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Objectives: The aim of this study was to assess the value of CO 2 laser vaporization in treating huge adult laryngeal vascular anomalies (HALVAs) by combining it with bleomycin injection., Materials and Methods: This study retrospectively reviewed the records of 13 adult patients who underwent 18 different procedures. Methods to treat HALVAs include traditional bleomycin injection and CO 2 laser vaporization combined with bleomycin injection between September 2009 and January 2023. Treatment results were evaluated by the grade of lumen constriction., Results: A total of five males and eight females, with an average age of 46.3 years (range, 22-66 years), were included in the study. The huge adult laryngeal vascular anomalies in our study were greater than 1633.71 mm 3 , and the long diameters of the bases were longer than 15 mm. Compared with the bleomycin injection-only group, the results with the CO 2 laser vaporization and bleomycin injection combined were better., Conclusions: Both bleomycin injection and CO 2 laser vaporization are safe treatment methods. Their combination may produce better results for huge adult laryngeal vascular anomalies., (© 2024 Wiley Periodicals LLC.)

A boy in his mid-teens presented with progressively increasing bleeding from the right eye and nostril intermittently over a period of 6 weeks. A complete ophthalmic examination revealed nothing significant. His otorhinological examination and haematological investigations were within normal limits. The patient came a month later with frank bleeding from the right eye. Ophthalmic examination revealed hyperaemia and maceration of the right lower palpebral conjunctiva. A histopathological examination of conjunctival scrapings from the site showed abnormal dilated blood vessels suggestive of a vascular malformation. Digital subtraction angiography confirmed the presence of a conjunctival micro arteriovenous malformation supplied by the external carotid and ophthalmic artery branches. He underwent successful transarterial Onyx embolisation resulting in complete resolution of the haemolacria., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Photodynamic therapy (PDT) is a minimally invasive therapy that was gradually established as a first-line treatment for vascular abnormalities. Its action depends on the appropriate wavelength of light and photosensitizer to produce toxic oxygen species and cause cell death. Objective: Several new clinical improvements and trends in PDT have been described in recent years. The aim of this review is to provide an overview of the current data from clinical trials. Methods: In this review, we introduce and generalize the wavelength, duration, dose, strength, and photosensitizer of PDT for the treatment of vascular abnormalities, such as circumscribed choroidal hemangiomas (CCH), choroidal neovascularization (CNV) and capillary malformation (CM). Results: The systematic review findings indicate that the application of PDT is a safe effective method to treat CCH, CNV and CM. However, PDT also has early onset side effects and late onset side effects. Conclusions: Based on the discussion of the effectiveness of PDT, we conclude that PDT has great potential for clinical use, although PDT has possible side effects.

Craniomaxillofacial vascular anomalies encompass a diverse and complex set of pathologies that may have a profound impact on pediatric patients. They are subdivided into vascular tumors and vascular malformations depending on biological properties, clinical course, and distribution patterns. Given the complexity and potential for leading to significant functional morbidity and esthetic concerns, a multidisciplinary approach is generally necessary to optimize patient outcomes. This article reviews the etiology, clinical course, diagnosis, and current management practices related to vascular anomalies in the head and neck., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

A series of randomized controlled trials (RCTs) have advanced the therapeutic approaches for vascular anomalies (VA). However, a notable obstacle in applying the findings of these trials to real-world patient care is trial waste (TW). To date, the extent of TW in RCTs for VA is not clear. In June 2024, we searched the ClinicalTrials database using the entity names defined by ISSVA classification as search terms. We documented the data available and then explored PubMed and Scopus for the publication status. Reporting adequacy was evaluated using the CONSORT checklist. Design limitations were analyzed based on bias risk and whether the article referenced a relevant systematic review. One hundred fifty-nine RCTs met the inclusion criteria. The majority of RCTs focused on benign VA (81.1%) and utilized pharmacotherapy (79.9%). Over 90% of these trials were conducted in North America, Europe, and Asia as single-center studies (68.6%), with funding primarily from official institutions (83.7%). The analysis of TW excluded 61 RCTs completed after June 2020 that remained unpublished. Among the remaining 98 RCTs, 53 were published, 41 had adequate reporting, and 16 had design limitations. In total, 67 RCTs exhibited at least one characteristic of TW. The 31 RCTs without waste tended to enroll more participants (P = 0.014) and conduct studies across multiple centers (P < 0.001) and countries (P = 0.022). Multicenter participation (P = 0.028) emerged as an independent protective factor against TW., Conclusion: We delineated the features of 159 VA RCTs and revealed that 68.4% of them exhibited TW. The diverse traits of the different TW indicators identified could serve as valuable insights for conducting future VA RCTs more rationally and efficiently., What Is Known: • Currently, a number of RCTs have been conducted on vascular anomalies (VA). However, there has been no study analyzing the situation of trial waste in VA-related RCTs., What Is New: • This study is the first to describe the characteristics of VA-related RCTs globally over the past 20 years and has identified a high burden of trial waste in this field. Multicenter participation was an independent protective factor against trial waste., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Over the past several centuries, the integration of contemporary medical techniques and innovative technologies, like genetic sequencing, have played a pivotal role in enhancing our comprehension of congenital vascular and lymphatic disorders. Nonetheless, the uncommon and complex characteristics of these disorders, especially considering their formation during the intrauterine stage, present significant obstacles in diagnosis and treatment. Here, we review the intricacies of these congenital abnormalities, offering an in-depth examination of key diagnostic approaches, genetic factors, and therapeutic methods., Competing Interests: Disclosures None.

Background: Despite the efficacy of absolute ethanol (EtOH), its radiolucency introduces several risks in interventional therapy for treating vascular malformations. This study aims to develop a novel radiopaque ethanol injection (REI) to address this issue., Methods: Iopromide is mixed with ethanol to achieve radiopacity and improve the physicochemical properties of the solution. Overall, 82 male New Zealand white rabbits are selected for in vivo radiopacity testing, peripheral vein sclerosis [animals were divided into the following 5 groups (n = 6): negative control (NC, saline, 0.250 ml/kg), positive control (EtOH, 0.250 ml/kg), low-dose REI (L-D REI, 0.125 ml/kg), moderate-dose REI (M-D REI, 0.250 ml/kg), and high-dose REI (H-D REI 0.375 ml/kg)], pharmacokinetic analyses (the blood sample was harvested before injection, 5 min, 10 min, 20 min, 40 min, 1 h, 2 h, 4 h, and 8 h after injection in peripheral vein sclerosis experiment), peripheral artery embolization [animals were divided into the following 5 groups (n = 3): NC (saline, 0.250 ml/kg), positive control (EtOH, 0.250 ml/kg), L-D REI (0.125 ml/kg), M-D REI (0.250 ml/kg), and H-D REI (0.375 ml/kg)], kidney transcatheter arterial embolization [animals were divided into the following 4 groups (n = 3): positive control (EtOH, 0.250 ml/kg), L-D REI (0.125 ml/kg), M-D REI (0.250 ml/kg), and H-D REI (0.375 ml/kg); each healthy kidney was injected with saline as negative control], and biosafety evaluations [animals were divided into the following 5 groups (n = 3): NC (0.250 ml/kg), high-dose EtOH (0.375 ml/kg), L-D REI (0.125 ml/kg), M-D REI (0.250 ml/kg), and H-D REI (0.375 ml/kg)]. Then, a prospective cohort study involving 6 patients with peripheral venous malformations (VMs) is performed to explore the clinical safety and effectiveness of REI. From Jun 1, 2023 to August 31, 2023, 6 patients [age: (33.3 ± 17.2) years] with lingual VMs received sclerotherapy of REI and 2-month follow-up. Adverse events and serious adverse events were evaluated, whereas the efficacy of REI was determined by both the traceability of the REI under DSA throughout the entire injection and the therapeutic effect 2 months after a single injection., Results: The REI contains 81.4% ethanol (v/v) and 111.3 mg/ml iodine, which can be traced throughout the injection in the animals and patients. The REI also exerts a similar effect as EtOH on peripheral venous sclerosis, peripheral arterial embolization, and renal embolization. Furthermore, the REI can be metabolized at a similar rate compared to EtOH and Ultravist® and did not cause injury to the animals' heart, liver, spleen, lungs, kidneys and brain. No REI-related adverse effects have occurred during sclerotherapy of VMs, and 4/6 patients (66.7%) have achieved complete response at follow-up., Conclusion: In conclusion, REI is safe, exerts therapeutic effects, and compensates for the radiolucency of EtOH in treating VMs., Trial Registration: The clinical trial was registered as No. ChiCTR2300071751 on May 24 2023., (© 2024. The Author(s).)

Fibro-Adipose Vascular Anomaly (FAVA) is a recently identified type of vascular malformation predominantly affecting adolescent females. Comprising abnormal adipose and vascular components, FAVA is frequently misdiagnosed as other vascular anomalies. It primarily manifests with pain, functional impairment, and musculoskeletal symptoms, particularly in the lower extremities. Accurate diagnosis requires a combination of clinical, radiologic, and histopathologic evaluation, with MRI and ultrasound being the primary imaging tools. Management of FAVA is multidisciplinary and tailored to individual patients. Interventional radiology procedures, such as percutaneous cryoablation, sclerotherapy, and embolization, are effective in long term control of symptoms. Cryoablation is particularly successful in alleviating pain and improving function. Surgical resection is reserved for specific cases with extensive lesions involving joints or when there is severe muscle or joint dysfunction. Additionally, sirolimus, an mTOR inhibitor, has shown promise in symptom relief, although further research is needed to confirm its long-term efficacy. Early diagnosis and treatment are essential for improving the quality of life in FAVA patients. Advances in imaging and treatment strategies have enhanced the ability to manage this complex and rare condition effectively., (Copyright © 2024. Published by Elsevier Inc.)

Overgrowth syndromes, particularly within the PIK3CA-related overgrowth syndrome (PROS) spectrum, are commonly associated with venous anomalies. The anomalies include spongiform venous malformations and persistent embryonic veins, such as the lateral marginal vein (of Servelle). The anomalous veins pose a significant risk of thromboembolic disease and should be occluded, preferably earlier in life. A thorough understanding of the conditions, anatomy, and interdisciplinary treatment of these complex anomalies is essential for optimal management. This review explores the clinical and imaging diagnosis of overgrowth syndromes and techniques for assessing and treating associated venous anomalies, particularly the endovenous closure of anomalous veins., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Venous malformation (VM) stands as the most prevalent form of vascular malformation, characterized by its diverse morphology. These lesions can manifest in any part of the body, affecting different tissue planes and giving rise to symptoms such as pain, swelling, or physical dysfunction. In the realm of treatment, direct puncture VM sclerotherapy holds its place as the primary approach. This technique involves the administration of a sclerosing agent into the VM channels during contrast phlebography while simultaneously managing the outflow veins through different methods. The process of VM sclerotherapy induces endothelial damage, thrombosis, and fibrosis, resulting in symptom relief through lesion shrinkage. It is crucial to exercise caution techniques and sclerosing agents during VM sclerotherapy to minimize procedural complications, enhance clinical outcomes, and ultimately improve the patient's overall quality of life., (Copyright © 2024. Published by Elsevier Inc.)

Background: Lower extremity deep venous insufficiency (DVI) occurs secondary to structural or functional abnormalities of deep venous valves in the affected extremities. The effectiveness of surgical treatment for improvement of the hemodynamic status in these patients remains controversial in clinical practice., Method: In this case report, we describe a patient who presented with severe right lower extremity edema and liposclerosis and underwent venography, which suggested a variation in the number of femoral veins. The valve within the duplicated femoral vein was significantly incompetent; however, the valve of the main trunk of the femoral vein showed normal function. We performed embolization of the duplicated femoral vein., Results: The patient tolerated the procedure well without recurrent symptoms., Conclusions: Individualized assessment based on venography findings is useful to establish the therapeutic approach in patients with DVI., 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.

Background: Klippel-Trenaunay-Weber syndrome (KTWS) is a rare disease with a wide range of manifestations. KTWS is characterized by a clinical triad of varicosities of the extremities, cutaneous vascular malformations, and hypertrophy of soft tissues and long bones. The diagnosis is made clinically supplemented with magnetic resonance imaging and computed tomography., Aim: Hereby we aim to highlight the significance of the possible life-threatening first-time presentations associated with the GI system in previously undiagnosed KTWS patients., Patient: We report the case of a 47-year-old male with KTWS, who presented with various symptoms such as rectorrhagia since childhood, digestive problems and abnormal lateral vascular malformations of the left buttock which extended all the way to the leg, vascular malformations of the left fourth and fifth toes as well as soft tissue swelling of the left foot. There was no evidence of other clinical presentations. The patient was hospitalized with severe rectorrhagia and a hemoglobin level of 3/9. Physical examination revealed a blood pressure of 85/55 and pulse rate of 115. Ruptured aneurysm of the superior mesenteric artery was found on angiography and subsequently treated with embolization. Dermatologic evaluation showed pitting edema of the left leg and foot and multiple vascular lesions. Thus a diagnosis of KTWS was established. Pulsed dye laser therapy and compression bandage was performed for the patient. The patient's follow-up was done 3 months after discharge for which the patient was again consulted by a dermatologist and gastroenterologist. Lymphedema of the left leg had improved to a great extend so treatment with compression bandage was continued. Colonoscopy was repeated for the patient to evaluate and control possible active sources of bleeding, due to potential life-threating complications., Results: According to previous findings, there have been few case reports of KTWS presenting with gastrointestinal manifestations, fewer of which have covered acute life-threatening bleedings associated with this system., (© 2024 The Authors. Journal of Cosmetic Dermatology published by Wiley Periodicals LLC.)

Vascular anomalies in the head and neck area are usually rare diseases and pose a particular diagnostic and therapeutic challenge. They are divided into vascular tumours and vascular malformations. A distinction is made between benign tumours, such as infantile haemangioma, and rare malignant tumours, such as angiosarcoma. Vascular malformations are categorised as simple malformations, mixed malformations, large vessel anomalies and those associated with other anomalies. Treatment is interdisciplinary and various modalities are available. These include clinical observation, sclerotherapy, embolisation, ablative and coagulating procedures, surgical resection and systemic drug therapy. Treatment is challenging, as vascular anomalies in the head and neck region practically always affect function and aesthetics. A better understanding of the genetic and molecular biological basis of vascular anomalies has recently led to clinical research into targeted drug therapies. This article provides an up-to-date overview of the diagnosis, clinic and treatment of vascular anomalies in the head and neck region., Competing Interests: S.M. gibt folgendes an: Firma Medtronic GmbH, Meerbusch - Dozenten und Beratertätigkeit; Firma Olympus, Hamburg - Dozenten und Beratertätigkeit I.W. gibt an, dass kein Interessenkonflikt besteht., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)

Purpose: To investigate quantitative changes in MRI signal intensity (SI) and lesion volume that indicate treatment response and correlate these changes with clinical outcomes after percutaneous sclerotherapy (PS) of extremity venous malformations (VMs)., Methods: VMs were segmented manually on pre- and post-treatment T2-weighted MRI using 3D Slicer to assess changes in lesion volume and SI. Clinical outcomes were scored on a 7-point Likert scale according to patient perception of symptom improvement; treatment response (success or failure) was determined accordingly., Results: Eighty-one patients with VMs underwent 125 PS sessions. Treatment success occurred in 77 patients (95 %). Mean (±SD) changes were -7.9 ± 24 cm 3 in lesion volume and -123 ± 162 in SI (both, P <.001). Mean reduction in lesion volume was greater in the success group (-9.4 ± 24 cm 3 ) than in the failure group (21 ± 20 cm 3 ) (P =.006). Overall, lesion volume correlated with treatment response (ρ = -0.3, P =.004). On subgroup analysis, volume change correlated with clinical outcomes in children (ρ = -0.3, P =.03), in sodium tetradecyl sulfate-treated lesions (ρ = -0.5, P =.02), and in foot lesions (ρ = -0.6, P =.04). SI change correlated with clinical outcomes in VMs treated in 1 PS session (ρ = -0.3, P =.01) and in bleomycin-treated lesions (ρ = -0.4, P =.04)., Conclusions: Change in lesion volume is a reliable indicator of treatment response. Lesion volume and SI correlate with clinical outcomes in specific subgroups., 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 B.V. All rights reserved.)

The diagnosis of vascular anomalies remains challenging due to significant clinical heterogeneity and uncertain etiology. Evaluation using biopsy and/or genetic testing for somatic variants is invasive, expensive, and prone to sampling error. There is great need for noninvasive and easily measured blood laboratory biomarkers that can aid not only in diagnosis, but also management of treatments for vascular anomalies. Angiopoietin-2, a circulating blood angiogenic factor, is highly elevated in patients with kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon and kaposiform lymphangiomatosis. Here, we describe our clinical experience using serum angiopoietin-2 as a biomarker for diagnosis and monitoring response to treatment., (© 2024 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

Vascular malformations can present with a variety of symptoms and an unpredictable course with the occurrence of wounds. Ulcerations in patients with vascular malformations are fortunately rare. Although few data exist, complications may involve a variety of mechanistic or hemodynamic factors. A rigorous etiological and vascular assessment is therefore essential. In view of the paucity of recommendations, the Wound and Healing Group of the French Society of Vascular Medicine, based on the literature on the subject, presents a number of suggestions for the diagnosis and management of wounds associated with vascular malformations., (Copyright © 2024 The Author(s). Published by Elsevier Masson SAS.. All rights reserved.)

Vascular anomalies represent a diverse group of disorders of abnormal vascular development or proliferation. Vascular anomalies are classified as vascular tumors and vascular malformations. Significant advances have been made in the understanding of the pathogenesis, natural history, and genetics of vascular anomalies, allowing for improvements in management including targeted molecular therapies. Infantile hemangiomas are the most common vascular tumor of childhood and follow a distinct natural history of proliferation and involution. Although benign, infantile hemangiomas can be associated with important complications. The use of beta-blockers has revolutionized the management of infantile hemangiomas. Other vascular tumors include pyogenic granulomas, congenital hemangiomas, and kaposiform hemangioendotheliomas, among others. Vascular malformations are categorized based on the type of involved vessel, including capillary malformations, venous malformations, lymphatic malformations, arteriovenous malformations, and mixed vascular malformations. Expert multidisciplinary management of vascular anomalies is critical to optimize outcomes in these patients. [ Pediatr Ann . 2024;53(4):e129-e137.] .

Nanotechnology has demonstrated immense potential in various fields, especially in biomedical field. Among these domains, the development of nanotechnology for diagnosing and treating vascular anomalies has garnered significant attention. Vascular anomalies refer to structural and functional anomalies within the vascular system, which can result in conditions such as vascular malformations and tumors. These anomalies can significantly impact the quality of life of patients and pose significant health concerns. Nanoscale contrast agents have been developed for targeted imaging of blood vessels, enabling more precise identification and characterization of vascular anomalies. These contrast agents can be designed to bind specifically to abnormal blood vessels, providing healthcare professionals with a clearer view of the affected areas. More importantly, nanotechnology also offers promising solutions for targeted therapeutic interventions. Nanoparticles can be engineered to deliver drugs directly to the site of vascular anomalies, maximizing therapeutic effects while minimizing side effects on healthy tissues. Meanwhile, by incorporating functional components into nanoparticles, such as photosensitizers, nanotechnology enables innovative treatment modalities such as photothermal therapy and photodynamic therapy. This review focuses on the applications and potential of nanotechnology in the imaging and therapy of vascular anomalies, as well as discusses the present challenges and future directions., (© 2024. The Author(s).)

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.)

A young male patient presented with an incidental finding of a large supraglottic vascular lesion. The lesion was initially noted during intubation 4 years ago. Although originally listed for elective excision, there was a significant delay and at the time of surgery, the lesion proved too large to remove and a significant threat to the patient's airway. An emergency tracheostomy was performed, followed by two consecutive treatments with sclerotherapy agents to reduce the size of the lesion. It was then successfully excised using a Thunderbeat ultrasound and bipolar dissection and cautery device., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

The discovery of inherited and somatic genetic mutations, along with advancements in clinical and scientific research, has improved the understanding of vascular anomalies and changed the treatment paradigm. With the aim of minimizing the need for invasive procedures and improving disease outcomes, molecularly targeted medications and anti-angiogenesis agents have become important as both adjuncts to surgery, and increasingly, as the primary treatment of vascular anomalies. This article highlights the commonly used and emerging therapeutic medications for nonmalignant vascular tumors and vascular malformations., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Pediatric intracranial arteriovenous shunts are rare vascular malformations that can be diagnosed prenatally or postnatally, as an incidental finding or due to complications. We propose a review of cerebral vascular malformations in newborns and infants with special emphasis on neurosonography and Doppler ultrasound as the first diagnostic method. Sonography can thus contribute in the planning of further studies that are always necessary, and in post-therapy follow-up., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Discovery of inherited and somatic genetic mutations, along with advancements in clinical and scientific research, has improved understanding of vascular anomalies and changed the treatment paradigm. With aim of minimizing need for invasive procedures and improving disease outcomes, molecularly targeted medications and anti-angiogenesis agents have become important as both adjuncts to surgery, and increasingly, as the primary treatment of vascular anomalies. This article highlights the commonly used and emerging therapeutic medications for non-malignant vascular tumors and vascular malformations in addition to medical management of associated hematologic abnormalities., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Venous malformations (VMs) are the most common vascular malformations, which can be classified as focal, multifocal, or diffuse types. But the risk of focal venous malformations with muscle involvement is not well defined., Methods: This is a single-center, retrospective review of patients treated for focal VMs between February 2021 and February 2022., Results: We assessed 26 patients focal VMs with 47 lesions; 18 (69%) were unifocal, 3 (12%) were dual-focal, and 5 (19%) were multifocal type VMs, and 29 (62%) were intramuscular VMs. The lower limbs intramuscular VMs had a significantly elevated risk of focal VMs (relative risk [RR],1.7; 95% confidence interval [CI], 1.148-2.394)., Conclusion: Intramuscular involvement of the body should be considered in focal VMs. The lower limbs intramuscular VMs had a significantly elevated risk of focal VMs., 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.

Vascular anomalies, broadly classified as nonmalignant tumors and malformations, consist of a multitude of disorders that have a wide range of symptoms and complications as well as overlapping clinical, radiologic, and histologic findings. Although usually difficult, distinguishing between nonmalignant vascular tumors and malformations, as well as the precise diagnosis within these distinctions, is critical because prognosis, therapy, and chronicity of care vary greatly. In contrast to normal endothelial turnover in vascular malformations, vascular tumors are characterized by the abnormal proliferation of endothelial cells and aberrant blood vessels., Competing Interests: Disclosure The authors have no conflicts of interest to disclose., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Purpose:  Analysis of clinical and diagnostic findings in rare urogenital and perineal vascular malformations only occurring in 2-3 % of vascular anomalies with regard to clinical symptoms and treatment decisions., Materials and Methods:  All 25 out of 537 patients presenting with congenital urogenital and perineal vascular malformations at our institution from 2014 to 2021 were included. Vascular anomaly classification, anatomical location, clinical symptoms at presentation, diagnostic imaging, and pain intensity were retrospectively assessed from the patient record and therapy management was evaluated., Results:  In total, 25 patients (10 females (40 %), 15 males (60 %)), aged 6 to 77 years were included. Diagnoses were: 10 (40 %) venous malformations (VMs), 5 (20 %) lymphatic malformations (LMs) and 10 (40 %) arteriovenous malformations (AVMs). Malformation manifestations were: 12 (32 %) lesser pelvis, 12 (32 %) external genitalia, and 13 (34 %) perineal/gluteal region. One AVM was located in the kidney. The leading clinical symptom was pain. The mean intensity was 6.0/10 for LM, 5.7/10 for VM, and 4.5/10 for AVM. Further major symptoms included physical impairment, local swelling, and skin discoloration. Bleeding complications or sexual dysfunction were rare findings. Patients with VM reported significantly more symptoms than patients with AVM (p = 0.0129). In 13 patients (52 %) minimally invasive therapy was indicated: 10 (77 %) sclerotherapies and 3 (23 %) transcatheter embolization procedures. Complete symptomatic remission was achieved in 9 (69 %) patients, partial response in 3 (23 %) patients, and 1 patient showed no clinical response to therapy. Follow-up appointments without the need for immediate minimally invasive therapy were significantly more common in patients with AVMs than in patients with VMs (p = 0.0198)., Conclusion:  To create a higher awareness of congenital urogenital and perineal vascular malformations. Awareness of this rare condition avoids misdiagnosis. Therapy decisions should be symptom-oriented. Emergency intervention is rarely required, even in fast-flow vascular malformations., Key Points:   · Venous malformations cause more symptoms with higher pain intensity than arteriovenous malformations.. · Diagnosis and adequate treatment can be hampered by a lack of awareness of the clinical presentation.. · Bleeding complications are rare, even in high-flow vascular malformations.. · Pain and physical impairment are the most commonly observed symptoms in these patients.., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

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.)

Background: Vascular malformations are rare diseases that should be treated in dedicated vascular anomaly centers (VAC). There is only a small amount of data on the diagnostic and therapeutic handling of these patients, before they are referred to a VAC., Purpose: To demonstrate the disease-specific patient characteristics in a German VAC, which are required to determine diagnostic and therapeutic steps., Material and Methods: In a retrospective study, all patients who were treated in the VAC from April 2014 until August 2021 were identified. In total, 593 patients were included in this study., Results: Almost all patients had previously consulted a physician (591/593, 99.7%). A mean of two different physicians had been consulted (range 0-10). Patients with more complex, syndromal vascular malformations had significantly more previous appointments ( P  = 0.0018). In only 44% (261/593) of patients, the referral diagnosis was made correctly. Most patients had been previously treated for their vascular anomaly: pharmacotherapy (n = 130; 21.9%), compression garments (n = 141; 23.8%), surgical resection (n = 80; 17.3%) and sclerotherapy (n = 68; 11.5%). Fifty-two patients who had been falsely diagnosed had also received therapy prior to their referral to the VAC (8.8%). Most patients received an ultrasound examination in the VAC (n = 464; 78.2%). Most frequently, compression therapy was prescribed (n = 256; 43.2%), followed by sclerotherapy (n = 175, 29.5%) and pharmacotherapy (n = 55; 9.3%)., Conclusion: Patients suffering from vascular anomalies often go through a complicated scheduling with numerous outpatient appointments and have a high risk of misdiagnosis and mistreatment prolonging the medical condition. Therefore, patients with vascular anomalies should be treated in a dedicated vascular anomaly center., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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.

Aim: Congenital tumors of the tongue are rare in pediatric patients but encompass a diverse range of entities. Each tumor type exhibits distinct clinical behaviors, necessitating a precise approach to differentiating the tumor types and a tailored, tumor-specific treatment regimen. Advanced imaging techniques, such as diffusion-weighted imaging and perfusion studies, play a vital role in differentiating benign and malignant tongue tumors. This review summarizes current knowledge regarding the presentation, imaging features, and treatment of congenital tongue tumors., Methods: A literature review was conducted by searching studies on congenital tongue tumors in databases such as PubMed, Embase, Web of Science, and Scopus. Relevant data, such as clinical features, radiologic characteristics, treatment modalities, and outcomes for different tumor types, were extracted from the selected articles., Results: Our literature review reveals the various entities of congenital tongue tumors, which can be categorized in terms of hereditary pattern, phenotype, and rarity. Congenital tongue tumors include a range of vascular malformations, such as hemangiomas, lymphatic malformations, arteriovenous malformations, and venous malformations. Another entity is represented by cystic lesions, including dermoid cysts, epidermoid cysts, ranulas, and mucous retention cysts. Rare malignant neoplasms include teratomas and rhabdomyosarcomas. These tumor types vary in terms of swelling, respiratory distress, or impaired oral function, depending on size and location. The detection of these tumors can be carried out using imaging modalities, such as ultrasound, magnetic resonance imaging, and computed tomography, which are utilized to facilitate diagnosis and differentiation. At present, surgical excision remains the cornerstone of treatment, while other modalities may be adopted, depending on tumor type and extent. The prognosis of congenital tongue tumors can be affected by tumor's site, size, involvement of vital structures, and malignancy., Conclusions: Given their diversity and complexity, congenital tongue tumors, albeit uncommon, require specialized clinical treatments tailored to each tumor type's characteristics. Understanding the variable presentations and imaging features enables accurate diagnosis, while customized treatment strategies are key to optimizing outcomes and minimizing morbidity in pediatric tongue tumors. This review summarizes current knowledge aimed at enhancing differential diagnosis and management of these diverse entities.

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.)

Purpose: To assess the effectiveness, safety, and predictors of outcomes and adverse events for percutaneous sclerotherapy using polidocanol for the treatment of venous malformations (VMs)., Methods: A retrospective single-center analysis was performed, including patients with VMs who were treated with sclerotherapy using polidocanol between January 2011 and November 2021 at a tertiary center. Demographic characteristics, clinical data, and radiologic features were analyzed, and the influence of patient- and VM-related factors on the subjective clinical outcome and adverse events were investigated using a multivariate logistic regression analysis., Results: In total, 167 patients who received 325 treatment sessions were included in this study. Overall symptom improvement was observed in 67.5%, stable symptoms were observed in 25.0%, and worsening was reported in 7.5% (clinical follow-up, 1.04 ± 1.67 years). The total adverse event rate was 10.2%, with an overall rate of 4.2% for permanent adverse events within the cohort. In multivariate analysis, the clinical outcome was worse in children (P = .01; 57.1% symptom improvement in children [age, <18 years] and 79.7% in adults), and adverse events were more frequently observed after the treatment of VMs located at the extremities (P < .01; 8.4% for VMs of the extremities and 1.2% for VMs in other locations)., Conclusions: Sclerotherapy using polidocanol can be an effective treatment option for VMs with an acceptable safety profile. However, it can be less effective in children, and adverse events can be more frequently expected for VMs of the extremities., (Copyright © 2023 SIR. Published by Elsevier Inc. All rights reserved.)

Vascular anomalies consist of tumours or malformations made up of abnormal growth or collections of blood vessels that can result in functional or cosmetic problems. While many vascular anomalies are present at birth, some do not appear until later in life, making diagnosis more challenging. Although many vascular anomalies are benign, some are associated with serious complications and may involve multiple organ systems. This article highlights the important features of clinically significant vascular anomalies to help physicians promptly identify and refer these cases to a specialised multidisciplinary team for evaluation and management. The discussion includes the various presenting complaints of vascular anomalies in children, namely, rapidly growing birthmarks, painful lesions, seizures/neurological manifestations, bleeding diathesis, cardiac/airway abnormalities and part of an overgrowth syndrome., Competing Interests: None

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.)

Objective: Puberty has been shown to accelerate growth of vascular malformations, including lymphatic (LM) and venous malformations (VM). This study aims to compare the number of procedures performed before and after puberty in patients with LM and VM to assess whether the onset of puberty results in higher treatment frequency., Methods: A retrospective review of head and neck LM and VM patients who were evaluated between January 2009 and December 2019 was performed. Patient demographics, lesion characteristics, and procedural details were recorded. For the purposes of this study, 11 years or older in females and 12 years or older in males were the established cut-offs for the onset of puberty., Results: After initial screening of 357 patients, 83 patients were included in the study based on inclusion criteria. There were 34 patients with LM (41 %) and 49 with VM (59 %). The mean age at diagnosis was 6.1 ± 10.9 years (LM: 4.2 ± 7.0, VM: 7.4 ± 12.9, p = 0.489). 68 patients underwent treatments, which included sclerotherapy, surgical excision, and/or laser. For all patients, the average number of lifetime treatments when initiated before puberty was 3.78 ± 2.81 and when initiated after puberty was 2.17 ± 1.37 (p = 0.022). Patients diagnosed pre-puberty were more likely to undergo treatments vs. those diagnosed after puberty (OR 10.00, 95 % CI: 2.61-38.28, p < 0.001)., Conclusion: We found that the number of treatments was fewer in those who started treatment after puberty. This finding suggests that providers may elect to proceed with observation in asymptomatic patients, given that waiting until after the onset of puberty has not shown an increase in the procedural load on patients., (Copyright © 2023 Elsevier Inc. All rights reserved.)

A cross-sectional study was performed to evaluate health-related quality of life (HRQOL) in children with congenital vascular malformations (CVM) and to investigate factors associated with an impaired HRQOL. Children (2-17 years) with CVMs who visited the HECOVAN expertise center between 2016-2018 were included. The PedsQL 4.0 Generic Core Scales were used and a score ≥ 1.0 SD below the normative mean was defined as an impaired HRQOL. Factors associated with impairment were investigated using univariate and multivariate logistic regression analysis. The median overall HRQOL was 84.8/100 (n = 207; 41% boys, 59% girls; self-reported IQR 73.9-92.4 and parent-reported IQR 71.4-92.4). Patients aged 13-17 years reported significantly worse physical functioning than those aged 8-12 years (median 84.4, IQR 71.1-93.8 versus median 90.6, IQR 81.3-96.9; p = 0.02). Parents reported a significantly lower overall HRQOL than their children (median 80.4, IQR 70.7-90.8 versus median 85.9, IQR 76.1-92.4; p = 0.001). HRQOL was impaired in 25% of patients. Impairment occurred significantly more often in lower extremity CVMs (38%, p = 0.01) and multifocal CVMs (47%, p = 0.01) compared to CVMs in the head/neck region (13%). Other associated factors included invasive management (31% versus 14%; p = 0.01), age at first treatment ≤ 5 years (48% versus 25%; p = 0.02) and ongoing treatment (38% versus 18%; p = 0.004). After correction for other factors, significance remained for lower extremity CVMs and ongoing invasive treatment., Conclusions: Overall median HRQOL was reasonable and not significantly different from the norm sample. Parental ratings were significantly lower than their children's ratings. A quarter of the patients had an impaired HRQOL, which seemed to worsen with age. Independently associated factors included a lower extremity CVM and invasive management., What Is Known: • Congenital vascular malformations could affect health-related quality of life (HRQOL). • Studies on pediatric patients are limited and either very small or in combination with adult patient series., What Is New: • This study raises awareness of an impaired HRQOL in 25% of pediatric patients with congenital vascular malformations. • Associated factors included a lower extremity CVM and invasive management., (© 2023. The Author(s).)

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).)

Objective: To characterize the current distribution, composition, and practice patterns of multidisciplinary vascular anomalies (VAs) teams in the US., Study Design: This is a cross-sectional survey of children's hospitals in the US offering VAs care. We approached 142 children's hospitals that provided care for VAs via email. The survey evaluated VA clinic location, medical staffing, research participation, and treatments offered. The survey was administered between October 2021 and July 2022., Results: Participants from 95 eligible hospitals responded to the survey (response rate = 67%). Large areas of the Midwest and Northwest US had no available multidisciplinary VA teams or clinics. Most respondents worked at academic centers (89%), with 66% at a freestanding children's hospital, and 56% reported having a multidisciplinary clinic. Most common physician participants in clinic included hematology-oncology (91%), interventional radiology (87%), dermatology (85%), plastic surgery (81%), and otolaryngology (74%). Only 38% of programs included medical geneticists. Smaller hospitals had fewer medical and ancillary staff and offered fewer therapeutic options. Research was available at most larger institutions (69%) but less commonly at smaller hospitals (34%)., Conclusions: Major portions of the US lack multidisciplinary VA care. Furthermore, VA programs vary in composition and geneticists are absent from the majority of programs. These findings should inform efforts to address disparate access and develop standards of care for multidisciplinary VA care in the US., Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interests., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Vascular malformations are due to abnormal development of blood and/or lymphatic vessels during embryonic life without endothelial cell proliferation. Most of the previous treatments were symptomatic methods as surgery and sclerotherapy because the pathogenic mechanism was not clearly understood. With advances in molecular biology, the pathogenesis of vascular malformations is thought to be related to inherited and/or somatic mutations that eventually activate the PI3K/ATK/mTOR, Ras/Raf/MEK/ERK pathways. Also, related studies have promoted the use of targeted inhibitors. This article provides a review of current causative genes and targeted drugs for pediatric vascular malformations, aiming to provide a basis for promoting accurate molecular diagnosis and precision targeted therapy for these diseases.

This study aims to examine transition of care (TOC) practices of multidisciplinary vascular anomalies centers (VACs). Thirty-seven of 71 VAC leaders to whom the survey was sent completed the questionnaire. TOC and transfer practices varied with only 16% of VACs having TOC programs. The most frequently cited barriers to developing a TOC program were lack of resources and difficulty finding expert adult providers., (© 2023 Wiley Periodicals LLC.)

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 less 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 for diagnosis and treatment. Part 2 of this review discusses embolization, endovenous laser ablation, and image-guided percutaneous biopsy. Please see Part 1 for a discussion of sclerotherapy and cryoablation. Select vascular anomalies will be discussed as examples to highlight IR diagnostic and/or treatment techniques., (© 2022 Wiley Periodicals LLC.)

Sinus pericranii is a rare vascular anomaly involving a venous sinus that drains into a subgaleal collection of veins through an emissary vein. Data regarding presentation, management, and outcomes are limited to case reports and small case series. There are no technical videos detailing the technique for percutaneous embolization. We present the case of a child with an enlarging, symptomatic accessory type sinus pericranii with connection to the torcula, who underwent percutaneous embolization after unsuccessful transvenous embolization. Embolization was performed with 3.4 cc Onyx-34 under live fluoroscopy and serial control superior sagittal sinus venograms . Significant reduction of flow into the sinus pericranii was achieved and the lesion had nearly completely resolved at the 3-week follow-up. Percutaneous embolization of the sinus pericranii is a reasonable alternative to transvenous embolization, but additional data are needed to determine the optimal treatment. The technical details and practical considerations discussed here may help neurointerventionalists adopt this treatment. The video also includes references 1-4 which are relevant to this topic. neurintsurg;15/8/828/V1F1V1Video 1Case presentation and technique for percutaneous embolization of sinus pericranii., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To evaluate the efficacy of sclerotherapy for congenital vascular malformation (CVM) using a combination of polidocanol foam and bleomycin liquid., Methods: A retrospective review of a prospectively collected data on patients who had sclerotherapy for CVM from May 2015 to July 2022 was performed., Results: A total of 210 patients with a mean age of 24.8 ± 2.0 years were included. Venous malformation (VM) was the most common type of CVM, accounting for 81.9% (172/210) of all patients. At 6 months follow-up, the overall clinical effective rate was 93.3% (196/210), and 50% (105/210) of patients were clinically cured. The clinical effective rates in VM, lymphatic, and arteriovenous malformation group were 94.2%, 100%, and 100%., Conclusion: Sclerotherapy using a combination of polidocanol foam and bleomycin liquid is an effective and safe treatment for venous and lymphatic malformations. It is a promising treatment option with satisfactory clinical outcome in arteriovenous malformations.

Depending on the diagnostic modality, the classification of vascular anomalies varies and so does the nomenclature. The 'International Society for the Study of Vascular Anomalies' (ISSVA) is the most widely accepted classification in the literature and is mainly based on the radiologic and clinical presentation. The aim of this article is to review the clinical practice of diagnosis and treatment of vascular anomalies in the head and neck region in a university hospital, with special focus on the nomenclature. All patients with a vascular anomaly presenting to the department of oral and maxillofacial surgery were reviewed in a retrospective manner. Nomenclature, diagnostic process, lesion characteristics, treatment and outcome were examined. The lesions were (re)classified according to the ISSVA classification. A total of 185 patients were identified, of which 12.4% (n = 23) had a congenital anomaly. After reclassification, the most common lesions were venous malformations (n = 47, 25.4%), followed by lobular capillary hemangiomas (n = 17, 9.2%). A group of 39 anomalies could not be further specified. One hundred and one patients (54,6%) received treatment, of which 93 were treated surgically (92,1% of treated patients). Endovascular treatment was considered in 41 patients but applied in only eight. This strict selection led to a low a complication rate. We provide an overview of the clinical practice in the management of vascular anomalies in a university hospital. The histology report is a source of miscommunication because clinicians use the ISSVA classification, while pathologists use the WHO classification. Every professional involved should be aware of the differences in classification and nomenclature., (© 2023. The Author(s).)

Introduction:  The objective of this study is to summarize the clinical characteristics and management of rare diseases of colorectal vascular malformation (CRVM) in children., Methods:  We retrospectively analyzed the clinical data of CRVM patients admitted to the Children's Hospital of Fudan University from 2004 to 2019., Results:  A total of 23 cases (16 males, 7 females) were enrolled. The median age of symptom onset was 1.4 years. Hematochezia and anemia were cardinal symptoms. Fourteen patients (60.9%) were misdiagnosed as anal fissures ( n  = 4), internal hemorrhoids ( n  = 3), rectal polyps ( n  = 2), inflammatory bowel disease ( n  = 2), portal hypertension ( n  = 2), and Meckel's diverticulum ( n  = 1), respectively. The average time from symptom onset to diagnosis was 4.5 ± 4.4 years. Other vascular malformations were detected in eight patients (34.8%). All patients showed a positive anomalous vascular image on contrast-enhanced computed tomography (CT) or magnetic resonance imaging (MRI). The sensitivity of colonoscopy in the diagnosis of CRVM was 82.6% (19/23). A total of 21 patients underwent a modified Soave procedure. The lesions were mostly restricted to the colorectum and showed transmural diffuse distribution, with an average length of 20 ± 5.4 cm. Two patients (9.5%) experienced surgical complications. Bloody stools reappeared in two patients (9.5%), and colonoscopy showed abnormal angiogenesis at the anastomotic site, which were cured by sclerotherapy and/or electrocautery. The median follow-up time was 78 months. Bloody stools were absent at the last time of follow-up, and hemoglobin was in the normal range for all patients., Conclusion:  The identification of CRVM in children often is delayed. Colonoscopy, CT, and MRI are essential in making the correct diagnosis. The modified Soave procedure is safe and feasible to treat CRVM in children. Endoscopic sclerotherapy and/or electrocautery can be used for residual lesions., Competing Interests: None declared., (Thieme. All rights reserved.)

Vascular malformations are congenital vascular anomalies that originate because of disorganized angiogenesis, most commonly from spontaneous somatic genetic mutations. The modern management of vascular malformations requires a multidisciplinary team that offers patients the gamut of medical, surgical, and percutaneous treatment options with supportive care. This manuscript discusses the standard and contemporary management strategies surrounding extracranial vascular malformations and overgrowth syndromes., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)