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Competing Interests: Competing interests: None declared.

Purpose: There is paucity of data on patient-perceived outcomes of bleomycin sclerotherapy for low-flow vascular malformations. In this study, the long-term outcomes of bleomycin sclerotherapy were investigated in terms of quality of life (QoL) and patient-perceived changes in health., Materials and Methods: A cohort of Dutch patients with vascular malformations treated with bleomycin sclerotherapy (June 2010-November 2015) completed a questionnaire evaluating disease symptoms, QoL (Short Form 36), patient-perceived change in health status (Global Rating of Change scales) and treatment satisfaction. QoL was assessed for the patient's status before and after treatment and was analyzed relative to an age and sex-matched Dutch reference population. Predictive factors associated with QoL and patient-perceived improvement in overall health status were assessed using multivariable linear and logistic regression analyses, respectively., Results: Seventy-seven patients, with a median follow-up of 22 months, were enrolled. About half of the respondents (49.3%) indicated that they perceived (any form of) improvement in their overall health status. Most often improved were the specific health aspects 'pain' (54.5%) and 'overall severity of symptoms' (57.1%). No factors were significantly predictive for patient-perceived improvement in health with respect to the vascular malformation. Impairment in work- or study-related activities prior to sclerotherapy was found to negatively impact physical QoL at follow-up (p = 0.03)., Conclusion: Approximately half of patients with low-flow vascular malformations indicate an improvement in overall health status following bleomycin sclerotherapy, particularly concerning pain and severity of symptoms. However, most patients only perceived little to moderate improvement to their health and desire further treatment.

Purpose: Multiple treatment options have been described for intramuscular venous malformations (VMs) of the limbs. At the current time, there are no clear management guidelines. The aim was to evaluate efficacy and safety of sclerotherapy in this type of VMs., Materials and Methods: This is a single-center, retrospective review of patients treated for extremity intramuscular VMs between January 2013 and June 2017. The primary outcomes were the improvement of symptoms self-assessed by questionnaires, and the reduction in VM size measured by magnetic resonance (MRI)., Results: Sclerotherapy was performed in 81 patients with extremity intramuscular VMs. The sclerosing agent was ethanol in 46 cases (56.8%), polidocanol in 27 cases (33.3%), a combination of both in 8 cases (9.9%). The mean follow-up was 26 months (range 3-52). Overall quality of life was improved in 62 patients (76.5%). The postoperative MRI showed a minimum change of VM size in 68 patients (83.9%). A major complication (peripheral nerve injury) was observed in 1 case (1.2% of patients, 0.5% of procedures). Minor complications occurred in 9 cases (11.1% of patients, 4.1% of procedures)., Conclusions: Sclerotherapy is a low-invasive, effective and safe treatment for intramuscular VMs of the extremities. It induces a significant improvement in symptoms, also when the VM size is unchanged., Level of Evidence: Level 4, Case Series.

Vascular malformations are poorly recognized constitutional anomalies which arises during early childhood. Several classifications tried to draw a distinction across the different entities. Recent advances in molecular biology have contributed to the update of their nosology. Syndromic vascular malformations are an example: while Klippel-Trenaunay syndrome, Proteus or CLOVES syndrome share many common features, understanding of pathological mechanism and specially the role of the PIK3/AKT/mTOR pathway enables us to rethink their classification. Then, some syndromes associated with overgrowth and vascular malformation have been grouped under a single term: "PIK3CA-related overgrowth spectrum" (PROS), and this group continues to grow. This new approach suggests new treatment options. Rapamycin, a PIK3/AKT/mTOR pathway inhibitor, demonstrated its efficiency for some forms of PROS. Targeted therapies such as PIK3 or mTOR selective inhibitor are still in a developmental phase and results are encouraging. This is an example of personalized medicine with significant therapeutic benefit for some patients. However, genotype relation with therapeutic efficiency must be clarified and physicians should pay attention to possible negative effects of these drugs, especially for young patients., (Copyright © 2018 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

The recent International Society for the Study of Vascular Anomalies (ISSVA) classification of vascular anomalies can be applied to orbital lesions, dividing them into vascular tumors and vascular malformations. Orbital cavernous hemangiomas are probably best considered cavernous venous malformations under this classification. Management of symptomatic lesions can be with surgical excision or stereotactic fractionated radiotherapy in selected cases. Beta-blockers including propranolol and topical timolol maleate represent first-line therapy for infantile hemangiomas, although surgery has a role in selected cases. Orbital venous-lymphatic malformations are problematic but with improved imaging, neuroradiological intervention, and a multidisciplinary approach to management, outcomes are improving., (Copyright 2018 Asia-Pacific Academy of Ophthalmology.)

Background:  Vascular anomalies are a diagnostic and therapeutic challenge. They require dedicated interdisciplinary management. Optimal patient care relies on integral medical evaluation and a classification system established by experts in the field, to provide a better understanding of these complex vascular entities., Method:  A dedicated classification system according to the International Society for the Study of Vascular Anomalies (ISSVA) and the German Interdisciplinary Society of Vascular Anomalies (DiGGefA) is presented. The vast spectrum of diagnostic modalities, ranging from ultrasound with color Doppler, conventional X-ray, CT with 4 D imaging and MRI as well as catheter angiography for appropriate assessment is discussed., Results:  Congenital vascular anomalies are comprised of vascular tumors, based on endothelial cell proliferation and vascular malformations with underlying mesenchymal and angiogenetic disorder. Vascular tumors tend to regress with patient's age, vascular malformations increase in size and are subdivided into capillary, venous, lymphatic, arterio-venous and combined malformations, depending on their dominant vasculature. According to their appearance, venous malformations are the most common representative of vascular anomalies (70 %), followed by lymphatic malformations (12 %), arterio-venous malformations (8 %), combined malformation syndromes (6 %) and capillary malformations (4 %)., Conclusion:  The aim is to provide an overview of the current classification system and diagnostic characterization of vascular anomalies in order to facilitate interdisciplinary management of vascular anomalies., Key Points:   · Vascular anomalies are comprised of vascular tumors and vascular malformations, both considered to be rare diseases.. · Appropriate treatment depends on correct classification and diagnosis of vascular anomalies, which is based on established national and international classification systems, recommendations and guidelines.. · In the classification, diagnosis and treatment of congenital vascular anomalies, radiology plays an integral part in patient management.., Citation Format: · Sadick M, Müller-Wille R, Wildgruber M et al. Vascular Anomalies (Part I): Classification and Diagnostics of Vascular Anomalies. Fortschr Röntgenstr 2018; 190: 825 - 835., Competing Interests: Disclosure The authors report no conflicts of interest in this work., (© Georg Thieme Verlag KG Stuttgart · New York.)

Congenital portosystemic shunt (CPS) is classified into type I (congenital absence of the portal vein) and type II, depending on the presence or absence of the intrahepatic portal vein. Reports still exist on cases in which psychiatric diseases or mental retardation was diagnosed or cases in which diagnosis and treatment are not performed until severe pulmonary complications occur, indicating the need to recognize CPS. Herein, we reviewed epidemiology, etiology, classification, symptom, diagnosis, and treatment based on clinical view points of CPS. For clinical view points, classification according to (1) the presence or absence of the intrahepatic portal venous system (IPVS) and (2) the extrahepatic or intrahepatic of shunt sites, facilitates the understanding of pathophysiological conditions and is useful in selecting a treatment for symptomatic CPS. Radiological and pathological examinations are important in IPVS evaluations, and IPVS evaluations are currently essential to make diagnoses by portography with balloon occlusion and liver biopsy. Symptomatic CPS (hepatic nodular lesions, portosystemic encephalopathy, and pulmonary complications, etc.) is an indication of treatment by shunt closure, but an indication of treatment for asymptomatic CPS is the challenge going forward., (© 2018 Japanese Society of Hepato-Biliary-Pancreatic Surgery.)

Congenital portosystemic shunts (CPSS) are rare vascular malformations that create an abnormal connection between portal and systemic veins resulting in complete or partial diversion of the portal flow away from the liver to the systemic venous system. Different anatomic types exist and several classifications have been proposed. They can be associated with other malformations especially cardiac and heterotaxia. The main complications include hepatic encephalopathy, liver tumors, portopulmonary hypertension, and pulmonary arteriovenous shunts. Diagnosis relies on imaging, and prenatal diagnosis is possible. Spontaneous closure of the CPSS is possible in some anatomic forms during the first year of life. When the CPSS remains patent, radiologic or surgical closure of the CPSS may prevent, resolve, or stabilize complications. Interventional radiology plays a key role for both the preoperative evaluation with occlusion test to assess the exact anatomy and to measure portal pressure after occlusion of the CPSS. Endovascular closure is the first option for treatment when possible.

Background Limited information is available on mid-term results and quality of life (QOL) after endovascular sclerotherapy of venous malformations. Purpose To compare two agents-polidocanol and ethanol-with a focus on the influence on QOL after sclerotherapy. Material and Methods Forty-one consecutive patients with a venous malformation in the head and neck area or in the extremities were treated with polidocanol between 2008 and 2013. Pre- and post-treatment magnetic resonance imaging (MRI) scans were compared. All patients completed a self-evaluation form on symptoms as well as a QOL questionnaire. The results were compared with previously obtained material during 1991-2001, comprising 44 consecutive, similarly located venous malformation patients subject to ethanol sclerotherapy. Results No significant clinical complications were observed. Subjectively, 19 (46%) of the patients benefitted from the treatment. QOL results showed that 85% of patients had an index < 39 - where 0 represents the highest and 100 the lowest QOL. Patients in the ethanol group had marginally better overall post-treatment QOL results. Post-treatment MRI in 35 patients showed the size of the malformation unchanged in 19 (54%) patients, in ten (29%) there was a decrease (<50%) while in six (17%) the decrease was more significant (>50%). Post-treatment MRI results did not correlate with either subjective symptoms or QOL results. Conclusion Polidocanol sclerotherapy were found to be an effective, safe, and well tolerated treatment option for low flow venous malformations. Routine MRI for follow-up appears redundant and may be omitted.

Objective: Sclerotherapy has become an important treatment option in the management of vascular malformations. However, little is known about success rate for treatment of venous malformations. This systematic review assesses the available published literature on outcome measures of sclerotherapy for venous malformations., Data Source: PubMed and EMBASE., Review Method: A systematic search was conducted, and studies from March 2008 to October 2016 were included. Based on the identified search results, study selection, data extraction, and assessment of study quality were, according to PRISMA, undertaken independently by two reviewers., Results: One randomized controlled trial and 44 cohort studies were included. The most frequently used measuring methods were subjective measurements, objective measurements, imaging like MRI and ultrasound, and finally patient reported outcome measure survey. Approximately 60% of the studies used more than one measuring method. Outcome measures were pain, swelling, disfigurement, cosmetic complains, patient satisfaction, physical difficulties, volume, diameter, mass, color, shape, size, venous flow, occlusion of venous space, blood pool ratio, and radioisotope uptake., Conclusions: The published literature over the past 10 years shows no clear method to evaluate the effect of sclerotherapy for venous malformation. We suggest that a standard set of outcome measures should be defined.

Orbital venous malformation (OVM) ranks as the most common form of vascular lesions in the orbit. OVM may cause functional impairment as well as disfigured appearance in orbital and periorbital regions. A variety of treatment methods has been used to deal with OVM, including sclerotherapy, laser therapy, embolization, surgical resection. The treatment scenario should be made based on hemodynamics, location and extent of lesions in order to achieve encouraging results. With increased understanding on hemodynamics of OVM and application of newly proposed techniques, a combined modality of multiple approaches with defined order has been advocated to be applied in the treatment of OVM. In this paper, the latest advancement in treatment of OVM is reviewed. (Chin J Ophthalmol, 2018, 54: 548-550) .

Objective: To summarize the treatment outcomes of embolizing orbital vascular malformation with intracavitary injection of medical glue during surgery. Methods: A retrospective case series study was performed on 25 patients with orbital vascular malformation who were treated at Tianjin Medical University Eye Hospital during March 2008 and March 2016. In the 25 patients(25 eyes), 7 were male and 18 were female. The range of age was 2-64 years and the median age was 29 years. The clinical features, operation records, pathological reports and follow-up data were analyzed. Results: The location of vascular malformation involved intraorbital in 11 cases and superficial area of eyelid and(or) face in 5 cases, as for the rest 9 cases, both intraorbital and superficial area were involved. Conjunctiva was involved in 4 patients. Clinical manifestation included intermittent protopsis or mass volumn changed (7 cases), pulsating exophthalmos and vascular murmur (1 case). Imaging examination showed solitary mass with regular shape in 5 cases and space occupying lesion with irregular shape and ill-defined margins in 20 cases. Optic nerve was involved in 7 cases. Surgical debulkling were performed via skin incision on the mass surface (in 3 cases), lateral orbitotomy (in 2 cases), and anterior orbitotomy (in 20 cases). The anterior orbitotomy approaches include skin incision under eyebrow (9 cases), skin incision under lower eyelid eyelash (3 cases), transconjunctiva joint lateral canthus incision (6 cases) and transconjunctiva incision only(2 cases). The intra-orbital part of vascular malformation involved intraconal compartment (10 cases) and extraconal compartment (10 cases). During the operation, vascular malformations were exposed and injected with medical aural and encephalic glue. The amount of injected glue ranged from 0.25 ml to 2.50 ml in divided doses. When the soft venous malformations turned hard, the lesions and remnant glue were fully removed. The whole procedure cause less bleeding and shorter time of operation. Histopathologic diagnosis were intraosseous hemangioma (1 case), arteriovenous malformation (2 cases), venous malformation (22 cases), including varix (4 case) and venous hemangioma (18 cases). One patient suffered from sudden central retinal artery embolism on the third day postoperatively, in which case, visual acuity recovered to 0.6 by timely rescue and appropriate procedure. Topical skin aseptic inflammation took place at the same side of medical glue injection in 3 cases which might be caused by medical glue. These 3 cases had superficial eyelid lesions. Postoperative follow up for patient was conducted from 6 months to 5 years. One young patient underwent recurrent orbital hematoma and many times surgery. Conclusions: Embolization of orbital vascular malformation with medical glue injection intraoperatively can be utilized as an easy approach to control hemorrhage. The surgeon should be careful with the application methods, for instance not to push too quickly, not to pass through the vascular malformation, and to remove the lesions and remnant glue completely, in order to avoid complications. (Chin J Ophthalmol, 2018, 54: 502-508) .

Venous malformations are the most common of all vascular anomalies. Venous malformations are treated using a multidisciplinary approach with sclerotherapy representing the primary treatment modality. While rare, sclerotherapy in the central nervous system carries the risk of venous thrombosis, non-target embolization, and venous infarction and hemorrhage. Options for controlling venous outflow when treating venous malformations in the head and neck are limited. This report details the use of balloon and coil occlusion of a superior sagittal sinus cortical vein to facilitate percutaneous sclerotherapy of a supraorbital venous malformation.

Hematochezia is a rare clinical presentation of congenital extrahepatic portosystemic shunt (CEPS). We describe a series of three patients with type II CEPS presenting as hematochezia that were treated by catheter embolization, followed by a brief review of published articles. Hematochezia of the patients was due to the giant inferior mesenteric vein, superior rectal vein and colonic varices. The catheter embolization was successfully accomplished in all of the patients. After a mean follow-up of 27 months, no serious adverse effects were observed. For unexplained massive hematochezia, CEPS needs to be considered as a differential diagnosis. Based on our present results and the review of the literature, transcatheter permanent embolization of the giant inferior mesenteric vein might be an effective and safe treatment for type II CEPS.Level of Evidence Level 4, case series.

Objective: Patients with Klippel-Trénaunay syndrome (KTS) and congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and spinal/skeletal abnormalities (CLOVES) syndrome have central phlebectasia and enlarged persistent embryonic veins that are often incompetent and prone to thromboembolism. The purpose of the study was to determine the presence of phlebectasia and the incidence of symptomatic pulmonary embolism (PE)., Methods: A retrospective review was conducted of patients referred to the Vascular Anomalies Center at our institution during a 21-year period who were diagnosed with KTS and CLOVES syndrome. Of these, the patients who had PE were screened for thromboembolic risk factors in addition to phlebectasia and the presence of persistent embryonic veins. Treatment outcomes following subsequent endovascular and medical therapies were reported., Results: A total of 12 KTS patients of 96 (12.5%) and 10 CLOVES syndrome patients of 110 (9%) suffered PE. Fourteen patients (64%) developed PE after surgery or sclerotherapy. All of the patients had abnormally dilated central or persistent embryonic veins; 20 patients were treated with anticoagulation (1 died at the time of presentation, and no information was available for 1) after PE, and 14 (66%) patients underwent subsequent endovascular treatment. Five patients developed recurrent PE despite anticoagulation. Two of the patients died of PE. No patients treated with endovascular closure of dilated veins had subsequent evidence of PE., Conclusions: Patients with KTS and CLOVES syndrome are at high risk for PE, particularly in the postoperative period., (Copyright © 2018 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Extracorporeal membrane oxygenation (ECMO) is an accepted treatment modality for life support refractory to conventional efforts in neonates with complex congenital heart lesions. Cannulation for ECMO can be accomplished by venovenous (VV) access where patients receive primarily respiratory support or venoarterial (VA) access which provides complete cardiopulmonary support. VV ECMO delivered by a single-vessel cannulation with a dual-chamber venous cannula allows for respiratory support while the patient remains dependent upon intrinsic cardiac function to support hemodynamics. We present a case of support of a newborn with single ventricle physiology and intraparenchymal near-atresia of the pulmonary veins using VV ECMO.

Purpose: The therapeutical management of low-flow vascular malformations (LFVMs) is challenging because of high recurrence rate; multiple strategies have been proposed. This paper aims to report a single-center experience of direct puncture sclerotherapy of peripheral LFVMs, focusing on technical aspects and clinical outcome in mid-term follow-up., Materials and Methods: 16 patients have been treated for peripheral LFVMs (mean age 36.1 years), complaining mild pain, swelling of the region of interest, and cosmetic nuisance. Preprocedural US and MR were acquired; angiography performed only in doubt vascular supply. Standard procedure consisted of direct puncture of the nidus using 20-23 gauge needles under US guidance and injection of up to 15 ml foam of sodium tetradecyl sulphate under fluoroscopic guidance. Clinical and radiological follow-up were assessed at 1, 3, and 6 months., Results: Lesions were localized: 8 in the upper and 5 the in lower limbs, 2 in the cheeks, and 1 in the vaginal labia. All procedures have been technically accomplished (100%). At 6 month follow-up, technical and clinical success were obtained in all cases, while radiological follow-up showed 81.2% (13 patients) complete vessels thrombosis after multiple sclerotherapy sessions. No major complications have been recorded; five patients (31.2%) referred minor complications., Conclusions: Sclerotherapy via direct puncture of LFVMs is a clinically effective procedure, well tolerated by patients, with reduced costs and mild minor complications rate; interventionalists should always clarify to the patients that multiple sessions would be performed and recurrences are expected at imaging follow-up despite clinical improvement.

Purpose This manuscript describes the technique of real-time MRI-guided sclerotherapy for low-flow venous malformations in the head and neck based on our institutional experience. Materials and methods Ethanolamine oleate is used as the sclerosant and is mixed with gadolinium for visualization during the procedure. The five procedural steps include: (I) an initial tri-plane T2-weighted sequence to visualize the lesion; (II) a T1 FSE or trueFISP sequence to assess needle placement and advancement within the lesion; (III) a tri-plane T1 FLASH sequence to monitor sclerosant injection; (IV) a T1 FSE or VIBE sequence to assess sclerosant coverage of the malformation before needle removal; (V) a post-procedural tri-plane T1 fat-saturated sequence to confirm sclerosant coverage of the lesion. Periprocedural medications typically include steroids, antibiotic prophylaxis, and non-steroidal anti-inflammatory medication. Patients are typically admitted for overnight observation. Conclusion Real-time MRI-guided sclerotherapy for low-flow venous malformations in the head and neck is effective and safe.

Superficial vascular anomalies constitute a large group of malformative and tumoral conditions developed from all types of vessels. Vascular tumors are the result of cellular hyperplasia, whereas vascular malformations (VMs) are constituted of dysplastic vessels. The classification from International Society for the Study of Vascular Anomalies (ISSVA) is based on this pathogenic difference. The most common vascular tumor is infantile hemangioma, which treatment, when necessary, is propranolol. Congenital hemangiomas and tumors that might be complicated with Kasabach-Merritt phenomenon, i.e. deep thrombocytopenia, are much rarer. Management of Kasabach-Merritt phenomenon is now largely based on sirolimus. Low-flow VMs include capillary, venous and lymphatic malformations; arteriovenous malformations are high-flow malformations. These different types of VMs might be combined. Currently, there is an increasing work in delineating the different entities based on molecular findings. Treatment of VMs depends on the impairment linked to them, and is decided case by case, in pluridisciplinary consultations. Interventional treatments, especially surgery and sclerotherapy, are usually partially efficient, and management of patients with VMs increasingly involves medical drugs. First-line treatment of coagulation disorders associated with venous malformations is based on low molecular weight heparin; sirolimus seems efficient in hemorrhagic complications refractory to usual treatment. Sirolimus is about to become the standard treatment in painful inflammatory manifestations of mixed and/or complicated lymphatic malformations., (Copyright © 2018 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Venous malformations are the most common type of congenital vascular malformation. The diagnosis and management of venous malformations may be challenging, as venous malformations may be located anywhere in the body and range from small and superficial to large and extensive lesions. There are many treatment options for venous malformations including systemic targeted drugs, open surgery, sclerotherapy, cryoablation, and laser photocoagulation. This article reviews the natural history, clinical evaluation, imaging diagnosis, and treatment modalities of venous malformations., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Vascular malformations (VM) are congenital lesions that can be debilitating and cause significant aesthetic and functional limitations. The chemotherapeutic agent bleomycin has been utilized as a sclerosant, directly injected percutaneously into the VM. Unfortunately, little is known about the benefits and short-term side effects of bleomycin with intralesional injections., Procedure: An IRB approved, retrospective chart review was performed on patients with VM who had been treated with intralesional bleomycin. Data included type of VM, number of treatments, total bleomycin dose per m², and adverse effects. A questionnaire was administered to available patients to assess subjective outcomes and side effects., Results: Forty-six patients were treated with 141 procedures of bleomycin sclerotherapy for VM. Patient ages ranged from 1 to 20 years (median age 10 years). The median cumulative bleomycin dose was 16.3 units/m²/person (range of 1.7-97.0 units/m²/person). Sixty-three percent of patients were reached for a questionnaire to assess short-term side effects. Ninety percent of patients surveyed were satisfied to very satisfied with the results from the procedure. About 24% of patients experienced transient nausea, vomiting and/or local hyperpigmentation., Conclusion: Bleomycin sclerotherapy can be an effective treatment of VM with repeat exposure with minor risk of short-term side effects, however, long-term risks are of great concern. Further studies are required to assess systemic absorption and long-term risks., (© 2018 Wiley Periodicals, Inc.)

Purpose: To evaluate the efficacy and safety of sclerotherapy with sodium tetradecyl sulfate (STS) and bleomycin for treatment of venous malformations (VMs) of the oropharyngeal region., Materials and Methods: A retrospective study of 33 patients with 46 VMs of the buccal and pharyngolaryngeal cavity associated with impairment of eating, respiration, or elocution was performed. Individual lesions were divided based on their anterior or posterior location, using the base of the tongue as an anatomic landmark. Lesion size was estimated with the use of orthogonal measurements on magnetic resonance or ultrasound images before and after treatment to assess radiologic response. Sclerotherapy sessions were performed under ultrasound, fluoroscopic, and, if needed, endoscopic guidance. Clinical response was assessed with the use of the Manchester Orofacial Pain Disability Scale. Methods for airway management were also compiled., Results: Following sclerotherapy, average VM diameter was reduced by 31.4% (P < .0001) on a per-patient basis and by 30.8% (P < .0001) on a per-lesion basis. The Manchester score improved by an average of 37.0% (P = .013). Four patients reported a worsening of symptoms, and 11 patients experienced symptomatic recurrence. Complications include pneumonia (5 patients) and urgent placement of a post-procedure tracheostomy (4 patients). Patients with posterior malformations experienced more complications (emergency tracheostomies in 4 and pneumonias in 4)., Conclusions: Sclerotherapy using STS is an efficient treatment for venous malformations of the buccal and pharyngolaryngeal cavity but can lead to significant complication for posterior lesions. Careful assessment of the airway is needed before treatment, and prophylactic tracheotomy should be considered in patients with posterior lesions., (Copyright © 2018 SIR. Published by Elsevier Inc. All rights reserved.)

Vascular anomalies are a morphologically and biologically diverse group of vascular channel abnormalities that are often congenital but may evolve or change over time in the developing child. Classification is based on a combination of physical and biological properties and clinical behavior that differentiate primarily between tumors and malformations and includes a few provisionally unclassified lesions. Anomalies of the gastrointestinal (GI) tract may present clinically with GI bleeding, abdominal pain, high-output cardiac failure, and malabsorption. This review focuses on new therapies for the treatment of GI bleeding. Important new pharmacological therapies include treatment of hemangioma with non-selective and selective beta-antagonist agents, propranolol and atenolol, and treatment of blue rubber bleb nevus syndrome and cutaneo-visceral angiomatosis with thrombocytopenia (also known as multifocal lymphangioendotheliomatosis with thrombocytopenia) with sirolimus, an inhibitor of the mammalian target of rapamycin. Therapeutic endoscopy may offer an effective alternative to bowel resection for colonic varices and other focal vascular anomalies of the GI tract that fail to respond to pharmacological therapy.

Low-flow vascular malformations are congenital lesions that can occur throughout the body. Treatment of these lesions is indicated to ameliorate pain, cosmetic disfigurement, and functional impairment. The first-line treatment of low-flow vascular malformations is percutaneous sclerotherapy. Traditionally, sclerotherapy is performed with a combination of ultrasound and fluoroscopy. However, malformations that are deep in the abdomen and pelvis or are obscured by overlying fascia or scar may be difficult to be visualized with ultrasound and fluoroscopy. MR-guided sclerotherapy has emerged as an alternative modality that can be used to needle guidance and sclerosant monitoring. In this review, we discuss the historical and current use of MR-guided sclerotherapy for the treatment of low-flow vascular malformations.

Background: Pulmonary sequestration is a rare congenital pulmonary dysplasia, which requires surgical resection (either via open thoracotomy or video-assisted thoracoscopic surgery [VATS] or via endoluminal occlusion of the abnormal feeding vessel)., Case Presentation: We described a 51-year-old female patient with a history of recurrent cough and repeated pneumonia. She was referred to our hospital for further work-up of pulmonary sequestration. We performed a hybrid surgery (i.e., embolization of the aberrant feeding vessel of the sequestration combined with wedge resection of the left lower lobe lesion through VATS). The patient was discharged on the sixth postoperative day in good condition and without complications., Conclusions: We believe that a hybrid operation is safer, more feasible, and more comprehensive than other treatments.

Introduction: Extensive cervicofacial venous malformations (VM) pose significant challenges to a patient's quality of life (altered breathing, dysphagia, dysarthria). Treatment options include: 1) Surgical debulking; 2) Sclerotherapy; 3) laser therapy; or 4) Combined modalities. Recent studies have demonstrated the importance of multimodality and multidisciplinary management of these patients. However, no studies have described combined single anesthetic laser and sclerotherapy treatment. We sought to demonstrate the safety and efficacy of combined Nd:YAG laser and sclerotherapy under the same anesthetic administration., Methods: Retrospective review of 8 patients (Age 6 mo -74 yrs, x͂ 31) with extensive cervicofacial VM with significant airway involvement. Patients were treated with combined suspension laryngoscopy with Nd:YAG laser of airway VM followed by image guided direct puncture sclerotherapy using bleomycin in the airway VM and sodium tetradecyl sulfate (STS) foam in the cervicofacial VM during the same anesthetic encounter., Results: All 8 patients had extensive cervicofacial VMs that were symptomatic with snoring or orthopnea. Four of the patients had previously been treated at outside institutions with residual disease or significant complications. All patients remained intubated post procedure (Avg. 1.07 days) and tolerated extubation without re-intubation or any major complications. The average length of hospital stay was 3.2 days, of which 1.9 days were spent in the ICU. Patients reported symptomatic improvement or had decreased VM disease on MRI follow up., Conclusion: Combined Nd:YAG laser therapy and sclerotherapy allows treatment of both superficial and deep components of VMs in a safe and efficient manner. In addition, suspension laryngoscopy provides improved visualization and access for the interventional radiologist in difficult to reach areas for sclerotherapy., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: Vascular malformations isolated to skeletal muscles are rare and often debilitating due to pain and very challenging to treat. Multi-modal management options include compression garments, medical therapy, sclerotherapy, and surgical resection., Methods: A retrospective review of patients who underwent sclerotherapy for intramuscular venous malformations (IVM) between 2008 and 2016 was performed. Demographics, indications, and clinical follow-up were analyzed., Results: Twenty patients underwent sclerotherapy for IVM. Six males and 14 females underwent 58 procedures. All patients presented with pain and were treated initially with compression garments. Median age at first treatment was 13years (+/- 5.06years). Initial protocol consisted of 2 sclerotherapy procedures with sodium tetradecyl sulfate (STS) within a 2-3month interval. Median volume of the lesion was 40cm 3 (+/- 28.7), mostly located in the lower extremities (15/20). Median number of treatments was 2 (+/- 1.95). Treatment prior to puberty resulted in a median symptom-free time of 4years (+/- 2.18), while after puberty resulted in a symptom-free time of 2years (+/- 2.28). Two patients had an underlying coagulopathy and were admitted for observation and peri-procedural Lovenox. No procedure related complications were noted with a median follow-up of 4years (+/- 2.27)., Conclusion: IVMs are rare but can be incapacitating secondary to pain. Sclerotherapy is a useful minimally invasive procedure generally requiring at least two consecutive treatments. Treatment of patients prior to puberty appears to provide a more durable result, and surgical resection may be avoided., Type of Study: retrospective., Level of Evidence: IV., (Copyright © 2018. Published by Elsevier Inc.)

Aim: Congenital portosystemic shunts (CPSS) are rare, congenital malformations that are increasingly often discovered during the fetal period, and for which, the manifestations and evolution are poorly understood. The objective of this review is to describe the phenotype and evolution of forms diagnosed in the antenatal period., Materials and Methods: We performed a systematic review of the literature cited in Pubmed between 1982 and 2016 for CPSS cases diagnosed during the fetal period., Results: We identified 123 cases. The median age at diagnosis was 25 GA (14-38 weeks GA). Eighty patients had 128 associated congenital anomalies. The congenital abnormalities most frequently associated with antenatal diagnosis of CPSS were congenital cardiac disease (30 cases), intrauterine growth restriction (21 cases), vascular anomalies (14 cases), and trisomy 21 (7 cases). Seventy-five complications were reported in the literature. The most frequent were antenatal hemodynamic abnormalities (27 cases), neonatal cholestasis (11 cases), and hyperammonemia (10 cases). Twenty-nine patients had no complications. The choice of treatment was conservative in 29/56 cases, interventional radiology in 15 cases and surgery in 15 cases (three of the latter after failure of embolization)., Conclusion: From this review, we propose an algorithm for the perinatal management of this congenital abnormality.

Purpose: The purpose of this study was to evaluate the safety and efficacy of preoperative percutaneous n-butyl cyanoacrylate (nBCA) embolization of venous malformations in children., Material and Methods: Clinical data were retrospectively reviewed in children who underwent embolization using nBCA followed by resection of venous malformations., Results: A total of 17 embolizations were performed in 14 patients (9 females, mean age: 5.5 years; median age: 3 years; range 0.1-16 years). The venous malformations involved the lower extremity and the knee joint (n = 7), the trunk (n = 4), head and neck (n = 2), and hand (n = 1). n-Butyl cyanoacrylate was diluted with iodized oil at a ratio of 1:3 to 1:5. The mean and median volume of nBCA per procedure were 2.1 and 2 mL, respectively (range: 0.5-8 mL). There were no complications associated with the procedures. The mean and median time between final embolization and resection were 3.6 and 2 days, respectively. All children underwent successful resection of the symptomatic lesions. The estimated mean and median blood loss were 75 and 50 mL, respectively (range: 5-350 mL). The postprocedure course was uneventful, the days to discharge ranged between 1 and 6 days (mean 3 days)., Conclusion: Initial results suggest that preoperative percutaneous n-butyl cyanoacrylate embolization of venous malformations is safe and effective in children, with the potential for minimizing blood loss and inpatient stay.

A patient with extensive multisystem overgrowth caused by a somatic gain of function PIK3CA-mutation is described. This case is an example of the clinical diversity of the PIK3CA-Related Overgrowth Spectrum (PROS) as the patient had overlapping features of Congenital Lipomatous Overgrowth Vascular malformations Epidermal nevi and Skeletal abnormalities (CLOVES) syndrome and Megalencephaly-Capillary malformation Polymicrogyria (MCAP) syndrome and underlines the utility of this umbrella term., (© 2018 Wiley Periodicals, Inc.)

Objective: No standardized therapeutic algorithm or embolic agent of choice has yet been identified for management of congenital peripheral venous malformations (VMs). Treatment options and reported outcomes therefore vary widely. Herein, we present an institution-wide algorithm for management of symptomatic congenital peripheral VMs using a single embolotherapeutic modality., Methods: During 36 months, patients with symptomatic congenital peripheral VMs underwent contrast-enhanced magnetic resonance imaging. Hematologic monitoring for localized intravascular coagulopathy was performed in all. Perioperative anticoagulation was administered accordingly. When applicable, venous duplex ultrasound was performed to assess for presence and patency of a deep venous system and superficial venous reflux. If superficial venous reflux was identified, radiofrequency ablation was performed per standard protocol before or at the time of initial embolization. Direct-stick embolizations (DSEs) were performed by a single operator using two concentrations (1% and 3%) of sodium tetradecyl sulfate (STS; Sotradecol; AngioDynamics, Latham, NY) without foam preparation. Patients were followed up clinically for resolution of symptoms, coagulopathic monitoring, and development of complications. All data were prospectively maintained and retrospectively reviewed., Results: There were 71 DSEs performed in 40 patients (1.8 procedures per patient [range, 1-8]; 12 male patients; mean age, 22 years [range, 2-53 years]). Mean follow-up was 17.1 months (range, 0.8-31.6 months). Presenting symptoms included pain (n = 40 [100%]), swelling (n = 36 [90%]), and cosmetic disfigurement (n = 32 [80%]). Anatomic distribution was upper extremity (n = 16 [23%]), lower extremity (n = 37 [52%]), head and neck (n = 7 [10%]), trunk (n = 10 [14%]), and visceral (n = 1 [1%]). There were 33 sporadic cases, 4 (10%) Klippel-Trénaunay syndrome cases, 2 (5%) blue rubber bleb nevus syndrome cases, and 1 (2.5%) CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevus, and skeletal deformities) syndrome case. Four patients presented with localized intravascular coagulopathy, two of whom required perioperative enoxaparin. Twenty-six patients (65%) required a single DSE session with complete symptom relief. Fourteen patients (35%) required repeated DSE. Two patients (5%) required adjunctive surgical excision. There was one postoperative death (1.4%) secondary to massive pulmonary embolism. Complications were otherwise limited to skin necrosis (n = 2 [3%]). Mean volume of sclerosant per session was 7 mL of 1% STS (range, 3-14 mL), and 15 mL of 3% STS (range, 3-42.5 mL)., Conclusions: In the absence of allergic reactions, most congenital peripheral VMs can be safely embolized with liquid STS, thereby avoiding the well-documented toxicity of ethanol. Venous thromboembolism remains a major source of morbidity and mortality in this population of patients despite close hematologic scrutiny. Prospective randomized trials are needed for embolotherapeutic standardization., (Published by Elsevier Inc.)

The aim of this study was to evaluate the efficacy of fluoroscopy-guided percutaneous injection of bleomycin as the primary treatment for low-flow vascular malformations. A total of 34 patients (mean (range) age 24 (8-51) years) with orofacial vascular lesions were treated in the Department of Interventional Radiology and Maxillofacial Surgery. There were 20 low-flow venous malformations, 11 lymphatic malformations, and three of mixed type. All patients were treated by fluoroscopy-guided percutaneous injection of a mixture of bleomycin (mean (range) 15 (5-15)mg) and a radio-opaque agent (Ultravist ® (iopromide), Bayer)/session. The number of sessions ranged from one to six. The clinical response was complete in 21 patients, obvious in nine, and of clinical benefit in four. Patients were reviewed within the first week, third week, and at three-month periods until 24 months. There were no serious complications such as pulmonary fibrosis. Fluoroscopy-guided intralesional injection of bleomycin should be considered as the first-line treatment for lymphatic malformations because it is effective and reliable with few complications., (Crown Copyright © 2018. Published by Elsevier Ltd. All rights reserved.)

Vascular malformations (VMs) of the head and neck can lead to aesthetic problems as well as cranial nerve damage, airway compromise, and vision loss. Large VMs are typically managed surgically, with sclerotherapy or embolization performed in the perioperative period to decrease the risk of excessive blood loss and minimize the size of the VM. However, this initial treatment is frequently insufficient leading to excessive blood loss intraoperatively, poorer margin visualization for the surgeon, and decreased likelihood of complete resection. As a result, resections of large VMs are often performed in a multistage approach. This article introduces a new hybrid approach for the management of head and neck VMs entailing the use of an endovascular operating room where a neuroendovascular surgeon performs embolization or sclerotherapy intraoperatively as needed in conjunction with surgical excision. Three patients with large VMs in the facial region underwent successful use of the hybrid approach. The hybrid approach improved visualization, leading to complete resection in 1 patient and nearly complete resections (70% and 90%) in the other patients. The technique also helped minimize blood loss because only the youngest patient (23 months old) required a blood transfusion. Implications of these findings include the transition from a multistaged approach for large VMs to a single-stage approach. In addition, decreases in blood loss may allow for the development and use of minimal access techniques, leading to a decrease in visible scarring for patients. We suggest the consideration of the hybrid approach for large head and neck VMs.

Objective: Congenital portosystemic venous shunt (CPSVS) is a rare vascular malformation with a high risk of mortality from pulmonary arterial hypertension (PAH), but the treatment outcome of CPSVS closure remains elusive. Our aim was to investigate the clinical features and establish the optimal management of CPSVS with or without PAH., Methods: Twenty-four patients with CPSVS treated in Kyushu University Hospital between 1990 and 2015 were enrolled in this study. The patients were divided into a PAH group (n = 9) and a non-PAH group (n = 15). Clinical characteristics and outcomes were evaluated., Results: The first manifestation of CPSVS at diagnosis (28.5 [1-216] months) was hypergalactosemia in 13 (54%) or PAH in six (25%) patients. PAH was the cause of all three deaths. The PAH group had higher levels of serum total bile acid, manganese, and total bilirubin, along with higher pulmonary vascular resistance index (PVRI) than the non-PAH group (7.2 [5.1-38.1] vs 1.2 [0.5-3.3] unit/m 2 , P < 0.001). Five of nine PAH patients underwent CPSVS closure at a median of 38 months (range 21-118) after PAH diagnosis. Pulmonary artery pressure improved after CPSVS closure with PAH-specific therapy, but normal range was not achieved. CPSVS closure improved the hepatic synthetic function of four PAH patients. Eigh-t of 15 non-PAH patients who received CPSVS closure did not develop PAH for a median of 34.5 months (range 6-164) after the procedure., Conclusions: CPSVS closure with PAH-specific therapy successfully controlled PAH. Early CPSVS closure may prevent the occurrence and progression of PAH with CPSVS., (© 2018 Wiley Periodicals, Inc.)

Introduction: Vascular malformations are congenital lesions with complex clinical presentations and management. Their classification and treatment options have considerably changed throughout the years, with conflicting evidence in the literature. In this article, we aim to review the classification, diagnosis and treatment of the main vascular malformations., Evidence Acquisition: A thorough non-systematic review of the literature was conducted using PubMed/Medline. Seventy-nine articles were selected and included, according to their scientific relevance and relation with the subject., Evidence Synthesis: Vascular malformations occur due to errors during vasculogenesis. They are present at birth, though they may not be apparent until later in life. These malformations are most frequently found in the head and neck, but can occur in any part of the body. They can be divided according to the predominant vessel type into arteriovenous, venous, capillary and lymphatic. Combined malformations occur when more than one type of vessel is present. Clinical presentation is variable and depends on the type of malformation, as well as location, size and relation with other structures. Symptoms such as ischemia, swelling, pain, thrombosis, deformity and functional impairment can be caused by these lesions. The diagnosis of vascular malformations is based in both clinical presentation and complementary imaging techniques, with special emphasis on magnetic resonance imaging. Depending on the malformation and clinical presentation, treatment may be medical or interventional, by means of either interventional techniques or surgery., Conclusions: Vascular malformations are a complex group of pathologies, with different clinical presentations and treatment options, and therefore management by a multi-disciplinary team is essential. Their cure is often challenging and when not possible, treatment should aim at symptomatic control and improvement of patient's quality of life.

Vascular malformations are congenital vascular anomalies with normal endothelial turnover, which can affect the whole vessel tree (arterial, capillary or lymphatic) both as isolated defects or as part of a syndrome. Developmental errors during embryogenesis, such as abnormal signaling processes that control the apoptosis, maturation and growth of vascular cells, lead to the persistence of vascular plexus cells with some degree of differentiation. Vascular malformations are usually present at birth, although symptoms or complications may be manifested later. Since the overwhelming majority are symptomatic, treatment is indicated which is often aimed only at improving the symptoms by controlling the size.

Objective: To evaluate the safety and efficacy of foam sclerotherapy with polidocanol for the treatment of venous malformation in the oropharynx. Methods: The clinical data of 21 children with venous malformation in the oropharynx treated by foam sclerotherapy were retrospectively analyzed. There were 10 males and 11 females, ranging in age from 1 month to 13 years, with a median age of 2.3 years. MRI was performed in all children, and the diagnosis was further confirmed by radiography. After general anesthesia, the oropharynx was exposed by opening device. Scalp acupuncture was used to pucture lesions and polidocanol foam was injected after the nidus was confirmed by digital subtraction angiography(DSA). The follow-up time ranged from 2-29 months, with a mean time of 15 months. The clinical symptoms, imaging data, therapeutic effects and postoperative complications were evaluated. Results: Total numbers of treatment were 52 times, 1-6 times/case; 13 cases were cured, 7 cases was relieved and no response in one case. Postoperative swelling in 13 cases, fever in 3 cases, local mucosal ulcer in 2 cases, difficult extubation in 2 cases. No nerve injury, swallowing function damage and cardiopulmonary accidents were found in all patients. Conclusion: Foam sclerotherapy with polidocanol in the treatment of venous malformation in the oropharynx is a safe and effective method.

Congenital portosystemic venous shunts are rare developmental anomalies resulting in diversion of portal flow to the systemic circulation and have been divided into extra- and intrahepatic shunts. They occur during liver and systemic venous vascular embryogenesis and are associated with other congenital abnormalities. They carry a higher risk of benign and malignant liver tumors and, if left untreated, can result in significant medical complications including systemic encephalopathy and pulmonary hypertension., Conclusion: This article reviews the various types of congenital portosystemic shunts and their anatomy, pathogenesis, symptomatology, and timing and options of treatment. What is Known: • The natural history and basic management of this rare congenital anomaly are presented. What is New: • This paper is a comprehensive review; highlights important topics in pathogenesis, clinical symptomatology, and treatment options; and proposes an algorithm in the management of congenital portosystemic shunt disease in order to provide a clear idea to a pediatrician. An effort has been made to emphasize the indications for treatment in the children population and link to the adult group by discussing the consequences of lack of treatment or delayed diagnosis.

Objective: An accurate "molecular" diagnosis and classification of similar but distinct diseases is sometime challenging but often crucial for the definition of the appropriate patient medical management and treatment as well as for genetic counseling and risk assessment in families. The advent of next-generation sequencing (NGS), which analysed all known disease-associated genes in parallel in a cost- and time-effective manner, eased this process of disease definition and also for vascular anomalies that are a heterogeneous group of vascular tumors and congenital circulatory malformations and often characterized by overlapping phenotypes., Methods: We designed a NGS-based screening of the 25 currently most prevalent genes identified in patients with vascular anomalies with Mendelian inheritance and applied this panel to study the DNA of 150 patients affected with vascular anomalies for autosomal recessive and autosomal dominant variants and to analyse the paired blood and DNA from intralesional biopsy specimens in 17 patients for somatic unbalance. Results were confirmed with Sanger sequencing., Results: We identified 14 pathogenic variants in 13 of 150 patients. Eight variants were previously reported as a disease-causing variant, and six were new. In 55 additional probands we detected 75 variants with unknown significance. Moreover, a previously reported somatic variant was detected in five of 17 available tissue biopsy specimens., Conclusions: Our results show that many genes can cause a wide variety of syndromic and nonsyndromic disorders, confirming that genetic testing by NGS is the approach of choice to diagnose heritable vascular anomalies, especially, but not only, when an intralesional biopsy specimen is available. The identification of the causative genes and the possibility of tracing somatic variants in tissues provide important information about etiology, patient clinical management, and treatment, and it could highlight otherwise unsuspected clinical situations., (Copyright © 2017 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Background and Aims: Until now, no study has focused exclusively on low-flow retrobulbar intraconal venous malformations (RIVMs) which may require treatment due to cosmetic defect, pain, and visual dysfunction. The treatment for RIVMs which surround the optic nerve remains challenging. This case series aimed to evaluate the technical feasibility, effectiveness, and safety of percutaneous sclerotherapy with polidocanol for low-flow RIVMs, using local anesthesia., Method: This is a prospective, non-comparative, single-center, interventional case series. All patients signed informed consent forms. Seven patients with RIVMs were treated with percutaneous sclerotherapy with polidocanol/air foam using CT guidance. Primary endpoints are reduction in the volume of RIVMs and pain relief assessed by visual analog scale (VAS). Secondary endpoints are exophthalmos and recording adverse events obtained in clinical follow-up during outpatient visits., Results: Results revealed that the mean volume of RIVMs was decreased from 12.05 ± 6.35 cm 3 preoperatively to 1.56 ± 0.43 cm 3 postoperatively, (p = 0.005), with a mean decrease of 87.05%. The intraocular pressure was decreased from 14.19 ± 2.99 to 11.79 ± 1.25 mmHg, (p = 0.043). The mean VAS score was decreased from 3.43 ± 2.37 preoperatively to 1.29 ± 0.76 postoperatively, (p = 0.023). The exophthalmos score was decreased from 1.75 ± 0.27 to 1.34 ± 0.31 cm, (p = 0.005). All patients were satisfied with the treatment, which did not leave a postoperative scar., Conclusions: The results of percutaneous intralesion injection of polidocanol for RIVMs are encouraging. The present results suggest that this method could be a safe and effective treatment option for patients with RIVMs.

Management of venous malformation (VM) in the pharynx is challenging because of difficulties in needle access and the presence of nerves as well as other critical anatomical structures around the lesion. We describe the successful treatment of a large pharyngeal VM using transoral sclerotherapy. The patient, a 21-yearold male, visited our hospital for sclerotherapy treatment consultation after complaining of difficulty in swallowing because of a pharyngeal VM. Transoral sclerotherapy was successful, and the symptoms showed complete alleviation. Using a flat detector-equipped angiographic C-arm CT (CACT) enabled the precise assessment of sclerosant placement, facilitating safer sclerotherapy in the pharynx.