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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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Abstract: We describe a rare case of right aortic arch with bilateral arterial duct and disconnected left pulmonary artery. Prenatal diagnosis allowed to plan delivery at the referral center and to maintain perfusion of the disconnected pulmonary artery through prostaglandin E1 infusion until surgical reconnection was performed. Early postnatal treatment avoided functional loss of the left lung that would have happened if malformation had not been identified before birth. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background Torcular dural sinus malformations (tDSMs) are described as slow flow dural arteriovenous fistulae with frequently poor outcomes in the neuroangiographic literature, but other etiologies have been proposed in the obstetric literature, where outcomes have been more favorable. Objective To review tDSMs reported in the literature of multiple specialties for features that support a common etiology, and to identify key prognostic factors, with an emphasis on tDSM trajectory highlighted in part I. Methods Analysis of imaging features and clinical outcome for 77 prenatal and 22 postnatal tDSMs reported in 37 papers from the literature. Results In addition to large venous lakes, 36% of prenatal and 96% of postnatal tDSMs had evidence of arterialization, where specifically assessed. For fetal cases, where there was an observable natural history, 97% underwent a spontaneous decrease--13% after an initial increase and only 1 case with subsequent enlargement after a decrease. Prenatal cases had 83% survival (62% with a favorable outcome) whereas postnatal cases had 59% survival (29% favorable). In addition to a postnatal diagnosis, unfavorable features included ventriculomegaly, parenchymal injury, arterialization, and need for intervention. Favorable features included decreasing tDSM size, presence of clot, and increasing clot percentage. Conclusions Neonatal and fetal tDSMs have overlapping imaging appearances, suggesting a common etiology, where neonatal tDSMs represent those rare fetal tDSMs that do not undergo spontaneous regression and have a propensity for worse outcomes. Decrease in tDSM size is a critical observation when managing a tDSM because it is generally irreversible and associated with a favorable outcome. A morphologically identical lesion to the tDSM has been reported in the fetal imaging literature, but the inability to directly demonstrate the angioarchitecture of these lesions has led to alternative etiologies being advanced for this population (eg, primary dural sinus dysplasia or thrombosis followed by dural sinus dilation).2-13 Some autopsy papers have also reported extraluminal localization of these fetal tDSMs.14 15 In distinction to the neuroangiographic literature, the outcome for these fetal tDSMs has been favorable in as many as 75-90% of cases.8 10 16 These differences in formulation and outcome cast doubt as to whether tDSMs seen in utero and postnatally represent the same entity. They also raise questions about how best to counsel late gestation pregnancies affected with a tDSM and how best to follow such pregnancies. In part I of this article,17we examined a local cohort of tDSM cases and found imaging commonalities across the fetal-neonatal age spectrum. For example, we described a fetal tDSM with directly visualized arteriovenous communications in utero, as well as postnatal tDSMs where AVFs were undetectable even with catheter angiography. We also showed that tDSM involution in our cohort was an irreversible process and one that may occur despite initial enlargement. In part II, we have conducted an analysis of the fragmented multidisciplinary literature on tDSMs to highlight prognostic factors identifiable from other cohorts and assess these in light of the prognosticators in our cohort. We have synthesized these data to offer a plausible model for tDSM evolution across fetal-neonatal life and provided a rational approach to following tDSM pregnancies and performing interventions when necessary. [ABSTRACT FROM AUTHOR]

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 cm3 preoperatively to 1.56 ± 0.43 cm3 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. [ABSTRACT FROM AUTHOR]

Introduction Low-flow vascular malformations are congenital abnormalities of the veins, capillaries or lymphatic vessels or a combination of the previous. Compressive garments are frequently used as a first-line treatment option for low-flow vascular malformations of the extremities with the purpose of relieving symptoms. Yet, the benefits and harms of compression stockings remain unclear. Methods A systematic search was performed in MEDLINE, Embase, Cochrane Central Register of Controlled Trials including a hand search for studies measuring the benefits and harms of compression garments in treating low-flow vascular malformations. Two investigators performed study selection, quality assessment and data extraction independently. Results Of the 565 studies found, eventually five (totalling 101 patients with venous malformations or Klippel–Trenaunay syndrome receiving compression therapy) observational studies were included in the systematic review. Although the overall quality of these studies was poor, results suggest that compression garments might lessen intravascular coagulation, improve symptoms and appearance, diminish oedema, and protect against minor trauma. None of the studies quantified any harms of compression therapy. Conclusion Even though compression therapy is commonly used in the treatment of low-flow vascular malformations, available literature does not provide high-quality evidence to validate its use. We therefore advocate the need for prospective comparative trials with standardised outcome measures to study the benefits and harms of this treatment option. [ABSTRACT FROM AUTHOR]

Vascular malformations are classified primarily according to their flow characteristics, slow flow (lymphatic and venous) or fast flow (arteriovenous). They can occur anywhere in the body but have a unique presentation when affecting the female pelvis. With a detailed clinical history and the proper imaging studies, the correct diagnosis can be made and the best treatment can be initiated. Lymphatic and venous malformations are often treated with sclerotherapy while arteriovenous malformations usually require embolization. At times, surgical intervention of vascular malformations or medical management of lymphatic malformations has been implemented in a multidisciplinary approach to patient care. This review presents an overview of vascular malformations of the female pelvis, their clinical course, diagnostic studies, and treatment options. [ABSTRACT FROM AUTHOR]

We analyzed the characteristics of the population with congenital portosystemic shunt diagnosed during the antenatal period and the organization of their perinatal care. This multicentric retrospective study included all the patients with a prenatal diagnosis of congenital portosystemic shunt. Between 1999 and 2015, 12 patients were included. Prenatal diagnosis was done at a median 26.5 weeks of gestation (21-34). All the patients presented intrahepatic CPSS, three of them had associated congenital cardiopathy, and one a Bannayan-Zonana syndrome. Ten patients had simple outcome on conservative treatment, eight of them having a spontaneous closure of their portosystemic shunt within the first 2 years of life. One patient had surgical treatment which failed and he developed a focal nodular hyperplasia. Another patient had radiological interventional closure of his shunt which was complicated by a venal portal thrombosis.Conclusion: Outcome of intrahepatic portosystemic shunt diagnosed prenatally is good in the majority of cases. What is known: • Multiples studies exist on congenital porto systemic shunt but when the diagnosis is done after birth. • The evolution, management, and complication are well known. What is new: • There is very few studies with only patients diagnosed in antenatal and it is a large series of cases. • Outcome of intrahepatic portosystemic shunt diagnosed prenatally is good in the majority of cases. [ABSTRACT FROM AUTHOR]

Understanding and management of vascular anomalies has always been intriguing. These disorders exhibit an expected pattern of clinical presentation and progression, and characteristic imaging findings. Significant progress in understanding and treating patients with vascular anomalies has been made in the past quarter century. Newer multidisciplinary domains for treating these disorders with medical drugs and less invasive image-guided or surgical procedures are constantly evolving. Vascular anomalies can exhibit aggressive tumor-like behavior resulting in recurrence or persistent symptoms after treatment. Thermal ablation has been widely used in tumor treatment. This has generated interest on using thermal ablation for treating vascular anomalies. Percutaneous image-guided cryoablation is increasingly used for this purpose as compared with other ablation technologies. Availability of small caliber cryoprobes and the ability to monitor the freeze zone in real time have made this an attractive option to interventional radiologists. These experiences are relatively new and limited. It is helpful to understand the emerging role of this technology in the treatment of vascular anomalies. [ABSTRACT FROM AUTHOR]

It is very important that patients seeking sclerotherapy for the treatment of venous malformations are aware of the expected course of the therapy. They should be thoroughly counseled about the complications, the need for multiple sessions of therapy, and also about the expected clinical outcome. The aim of this review is to discuss the long-term outcomes of sclerotherapy for the treatment of venous malformation. Many studies have discussed their individual center's experiences and short-midterm results, but there is a relative paucity of data on long-term outcomes. We have reviewed the literature and also shared our experience of a large cohort of patients (n = 116) with a relatively longer follow-up period of more than 1 year. Venous malformations are very complex lesions and their treatment is quite variable depending on its extent and complexity. As a result, outcome studies vary considerably in the choice of sclerosant, study methodology, outcome assessment (clinical vs. imaging), and grading scales. This review also highlights this extreme heterogeneity in the literature of the sclerotherapy outcome and summarizes a few national and international studies for comparison. [ABSTRACT FROM AUTHOR]

Vascular anomalies comprise a spectrum of diseases that are broadly classified as tumors and malformations. Diagnosis is often challenging, given a wide range of clinical presentations with overlapping signs and symptoms. Accurate diagnosis is critical to determine prognosis and to generate a management plan, which frequently involves multiple subspecialists during different phases of treatment. An updated classification system provides structure and clear, consistent terminology, allowing for improved diagnosis, provider communication, and collaboration. Historically, treatment of vascular anomalies was primarily surgical and medical therapies were limited or ineffective. Recent discoveries of pharmacologic agents effective in treating vascular anomalies have broadened our medical therapeutic options, limiting the need for unnecessary or high-risk procedures and improving patients' quality of life. [ABSTRACT FROM AUTHOR]

Objectives Parkes Weber syndrome is a congenital vascular malformation which consists of capillary malformation, venous malformation, lymphatic malformation, and arteriovenous malformation. Although Parkes Weber syndrome is a clinically distinctive entity with serious complications, it is still frequently misdiagnosed as Klippel–Trenaunay syndrome that consists of the triad capillary malformation, venous malformation, and lymphatic malformation. Methods We performed a systematic review investigating clinical, diagnostic, and treatment modalities of Parkes Weber syndrome (PubMed/MEDLINE, Embase, and Cochrane databases). Thirty-six publications (48 patients) fulfilled the eligibility criteria. Results The median age of patients was 23 years (IQR, 8–32), and 24 (50.0%) were males. Lower extremity was affected in 42 (87.5%) and upper extremity in 6 (12.5%) patients; 15 (31.3%) patients developed high-output heart failure; 12 (25.0%) patients had chronic venous ulcerations, whereas 4 (8.3%) manifested distal arterial ischemia. The spinal arteriovenous malformations were reported in six (12.5%) patients and coexistence of aneurysmatic disease in five (10.4%) patients. The most frequently utilized invasive treatments were embotherapy followed by amputation and surgical arteriovenous malformation resection, and occasionally stent-graft implantation. All modalities showed clinical improvement. However, long follow-up and outcome remained unclear. Conclusion A diagnosis of Parkes Weber syndrome should be made on the presence of capillary malformation, venous malformation, lymphatic malformation, and arteriovenous malformation (as main defect) in overgrowth extremity. Arteriovenous malformation presents the criterion for distinguishing Parkes Weber syndrome from Klippel-Trenaunay syndrome, which is substantial for treatment strategy. The primary management goal should be patient's quality of life improvement and complication reduction. Embolization alone/combined with surgical resection targeting occlusion or removal of arteriovenous malformation “nidus” reliably leads to clinical improvement. [ABSTRACT FROM AUTHOR]

Objectives: To assess the treatment-induced changes of health-related quality-of-life (HRQoL) in patients with venous malformations (VM) who underwent ethanol gel sclerotherapy.Methods: The prospective study in children and adults was approved by the local ethics committee. 31 patients (mean age 23.42 years, range 6.6 - 46.5; 26 female, 5 male) with VM were included. Patients' self-assessed HRQoL was measured before and after treatment using psychometrically validated questionnaires for adults and children. Differences were analysed with a paired t test.Results: 58 sclerotherapy sessions were performed. The Physical Component Summary (PCS) at baseline was 43.69 and increased significantly (p = 0.01122) to 48.95 after treatment. The bodily pain (BP) scale increased significantly from 37.94 to 48.56 (p = 0.00002), the general health (GH) scale increased significantly from 46.69 to 52.17 (p = 0.00609). Baseline Physical Summary Score (PHS) in children increased significantly after treatment (p < 0.00001) from 25.25 to 45.89. The baseline Psychosocial Summary Score (PSS) in children was 51.08 and increased significantly (p = 0.00031) after treatment to 58.84.Conclusion: Paediatric and adult patients with VM suffer from bodily pain with overall reduced physical functioning. After sclerotherapy, these restrictions are successfully returning to normal levels with a positive effect on mental and psychosocial domains.Key Points: • Sclerotherapy in venous malformation patients has an unknown effect on health-related quality-of-life • Prospective study showed improvements in bodily pain and general health in adults • Children and adults improved from treatment with ethanol gel • Sclerotherapy is an appropriate therapy. [ABSTRACT FROM AUTHOR]

Aim Localized intravascular coagulopathy (LIC) has been described in adults with venous malformation (VM) but rarely reported in children. This study aims to determine the prevalence of LIC in children with VM and associated risk factors. Methods Patients younger than 18 years with VM from 2010 to 2014 were reviewed. Diagnosis was confirmed by Doppler ultrasound and/or magnetic resonance imaging. Demographics data and VM characteristics including volume, site, extension, painful symptoms, and palpable phleboliths were studied. Plasma D-dimer level of greater than 500 ng/mL was considered as abnormal. Results Total 24 children were included, of whom 8 were boys. Median age of presentation was 9 months (range: 0-12 years). Head-and-neck VM occurred in 17 (70.8%) patients and 3 (12.5%) had multifocal lesions. Seven (29.2%) patients had VM volume greater than 10 mL. Five (20.8%) patients had painful symptoms. Palpable phleboliths were found in two patients. Plasma D-dimer was raised in eight cases (33.3%). One patient with Klippel-Trenaunay syndrome (KTS) had D-dimer level of 5,000 ng/mL. Raised D-dimer was found in 23.5% of small VM (volume < 10 mL) and 57.1% of large VM (p = 0.167). D-dimer was significantly raised in multifocal VM (p = 0.028) and showed increasing trend in lesions with palpable phleboliths (p = 0.101). All patients had sclerotherapy performed with indications (cosmesis 41.7%, enlarging lesion 29.2%, pain 20.8%, bleeding 8.3%). Perioperatively, bolus intravenous fluid and mannitol were given to selected patients. All patients had VM volume reduction after sclerotherapy. There were no major thromboembolic complications. Conclusion LIC with raised D-dimer level occurred in one-third of pediatric VM. It was more common in large, multifocal VM and in those with palpable phleboliths or KTS. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to define morbidities and costs related to modern-day medical care for children with vascular anomalies.Methods: We reviewed the 2003-2009 Kids' Inpatient Database for pediatric patients (age < 21 years) hospitalized with hemangioma, arteriovenous malformation (AVM), or lymphatic malformation (LM). Patient characteristics, hospital complications, and hospital charges were compared by vascular anomaly type. Multivariable linear regression modeling was used to determine predictors of increasing hospital costs for patients with AVMs.Results: In total, 7485 pediatric inpatients with vascular anomalies were identified. Frequently associated complications included chronic anemia (4.0%), sepsis (4.6%), and hypertension (2.4%). Children with AVM had the highest rate of in-hospital mortality, compared to those with hemangiomas or LM (1.0% vs. 0.1% vs. 0.3%, p < 0.001). AVMs were also associated with the highest median hospital charge, more than twice the cost for hemangiomas or LM ($45,875 vs. $18,909 vs. $18,919; p < 0.001).Conclusions: There is a significant rate of morbidity in children with vascular anomalies, most often from blood loss and infection. The greater cost of AVM care may be related to the higher mortality rate, associated complications, and complexity of procedures required treating them. Cost-effective management of vascular anomalies should target prevention and the early recognition of both chronic comorbidities and acute complications. [ABSTRACT FROM AUTHOR]

Objective To summarise the role of fine needle aspiration cytology in the differential diagnosis of vascular anomalies from other lesions in the head and neck region by presenting five interesting cases reported from our hospital data. Method Five patients ranging in age from 3 months to 25 years old were diagnosed with vascular anomalies based on their histories, clinical examinations, imaging examinations and ultrasonic testing. The previous diagnosis of these patients were corrected by fine needle aspiration cytology in our hospital and the proper treatment was administered. Result Two cases were wrongly treated in a local hospital. All five of the patients underwent operations after excluding vascular anomalies by fine needle aspiration cytology. Conclusion Fine needle aspiration cytology should be used in the following situations to prevent misdiagnoses and the mistreatment as vascular anomalies: when imaging examination and ultrasonic testing lead to a diagnosis of vascular malformation, but the history or clinical examination does not confirm this diagnosis; when lesions are treated as “vascular malformations” by sclerotherapy, and the hydatid fluid is not like blood or lymph fluid; and when propranolol is used to treat the proliferation stage of “haemangiomas” with without any effect. [ABSTRACT FROM AUTHOR]

Aim of the Study mTOR inhibitors are showing promising results in the management of vascular anomalies. Although current controlled trials remain to be completed, many individual experiences are being published. We present our series of children with complex vascular anomalies treated with sirolimus. Patients and Methods A retrospective review of 41 patients treated with sirolimus between January 2011 and December 2015 was performed: 15% (n = 6) had vascular tumors (4 kaposiform hemangioendotheliomas, 1 PTEN) and 85% (n = 35) had malformations (13 generalized lymphatic anomalies/Gorham-Stout diseases [GSD], 1 kaposiform lymphangiomatosis [KLA], 11 large lymphatic malformations (LMs) in critical areas, 2 lymphedemas, 4 venous malformations, and 4 aggressive arteriovenous malformations [AVM]). Several variables were collected: type of vascular anomaly, duration of treatment, dosage, response, and secondary effects. Results There was a female predominance (1.4:1). All patients received sirolimus, at initial dosage of 0.8 mg/m2/12 hour. Overall successful response rate was 80.4% of cases, presenting improvement in radiologic imaging and reduction of symptoms, at a median time of 10 weeks. Patients showing no response included four AVMs, one GSD, one LM, one KLA, and one unknown tumor. Sirolimus was well tolerated, even in neonates, with insignificant side effects. No patients had complete resolution and no patients worsened on therapy. Thirty patients remain under treatment at the present moment. Conclusion Sirolimus has become a new therapeutic option for patients with vascular anomalies that do not respond to other treatments. Unfortunately, important questions as what is the most appropriate dosage and for how long should the patient be treated remain unanswered. An international registry followed by customized controlled trials is mandatory to clarify the future of this therapy. [ABSTRACT FROM AUTHOR]

Background and Aims. Vascular malformations are a vast group of congenital malformations that are present at birth. These malformations can cause pain, pressure, and cosmetic annoyance as well as downturn growth and development in a child in the case of high flow. Sclerotherapy has become an important tool in the treatment of vascular malformations. However, little is known about the success rate of sclerotherapy. Material and Methods. In this study, the efficiency of sclerotherapy in the treatment of vascular anomalies was investigated retrospectively in 63 patients treated in Turku University Hospital between 2003 and 2013. Results. Out of the 63 patients investigated, 83% (53) had venous malformations (VMs) and 9% (5) were defined as having arteriovenous malformations (AVMs). Patients with a VM were operated on, in 14% (8) out of all VM cases. Hence 86% (45) of patients with a VM received adequate help to their symptoms solely from sclerotherapy. The duration of treatment for the 14% of the VM patients that needed a surgical procedure was prolonged by 7–9 months, that is, by 41%. Conclusions. Sclerotherapy is an effective method in the treatment of VMs with a satisfactory clinical response in patients symptoms in 84% of cases. [ABSTRACT FROM AUTHOR]

Background Arteriovenous fistulas are critical for haemodialysis, but maturation rates remain poor. Experimental and anecdotal evidence has supported the use of transdermal glyceryl trinitrate ( GTN) patches. The aim of this RCT was to determine whether use of a GTN patch aids arteriovenous fistula maturation. Methods Patients referred for arteriovenous fistula formation were eligible. The GTN or placebo patch was applied immediately after surgery and left in situ for 24 h. The primary outcome measure was the change in venous diameter at 6 weeks after fistula formation. The secondary outcome measure was clinical fistula patency at 6 weeks. Results Of 200 patients recruited (533 screened), 101 were randomized to the placebo group and 99 to the GTN group. Of these, 81 and 86 respectively completed surgery, and had follow-up data available at 6 weeks. Improvements in venous diameter were similar in the two groups: mean(s.d.) increase 2·3(1·9) mm in the placebo group compared with 2·2(1·8) mm in the GTN group ( P = 0·704). The fistula failure rate did not differ significantly between the two groups: 23 per cent for placebo and 28 per cent for GTN ( P = 0·596). Conclusion GTN transdermal patches used for 24 h after surgery did not improve arteriovenous fistula maturation. Registration number: NCT01685710 (). [ABSTRACT FROM AUTHOR]

Stereotactic radiosurgery has long been recognized as the optimal form of management for high-grade arteriovenous malformations not amenable to surgical resection. Radiosurgical plans have generally relied upon the integration of stereotactic magnetic resonance angiography (MRA), standard contrast-enhanced magnetic resonance imaging (MRI), or computed tomography angiography (CTA) with biplane digital subtraction angiography (DSA). Current options are disadvantageous in that catheter-based biplane DSA is an invasive test associated with a small risk of complications and perhaps more importantly, the two-dimensional nature of DSA is an inherent limitation in creating radiosurgical contours. The necessity of multiple scans to create DSA contours for radiosurgical planning puts patients at increased risk. Furthermore, the inability to import two-dimensional plans into some radiosurgery programs, such as Cyberknife TPS, limits treatment options for patients. Defining the nidus itself is sometimes difficult in any of the traditional modalities as all draining veins and feeding arteries are included in the images. This sometimes necessitates targeting a larger volume, than strictly necessary, with stereotactic radiosurgery for treatment of the AVM. In this case report, we show the ability to use a less-invasive and three-dimensional form of angiography based on time-lapsed CTA (4D-CTA) rather than traditional DSA for radiosurgical planning. 4D-CTA may allow generation of a series of images, which can show the flow of contrast through the AVM. A review of these series may allow the surgeon to pick and use a volume set that best outlines the nidus with least interference from feeding arteries or draining veins. In addition, 4D-CTA scans can be uploaded into radiosurgery programs and allow three-dimensional targeting. This is the first reported case demonstrating the use of a 4D CTA and an MRI to delineate the AVM nidus for Gamma Knife radiosurgery, with complete obliteration of the nidus over time and subsequent management of associated radiation necrosis with bevacizumab. [ABSTRACT FROM AUTHOR]

Background We have introduced and performed percutaneous sclerotherapy on pediatric patients, and information regarding the mid- and long-term results after percutaneous treatment of peripheral venous malformations is necessary to counsel patients and their parents about the outcome of the therapy. This study was designed to retrospectively evaluate the long-term satisfaction of pediatric patients following percutaneous sclerotherapy for venous malformations (VMs). Methods A total of 53 children younger than 16 years of age with VMs who underwent sclerotherapy were included in this study. Self-assessment questionnaires regarding the treatment effectiveness and patients' satisfaction were sent to 50 of 53 patients. Sclerotherapy was performed using 3% polidocanol, absolute ethanol, or 5% ethanolamine oleate (EO). Results The median numbers of sessions per patient was 2.0 (range, 1-12), and the median follow-up time was 1.8 years (range, 6months-8.3years). Forty-two patients (84%) returned the questionnaire. The most frequent symptom was swelling (95%, 40/42) before sclerotherapy. After treatment, patients felt less pain (82%, 23/28), functional limitation (78%, 14/18), swelling (70%, 28/40), and cosmetic disfigurement (59%, 13/22). None of the patients responded 'worse' for any symptoms, and 30 patients (71%) reported being 'very satisfied' or 'satisfied' with the treatment. Conclusion Percutaneous sclerotherapy of VMs was safe and effective for relieving symptoms in our pediatric patients, and many of them were satisfied with the outcomes. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the characteristics of enhancement of focal nodular hyperplasia (FNH) of the liver by analyzing the dynamic contrast-enhanced multislice computed tomography (MSCT) features and correlating them with pathological findings. Patients and methods: Nine males and 16 females with pathologically confirmed FNH and complete preoperative contrast-enhanced MSCT data were recruited for this study. The imaging features of FNH on the pre-and postcontrast MSCT were analyzed by two experienced radiologists by consensus. Results: Pathology showed central scars and abnormal blood vessels in 17 and 21 of 25 lesions, respectively, while MSCT with multiphase enhancement showed central scars in eight of the 17 lesions (47.1%) and abnormal arteries or draining veins in 13 of the 21 lesions (61.9%). Furthermore, abnormal draining veins in five lesions were found to be diagnostic, which is another important finding. Conclusion: Multiphase scanning can provide the panorama of FNH lesions and reveal their enhancement patterns and pathological characteristics. Abnormal blood vessels within or around the lesion are demonstrated more often than central scar, and both should be observed for FNH diagnosis. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate clinical outcomes and their predictors in patients with venous malformation (VM) treated with foam sodium tetradecyl sulfate (STS) sclerotherapy.Methods: We retrospectively evaluated clinical outcomes of foam STS sclerotherapy in 86 patients with 91 VMs to assess reduction in pain and mass after treatment. Univariate and multivariate analysis was performed to determine possible predictors of clinical outcome with foam STS sclerotherapy.Results: A positive response of 49.5% in pain reduction and 52.7% in mass reduction was observed. The numerical rating scale (NRS) score improved from 4.36 ± 2.64 to 1.74 ± 1.57, and VM mass volume decreased to 41.7 ± 35.52% of the initial size. On multivariate analysis, a high baseline NRS score (odds ratio: 1.12, 95% confidence interval: 1.09-1.15) and VM location in the trunk versus the head and neck (odds ratio: 1.30, 95% confidence interval: 1.00-1.69) were positive predictors of pain improvement. Minor complications occurred in 11 (12.1%) patients and recurrence in 12 (13.2%) patients.Conclusions: Foam STS sclerotherapy is an effective treatment in venous malformation, with low complication risk. A high baseline NRS score and location in the trunk versus the head and neck were positive predictors in improvement of pain.Key Points: • Foam STS sclerotherapy is effective in VM, with low risk of complications. • Relief of pain tends to be dramatic in patients with severe pain. • Location of VM is a predictor of pain improvement. • The presence of a draining vein does not affect foam sclerotherapy. [ABSTRACT FROM AUTHOR]

Purpose: Venous malformations (VM) involving the hand and forearm often lead to chronic pain and dysfunction, and the threshold for treatment is high due to the risk of nerve and skin damage, functional deterioration and compartment syndrome. The purpose of this study is to demonstrate that sclerotherapy of diffuse and infiltrative VM of the hand is a safe and effective therapy.Materials and Methods: A retrospective review of all patients with diffuse and infiltrative VM of the hand and forearm treated with sclerotherapy from 2001 to 2014 was conducted. All VM were diagnosed during the clinical visit by a combination of physical examination and imaging. Sclerotherapy was performed under imaging guidance using ethanol and/or sodium tetradecyl sulfate foam. Clinical notes were reviewed for signs of treatment response and complications, including skin blistering and nerve injury.Results: Seventeen patients underwent a total of 40 sclerotherapy procedures. Patients were treated for pain (76%), swelling (29%) or paresthesias (6%). Treatments utilized ethanol (70%), sodium tetradecyl sulfate foam (22.5%) or a combination of these (7.5%). Twenty-four percent of patients had complete resolution of symptoms, 24% had partial relief of symptoms without need for further intervention, and 35% had some improvement after initial treatment but required additional treatments. Two skin complications were noted, both of which resolved. No motor or sensory loss was reported.Conclusion: Sclerotherapy is a safe and effective therapy for VM of the hand with over 83% of patients experiencing relief. [ABSTRACT FROM AUTHOR]

Purpose: To determine the efficacy of thoracic endovascular aortic repair (TEVAR) for degenerative aneurysm involving only the descending thoracic aorta (DTAA).Methods: An English-language literature review was performed through PubMed, Scopus, and Google Scholar to identify any study evaluating the outcomes of TEVAR for DTAA. The main endpoints of this analysis were all-cause 30-day and late postoperative mortality. Secondary outcome measures were procedure success, vascular access complications, paraplegia, stroke, early endoleaks during the index hospitalization, aneurysm-related death, reinterventions, and conversion to open repair. To control for the anticipated heterogeneity among small observational studies, absolute values and means were pooled using random effects models; the results are expressed as pooled proportions, means, or risk ratio (RR) with 95% confidence intervals (CIs).Results: Eleven studies reporting on 673 patients (mean age 72.6 years, mean aneurysm diameter 62.9 cm) with DTAA were selected for the analysis. Technical success was reported in 91.0% of patients, and vascular access complications requiring repair were encountered in 9.7% of cases. Pooled overall 30-day, 1-year, 2-year, and 3-year survival rates were 96.0%, 80.3%, 77.3%, and 74.0%, respectively. Five studies compared the results of TEVAR after elective (n=151) and urgent/emergent procedure (n=77); the latter was a predictor of 30-day mortality (17.1% vs 1.8%, RR 3.83, 95% CI 1.18 to 12.40, p=0.025). Paraplegia occurred in 3.2% of patients and was permanent in 1.4% of patients. The stroke rate was 2.7%. Early type I endoleak was observed in 7.3%, type II endoleak in 2.0%, and type III in 1.2% of patients. The mean follow-up of 9 studies was 22.3 months. At 3 years, freedom from reintervention was 90.3%. Death secondary to aneurysm rupture and/or fistula was reported in 3.2% of patients.Conclusion: Current results indicate that TEVAR for DTAA can be performed with rather high technical success, low postoperative morbidity, and good 3-year survival. [ABSTRACT FROM AUTHOR]

PHACE (posterior fossa brain malformation, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities) syndrome is a neurocutaneous disorder often involving the cerebral vasculature. PHACE patients appear to have early-onset and severe headaches more commonly than children without PHACE. The objective of this study was to characterize the clinical features and prevalence of headache by conducting a cross-sectional survey of families in 2 large PHACE registries. Sixty-six percent of eligible families completed the survey in which 62.7% of respondents reported headaches. Average age of headache onset was 48.8 months. Females were more likely to have headaches (68.6% vs 30.8%, P = .014). Families reported associated migrainous features including nausea (62.5%), vomiting (37.5%), photophobia (75%), and phonophobia (75%). Headaches occurred at least weekly in 29.4%, lasted ≥1 hour in 85.4%, and led to ≥1 hospital admission in 15.7%. Three respondents with headaches had at least 1 ischemic stroke. We demonstrated that headaches are common among PHACE patients, develop at an early age, and have migrainous features. [ABSTRACT FROM AUTHOR]

Background: Venous and lymphatic malformations of the head and neck can be successfully treated with percutaneous sclerotherapy.Purpose: To examine the utility of three-dimensional volumetric analysis to assess these lesions and their response to therapy.Material and Methods: Prospectively maintained procedure records were retrospectively reviewed to identify all patients with vascular malformations who underwent percutaneous sclerotherapy. Clinical data were used to classify lesions by apparent size and degree of visible physical asymmetry due to the lesions. Lesion volume was calculated using magnetic resonance images. Cohen's weighted kappa coefficients were calculated to assess both intra- and inter-rater agreement. Pearson coefficients were calculated to identify correlation between clinical and volumetric measures, both at initial diagnosis and following treatment.Results: Thirty-seven patients with head and neck venous or lymphatic malformations underwent 55 treatment sessions. Cohen's weighted kappa coefficients were 0.84 and 0.77 for intra- and inter-rater agreement, respectively. Clinical size did not significantly correlate with measured volume at diagnosis (ρ = 0.08, P = 0.57). For lymphatic malformations, total lesion volume correlated with volume of macrocystic components (ρ = 0.47, P < 0.01). Total volume reduction significantly correlated with clinical response grade (ρ = 0.46, P = 0.02). For lymphatic malformations, reduction of volume of the macrocystic component significantly correlated with clinical response grade (ρ = 0.44, P = 0.03).Conclusion: Changes in calculated volume corresponded to clinical measures of treatment response. Variability of qualitative approaches to lesion analysis may have led to the lack of correlation between initial size of a lesion based on clinical measures and calculated volume. Future research should include quantitative metrics to augment qualitative clinical results. [ABSTRACT FROM AUTHOR]

Introduction: Congenital portosystemic shunt (CPSS) is a rare entity without insufficiency in treatment issues. The aim of this article is to show our experience in the heterogeneity of this condition.Material and Methods: A retrospective study of 25 CPSS in the period 1995 to 2014 was conducted. Description of the morphology, clinical impact, and treatment is given.Results: According to the imaging techniques (IT), the shunt was apparently intrahepatic in 14 patients, extrahepatic in 10 patients, and mixed in 1 patient. In 14 children, IT showed hepatic portal circulation. In total shunts in which radiological examination was performed, invasive radiological techniques were able to demonstrate intrahepatic portal vein. In other patients, it was not investigated as they are asymptomatic. A child presented multiorgan failure with fulminant hepatic failure at birth. The shunt was radiologically closed and clinical impairment reversed rapidly. He is now asymptomatic with no longer images of CPSS in ultrasound scan controls. Also, seven children are asymptomatic at this time and are monitored periodically. Seven children had prenatal diagnosis, in five the shunt closed spontaneously. Nine children were symptomatic in their evolution (hyperammonemia, regenerative nodules, cholestasis, gastrointestinal bleeding). Of these, in five we performed balloon test occlusion, tolerated in all patients, followed by radiological closure. In our experience, the advancement of interventional radiology techniques avoided surgery to close the shunt.Conclusions: Morphologically, the CPSS is extremely heterogeneous, with multiple possible connections established. CPSS has multiple clinical presentations, from asymptomatic patients to acute liver failure. The therapeutic approach should be individualized and therefore held in overspecialized centers. [ABSTRACT FROM AUTHOR]

This study describes a new method for detecting retinal vasculature. This is a useful approach for ocular diseases diagnosis. In order to determine the accuracy of the proposed method, the output of the Canny, Gabor, Frangi and LoG filters was evaluated and compared to the results generated by a matched filter. The performance of this method is evaluated by measuring the diameter of the retinal vessel in images processed by the four classic techniques and matched filter. The vessel diameter values are compared to the values provided by the ground truth retinal images. In order to underline the accuracy of the matched filter, a qualitative assessment using the Pratt's figure of merit and percentage error rate was made. [ABSTRACT FROM AUTHOR]

Background Persistent left superior vena cava (PLSVC) occurs in approximately 1 in every 200 people, and with various malformations. Nurses who insert peripherally inserted central catheters (PICCs) may need to place a PICC line in this venous malformation. Aims To review the literature and assess the safety of positioning a PICC line in a PLSVC, and to also assess the ideal placement of a PICC line in a PLSVC with reference to a chest radiograph and intravenous electrocardiogram (IVECG) navigation. Methodology Literature search across 5 main databases, alongside hand-searched articles. Results No literature was found that prohibits placement of a PICC line in a PLSVC, unless the PLSVC enters the left atrium, and no literature was found that identifies an ideal position for the PICC tip in a PLSVC. Two approximate positions were highlighted: high in the PLSVC or passed through a bridging brachiocephalic vein to a right superior vena cava. Placing a PICC line in a PLSVC using IVECG navigation is shown to produce abnormal electrocardiogram readings. Conclusions A PICC line can be safely placed in a PLSVC as long as the PLSVC does not enter the left atrium, avoiding potential systemic embolization. Final positioning of the PICC tip on a chest radiograph is proposed to be in the midregion between the carina and the junction of the PLSVC/coronary sinus, with the aim of avoiding coronary sinus thrombosis and providing satisfactory dilution of infusate. If abnormal electrocardiogram readings during IVECG placement are seen, then PLSVC should be suspected. Further research and data are needed due to limited research in this area. [ABSTRACT FROM AUTHOR]

Background: The incidence of vascular anomalies in the head and neck is higher than in the extremities. It is especially common for vascular anomalies to involve the lip. The lips are a functionally and aesthetically important component of the head and neck area. A retrospective analysis of data from our vascular anomaly center was performed in order to understand the characteristics and treatment requirements of vascular anomalies of the lip and to establish which treatments are likely to lead to the best outcomes. Methods: A retrospective review was performed of the medical records of patients diagnosed with vascular anomalies of the upper or lower lip from January 2001 to September 2013. Using clinical photographs, radiologic findings, and patient records, the diagnosis of each case and the location of the vascular anomaly were recorded, along with information about treatment and follow-up. Results: A total of 1,606 patients were diagnosed with vascular anomalies over this time period, of whom 127 (7.9%) were found to have vascular anomalies in the lip only. Surgical treatment with or without embolization, sclerotherapy, laser therapy, medication only, and observation were the treatment strategies adopted in these cases. Conclusions: Vascular anomalies of the lip should be diagnosed accurately. Radiologic diagnosis played a crucial role in treatment planning, and several techniques were used to treat vascular anomalies of the lip. When surgical excision is indicated for the correction of vascular anomalies of the lip, the aesthetic and functional characteristics of the lip should be considered. [ABSTRACT FROM AUTHOR]

Aim: Patients with vascular anomalies are often misdiagnosed, leading to delayed or improper treatment. The aim of this study was to evaluate the impact of an interdisciplinary team on the diagnosis and treatment of paediatric patients with vascular anomalies.Methods: We reviewed the paediatric patients evaluated by our interdisciplinary team between 2002 and 2012, analysing the referral diagnosis, final diagnosis, patient age, sex, clinical history, laboratory tests, imaging studies and treatments.Results: Of the 480 patients who were evaluated, 435 (90.6%) had a vascular anomaly: 30.7% of all patients had a tumour and 55.2% had a malformation. Haemangiomas comprised 93.2% of all tumours, while malformations included capillary (9.8%), lymphatic (30.1%), venous (36.8%), arteriovenous (3.8%) and combined slow-flow (7.9%) malformations. Tumours were initially diagnosed correctly in 89.2% of the patients, but only 38.0% of the malformations were diagnosed correctly. Improper treatment was given to 1.4%, due to incorrect diagnoses.Conclusion: This study showed that haemangiomas were likely to be diagnosed correctly, but other tumours and vascular malformations were likely to be misdiagnosed. Misdiagnosis seldom led to improper treatment, but probably led to delayed treatment in many cases. The interdisciplinary approach led to improved diagnostics and treatment. [ABSTRACT FROM AUTHOR]

Objective: Venous malformation (VM) is a common vascular malformation in soft tissue. Its morphological and draining features, relationship with main vein, and hemodynamics in the lesion are not completely clear till now. Above information is important for choice of treatment. Methods: From January 2012 to December 2013, 23 patients were enrolled into this study. Percutaneous puncture into the lesion was made and contrast media was injected into venous sinus. Immediately after that CT scan was performed, and repeated several minutes later. Then three-dimensional imaging was performed to show the morphology and draining veins of VMs, and the relevant main veins. The hemodynamics in the lesion was also evaluated. Results: All patients underwent successful examination. Three-dimensional imaging showed an irregular shape of VM. Based on the draining features, VMs were classified into three types: (1) Type I (7/23): malformation without visible draining vein; (2) Type II (10/23): malformation with normal draining vein; (3) Type III (6/23): malformation with abnormal dilated draining vein. The flow in type I VMs was slow. Contrast media retained in the lesion for more than 1 day. The flow in type III VMs was fast. Contrast media flowed away rapidly. The flow of type II VMs was between type I and type III. According to above information and adjacent anatomy, all patients received suitable treatment, including sclerotherapy with absolute ethanol and bleomycin A5, intralesional copper wires retention, surgical removal, and combinational therapy of the above methods. Conclusions: This procedure can clearly show the morphological and draining features of VM and its relationship with main vein, and further evaluate the hemodynamics in the lesion, which are helpful for the choice of treatment. [ABSTRACT FROM AUTHOR]

Bevacizumab neutralizes a key stimulation factor in tumour angiogenesis - vascular endothelial growth factor (VEGF). The origin of VEGF is related to platelets, and the basic stimulus for its production is hypoxia. By neutralizing VEGF, changes occur in the blood that are manifested at the platelet and haemoglobin levels. Study results indicate that proteinuria is a side effect of bevacizumab treatment. The aim of the present study was to examine the percentages of proteinuria in patients with metastatic colorectal cancer after the administration of bevacizumab. The correlation between the degree of proteinuria and changes in the number of platelets and concentration of haemoglobin in patients, as well as in relation to the age (< 60 i ≥ 60) and gender of the patients, was examined. The study group of respondents included 20 patients with metastatic colorectal cancer (9 men and 11 women) ranging in age from 43 to 73 years. Proteinuria was tested using 'Laboquick' test strips. The levels of platelets and haemoglobin were measured using an autoanalyser, the type Cell Dyn 3700 - Abbott, according to the protocol prescribed by The International Federation of Clinical Chemistry and Laboratory Medicine (IFCC). Statistically significant differences were recorded for the platelet measurements before and after therapy in the overall group of respondents (p= 0.025). Statistically significant decreases in platelet counts and increases in haemoglobin concentrations were also noted in women (Plt: p=0.0036; Hb: p=0.0286) and in patients < 60 years old (Plt: p=0.0270; Hb: p=0.0553). No significant differences were identified in men or in patients ≥ 60 years of age. Proteinuria was detected in one case in which demonstrating an increase in only the recorded number of platelets and a decrease in the level of haemoglobin. Platelets and haemoglobin may serve as prognostic biomarkers of the risk of proteinuria. Based on the initial values of these parameters, we can predict the target group, in terms of gender and age, that may obtain the most benefit from bevacizumab therapy. [ABSTRACT FROM AUTHOR]

Defective paracrine Transforming Growth Factor-β (TGF-β) signaling between endothelial cells and the neighboring mural cells have been thought to lead to the development of vascular lesions that are characteristic of Hereditary Hemorrhagic Telangiectasia (HHT). This review highlights recent progress in our understanding of TGF-β signaling in mural cell recruitment and vessel stabilization and how perturbed TGF-β signaling might contribute to defective endothelial-mural cell interaction affecting vessel functionalities. Our recent findings have provided exciting insights into the role of thalidomide, a drug that reduces both the frequency and the duration of epistaxis in individuals with HHT by targeting mural cells. These advances provide opportunities for the development of new therapies for vascular malformations. [ABSTRACT FROM AUTHOR]

Purpose: We present a technical development of the endovascular treatment technique for vein of Galen aneurysmal malformation, using Onyx™ (ethylene vinyl alcohol copolymer) delivered under flow control through double-lumen balloon microcatheters. Materials and methods: An 11-month-old patient that initially presented with increasing head circumference was diagnosed with mural type vein of Galen aneurysmal malformation. Complete cure was obtained in a single endovascular treatment session. Onyx was delivered simultaneously through two double-lumen balloon microcatheters (Scepter™, Microvention, Terumo Group). In both arterial feeders, balloon inflation occluded the high-flow arterial-venous shunting and thus facilitated the controlled propagation of the embolic product in the afferent artery and the shunt point while preventing distal migration into the venous system. Results: Two years post-procedure, MR imaging showed persistent occlusion of arterial-venous shunts with complete regression of the venous dilatation. On clinical examination, the patient had no neurological deficits, and no cognitive impairment was detected at neuropsychological testing. Conclusion: The use of double-lumen balloon microcatheters for flow control during delivery of Onyx represents a viable alternative for the endovascular treatment of vein of Galen aneurysmal malformations. [ABSTRACT FROM AUTHOR]

carotid-cavernous fistulas are spontaneous or acquired connections between the carotid artery and the cavernous sinus, being classified as direct or indirect; being usually diagnosed in postmenopausal women, but are also associated with other pathologies such as pregnancy, sinusitis and cavernous sinus thrombosis. They are clinically characterized by ophthalmological symptoms and pulsatile tinnitus. A 51-year-old woman who started her current condition about 4 years ago with pulsatile tinnitus, to which were added progressively: Pain, conjunctival erythema, right eye proptosis and the occasional headache of moderate intensity. Carotid-cavernous fistula was diagnosed, for the technical difficulty inherent in the case was made a contralateral transvenous approach and embolization with 360° GDG coils, with successful evolution of the patient. The endovascular management of these lesions is currently possible with excellent results. [ABSTRACT FROM AUTHOR]

Background: Thalidomide is effective in the treatment of angiodysplasia. The mechanisms underlying its activity may be associated with inhibition of angiogenic factors. It was recently shown that Slit2/Robo1 signaling plays a role in angiogenesis. Purpose: The aim of this study was to explore the expression and effects of Robo1 and Slit2 in angiodysplasia and to identify the possible therapeutic mechanisms of thalidomide. Method: Slit2 and Robo1 expression were analyzed in tissue samples and human umbilical vein endothelial cells (HUVECs) treated with thalidomide using a combination of laboratory assays that were able to detect functional activity. Results: Slit2, Robo1 and vascular endothelial growth factor (VEGF) were strongly expressed in five angiodysplasia lesions out of seven cases, while expression was low in one out of seven normal tissues. Exposure of HUVECs to recombinant N-Slit2 resulted in an increase in VEGF levels and stimulated proliferation, migration and tube formation. These effects were blocked by an inhibitor of PI3K and thalidomide. Conclusions: Robo1 and Slit2 may have important roles in the formation of gastrointestinal vascular malformation. High concentrations of Slit2 increased the levels of VEGF in HUVECs via signaling through the PI3K/Akt pathway-an effect that could be inhibited by thalidomide. [ABSTRACT FROM AUTHOR]

Introduction: Most vascular anomalies are clinically easy to recognize. The age is the most prominent element to take into consideration for the diagnostic. Lesions differ by the moment of appearance: congenital hemangiomas and hemangioendotheliomas (occurred at birth); lymphatic- and venous malformations (emerged in the first week of life-unprogressive), infantile hemangiomas (emerged in the first week of life-fast evolutive) and arteriovenous malformations (appeared throughout childhood). One may diagnose up to 90% of cases based on aspect (color, depth, local temperature, presence of murmur). Doppler ultrasound and MRI may help in difficult cases. Materials and methods: We performed a prospective study of 54 cases with vascular anomalies from May 2010 to April 2013. Thirty-six girls and 18 boys treated with Propranolol - unselective beta-blocker. There were 50 vascular tumors and 4 vascular malformations. All patients underwent ECG and echocardiography before treatment. Four patients excluded based on consent withdrawal. Results: Treatment: age m=9,24 months, duration m=9,15 months and response: very good-66,66%, good-12,96%, partial-7,40%, no response-9,25% and aggravation-3,70%. As a conclusion, our results support the use of Propranolol in vascular anomalies. [ABSTRACT FROM AUTHOR]

Vascular anomalies (VAs) comprise a large variety of individual diagnoses that in different phases of treatment require a diverse number ofmedical specialists to provide optimal care. Medical therapies include agents usually associated with cancer chemotherapy, such as vincristine, as well more immunomodulatory types of drugs, such as glucocorticoids and sirolimus. These immunomodulating drugs are being successfully applied in cases that are typically categorized as vascular tumors, including kaposiform hemangioendothelioma (KHE) and tufted angioma (TA), as well as some of the more invasive types of vascularmalformations (i.e., microcystic lymphatic malformations and blue rubber bleb nevus syndrome (BRBNS). These therapies need to be combined with good supportive care, which often involves anticoagulation, antimicrobial prophylaxis, and comprehensive pain and symptom-relief strategies, as well as appropriate drug monitoring and management of side effects of medical treatment. The optimal care of these patients frequently involves close collaboration between surgeons, interventional and conventional radiologists, medical subspecialists, and nurses. [ABSTRACT FROM AUTHOR]

Children with vascularmalformations are bestmanaged with amultidisciplinary teamof specialists. Interventional radiology may deliver primary treatment such as staged sclerotherapy and embolization for malformations that are poor candidates for primary surgical resection or play a supportive role such as preoperative or intraoperative embolization. A thorough understanding of vascular morphology and flow dynamics is imperative to choosing the best treatment tool and technique. In this review, the author discusses the selection of techniques and tools used to treat vascular malformations based on their angiographic morphology. [ABSTRACT FROM AUTHOR]

Objectives: Complex venous malformations (VMs) may extensively involve the soft tissue. The treatment remains a challenge till now. Here we introduce a combinational therapy of copper wires and pingyangmycin (bleomycin A5,PYM). Methods: Copper wires were retained in VMs by repeated penetration with a straight needle. Subsequently, PYM solution was injected into the lesion. Eight to 10 days later, copper wires were removed. The dressing was changed every day until the puncture pores healed. Magnetic resonance imaging scanning was performed to observe the change of VMs. Results: From January 2001 to December 2011, 56 patients were treated. During the follow-up period, most of the VMs shrunk obviously. The symptoms were relieved or disappeared. The complications included local pain, temporary paraesthesia and moderate fever, which disappeared quickly after the removal of copper wires. Conclusions: This combinational therapy is a safe and effective approach for the complex VMs in soft tissue. [ABSTRACT FROM AUTHOR]

Introduction : Deep AVMs are more difficult to operate compared with other supratentorial locations due to the long working corridor through the brain, location in eloquent areas and vicinity with brain critical structures. Material and Method : We report a series of 7 cases with deep AVMs operated in the Fourth Department of Neurosurgery, Emergency Clinical Hospital Bagdasar-Arseni, between 2009 and 2013. Results : Six patients had ruptured AVMs and one presented frequent, refractory seizures due to a deep large porencephalic cyst. At admission, one patient presented mRS 2, 3 patients had mRS 4 and 3 patients had mRS 5. Patients underwent surgery and we achieved total resection in 5 patients and we left a residual nidus in two cases. These last 2 patients with residual nidus were referred to stereotactic radiosurgery Gamma Knife with good results. Following surgery mRS improved in all 7 patients, 3 had mRS 1, 3 had mRS 3 and one mRS 4. Outcome was favorable in 3 cases and slowly favorable in 4 patients. Conclusions : Deep AVMs frequently present sudden onset, with altered mental state, motor deficits and large intraparenchymatal hematomas. Surgery is mandatory for deep ruptured AVMs, being the only treatment that can provide neurological improvement through hematoma evacuation and cure through nidus resection. In experienced hands, deep AVMs can be successfully resected. Postoperative results are good, with improvement of state of consciousness and motor deficits. Adequate patient selection is essential in order to achieve a good postoperative outcome. [ABSTRACT FROM AUTHOR]

Arteriovenous malformations occur when abnormalities of vascular patterning result in the flow of blood from arteries to veins without an intervening capillary bed. Recent work has revealed the importance of the Notch and TGF-β signaling pathways in vascular patterning. Specifically, Notch signaling has an increasingly apparent role in arterial specification and suppression of branching, whereas TGF-β is implicated in vascular smooth muscle development and remodeling under angiogenic stimuli. These physiologic roles, consequently, have implicated both pathways in the pathogenesis of arteriovenous malformation. In this review, we summarize the studies of endothelial signaling that contribute to arteriovenous malformation and the roles of genes implicated in their pathogenesis. We further discuss how endothelial signaling may contribute to vascular smooth muscle development and how knowledge of signaling pathways may provide us targets for medical therapy in these vascular lesions. [ABSTRACT FROM AUTHOR]