine patients had complete remission (67.2%), 19 patients had partial remission (32.8%), and the effective treatment rate was 100%. Conclusion: Bleomycin lavage combined with N-butyl-2-cyanoacrylate glue embolization can achieve good therapeutic effects on orbital vascular malformations.

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.

Background/aim: Vascular malformations are congenital abnormalities that result from disturbances in the embryologic development of the vascular system. A retrospective study at a single institution was performed to determine the localization and treatment patterns for vascular malformations in children., Patients and Methods: A total of 198 pediatric patients were identified. Age at diagnosis and presentation, sex, localization, diagnostics, and therapy were described., Results: The most common diagnosis was lymphatic malformation (LM, 58.6%), followed by venous (VM, 31.8%) and arteriovenous malformation (AVM, 4.5%). The mean age at diagnosis was 2.2 years, while the mean age at presentation at our hospital was 7.2 years. The sex ratio showed a female predominance (1.44:1), which was most evident in children with AVM. The neck, cheek/parotid gland and oral cavity were the most predominant locations. Half of the patients required at least one intervention at our hospital. Especially, CM and LM were managed by watch-and wait, whereas lymphovenous malformation (LVM) and AVM were most often treated. Treatment differed between the various malformation types, the most common used treatment was conventional surgery followed by laser therapy. In case of treatment, the average number of procedures in our hospital was 1.58 for VM, 1.53 for LM, 1.33 for AVM, and 1.0 for LVM., Conclusion: In children with vascular malformations interventional treatment is often necessary, in many cases more than one treatment step is needed. Correct identification of the malformation type is important for optimal treatment and appropriate care of patients with vascular malformations., (Copyright © 2023, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

We describe the case of a term newborn who presented with congenital testicular torsion at 10 hours of age. During the evaluation of this problem, additional malformations were encountered. Diagnostic and therapeutic considerations are addressed.

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.)

Background/objective: Oral malformations of the tongue are exceedingly rare. The aim of this study was to evaluate the effectiveness of individualized treatment for patients with vascular malformations of the tongue., Methods: This retrospective study is based on a consecutive local registry at a tertiary care Interdisciplinary Center for Vascular Anomalies. Patients with vascular malformations of the tongue were included. Indications for therapy of the vascular malformation were macroglossia with the impossibility to close the mouth, bleeding, recurrent infection and dysphagia. Size regression of the malformation (volume measurement) and symptom improvement were investigated., Results: Out of 971 consecutive patients with vascular malformations, 16 patients suffered from a vascular malformation of the tongue. Twelve patients had slow-flow malformations and 4 fast-flow malformations. Indications for interventions were bleeding (4/16, 25%), macroglossia (6/16, 37.5%), and recurrent infections (4/16, 25%). For two patients (2/16, 12.5%), there was no indication for intervention due to absence of symptoms. Four patients received sclerotherapy, 7 patients Bleomycin-electrosclerotherapy (BEST) and 3 patients embolization. Median follow-up was 16 months (IQR 7-35.5). In all patients, symptoms had decreased after two interventions at a median (IQR 1-3.75). Volume reduction of the malformation of the tongue was 13.3% (from median 27.9 cm3 to median 24.2 cm3, p = 0.0039), and even more pronounced when considering only patients with BEST (from 86 cm3 to 59.1 cm3, p = 0.001)., Conclusion: Symptoms of vascular malformations of the tongue are improved after a median of two interventions with significantly increased volume reduction after Bleomycin-electrosclerotherapy.

Vascular anomalies of the head and neck is an evolving field, with more recent focus on identifying and understanding the underlying genetic and molecular causes for these lesions. Medical therapies for some of these vascular anomalies have been developed. Many complex vascular anomalies require multimodality therapy, and other lesions could be treated with any of a variety of the available therapies. High-quality outcomes research and establishing clinical practice guidelines to help guide management are essential., Competing Interests: Disclosure No disclosures or conflicts of interest., (Copyright © 2022 Elsevier Inc. All rights reserved.)

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.)

Vascular anomalies are a heterogenous group of vascular lesions that can be divided, according to the International Society for the Study of Vascular Anomalies Classification, into two main groups: vascular tumors and vascular malformations. Vascular malformations can be further subdivided into slow-flow and fast-flow malformations. This clinical and radiological classification allows for a better understanding of vascular anomalies and aims to offer a more precise final diagnosis. Correct diagnosis is essential to propose the best treatment, which traditionally consists of surgery, embolization, or sclerotherapy. Since a few years, medical treatment has become an important part of multidisciplinary treatment. Genetic and molecular knowledge of vascular anomalies are increasing rapidly and opens the door for a molecular classification of vascular anomalies according to the underlying pathways involved. The main pathways seem to be PI3K/AKT/mTOR and RAS/RAF/MEK/ERK. Knowing the underlying molecular cascades allows us to use targeted medical therapies. The first part of this article aims to review the vascular anomalies seen in the head and neck region and their underlying molecular causes and involved pathways. The second part will propose an overview of the available targeted therapies based on the affected molecular cascade. This article summarizes theragnostic treatments available in vascular anomalies., (© 2022 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Vascular anomalies of the head and neck comprise a wide spectrum of phenotypically diverse lesions. Optimal diagnosis and management of these lesions are critically dependent upon establishment of uniform and well-defined histopathologic, clinical, and radiological criteria, but these remain subject of debate. In this paper, we describe the International Society for the Study of Vascular Anomalies classification scheme, which was first published in 1996 and updated in 2014. The strength of this proposal rests on its distinction between vascular malformations and tumors, and is responsible for its wide adoption. This paradigm serves as a developing platform for diagnosis, inter-collegial communication, and treatment, and adhering to it will help clinicians to improve the management of vascular anomalies., (© 2022 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

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: Vascular anomalies affect up to 5% of children with the majority affecting the head and neck. They present at different ages as a wide variety of lesions. A careful evaluation with history, physical examination, and imaging assists in the proper diagnosis. Depending upon the condition treatment options for vascular anomalies include topical therapy, selective photothermolysis, sclerotherapy, embolization, surgical excision, and targeted systemic therapy., Conclusion: Staged multimodal therapeutic regimens have proven to best control disease and allow for the preservation of function and aesthetics. The timing, sequence, and combination of therapies are best determined by a multidisciplinary vascular anomalies team. Patients and families need to be counseled on anticipated positive outcomes following a protracted course of treatment for the majority of vascular anomalies., (© 2022 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Vascular anomalies (VAs) are morphogenesis defects of the vascular system (arteries, capillaries, veins, lymphatic vessels) singularly or in complex combinations, sometimes with a severe impact on the quality of life. The progress made in recent years with the identification of the key molecular pathways (PI3K/AKT/mTOR and RAS/BRAF/MAPK/ERK) and the gene mutations that lead to the appearance of VAs has allowed the deciphering of their complex genetic architecture. Understanding these mechanisms is critical both for the correct definition of the phenotype and classification of VAs, as well as for the initiation of an optimal therapy and the development of new targeted therapies. The purpose of this review is to present in synthesis the current data related to the genetic factors involved in the etiology of VAs, as well as the possible directions for future research. We analyzed the data from the literature related to VAs, using databases (Google Scholar, PubMed, MEDLINE, OMIM, MedGen, Orphanet) and ClinicalTrials.gov. The obtained results revealed that the phenotypic variability of VAs is correlated with genetic heterogeneity. The identification of new genetic factors and the molecular mechanisms in which they intervene, will allow the development of modern therapies that act targeted as a personalized therapy. We emphasize the importance of the geneticist in the diagnosis and treatment of VAs, as part of a multidisciplinary team involved in the management of VAs.

Venous malformations are the most common congenital vascular malformations. Because venous malformations can be complex, difficult to treat, and associated with late complications, it is important to know the basics of the different types of venous malformations and clinical differential diagnosis. Patients with complex lesions may be best served by a specialty vascular anomalies clinic., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: Current data on the nature and rate of major complications for embolo-sclerotherapy (EST) of vascular malformations are scarce. However, even fewer studies focus on vascular malformations specific to the head and neck, which confer an increased specific risk of airway compromise, neurologic and ophthalmologic injury. More understanding is required surrounding the type and incidence of complications to improve treatment planning and informed consent. Therefore, this study aimed to review major complications secondary to EST of head and neck vascular malformations over a 5-year period in a single specialized multidisciplinary centre for vascular anomalies., Methods: All interventions were decided by the multidisciplinary team. Demographic, procedural and complication data between 1st January 2013 and 31st December 2017 were prospectively documented in a dedicated database and analysed. EST of high-flow vascular malformations (HFVMs) was performed by selective catheter angiography or direct injection, and by direct injection only for low-flow vascular malformations (LFVMs). Major complications were defined as any tissue or functional damage caused by direct injection, distal embolization or tissue reaction and were decided by the multidisciplinary team., Results: Forty-eight patients (median age of 35 years; range of 14-70 years; 18 men and 30 women) had 100 EST procedures for head and neck vascular malformation. Of these, 14 patients had EST for HFVM and 34 patients for LFVM, total 43 and 57 procedures, respectively. Overall, five patients with HFVM developed major complications from EST when compared with two patients with LFVM ( p = 0.0167). Two patients required pre-emptive tracheostomy due to risk of post-operative airway compromise. Overall, seven (14.6%) patients experienced major complication from EST. In the HFVM group, major complications from EST occurred in five patients; four cases of tissue ulceration and necrosis (two needed debridement, one healed with resultant fibrosis that impeded speech and one resolved spontaneously) and one post-procedural airway compromise requiring tracheostomy. Meanwhile, in the LFVM group, major complications occurred in two patients; one case of severe necrosis involving the alar cartilage, lip and cheek requiring debridement and reconstruction under plastics and one simple cellulitis. No patients sustained stroke or vision impairment., Conclusions: EST is relatively safe for head and neck vascular malformations in a high-volume experienced centre. Our major complication rate of 14.6% per patient (35.7% for HFVM; 5.9% for LFVM) or 7% per procedure (11.6% for HFVM; 3.5% LFVM) compares favourably with published data from other centres. These data will improve treatment planning and informed consent for EST for both HFVM and LFVM of the head and neck.

Introduction: Genetically targeted drugs in vascular anomalies (VA) are used despite the absence of a validated severity score. The aim of this study was to evaluate the feasibility of grouping phenotypic VA clinical characteristics into a single severity score., Methods: A systematic literature review including children treated with sirolimus accompanied by a detailed description of phenotype and management was conducted. Demographic data and clinical features were extracted to define distinct categories of phenotypes., Results: Children with VA display two main phenotypes regardless of VA subtype, which may overlap. A systemic phenotype results from direct invasion and compression of vital structures generally leading to hospitalization and aggressive management in infancy. A functional phenotype is associated with chronic pain and disability manifesting mainly during early adolescence and managed in the outpatient setting., Conclusion: The two distinct phenotypes described could be the basis for developing a unified scoring system for VA severity assessment., (© 2022 Wiley Periodicals LLC.)

The treatment of vascular malformations and vascular anomalies is often complex, combining various approaches in the art of medicine to provide best outcomes and quality of life for these patients. Treatment may include but is not limited to the following: local control with compression garments and attire, pain control, surgical procedures and debulking, laser therapy, sclerotherapy, and medical management. In this article, the authors discuss the aspects of medical management, visiting the history of medical treatment, and the recent utilization and success of enzymatic pathway inhibitors, specifically sirolimus and new therapies that hold promise for the future for these patients., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Vascular anomalies encompass a spectrum of tumors and malformations that can cause significant morbidity and mortality in children and adults. Use of the International Society for the Study of Vascular Anomalies (ISSVA) classification system is strongly recommended for consistency. Vascular anomalies can occur in isolation or in association with clinical syndromes that involve complex multifocal lesions affecting different organ systems. Thus, it is critical to be familiar with the differences and similarities among vascular anomalies to guide selection of the appropriate imaging studies and possible interventions. Syndromes associated with simple vascular malformations include hereditary hemorrhagic telangiectasia, blue rubber bleb nevus syndrome, Gorham-Stout disease, and primary lymphedema. Syndromes categorized as vascular malformations associated with other anomalies include Klippel-Trenaunay-Weber syndrome, Parkes Weber syndrome, Servelle-Martorell syndrome, Maffucci syndrome, macrocephaly-capillary malformation, CLOVES ( c ongenital l ipomatous o vergrowth, v ascular malformations, e pidermal nevi, and s coliosis, skeletal, and spinal anomalies ) syndrome, Proteus syndrome, Bannayan-Riley-Ruvalcaba syndrome, and CLAPO ( c apillary malformations of the lower lip, l ymphatic malformations of the face and neck, a symmetry of the face and limbs, and p artial or generalized o vergrowth) syndrome. With PHACES ( p osterior fossa malformations, h emangiomas, a rterial anomalies, c ardiac defects and/or coarctation of the aorta, e ye abnormalities, and s ternal clefting or supraumbilical raphe) syndrome, infantile hemangiomas associated with other lesions occur. Diagnostic and interventional radiologists have important roles in diagnosing these conditions and administering image-guided therapies-embolization and sclerotherapy, and different ablation procedures in particular. The key imaging features of vascular anomaly syndromes based on the 2018 ISSVA classification system and the role of interventional radiology in the management of these syndromes are reviewed. Online supplemental material is available for this article. © RSNA, 2022.

Vascular tumors are classified into three categories by the International Society for the Study of Vascular Anomalies (ISSVA): benign, locally aggressive/borderline, and malignant. Many of these tumors are rare, cutaneous in nature, and present in childhood. The characterization and delineation of these distinct vascular tumors is an evolving area of clinical research. The diagnosis of these lesions relies on history and clinical presentation, location, histologic appearance, immunohistochemistry, and more recently, associated genetic mutations. This article provides a brief, yet comprehensive overview of all cutaneous vascular tumors currently recognized by the ISSVA, including presentation, diagnosis, and treatment., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Vascular anomalies are highly variable in their angioarchitecture, location, and flow dynamics. An individualized, multidisciplinary approach to treatment is required, focusing on improving patient quality of life. With appropriate percutaneous or endovascular treatment, patient satisfaction following interventional therapy is generally high, acknowledging that a complete cure may not always be possible., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Capillary malformations (CMs) are the most common vascular anomalies, composed of enlarged capillaries and venules with thickened perivascular cell coverage in skin and mucous membranes. These congenital anomalies represent an error in vascular development during embryogenesis. Most of the CMs occur without any syndromic findings; the association between CMs systemic anomalies in some patients, however, makes the recognition of additional syndrome features critical. Some genetic disorders discussed, which feature CMs, include Sturge-Weber syndrome, diffuse CMs with overgrowth, Klippel-Trenaunay syndrome, CLOVES syndrome, among others. This article can aid clinicians in better identifying CMs and associated syndromes and provide consistent terminology to facilitate interdisciplinary management., (Copyright © 2022 Elsevier Inc. All rights reserved.)

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.)