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During extracorporeal life support (ECLS) in infants, cannulation of the right common carotid artery may result in a devastating ischaemic neurological injury. Herein, we present a case of an infant who encountered bilateral cerebral infarction during ECLS via the right carotid artery due to a rare and tragic anomaly of the circle of Willis. The magnetic resonance angiography complemented computed tomography in diagnosing the infarction and identifying this unique anatomy., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare, severe, sporadically occurring disorder characterized by multiple venous malformations., Aims: To present and analyze a case series of pediatric patients with BRBNS and to describe diagnostic approaches and management options applied., Patients and Methods: Multicenter, retrospective study, evaluating the diagnosis and management of children with BRBNS., Results: Eighteen patients diagnosed with BRBNS were included. Cutaneous venous malformations were observed in 78% and gastrointestinal venous malformations in 89%. Lesions were also found in other organs including muscles, joints, central nervous system, eyes, parotid gland, spine, kidneys and lungs. Gastrointestinal lesions were more common in the small intestine than in stomach or colon. The management varied significantly among centers. Endoscopic therapy and surgical therapy alone failed to prevent recurrence of lesions. In younger children and in patients with musculoskeletal or other organ involvement, sirolimus was used with 100% success rate in our series (5 patients treated) although poor compliance with subtherapeutic sirolimus trough levels led to recurrence in a minority., Conclusions: Considering the multi-organ involvement in BRBNS, diagnosis and management requires a multidisciplinary approach. The treatment includes conservative, medical, endoscopic and surgical options. Prospective multicenter studies are needed to identify the optimal management of this rare condition., (Copyright © 2019 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.)

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

Congenital aortic arch anomalies are rare and may be associated with other congenital cardiovascular malformations. The authors report a rare case of anomaly in the aortic arch embryogenesis, presenting with a right aortic arch and an isolated innominate artery, associated with the subclavian steal phenomenon. This condition is discussed considering the Edwards hypothetical double embryonic arch and its clinical aspects., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Within the broad spectrum of pediatric hepatobiliary disorders, hepatic vascular malformations, portal hypertension, and hepatic transplant interventions pose numerous challenges. The role of interventional radiology within each of these conditions is discussed herein, beginning with endovascular management of high flow hepatic vascular malformations. Next, while becoming less common in adult populations, surgical portoportal and portosystemic shunts remain prevalent in many pediatric centers. Shunt anatomy is reviewed along with endovascular management techniques for shunt dysfunction. Next, the growing experience with pediatric transjugular intrahepatic portosystemic shunt placement is reviewed along with tips for success in pediatric patients. Finally, pediatric hepatic transplant interventions are discussed with technical notes pertinent to split liver anatomy., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: portosystemic intrahepatic venous connections (Abernethy syndrome) are rare anatomical variants, which are classified according to the type of union between the portal venous circulation and the central venous system. In adults, the diagnosis is often incidental, although some cases can be presented with an encephalopathy without associated liver disease., Case Reports: here we present two cases of portosystemic shunt, one with encephalopathy development, and the other casually caught. Its treatment by interventionist radiology, was decided in function of clinic symptoms. Both patients were asymptomatic at controls in the outpatient consultation. No complications derived from the therapeutic decision. The control is carried out annually with image tests and blood analysis., Discussion: given the low prevalence of malformation and its usual diagnosis at younger ages (associated with important cognitive alterations) its treatment in adults is not protocolized. In these cases the decision of the treatment would be conditioned to the associated symptomatology, being the minimally invasive treatment (by interventional radiology) a therapeutic option in the symptomatic adult. Observation by imaging tests and control in outpatient consultation (without associated treatment) would be a choice in asymptomatic adults.

Background: The use of monoethanolamine oleate 5% is effective for the treatment of vascular malformations with low blood flow., Objectives: To report a case series of vascular malformations in the mouth and oral cavity treated with monoethanolamine oleate 5%., Methods: A retrospective descriptive study was performed in electronic patient charts covering seven years. Patient demographics, diagnostic resources, lesion site, size, and number of applications of monoethanolamine oleate 5% were collected., Results: A total of 21 vascular malformations were recorded, located mostly on the lower lip (52.3%) and resolved in a single application in 14 patients. The authors found 19 patients treated with sclerotherapy. Thirteen were women and six were men, with a mean age of 61 years., Study Limitation: Small sample size., Conclusions: Sclerotherapy is an effective treatment for vascular malformations of the lips and oral cavity, with resolution after only one or two applications (n=16)., (Copyright © 2019. Published by Elsevier España, S.L.U.)

Purpose of Review: Vascular malformations (VaMs) are a consequence of disrupted morphogenesis that may involve arterial, capillary, venous, or lymphatic endothelium alone or in a combination. VaMs can have serious health impacts, leading to life-threatening conditions sometimes. Genetic mutations affecting proliferation, migration, adhesion, differentiation, and survival of endothelial cells, as well as integrity of extracellular matrix are believed to be the pathogenesis of these disorders. Here, we present an updated review of genetic mutations and potential therapeutic targets for VaMs., Recent Findings: Increased number of genetic mutations have been discovered in vascular anomalies via targeted deep sequencing. When a genetic defect is identified, it often presents in only a small percentage of cells within the malformation. In addition, mutations within the same gene may result in different clinical phenotypes. Management of VaMs can be challenging depending on the severity and functional impairment associated. There are no standard treatment algorithms available to date for VaMs, therefore the disorder has significant unmet clinical needs. Currently, the focus of therapeutic development is to target constitutively activated intracellular signaling pathways resulted from genetic mutations., Summary: Knowledge about the genetic mutations and altered signaling pathways related to VaMs have improved our understanding about the pathogenesis of vascular anomalies and provided insights to the development of new targeted therapies.

Objectives: Sclerotherapy is an essential component of the treatment for venous malformations, and ethanolamine oleate (EO) is known as a useful sclerosing agent. However, macroscopic haemoglobinuria (MH) and subsequent renal impairment are severe complications after sclerotherapy using EO. The present study aimed to clarify the MH risk factors for better peri-operative management of venous malformations., Methods: Data collected during 130 procedures involving 94 patients who were undergoing sclerotherapy using EO for venous malformation were retrospectively analysed. Pre-operative and operative variables, including sex, age, pre-operative body mass index, location, depth, type of lesion, size, number of procedures, type of drainage vein, ratio of sclerosant to air, and injected total dose of 5% EO per body weight (BW), were examined. Univariable analysis and multivariable logistic regression were performed to determine the possible risk factors for MH., Results: Following sclerotherapy, MH occurred in 27.7% of patients, but no patient developed post-operative renal impairment because of aggressive hydration and haptoglobin administration. On univariable analysis, diffuse lesion, lesion size ≥50 cm 2 , and total injected dose of 5% EO ≥ 0.18 mL/kg were found to be the MH risk factors. Multivariable logistic regression analysis identified a total injected dose of 5% EO ≥ 0.18 mL/kg as the significant independent factor contributing to MH risk., Conclusions: Macroscopic haemoglobinuria is a reversible complication if immediate and appropriate interventions with aggressive hydration and haptoglobin administration are performed; therefore, it should be closely monitored following sclerotherapy, especially when using 5% EO ≥ 0.18 mL/kg., (Copyright © 2019 European Society for Vascular Surgery. Published by Elsevier B.V. All rights reserved.)

Purpose: To review a representative case series and share a stepwise approach to the diagnosis and treatment of periorbital venolymphatic malformations., Methods: A case series of 9 patients with representative periorbital venolymphatic malformations. All patients managed at the University of California, Los Angeles Orbital Center for a vascular malformation over the last 5 years were reviewed. Cases representative of the varied pathologic findings and techniques were selected. The clinical, radiologic, and intraoperative findings are presented and a survey of techniques composed., Results: Venolymphatic malformations can have protean manifestations. Characteristics vital to the understanding and management of these lesions are the classification and vascular composition of the lesion, internal flow and drainage patterns, anatomic localization and association with distant components. Options for treatment include sclerotherapy, biologic therapy, embolization, surgical excision, or some combination thereof., Conclusions: A comprehensive understanding of each patient's unique lesion is critical to a rational treatment approach. Teamwork and creativity are key to effectively managing these lesions.

Purpose: To present a case series of percutaneous sclerotherapy of para-orbital and orbital venous malformations., Method: Seven patients with venous malformations in the orbital and para-orbital region were reviewed. Puncture venogram was performed on all patients and image guided bleomycin injections using biplane digital subtraction in angiography suite., Results: Average age of patients at the time of the procedure was 22 years old with the youngest being five years of age and the oldest being 50 years. Follow-ups ranged from 3 months to 18 months and the average follow-up duration was 13 months. The patients had no intraprocedural complications and reported symptomatic relief of pain as per the last follow-up. Patients have shown no symptoms or signs of recurrence of lesions with uneventful recovery so far., Conclusions: Percutaneous sclerotherapy of orbital Venous Malformations with bleomycin is a safe and well-controlled procedure done in real time; it has no major systemic adverse side effects and higher efficacy than other sclerosing agents.

The optical imaging guided tumor vessels and vascular malformation visualization by using the second near infrared emission beyond 1500 nm (NIR-II) is emerged as the next generation fluorescence imaging technique for early tumor diagnosis and identification of tumor-associated vascular features. On the other hand, developing theranostic probes for NIR-II imaging guided photothermal therapy (PTT) is of great significance, which is rarely explored. Herein, a high performance theranostic nanoplatform based on the core-shell structured NaLuF 4 nanorods@polydopamine (denoted as NRs@PDA) by integrating the new advanced NIR-II imaging beyond 1500 nm with PTT function was developed for tumor-associated vascular malformation visualization and imaging-guided PTT. Methods : In this work, the hydrophilic NaLuF 4 NRs@PDA therapeutic probe was synthesized by using a reverse microemulsion method. The crystal phase, morphology, emission spectra and photothermal performance of the synthesized samples were systematically characterized. The NIR-II optical imaging and photothermal properties were investigated by in vitro and in vivo experiments. Results : The NaLuF 4 NRs@PDA therapeutic probe possessed efficient NIR-II emission centered at 1525 nm with high quantum yield (QY), good photo-stability and high biocompatibility. In vivo NIR-IIb imaging based on the designed probe can clearly visualize the whole-body vessel and brain vessel with high spatial resolution, especially tumor-associated vessels. In addition, in vitro and in vivo experiments also demonstrated that the designed NaLuF 4 NRs@PDA probe possessed efficient photothermal conversion efficiency (40.18%) for PTT ablation of tumor. Conclusion : With the excellent NIR-II imaging ability and PTT of tumor, the designed theranostic nanoplatform successfully realize the simultaneous tumor vessel diagnosis and tumor therapy, which may provide the opportunity of designing new theranostic bioprobes with combination of the NIR-II optical imaging technique and PTT function for tumor diagnosis and therapy., Competing Interests: Competing Interests: The authors have declared that no competing interest exists.

Background: Atypical intracerebral hemorrhage is a common form of primary manifestation of vascular malformations., Objective: The aim of the present study is to determine clues to the cause of bleeding according to hemorrhage pattern (lobar, basal ganglia, infratentorial)., Methods: We retrospectively evaluated 343 consecutive neurosurgical patients with intracerebral hemorrhage (ICH), who were admitted to our neurosurgical department between 2006 and 2016. The study cohort includes only neurosurgical patients. Patients who underwent treatment by neurologists are not represented in this study. We assessed location of hemorrhage, hematoma volumes to rule out differences and predicitve variables for final outcome., Results: In 171 cases (49.9%) vascular malformations, such as arteriovenous malformations (AVMs), cavernomas, dural fistulas and aneurysms were the cause of bleeding. 172 (50.1%) patients suffered from an intracerebral hemorrhage due to amyloid angiopathy or long standing hypertension. In patients with infratentorial hemorrhage a malformation was more frequently detected as in patients with supratentorial hemorrhage (36% vs. 16%, OR 2.9 [1.8;4.9], p<0.001). Among the malformations AVMs were most common (81%). Hematoma expansion was smaller in vascular malformation than non-malformation caused bleeding (24.1 cm3 vs. 64.8 cm3, OR 0.5 [0.4;0.7], p < 0.001,). In 6 (2.1%) cases diagnosis remained unclear. Final outcome was more favorable in patients with vascular malformations (63% vs. 12%, OR 12.8 [4.5;36.2], p<0.001)., Conclusion: Localization and bleeding patterns are predictive factors for origin of the hemorrhage. These predictive factors should quickly lead to appropriate vascular diagnostic measures. However, due to the inclusion criteria the validity of the study is limited and multicentre studies with further testing in general ICH patients are required., Competing Interests: The authors have declared that no competing interests exist.

Purpose of Review: Capillary malformations, the most common type of vascular malformation, are caused by a somatic mosaic mutation in GNAQ, which encodes the Gαq subunit of heterotrimeric G-proteins. How the single amino acid change - predicted to activate Gαq - causes capillary malformations is not known but recent advances are helping to unravel the mechanisms., Recent Findings: The GNAQ R183Q mutation is present not only in endothelial cells isolated from skin and brain capillary malformations but also in brain tissue underlying the capillary malformation, raising questions about the origin of capillary malformation-causing cells. Insights from computational analyses shed light on the mechanisms of constitutive activation and new basic science shows Gαq plays roles in sensing shear stress and in regulating cerebral blood flow., Summary: Several studies confirm the GNAQ R183Q mutation in 90% of nonsyndromic and Sturge-Weber syndrome (SWS) capillary malformations. The mutation is enriched in endothelial cells and blood vessels isolated from skin, brain, and choroidal capillary malformations, but whether the mutation resides in other cell types must be determined. Further, the mechanisms by which the R183Q mutation alters microvascular architecture and blood flow must be uncovered to develop new treatment strategies for SWS in particular, a devastating disease for which there is no cure.

Background: Percutaneous therapy with various sclerosants is an established treatment of venous malformations in general. We investigated the safety and effectiveness of polidocanol in the craniofacial region., Purpose: To present and evaluate our subjective and objective mid- and long-term results of patients with craniofacial venous malformations (CFVM) after percutaneous sclerotherapy using polidocanol., Material and Methods: Twenty patients with CFVM treated by percutaneous sclerotherapy were followed up and asked to fill in a questionnaire comparing levels of the following CFVM-related symptoms before and after treatment: pain; functional impairment; cosmetic deformities; and impairment in daily life. Additionally, both size reduction as well as procedural-related complication rates were analyzed., Results: Evaluation of the questionnaire revealed an improvement or complete relief of CFVM-related symptoms with significant reduced impairment in daily life after percutaneous sclerotherapy. Eighteen (90%) patients noticed a post-sclerotherapy improvement of at least one of their corresponding symptoms and expressed satisfaction with regard to their treatment. For 13 patients, a > 50% size reduction of the CFVM could be observed, while seven exhibited a < 50% size reduction. One minor complication was encountered in 56 treatment sessions (1.8%)., Conclusion: Percutaneous sclerotherapy using polidocanol is a well-tolerated treatment for CFVM with a low complication rate. Size reduction and positive results with improvement of different clinical symptoms can be achieved.

Objective: Multidisciplinary vascular anomaly clinics (VACs) offer important value to pediatric patients with complex vascular anomalies whose care overlaps specialties. These clinics are labor intensive and costly to operate since providers see fewer patients compared to their individual specialty clinic. Our North American tertiary care institution's VAC specialists include a pediatric otolaryngologist, pediatric surgeon, pediatric plastic surgeon, pediatric dermatologist, and interventional radiologist. To assess financial feasibility, we conducted a cost analysis of our VACs comprised of 2 half-day multidisciplinary physician attended clinics (5 specialists at our main campus and 2 specialists at a satellite clinic) and a half-day nurse practitioner clinic., Method: Assessment of net revenue based on net collections for clinic, professional, operative, hospital setting, and facility charges generated during 12 consecutive monthly VACs beginning July 1, 2015. Expense calculations included provider and staff salaries, benefits, supply costs, and clinic leasing costs., Results: There were 469 clinic visits, of which 202 were new patient evaluations. Sixty-eight patients underwent 93 procedures under general anesthesia, including procedures performed by our interventional radiologist, most commonly sclerotherapy or embolization (n = 37), surgical interventions including endoscopy (n = 36), or laser procedures (n = 20). Three patients were admitted. Fifty-seven patients received a new diagnosis different from that for which they were referred. Gross revenue was $1 810 525, and net revenue was 42.5%, or $783 152. Expenses totaled $453 415 for a net positive revenue of $329 737., Conclusion: When including direct downstream revenue, particularly from operative procedures, our VAC program operates on a net positive margin, making the program financially feasible.

Purpose: We aimed to evaluate the safety and effectiveness of cryoablation in the treatment of low-flow malformations, specifically venous malformation (VM) and fibroadipose vascular anomaly (FAVA)., Methods: We conducted a retrospective review of 11 consecutive patients with low-flow malformations (14 lesions; 9 VM, 5 FAVA), median lesion volume 10.8 cm3, (range, 1.8-55.6 cm3) with a median age of 19 years (range, 10-50 years) who underwent cryoablation to achieve symptomatic control. Average follow-up was at a median of 207 days postprocedure (range, 120-886 days). Indications for treatment included focal pain and swelling. Technical success was achieved if the cryoablation ice ball covered the region of the malformation that corresponded to the patient's symptoms. Clinical success was considered complete if all symptoms resolved and partial if some symptoms persisted but did not necessitate further treatment., Results: The technical success rate was 100%. At 1-month follow-up, 13 of 14 lesions (93%) had a complete response and one (7%) had a partial response. At 6-month follow-up 12 of 13 (92%) had a complete response and 1 (8%) had a partial response. A total of 6 patients underwent primary cryoablation. Out of 9 VM cases, 7 had prior sclerotherapy and 2 had primary cryoablation. Out of the 5 FAVA cases, 1 had prior sclerotherapy and the remaining 4 cases underwent primary cryoablation. There were 3 minor complications following cryoablation including 2 cases of skin blisters and 1 case of transient numbness. These complications resolved with conservative management., Conclusion: Cryoablation is safe and effective in the treatment of low-flow vascular malformations, either after sclerotherapy or as primary treatment.

The discoveries of new genes underlying genetic skin diseases have occurred at a rapid pace, supported by advances in DNA sequencing technologies. These discoveries have translated to an improved understanding of disease mechanisms at a molecular level and identified new therapeutic options based on molecular targets. This article highlights just a few of these recent discoveries for a diverse group of skin diseases, including tuberous sclerosis complex, ichthyoses, overgrowth syndromes, interferonopathies, and basal cell nevus syndrome, and how this has translated into novel targeted therapies and improved patient care., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Introduction: Vascular anomalies encompass an extremely heterogeneous group of congenital abnormalities of the vascular system. They include vascular tumors and malformations and have a prevalence of 4.5%. Vascular anomalies are frequently sporadic and associated with somatic mutations and/or a double-hit mechanism and are characterized by considerable phenotypic and genetic heterogeneity. The aim of this review was to provide a genetic description of vascular anomalies, the sequencing technologies used for their diagnosis and the drugs that may potentially be used for their treatment., Evidence Acquisition: PubMed, OMIM, Orphanet, Genetic Testing Registry and ClinicalTrials.gov were searched for monogenic vascular anomalies in order to evaluate the genetic tests (based on sequencing) currently used for their diagnosis, and for any drugs that could be useful to treat them., Evidence Synthesis: From the search of the clinical synopsis section of OMIM and PubMed for vascular anomalies we selected 19 disorders with a known molecular etiology. From the search for pharmacological trials and therapies in the ClinicalTrials.gov and PubMed databases we selected 87 drugs., Conclusions: Most genetic tests with validated clinical utility are based on a next generation sequencing (NGS) approach. Targeted NGS is indeed the best approach for the analysis of disorders with complex phenotypes and genetics and involvement of somatic mutations. Genetic diagnosis provides data for determine genotype-phenotype correlations, segregation and recurrence risk in families, and new targets for gene- or mutation-specific pharmacological therapies. Improvement of diagnostic techniques is needed to offer patients appropriate care, more focused follow-up, and hopefully drugs to treat their disorders.

Beta-blockers are efficient for treating complicated infantile hemangiomas; propranolol is currently the first-line treatment. Superficial vascular malformations have to be managed by multidisciplinary teams. T2 FAT-SAT MRI is the most interesting sequence to explore superficial vascular malformations. Recent advances in molecular biology allow exploring new genetic mutations which could be involved in vascular malformations and be the target of new drugs. Mammalian target of rapamycin (mTOR) inhibitors are promising drugs for slow-flow malformations. Arteriovenous malformations are aggressive lesions with very few treatment options., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

The traditional classification of congenital aortic arch abnormalities was described by James Stewart and colleagues in 1964. Since that time, advances in diagnostic imaging technology have led to better delineation of the vasculature anatomy and the identification of previously unrecognized and unclassified anomalies. In this manuscript, we review the existing literature and propose a series of modifications to the original Stewart classification of congenital aortic arch abnormalities to incorporate this new knowledge. In brief, we propose the following modifications: (1) In Group I, we further divide subgroup B into left arch atretic and right arch atretic; (2) In Group II, we add three more subgroups, including aberrant right innominate artery, "isolated" right innominate artery (RIA), "isolated" right carotid artery with aberrant right subclavian artery; (3) In Groups I, II, and III, we add a subgroup of absence of both ductus arteriosus; and (4) In Group IV, we add three subgroups, including circumflex retro-esophageal aorta arch, persistent V aortic arch, and anomalous origin of pulmonary artery from ascending aorta., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Vascular anomalies are common in the upper extremities, but there continues to be a relative paucity of information about them in publications dealing with surgery in the hands and upper limbs. The wide spectrum of pathology and an inconsistent use of terminology make vascular anomalies susceptible to incorrect diagnosis and as a result, to misdirected management. This article aims to provide an update on vascular anomalies relevant to the upper limbs, focusing on significant advances in pathogenesis and genetics, classification systems, diagnosis and treatment.

Vascular malformations and tumors, also known as "vascular anomalies", comprise an extensive variety of lesions involving all parts of the body. Due to a lack of a complete understanding of the origin and histopathology of such lesions, this field has been traditionally obscured by the use of an unclear nomenclature. Knowledge of the classification and clinical and imaging characteristics of this group of lesions is paramount when managing these patients. The objective of this series of two articles is to review the current classification of vascular anomalies, to describe the role of imaging in their diagnosis, to summarize their distinctive histopathologic, clinical and imaging features, and to discuss the treatment options. High-flow lesions were discussed in the first article of this series. In this second article, we will focus on low-flow lesions, including complex syndromes with associated low-flow malformations., (Copyright © 2018 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.)

An anomalous systemic arterial supply to the normal lung parenchyma with normal bronchial connections is a rare malformation. We report a case of a systemic arterial supply to the normal basal segment of the left lower lobe. The patient underwent resection of the left lower lobe. Here, we propose a revised nomenclature for a systemic arterial supply to the normal lung with normal bronchial connections., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: To review simultaneous intra-operative sclerotherapy (IOS) with immediate surgical resection for the treatment of cervicofacial venous malformations (VMs) at a single institution. While pre-operative sclerotherapy (POS) has been reported in the literature, simultaneous intra-operative sclerotherapy and surgery in the operating room has not., Methods: The database from the Hemangioma and Vascular Birthmarks Clinic was reviewed. All patients in both groups had biopsy-proven VMs., Results: IOS was used in 11 surgical patients with average age 17 years. Sclerotherapy was performed with sodium tetradecyl sulfate 3%, absolute alcohol or bleomycin. Immediately after IOS, and under the same anesthetic, all patients had either complete resection or debulking of the VMs. Eight patients had complete resolution of their VM and 3 had improvement. Average duration of the combined procedures done under a single anesthetic was 121 min. The POS approach was used for 6 surgical patients with average age 7 years. Sclerotherapy agents used were absolute alcohol or sodium tetradecyl sulfate 3%. All patients underwent complete resection of the VM 24-72 h after sclerotherapy under a separate surgical session. Five patients experienced complete resolution of their VM and one has had further sclerotherapy for recurrent disease. Interventional Radiology suite sclerotherapy times were on average 70 min. Surgical times were on average 142 min. Total combined anesthesia times for the two procedures added together were 212 min. Treatment time was significantly shorter in the IOS group (p = 0.0015)., Conclusions: Simultaneous IOS at the time of surgical resection has been successful in our hands. IOS has the advantage of a single procedure and decreased cost to the patient. In the era of reducing pediatric exposure to anesthesia, this approach is especially attractive in the pediatric population. As well, at approximately $100/minute cost to the patient to be in either the Interventional Radiology Suite or in the operating room, the reduced length of the procedures seen in the IOS approach results in lower overall cost to the patient., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: The term hepatopulmonary syndrome typically applies to cyanosis that results from "intrapulmonary vascular dilatation" due to advanced liver disease. Similar findings may result from a congenital portosystemic shunt without liver disease. An adverse consequence of such shunts is intrapulmonary vascular dilatation, which affects the microvascular gas exchange units for oxygen., Case Presentation: Here, we describe a toddler with chronic cyanosis, exercise intolerance, and finger clubbing due to a malformation shunt between the portal vein and the inferior vena cava. A transcatheter embolization of the shunt resulted in resolution of his findings., Conclusions: Congenital portosystemic shunts need to be considered in the differential diagnosis of cyanosis.

Individuals with chronic illnesses must manage long-term uncertainty as they cope with the ways the illness influences their lives. In the context of pediatric illnesses, parents must manage uncertainty during the diagnosis and treatment of their child's illness. It is common for children with complex chronic illnesses to see multiple specialists for the treatment of their condition. While previous research has explored parents' uncertainty during a child's diagnosis and during end-of-life care, less is known about these experiences when the child is referred to a team of specialists for treatment. The aim of the current study was to explore how specialists, as credible authorities, influence parents' uncertainty during parents' first visit to a multidisciplinary clinic for the care of their child's complex chronic illness. Data were collected through semi-structured interviews with 29 parents after their child's first visit to a vascular anomaly clinic at a large Midwestern children's hospital. The results suggest parents' communication with credible authorities facilitates effective uncertainty management primarily through the mechanism of uncertainty reappraisal. The results also suggest that specialists, as credible authorities, are a key mechanism in the appraisal of uncertainty for conditions that are often misdiagnosed and mismanaged.

Purpose: To evaluate radiofrequency ablation (RFA) for closure of marginal veins in pediatric and adult patients with venous malformations., Materials and Methods: Medical records, imaging and procedure details were retrospectively reviewed in patients who underwent RFA of the marginal vein in a 17-month period. Additional sclerotherapy (n = 19) and coil embolization of the marginal vein were performed (n = 2)., Results: A total of 23 marginal veins were treated in 20 patients. Mean age at treatment was 16 years ± 9.4 (1-37 years). Pre-procedural magnetic resonance imaging revealed thoracoabdominal marginal veins in 3 patients. A type I marginal vein (draining in the great saphenous vein below the popliteal vein) was identified in 1 and type IIa/IIb (draining in a median/lateral accessory saphenous vein) in 2/8 cases. Type III (draining into the profunda femoral vein) was detected in 8, and type IV (draining into gluteal veins) in 1. Mean diameter of the marginal veins was 13.2 mm ± 4 (7-20 mm). Patency was found in 1 during follow-up (22 months ± 9.8). Complete or partial occlusion was achieved in 94.5% of the veins. One patient showed signs of thrombophlebitis after the procedure, and another incomplete paresis of the peroneal nerve., Conclusion: RFA is effective as minimally invasive treatment of the marginal venous system. These veins should be treated early in life. Marginal veins with large diameter, residual tributaries and the intrafascial courses usually require adjunct coil embolization and sclerotherapy., Level of Evidence: Case series, Level IV.

Pain is a common symptom and the major complaint in patients with venous malformations of the extremities, which may lead to joint dysfunction and even walking disabilities. Therefore, this study aimed to investigate determined independent risk factors for pain in these patients. We retrospectively collected data for 168 patients with venous malformations of the extremities from January 16, 2013 to August 13, 2015. They were categorized into painful and painless groups according to the symptom and pain scores. Associations between pain and candidate factors were determined using univariate and multivariate analyses. A total of 125 (74.4%) patients with an average pain score of 4.4 were included in the painful group. In univariate analysis, age, lesion size, tissue involvement, and phleboliths were associated with pain. In the multivariate analysis, only type-II tissue involvement (adjusted odds ratio 4.57; p = 0.001) and phleboliths (adjusted odds ratio 2.44; p = 0.039) were identified as the independent risk factors. In conclusion, this study revealed that prevalence of pain in patients with venous malformations of the extremities was high. Patients who presented with type-II tissue involvement and phleboliths are more likely to suffer from pain.

The aim of the study was to assess the effectiveness of ultrasound-guided use of the foam form of ethoxysclerol for the treatment of venous malformations of the maxillofacial area. The study involved clinical and ultrasound examination data of 60 patient allowing to determine treatment tactics and optimal volume of injected foam sclerosant as well as the role of ultrasound for monitoring of the treatment. The results proved the efficacy of 3% foam ethoxysclerol for less invasive treatment of venous maxillofacial malformations.

Vascular malformations and tumors, also known as "vascular anomalies", comprise an extensive variety of lesions involving all parts of the body. Knowledge of their classification and imaging characteristics is paramount. Whereas hemangiomas are benign vascular tumors, characterized by cellular proliferation and hyperplasia; vascular malformations are not real tumors and characteristically exhibit normal endothelial turnover. Vascular malformations are classified according to the predominant vascular channel as arterial, capillary, venous, lymphatic, or mixed. Ultrasound and MRI are the main imaging modalities used in the diagnosis and classification of the vascular anomalies. In this series of two articles we review the classification of vascular anomalies, describe the role of imaging, summarize their distinctive histopathogenic, clinical and imaging features, and discuss the treatment options. On the first article we discuss the high-flow lesions, whereas the slow-flow lesions will be reviewed on the second. Complex syndromes with associated vascular tumors and malformations will be also presented., (Copyright © 2018 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.)

Purpose: To evaluate the clinical outcome of patients with venous malformation (VM) involving the eyelid treated with bleomycin sclerotherapy., Methods: A retrospective review was performed of 18 consecutive patients with VM involving the eyelid who underwent bleomycin sclerotherapy. Patients' clinical presentation, details of sclerotherapy, and post-sclerotherapy resolution of the lesion as well as any procedure-related complications were evaluated., Results: Twelve women and six men of mean age 34.3±20.4 years underwent sclerotherapy with bleomycin. Chief complaints were cosmetic disfigurations with or without hemifacial deformity (n=2), pain in engorgement area (n=2), pain and swelling from venous thrombosis (n=2), swelling or engorgement obstructing their eyesight (n=2), or eyelid dysfunction (n=1). The lesions were only in the eyelid in three patients; otherwise they were extended out of the eyelid either superiorly (n=3), laterally (n=8), inferiorly (n=8), and/or posteriorly to the orbit (n=8) to various extents. Conjunctival involvement was present in 13 patients. 14 patients had received prior treatments including surgery, laser therapy, or non-bleomycin sclerotherapy. With an average three sessions of bleomycin sclerotherapy (average total dose 34.5 mg), more than 80% shrinkage was observed in seven patients (38.9%), 50-80% shrinkage in eight patients (44.4%), and 30-50% shrinkage in two patients (11.1%). One patient had recurrence, which was successfully treated again with bleomycin. No procedure-related complications were noted., Conclusions: The use of bleomycin appears to be a simple, safe, and effective treatment for venous malformations involving the eyelid, avoiding more elaborate and challenging surgical or laser interventions, and is even effective in full thickness lesions., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2019. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

A 25-year-old female was referred to the Interventional Radiology Department for investigation and treatment of a rectal venous malformation (RVM) causing large recurrent episodes of rectal bleeding and chronic anaemia. Magnetic resonance imaging (MRI) demonstrated a large venous malformation affecting the rectum, lower pelvis and left thigh. After three failed attempts at injection foam sclerotherapy using fluoroscopic colonoscopy, a multidisciplinary team proposed an embolization procedure of the arterial inflow to the venous malformation. Following discussion with the patient, embolization of the superior rectal arteries was undertaken with immediate on-table fluoroscopic improvement in the RVM. Post-treatment, a significant reduction in bleeding, was reported by day 10 with subsequent return to activities of daily living and full employment. Follow-up MRI at 1 year demonstrated significant reduction in bowel-wall thickening.

An in-utero re-classification of umbilical-portal-systemic venous shunt (UPSVS) has recently been proposed. We retrospectively reviewed the sonograms of a large cohort of fetuses, identified and analyzed UPSVS cases, and presented the prenatal sonographic characteristics, birth outcomes, and follow-up results following the new classification system.Sonograms and clinical data of all participants who visited our departments from April 2016 to July 2018 were retrospectively reviewed. Identified cases of UPSVS were analyzed according to the new classification: Type I: umbilical-systemic shunt (USS); Type II: ductus venosus-systemic shunt (DVSS); Type IIIa: intrahepatic portal-systemic shunt (IHPSS) and Type IIIb: extrahepatic portal-systemic shunt (EHPSS). Postnatal follow-ups ranged from 3 months to 1 year.A total of 10 UPSVS cases were identified in 61,082 fetuses: 4 with Type I, 3 with Type II and 3 with Type IIIa. All 4 cases of USS had complete agenesis of the portal venous system, and had the umbilical vein drained into the inferior vena cava. Two USS cases also had trisomy 21. Pregnancy was terminated in all cases with a Type I shunt. Two fetuses with DVSS had normal portal venous system and were born full term. The pregnancy of 1 DVSS case was terminated due to the detection of trisomy 21. Three cases were IHPSS with full-term birth. One had chromosomal abnormality and 1 had surgery to repair the shunt 12-days post birth. In the 2 cases that did not receive repair surgery, sonographic examination revealed the portal-hepatic venous shunt was not closed at the 6-month follow-up period. However, the 1 case that had repair surgery appeared healthy at the 3-month follow-up period.UPSVS is extremely rare. Type I shunts have the poorest prognosis, and the presence of the intrahepatic portal venous system is key to live birth in UPSVS regardless of types. Chromosomal abnormalities and other organ anomalies can occur in any types of UPSVS. Therefore, karyotyping and examination of other organs should be performed once UPSVS is detected.

Background: Orbital vascular malformation often encircles normal tissue with ill-defined borders. It is easy to bleed during resection operation, making surgical treatment difficult and lesions hard to be removed completely. In this study we aimed to summarize the treatment outcomes by embolizing orbital vascular malformation with intraoperative intracavitary injection of medical glue ., Methods: A retrospective observational and cross-sectional case series study enrolled 31 patients (male = 9, female = 22) with orbital vascular malformations, who were treated from March 2008 to September 2017 at our institution. The clinical features, operation records, pathological reports and follow-up data were analyzed., Results: The location of vascular malformations involved intraorbital (14 cases), superficial area of eyelid and/or face (7 cases), both intraorbital and superficial area (10 cases). Imaging examination showed a solitary mass with regular shape in 8 cases and a space occupying lesion with irregular shape and ill-defined margins in 23 cases. There were 9 cases had optic nerve involved. Surgical debulkling were performed via skin incision on the mass surface (5 cases), lateral orbitotomy (2 cases), and anterior orbitotomy (24 cases). During the operation, lesions were partly exposed and injected with medical glue. The amount of injected glue was 0.25 ml to 2.5 ml in divided doses. The lesions and remnant glue were removed after the glue had turned hard. The whole procedure caused less bleeding and was easier performing than usual. Topical skin aseptic inflammation took place on the same side of the superficial eyelid lesions in 3 cases. One patient suffered from sudden central retinal artery embolism on the third day post operation. With timely rescue and appropriate procedure, visual acuity recovered to 20/32. There were no recurrences in 29 cases., Conclusions: Embolization of orbital vascular malformation with medical glue intraoperatively made it easy to control hemorrhage. Surgeons should be careful with glue application methods in order to avoid complications.

Notch expression has been shown to be aberrant in brain arteriovenous malformations (AVM), and targeting Notch has been suggested as an approach to their treatment. It is unclear whether extracranial vascular malformations follow the same patterning and Notch pathway defects. In this study, we examined human extracranial venous (VM) (n = 3), lymphatic (LM) (n = 10), and AV (n = 6) malformations, as well as sporadic brain AVMs (n = 3). In addition to showing that extracranial AVMs demonstrate interrupted elastin and that AVMs and LMs demonstrate abnormal α-smooth muscle actin just as brain AVMS do, our results demonstrate that NOTCH1, 2, 3 and 4 proteins are overexpressed to varying degrees in both the endothelial and mural lining of the malformed vessels in all types of malformations. We further show that two gamma secretase inhibitors (GSIs), DAPT (GSI-IX) and RO4929097, cause dose-dependent inhibition of Notch target gene expression (Hey1) and rate of migration of monolayer cultures of lymphatic endothelial cells (hLECs) and blood endothelial cells (HUVEC). GSIs also inhibit HUVEC network formation. hLECs are more sensitive to GSIs compared to HUVEC. GSIs have been found to be safe in clinical trials in patients with Alzheimer's disease or cancer. Our results provide further rationale to support testing of Notch inhibitors in patients with extracranial vascular malformations.

Importance: Differential diagnosis of congenital vascular anomalies is challenging, and misdiagnosis is frequent. Vascular malformations are considered one of the most difficult vascular diseases to treat. A new imaging approach that visualizes anatomical features and quantitatively assesses molecular biomarkers noninvasively would aid diagnosis and monitoring of treatment response of vascular malformations., Objective: To evaluate multispectral optoacoustic tomography (MSOT) for noninvasive assessment of molecular biomarkers for diagnosis and therapeutic monitoring of vascular malformations., Design, Setting, and Participants: This pilot study examined 6 patients with arteriovenous malformation (AVM) and 6 patients with venous malformation (VM) diagnosed according to the classification system of the International Society for the Study of Vascular Anomalies. All patients underwent clinical hybrid MSOT/ultrasonographic (US) imaging before and after treatment at an interdisciplinary vascular malformations clinic by trained MSOT and US examiners. Examiners were blinded to the patient history and stage of disease. Data were collected from April 11 to 25, 2017, and analyzed from May 1 to October 31, 2017., Interventions: Clinical hybrid MSOT/US imaging was performed before or within 1 week after endovascular embolization (for AVM) or percutaneous sclerotherapy (for VM)., Main Outcomes and Measures: Region-of-interest analysis of the lesion and contralateral healthy tissue revealed quantitative values for oxygenated (HbO2) and deoxygenated (HbR) hemoglobin by spectral unmixing of optoacoustic data acquired at multiple wavelengths. The HbO2:HbR ratio was calculated for healthy tissue and for AVM and VM before and after treatment., Results: Twelve patients (9 female and 3 male; mean [SD] age, 23 [18] years; age range, 6-59 years) with vascular malformations (6 with AVMs and 6 with VMs) were included. Significantly higher HbO2:HbR ratios for AVMs (mean [SEM], 1.82 [0.08] vs 0.89 [0.03]; P < .001) and for VMs (mean [SEM], 1.12 [0.04] vs 0.89 [0.03]; P = .001) were found on MSOT tissue compared with healthy tissue. Significantly higher HbO2:HbR ratios for AVMs compared with VMs (mean [SEM], 1.82 [0.08] vs 1.12 [0.04]; P < .001) were also found. Therefore, MSOT provided intrinsic biomarker patterns to distinguish both vascular malformations. After therapy, the HbO2:HbR ratio dropped in correlation to treatment success validated by magnetic resonance imaging or angiography., Conclusions and Relevance: This study suggests that different types of vascular malformations are clearly distinguished by MSOT-based, noninvasive assessment of hemoglobin levels in vascular malformations. The therapy effects found in this study could be instantly visualized, and this may offer a new tool for noninvasive diagnosis and monitoring of vascular malformations.

Vascular anomalies encompass a wide variety of clinical conditions involving the vasculature. Over the past several decades, the terminology has changed as our understanding of these conditions has improved. A well-accepted classification scheme has evolved with constant additions, updates, and revisions. Imaging techniques have also advanced with greater resolution and a better understanding of the correlation between imaging, clinical behavior, and natural history. Meanwhile, the treatment of these conditions has also changed, with greater focus on minimally invasive techniques as opposed to radical surgery. Despite these changes, surgical intervention remains a high-value option for select patients. With greater understanding of the molecular basis of these diseases, medical treatment has improved especially in severe cases where options remain limited. A multidisciplinary approach to comprehensive patient care usually yields the best outcome and is strongly encouraged., (Copyright © 2018 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.)

Cutaneous vascular anomalies can be challenging to diagnose and treat given their rare presentation and that they do not always fall under the purview of a specific medical specialty. Patients with cutaneous vascular anomalies are best managed in vascular anomalies clinics, but to our knowledge, no reported publications exist on how to develop such a clinic. In this article, we describe the specifics steps our institution took to create a vascular anomalies clinic and discuss how it benefitted our patients and referring providers. We also highlight the roles of the diagnostic and interventional radiologist within a vascular anomalies clinic., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Foam sclerotherapy is an effective treatment strategy for venous malformations (VMs). Stability is one of the characteristics of sclerosant foams that determine the sclerosing power., Objective: To evaluate the clinical outcomes of sclerotherapy with prolonged half-life of hyaluronic acid (HA)-polidocanol (POL) foam for treating head and neck VMs., Materials and Methods: Seventy patients with head and neck VMs were enrolled between October 2013 and January 2015 in Qilu Hospital Shandong University. Sclerotherapy was performed with prolonged half-life POL foam by addition of 0.05% HA. All patients were followed up at 1, 3, and 6 months. Reviews on the site and size of the lesion, times and duration of treatments, therapeutic response, and complications were performed to evaluate the end result., Results: Sites of lesions included face, bucca, parotid region, neck, tongue, floor of mouth, lip, forehead, palate, and nose. An overall average of 2.5 treatments was required. Total response rate was 100%. "Resolution" was achieved in 21 cases (30%) and a significant response in 49 patients (70%). Immediate swelling was the most common complication. Epidermal extravasation was seen in 1 patient. Mucosal ulcer was observed in 1 patient. No generalized complications occurred., Conclusion: The prolonged half-life of HA-POL foam sclerotherapy of VMs in the head and neck is safe and effective.