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

Vascular anomalies are classified by the International Society for the Study of Vascular Anomalies (ISSVA). They result from an abnormal embryonic development of vessels, and in most cases, they are present at birth. A minority of VAs manifest at a later stage. They grow along with the child and may enlarge spontaneously during an infection. They often create aesthetic flaws, pain, impaired quality of life and require challenging treatment options that frequently do not provide patients with desired results. Part of these anomalies are classified as lymphatic. Proper diagnosis is based on radiological tests, including ultrasonography, computed tomography and magnetic resonance. It is vital to establish a correct diagnosis that precedes the appropriate treatment. The mammalian target of rapamycin (mTOR) inhibitor sirolimus is an effective treatment for difficult lymphatic anomalies. This article presents the current use of sirolimus and its antiproliferative properties that enables mTOR pathway inhibition, leading directly to stopping, reversing or minimising the effects of development of vascular anomalies. So far, it has been proven that sirolimus has a measurable effect on lymphatic malformations, is well tolerated, manageable and rarely produces adverse effects. Still, there are no guidelines for sirolimus care in patients with vascular anomalies as we lack prospective clinical trials. [ABSTRACT FROM AUTHOR]

Objectives: Contrast agents are used widely in the interventional setting and in particularly in the management of vascular anomalies and have also been used in combination with sclero-embolic agents. There is limited information on the interaction of contrast agents with sclerosant agents when used as mixtures. The aim of this study was to determine the effect of mixing radiological contrast agents with detergent sclerosants and measuring the effect on change in lytic activity of detergent sclerosants in vitro and by proxy the change in potency. Methods: Red blood cell lysis was assessed following the incubation of two commonly used contrast agents, LIPIODOL® and ULTRAVIST®, mixed with detergent sclerosants, FIBROVEIN®, sodium tetradecyl sulfate (STS), and AETHOXYSKLEROL®, polidocanol (POL). Results: The density of both contrast agents was higher than STS and POL and neither of the detergent sclerosants were miscible in LIPIODOL. LIPIODOL on its own caused cell lysis (1.01%, p < 0.05) whereas ULTRAVIST did not. Fifty per cent cell lysis for sclerosant and LIPIODOL mix occurred at concentrations of: 0.041% (2.4 times greater than the control, p < 0.05) and 0.08% (3.6 times greater than the control, p = 0.06) for STS and POL, respectively. Conclusions: LIPIODOL, when mixed with sclerosant detergents (ratio 1:1) causes a reduction in the lytic activity of sclerosants and this effect was statistically significant and most prominent in lower sclerosant concentration mixtures. [ABSTRACT FROM AUTHOR]

Background: Capillary malformations of the head and neck region often cause psychological and physical burden. As the effectiveness of modern laser and light therapies is still suboptimal, patients often seek different therapeutic strategies. Other recognized, but not routinely proposed therapies include cosmetic camouflage, surgery, and medical tattooing. Information on therapeutic outcomes is currently lacking for patients to adequately participate in the treatment decision-making process. Objective: The objective of this systematic review was to review the effectiveness and safety of recognized therapies for untreated capillary malformations of the head and neck: laser and light treatment modalities, photodynamic therapy, cosmetic camouflage, medical tattooing, and surgery. Methods: PubMed, Embase, and the Cochrane Central Register of Controlled Trials were searched up to 16 December, 2020 for observational and experimental studies examining recognized therapies for untreated capillary malformations of the head and neck. Two reviewers independently evaluated the risk of bias of included studies. Predefined treatment and safety outcomes of pooled data were scored using the Grading of Recommendations Assessment, Development and Evaluation (GRADE). Results: We included 48 observational and three randomized studies (totaling 3068 patients), evaluating nine different therapies. No studies on surgery or cosmetic camouflage matched our inclusion criteria. The pooled proportion of patients reaching a ≥75% clearance was 43% (95% confidence interval 24–64%; I2 = 55%) for the pulsed dye laser after three to eight treatment sessions (GRADE score: very low). Other therapies were less effective. Hyperpigmentation was most frequently described after the pulsed dye laser (incidences up to 40%). Pain was most common after photodynamic therapy, yet the intensity was unreported. Substantial heterogeneity among studies as to patient characteristics and outcomes limited pooling and data comparisons. Conclusions: The pulsed dye laser seems preferable for treatment-naive capillary malformations of the head and neck region, yet demonstrates greater hyperpigmentation rates compared with other therapies. Our results are, however, based on low-quality evidence. Future studies using uniform outcome measures and validated metrics are warranted for study comparability. Based on this systematic review, clinicians and patients should be aware of the limited evidence about the available options when making (shared) treatment decisions for capillary malformations. Trial Registration: Review registration number PROSPERO database: CRD42020199445. [ABSTRACT FROM AUTHOR]

Background: Based on clinical observations we hypothesized that patients with intramuscular venous malformations (VMs) did worse or needed more sclerotherapy sessions than patients with extramuscular VMs. Purpose: To evaluate the difference in treatment and quality of life (QOL) results after Polidocanol sclerotherapy of intra- and extramuscular low-flow VMs. Material and methods: Forty-one patients with a VM were treated with Polidocanol in two university hospitals. The results were retrospectively analyzed. Pre- and post-treatment magnetic resonance imaging (MRI) scans were compared. All patients completed a self-evaluating form on symptoms as well as a QOL questionnaire. The results were compared between two groups: intra- and extramuscular VM's. Results: No statistically significant differences between intra- and extramuscular groups concerning QOL and specific dimensions pain, functional problems or cosmetic appearance were found. Radiological changes in MRI examinations did not correlate with any of the subjective symptoms. 56% of the patients benefitted from the treatment according to patient self-evaluation. Conclusion: In this material intramuscular VM's responded to the treatment comparably to extramuscular malformations. Post-treatment MRI findings do not correlate with subjective symptoms or QOL results and thus, a routine post-treatment examination seems to be unwarranted. Subjective symptoms and QOL results are the most important parameters in evaluating the effectiveness of sclerotherapy. [ABSTRACT FROM AUTHOR]

There are not many error proof clinical scores to assess the native dialysis access. CAVeA2T2 score is a recent tool in use. Objective of the study is to assess the clinical utility of CAVeA2T2 scoring system in predicting the survival rate of brachiocephalic arteriovenous fistula (BC-AVF). Methods: All consecutive patients fulfilling the inclusion criteria for BC-AVF from January 2016 to January 2018 were included. According to their CAVeA2T2 score they were divided into two groups (Group A: < 2 and Group B: ≥2). Cumulative primary and secondary patency survival of BC-AVF for both groups were measured. Results: A total of 112 BC-AVFs were analysed. Mean age was 42±SD 14 years (M: F =5:1). Mean CAVeA2T2 score was 1.45±1.8. In terms of primary patency, there was no statistically significant difference between two groups (p=0.074, p = 0.229 and p=0.357 at 6 weeks, 6 months and 12 months respectively). However, the difference was significant in terms of secondary patency (p=0.002, p=0.036 and p=0.032 at 6 weeks, 6 months and 12 months respectively). On comparing the cumulative survival between two groups; a significantly low primary patency rate survival (Log Rank x2 = 12.9, p-value = 0.001) and secondary patency rate survival (Log Rank x2 = 7.6, p-value = 0.001) of BC-AVF was found in Group B. Conclusion: We found CAVeA2T2 score an easily applicable and useful tool to assess the patency and survival of BC-AVF. Patients have a poor patency and significantly low survival rate when their CAVeA2T2 score was ≥2. [ABSTRACT FROM AUTHOR]

Objective: Vascular anomalies are a diverse group of lesions, ranging from simple to complex, disfiguring anomalies. Our objective was to diagnose and provide comprehensive treatment to patients presenting with vascular anomalies, using a multi-disciplinary approach involving dermatologists, plastic surgeons, radiologists and pediatric surgeons. Methods: Patients presenting with vascular anomalies to The Indus Hospital, Karachi, from January 2017 to March 2019 were enrolled, using a pre-defined questionnaire. Assessment, diagnostic work up, management and clinical and photographic follow up was maintained to monitor outcomes. Results: One hundred eighty seven patients with a mean age of 4.6 years, (females 62%) were enrolled. Diagnoses included vascular tumors (n=89, 47.6%), lymphatic malformations (n=38, 20.3%), capillary malformations (n=19, 10%), venous malformations (n=16, 8.5%), arterio-venous malformations (n=14, 7.5%) and mixed anomalies (n=11, 5.9%). Treatment modalities, in isolation or combination, included oral propranolol, topical timolol, pulsed dye laser and intra-lesional sclerotherapy. Mean follow up was in 7.1 months, with 27 patients achieving treatment completion. 26 children were lost to follow-up. Conclusions: Vascular anomalies have mostly been managed successfully at VAC using single or multimodal treatment. Increasingly complex anomalies can be handled using a multi-disciplinary approach. Establishment of VAC has facilitated many patients who were earlier considered as diagnostic and therapeutic challenges. [ABSTRACT FROM AUTHOR]

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. [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]

Purpose This manuscript describes the technique of real-time MRI-guided sclerotherapy for low-flow venous malformations in the head and neck based on our institutional experience. Materials and methods Ethanolamine oleate is used as the sclerosant and is mixed with gadolinium for visualization during the procedure. The five procedural steps include: (I) an initial tri-plane T2-weighted sequence to visualize the lesion; (II) a T1 FSE or trueFISP sequence to assess needle placement and advancement within the lesion; (III) a tri-plane T1 FLASH sequence to monitor sclerosant injection; (IV) a T1 FSE or VIBE sequence to assess sclerosant coverage of the malformation before needle removal; (V) a post-procedural tri-plane T1 fat-saturated sequence to confirm sclerosant coverage of the lesion. Periprocedural medications typically include steroids, antibiotic prophylaxis, and non-steroidal anti-inflammatory medication. Patients are typically admitted for overnight observation. Conclusion Real-time MRI-guided sclerotherapy for low-flow venous malformations in the head and neck is effective and safe. [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: Sporadic vascular malformations (VMs) are complex congenital anomalies of blood vessels that lead to stroke, life-threatening bleeds, disfigurement, overgrowth, and/or pain. Therapeutic options are severely limited, and multidisciplinary management remains challenging, particularly for high-flow arteriovenous malformations (AVM).Methods: To investigate the pathogenesis of sporadic intracranial and extracranial VMs in 160 children in which known genetic causes had been excluded, we sequenced DNA from affected tissue and optimized analysis for detection of low mutant allele frequency.Results: We discovered multiple mosaic-activating variants in 4 genes of the RAS/MAPK pathway, KRAS, NRAS, BRAF, and MAP2K1, a pathway commonly activated in cancer and responsible for the germline RAS-opathies. These variants were more frequent in high-flow than low-flow VMs. In vitro characterization and 2 transgenic zebrafish AVM models that recapitulated the human phenotype validated the pathogenesis of the mutant alleles. Importantly, treatment of AVM-BRAF mutant zebrafish with the BRAF inhibitor vemurafinib restored blood flow in AVM.Conclusion: Our findings uncover a major cause of sporadic VMs of different clinical types and thereby offer the potential of personalized medical treatment by repurposing existing licensed cancer therapies.Funding: This work was funded or supported by grants from the AVM Butterfly Charity, the Wellcome Trust (UK), the Medical Research Council (UK), the UK National Institute for Health Research, the L'Oreal-Melanoma Research Alliance, the European Research Council, and the National Human Genome Research Institute (US). [ABSTRACT FROM AUTHOR]

Introduction While surgical resection remains a primary treatment for accessible arteriovenous malformations (AVMs), stereotactic radiosurgery (SRS) has become thoroughly integrated into the AVM armamentarium; however, delayed sequelae of this treatment have become evident with increased long-term follow-up. To our knowledge, this is the second case of an aberrant local arterial angiographic blush without early venous drainage or an associated lesion following AVM SRS. Case Description An 8-year-old female presented with a ruptured 4-cm right medial frontal periventricular Spetzler-Martin grade 3 AVM with isolated intraventricular hemorrhage. She underwent subtotal resection followed by SRS. Six years later, diagnostic cerebral angiography demonstrated a prominent arterial-phase filling microvasculature without early venous drainage in the region of the irradiated residual AVM nidus. Conclusion Although there is a paucity of information on angiographic blush following AVM SRS, consensus in the literature suggests that without early venous drainage, these lesions appear to pose an insignificant threat to the patient. These angiographic findings may be on a spectrum of delayed cerebrovascular radiation changes, and thus indefinite follow-up may be considered, especially in pediatric patients. [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]

Bleomycin has progressively been used to treat low-flow vascular malformations in children. No significant systemic side effects have been reported in large series after low doses, but some authors are still concerned about its use. We report a case of a severe acute lung toxicity after a low dose of a second bleomycin intralesional injection in a 5-year-old girl. She had no risk factors and presented a cervical low-flow venous malformation. Twenty-four hours after this second administration, she presented with fever and respiratory distress. A chest radiograph showed bilateral opacities and computerized tomography revealed extensive and diffuse lung ground-glass opacities. The patient started to receive intravenous methylprednisolone, but she experienced progressively increased dyspnea, and montelukast was added. She improved and was discharged from the hospital without oxygen support, with montelukast and prednisolone for tapering doses during months. Five months after onset, the patient is developing well, is active, and walks and talks without dyspnea. A new low-dose computed tomography shows improvement in radiologic findings. This is the second case of pulmonary toxicity observed in a child after bleomycin intralesional administration, and the first reported after the lowest dose of this drug to date (7 mg: 0.28 mg/kg; 10 U: 0.4 U/kg). A delay in the diagnosis and treatment of this complication can be fatal. Any physician who treats these patients must be alert and consider this complication in children with respiratory symptoms after bleomycin sclerotherapy. Early detection of pulmonary toxicity would allow prompt therapy and could avoid pulmonary damage. [ABSTRACT FROM AUTHOR]

Objective: (1) Discuss the presentation and management of persistent stapedial artery (PSA) discovered incidentally during cholesteatoma surgery. (2) Review use of carbon dioxide (CO2) laser for treatment of PSA in the setting of chronic ear disease. Patients: Two consecutive patients with PSA and primary acquired cholesteatoma. Intervention(s): Tympanomastoidectomy using a canal wall reconstruction technique. Main Outcome Measure(s): Surgical healing, collateral damage to surrounding anatomy, and postoperative hearing. Results: Persistent stapedial artery was detected incidentally in 2 patients, manifesting with heavy pulsatile bleeding arising during dissection of mesotympanic cholesteatoma. Preoperative computed tomography scans demonstrated absent foramen spinosum but no other aberrant vascular anatomy. Using CO2 laser, the PSAs were ablated and controlled, allowing complete resection of cholesteatoma and successful completion of the procedure. In both patients, surrounding anatomical structures suffered no iatrogenic injury. Postoperatively, no significant complications occurred. To date, neither patient has demonstrated evidence of recidivistic cholesteatoma. Conclusions: A CO2 laser can be useful for managing a PSA in the setting of chronic ear disease. [ABSTRACT FROM AUTHOR]

Scleroderma (systemic sclerosis, scleroderma) is a chronic, progressive autoimmune disease characterized by damage to blood vessels, the presence of autoantibodies, progressive hardening, atrophy of the skin and subcutaneous tissue and damage to many internal organs. In scleroderma we observe peripheral microcirculation disorders, in which small peripheral vascular abnormalities play an important role. Raynaud's phenomenon is the most common manifestation of peripheral microcirculation disorders in the course of systemic sclerosis and concerns mainly the fingers. Treatment of patients with systemic sclerosis should be comprehensive and include education of patients, use of medication and rehabilitation. Drugs of first choice for the treatment of peripheral microcirculation disorders include calcium channel blockers, phosphodiesterase inhibitors, and prostaglandins. From our clinical experience gained in the treatment of microvascular disorders, sulodexide [a mixture of heparin (80%) and dermatan sulfate (20%)] seems to be a good and safe drug worth recommending. It works as an anticoagulant, pro-fibrinolytic, anti-inflammatory, inhibits the fibrosis process, and has protective effects on the vascular endothelial cells. Sulodexide is a safe rheological drug successfully used to treat a number of diseases accompanied by microcirculation disorders, including scleroderma. [ABSTRACT FROM AUTHOR]

Venous malformations are slow-flow congenital vascular malformations that enlarge as the child ages and may be associated with localised intravascular coagulation, a consumptive coagulopathy characterised by elevated D-dimer and decreased fibrinogen levels. The authors review the known correlations between localised intravascular coagulation and venous malformation number, size and planes involved, and call attention to the concept of the progression of localised intravascular coagulopathy as the child ages and their venous malformations enlarge. The authors also discuss the identified therapeutic options for its investigation, management and treatment, including compression garments, anti-coagulation therapy, sclerotherapy, endovascular laser, surgical excision and sirolimus (rapamycin). Evidence for protocol improvements that may be instigated for the optimal physical and medical therapy of venous malformations complicated by localised intravascular coagulopathy is reviewed. [ABSTRACT FROM AUTHOR]

AIM: Comparison of different ultrasound elastography techniques for detection of changes after sclerotherapy within venous malformations. MATERIALANDMETHODS:In patients with venous malformations sonographywas executed at exactly the same position prior to and after ethanol-gel sclerotherapy. Both examinations included B-Mode, vascular sonography with Color-Coded Duplex Sonography, and additional sonography with different elastography techniques (strain, qualitative and quantitative Acoustic Radiation Force Impulse (ARFI) elastography) with a linear transducer (6-9 MHz). Qualitative elastograms were read in consensus and scored. Differences of elasticity scores were statistically analyzed, p-values < 0.05 were regarded significant. RESULTS: Elasticity scores of strain and qualitative ARFI elastography in 25 patients (21 females, averagely 24.4 years old) were comparable before treatment (p = 0.69). After therapy qualitative ARFI scores changed significantly compared to pre-treatment scores (p = 0.0017), whereas strain elastography scores revealed no significant changes (p = 0.13). Quantitative ARFI values obtained after sclerotherapy within the venous malformations were significantly higher compared to pretreatment values (p = 0.049), and significantly higher to values obtained in surrounding tissue (p = 0.030). Comparison of preand post-treatment ARFI values of the surrounding tissue was not significant (p = 0.67). CONCLUSION: Elasticity scores of qualitative ARFI elastography reliably detect ethanol-gel induced changes in venous malformations. Quantitative ARFI may be a tool for therapy planning, and for monitoring sclerotherapy outcome as well as the effect of sclerosing agents on malformation and surrounding tissue in patients with venous malformations. [ABSTRACT FROM AUTHOR]

Arteriovenous malformations (AVMs) are high-flow vascular anomalies that have demonstrated a very high recurrence rate after endovascular treatment, surgical treatment, or a combination of both. Surgical treatments have shown good response when they are small and well localized but a poor response when diffuse. A better understanding of the nature of the lesion has led to a better response rate and a safer treatment for these patients. This has been accomplished through a detailed understanding of the angioarchitecture of the lesion, enabling a tailored approach in reaching and targeting the nidus of the AVM with different liquid embolic agents, more specifically ethanol. Flow reduction techniques help in exposing the nidus to sclerosant agents. A clinical classification, the Schobinger classification, will help determine the appropriate time to start or to pursue therapy. [ABSTRACT FROM AUTHOR]

Congenital vascular malformation (CVM) between the external carotid artery and the internal jugular vein is a rare disease, it originates as a consequence of arrested development during various stages of embryogenesis. The natural history of CMVs is progressive growth, it can remain clinically silent until it progresses causing local swelling accompanied by symptoms and signs of arteriovenous shunting, mass effect, ischemic insult with ulceration of skin or bleeding. In literature only few cases of CMVs have been reported, therefore there is still no consensus about the correct surgical approach to this pathology. We report an uncomplicated case of CVMs in a 15-year-old female successfully treated with coils embolization. To our knowledge, this is the eighth reported case in the literature review. [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]

Background: To examined the curative effect of vitreous injection with ranibizumab,laser coagulation and cryotherapy in treating stage 3 Coats' disease with exudative retinal detachment.Methods: Seventeen patients with stage 3 Coats' disease were enrolled in the study. All eyes were treated with vitreous injection of ranibizumab as initial treatment, and subsequent treatment depended on the absorption of subretinal fluid, Including cryotherapy and laser photocoagulation. Repeat treatment for the two treatment intervals occurred in ≥1 month. The mean follow-up time was 24.12 ± 5.99 months. The main data evaluation and outcome measurements included the patient's vision, intraocular pressure(IOP), optical coherence tomography (OCT), slit lamp examination, indirect ophthalmoscopy, color Doppler imaging (CDI) and color fundus image analysis. The following variables were compared between groups: abnormal vascular changes, subretinal fluid and exudate absorption, retinal reattachment and complications. The final follow-up results were used to determine the effectiveness of treatment.Results: Of the 17 patients included, 88.24% were male and 11.76% were female. Visual acuity was less than 0.02 in 12 eyes before surgery and 8 eyes after surgery. Visual acuity improved in 7 eyes, accounting for 41.18% of cases, and remained unchanged in 7 eyes, accounting for 41.18% of cases. Three patients were too young to undergo the operation, accounting for 17.65% of cases. The best vision was 0.1. Patients were treated 1 to 5 times for an average of 2.82 ± 0.95 times each. There was no statistically significant difference (t = 1.580, p = 0.135) between the preoperative and postoperative intraocular pressures. However, there was a statistically significant difference between the preoperative and postoperative retinal detachment height (2- related samples Wilcoxon signed rank test with z = 3.517, p = 0.000). The results further showed that all patients had different degrees of subretinal fluid absorption, and some of the new blood vessels subsided. All patients were successfully treated with laser and cryosurgery. No ocular or systemic complications were observed during follow-up.Conclusions: Intravitreal ranibizumab (IVR), laser coagulation and cryotherapy were effective in the treatment of Coats' disease with exudative retinal detachment.Trial Registration Number: We retrospectively registered our study, The trial registration number (TRN) is ChiCTR-ONC-17011161 and date of registration is April 16, 2017. [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]

Aim To determine whether apoptosis is more common in previously punctured native veins than in non-punctured native veins among patients who undergo surgical creation of arteriovenous fistula (AVF) for dialysis access. Methods Cephalic vein specimens were obtained from January 1, 2013 to December 31, 2014 from 60 patients, 30 with previously punctured native veins and 30 with non-punctured native veins. Before AVF placement, a 1-cm vein segment was excised from distal part of the vein for histological, histochemical, and immunohistochemical analysis. Vein specimens were divided into two portions along the longitudinal axis and stained with hematoxylin and eosin for routine histological evaluation. Immunohistochemical analysis was used to localize Bax, p53, caspase 3, and Bcl-2 expression. Results The group with previously punctured veins showed significantly increased caspase 3 (P < 0.001, two-sided Fisher's Exact Test) and Bax expression (P = 0.002, two-sided Fisher's Exact Test) and significantly decreased Bcl-2 expression (P < 0.001, two-sided Fisher's Exact Test) compared with the control group. There were no significant differences between the groups in p53 expression (χ2 = 0.071, df = 1, P = 0.791). Fistula failure was significantly more common in the study group (26.7% vs 6.7%, χ2 = 4.32, df = 1, P = 0.038). Conclusion Our study indicates a possible role of veni-puncture in apoptosis development and a possible role of apoptosis in fistula failure, but we do not have sufficient evidence to conclude that it represents its main cause. [ABSTRACT FROM AUTHOR]

We report a simple endovascular repair for symptomatic aberrant right subclavian artery (ARSA) in an 11-year-old girl. She had undergone tracheostomy due to hypoxic encephalopathy after cardiac surgery and suffered from dyspnea. Computed tomography revealed tracheal stenosis caused by a vascular ring made with an ARSA and the right common carotid artery. Transcatheter ARSA embolization using an Amplatzer vascular plug and coils was performed. Symptoms improved within a few days after this intervention and no complications such as upper extremity ischemia were encountered. Computed tomography showed the ARSA around the trachea had reduced in size and tracheal stenosis was improved. [ABSTRACT FROM AUTHOR]

Background/purpose: Catheter-based renal sympathetic denervation (RDN) can reduce blood pressure (BP) and sympathetic activity in certain patients with uncontrolled hypertension. Less is known about the impact of renal anatomy and procedural parameters on subsequent BP response.Methods/materials: A total of 564 patients with resistant hypertension underwent bilateral RDN in 9 centers in Europe and Australia using a mono-electrode radiofrequency catheter (Symplicity Flex, Medtronic). Anatomical criteria such as prevalence of accessory renal arteries (ARA), presence of renal artery disease (RAD), length, and diameter were analyzed blinded to patient's characteristics.Results: ARA was present in 171 patients (30%), and RAD was documented in 71 patients (13%). On average 11±2.7 complete 120-s ablations were performed, equally distributed on both sides. After 6months, BP was reduced by 19/8mmHg (p<0.001 for both). Change of systolic blood pressure (SBP) was not related to the presence of ARA (-18 vs. -20mmHg; p=NS) or RAD (-16 vs. -20mmHg; p=NS). Patients with a bilateral diameter≤4mm had a more pronounced reduction of SBP compared to patients with a unilateral diameter≤4mm or a bilateral diameter>4mm (-29 vs. -26 vs. -17mmHg; p<0.001). Neither the length of the renal artery nor the number of RF ablations influenced BP reduction after 6months.Conclusions: The diameter of renal arteries correlated with SBP change after RDN at 6-month follow-up. Change of SBP was not related to the lengths of the renal artery, presence of ARA, RAD, or the number of RF ablations delivered by a mono-electrode catheter. [ABSTRACT FROM AUTHOR]

Spinal dural arteriovenous fistulas (SDAVFs) are the most common type of spinal vascular malformations. However, SDAVFs are still underdiagnosed entities because their clinical symptoms are usually non-specific, as they include low back pain or radiating pain to the limb. There have been several reports of acute paraplegia after lumbar epidural steroid injections in patients with SDAVFs. We present 4 patients with SDAVFs who received lumbar steroid injection. Among the 4 cases, acute paraplegia developed in 2 cases that received a larger volume of injectate than the other cases. Thus, we are suggesting that the volume of injectate may be a contributing factor for acute paraplegia after lumbar steroid injection in patients with SDAVFs. [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]

Exposure to particulate matter air pollution has been causally linked to cardiovascular disease in humans. Several broad and overlapping hypotheses describing the biological mechanisms by which particulate matter exposure leads to cardiovascular disease have been explored, although linkage with specific factors or genes remains limited. These hypotheses may or may not also lead to particulate matter-induced cardiac dysfunction. Evidence pointing to autocrine/paracrine signaling systems as modulators of cardiac dysfunction has increased interest in the emerging role of endothelins as mediators of cardiac function following particulate matter exposure. Endothelin-1, a well-described small peptide expressed in the pulmonary and cardiovascular systems, is best known for its ability to constrict blood vessels, although it can also induce extravascular effects. Research on the role of endothelins in the context of air pollution has largely focused on vascular effects, with limited investigation of responses resulting from the direct effects of endothelins on cardiac tissue. This represents a significant knowledge gap in air pollution health effects research, given the abundance of endothelin receptors found on cardiac tissue and the ability of endothelin-1 to modulate cardiac contractility, heart rate, and rhythm. The plausibility of endothelin-1 as a mediator of particulate matter-induced cardiac dysfunction is further supported by the therapeutic utility of certain endothelin receptor antagonists. The present review examines the possibility that endothelin-1 release caused by exposure to PM directly modulates extravascular effects on the heart, deleteriously altering cardiac function. [ABSTRACT FROM AUTHOR]

Background: Optimizing vascular access outcomes is still a challenge, since 30-60% of arteriovenous fistulas fail or do not mature and catheters are widely used in contemporary patients. Methods: This study reports on strategies and outcomes in a single center in which access planning, surgery and maintenance are managed by a team of nephrologists. We retrospectively analyzed 305 fistulas and 61 grafts created in 270 consecutive patients between 2002 and 2013. Results: The percentage of patients receiving a fistula or graft who initiated hemodialysis with a mature access was 68.6%. Among prevalent patients, 71.7% used a fistula, 15.7% a graft and 12.6% a catheter. Rates of primary failure and revision before cannulation were 14.4 and 1.6% for fistulas vs. 4.9 and 3.3% for grafts. After maturation, complications (1.040 vs. 0.188 per patient-year (py)) and interventions (0.743 vs. 0.066 per py) were greater for grafts than for fistulas (p < 0.001). Secondary patency did not significantly differ between grafts and fistulas (median survival 34.8 vs. 57.3 months, p = 0.36), unless primary failures were excluded from Kaplan-Meier analysis (median survival 34.9 vs. 70.9 months, p = 0.03). Conclusions: High fistula prevalence, low access-related morbidity and catheter dependence were achieved using individualized strategies, including midforearm or perforating vein fistula creation and selective graft placement in high risk patients. Direct involvement of nephrologists throughout all steps of access care can improve access outcomes, by promoting a patient-centered approach. [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]

Background and Purpose: The major determinants of hemorrhage in an arteriovenous malformation (AVM) include its angioarchitecture, as well as the patient's age and clinical presentation. The aim of this study was to determine whether or not the risk of hemorrhage in an AVM differs based on the different clinical presentations and the initial age at diagnosis.Methods: A consecutive case series of 496 patients having a brain AVM was retrospectively analyzed. The radiological evidence of hemorrhage was established in 84 of these cases after the initial diagnosis of AVM had been made. These cases were analyzed using univariate analysis and Kaplan-Meier curves with respect to age at diagnosis, the clinical presentation of patients harboring the AVM as well as the Spetzler-Martin grade of the AVM.Results: Hemorrage occurred in 84 patients with an AVM during a mean duration of 2.5 years (range, 1month-28 years). In univariate analysis, a subsequent hemorrhage within an AVM was associated with the following factors: When the patients initially presented with a hemorrhage, and, had a younger age (<30 years), female gender, and Spetzler-Martin III and IV grades. By analysis of the Kaplan-Meier curves, it was estimated that the annual hemorrhage rate was 12.3% in those cases who initially presented with hemorrhage, compared with 3.5% for those cases whose initial presentation was not associated with AVM bleed. In the latter category (i.e., patients who did not have hemorrhage as the initial presentation), the annual rate of subsequent hemorrhage was lower for those patients who initially presented with seizures than those who had other initial presentations (such as those having neurological deficits, headaches, or those incidentally detected to be having an AVM) (with the bleeding rate being 1.9% in those with seizures as the initial presentation vs. 4.6% for the other presentations or those with incidentally detected AVMs, P = 0.002).Conclusions: The subsequent occurrence of hemorrhage in an AVM was associated with the initial presentation of hemorrhage, a younger patient's age (<30 years), the female gender, and Spetzler-Martin grades III and IV. The risk of subsequent hemorrhage in patients with an initial presentation of a seizure was lower than the risk of hemorrhage that occurred with other presentations. Patients with an AVM who present with recurrent headaches, and those in whom the AVM has been detected incidentally, should also be kept under surveillance as they too manifest a risk of a subsequent hemorrhage. [ABSTRACT FROM AUTHOR]

Objective: The aim of the present study is to evaluate the relationship between demographic characteristics, hematological and biochemical parameters, and elements of the coagulation system that may predispose a person to thrombosis or to anatomical and functional parameters in Doppler ultrasonography scanning and the maturation and adequacy of arteriovenous fistulas. Materials and Methods: Overall, 36 patients who underwent a native arteriovenous fistula operation were included. Biochemical parameters, hematological parameters, the coagulation system, and a Doppler ultrasonography evaluation were performed before an arteriovenous fistula operation on day 1 after the fistula operation and at the time point when the arteriovenous fistula became dysfunctional. Results: A thrombus occurred between 1 and 73 days (mean: 28.4±26.1) in 10 patients. It was found that the female gender (p<0.001), presence of thrill (p=0.014), quality of vein (p<0.001), peak systolic velocity and end-diastolic velocity of the radial artery at the snuffbox region on day 1 after the operation (p=0.035 and p=0.049, respectively), and the cephalic vein diameter in the Brescia-Cimino region (p=0.011) were associated with thrombosis formation. No relation was found among blood pressure, fistula region, quality of artery, spasm of artery and vein, hematologic and biochemical parameters, coagulation parameters, active protein C resistance, anti-cardiolipin antibody, D-dimer, C-reactive protein, or erythrocyte sedimentation rate and thrombosis formation (p>0.05). Conclusion: Thrombosis occurs at a relatively early period in approximately a quarter of arteriovenous fistulas. Female gender, quality of vein, and the absence of thrill in the post-operative period are important factors in thrombosis formation. [ABSTRACT FROM AUTHOR]

Purpose Genital infantile hemangiomas are vascular anomalies that often require complex management and interdisciplinary care. Propranolol was first used to treat patients with infantile hemangiomas in 2008 and has since gained acceptance as first-line therapy. Materials and Methods We review the presentation, course, management and outcomes of all cases of genital infantile hemangiomas managed by propranolol administration at a single institution from April 2010 to July 2014. Results During the study period 9 patients with genital infantile hemangiomas were referred to our hemangioma treatment clinic. Propranolol was initially administered under careful outpatient monitoring at a dose of 1 mg/kg daily in 8 patients. One patient, a 700 gm premature infant, was started on therapy in the inpatient setting at 0.5 mg/kg daily, given the history of prematurity. All patients underwent successful increase of dose to at least 2 mg/kg for the observation phase after tolerating the starting doses. One patient discontinued propranolol prematurely per parental request due to concern regarding peripheral vasoconstriction. Otherwise, no patient demonstrated significant hypotension, symptomatic bradycardia, hypoglycemia or other major side effect requiring treatment discontinuation. All patients who continued the treatment protocol had excellent response to therapy. Conclusions Propranolol therapy for genital infantile hemangiomas was successfully initiated and the dosage increased in 9 young children without significant side effects and with marked improvement in all patients who continued on treatment. Propranolol is the only Food and Drug Administration approved therapy for treatment of patients with this vascular anomaly and should be considered first-line therapy for genital infantile hemangiomas. [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]

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]

Unlabelled: Vascular anomalies include a heterogeneous group of disorders that are categorized as vascular tumors or vascular malformations. Treatment options include resection, embolization, laser therapy, and sclerotherapy or medical treatment such as propranolol, steroids, interferon, and cytostatic chemotherapy. Mammalian target of rapamycin seems to play a key role in the signal pathway of angiogenesis and subsequently in the development of vascular anomalies. Recently, the successful use of sirolimus has been reported in children with lymphatic malformations and kaposiform hemangioendotheliomas. We report on six patients with different vascular anomalies (kaposiform hemangioendothelioma n = 2, combined lymphatico-venous malformation n = 2, pulmonary lymphangiectasia n = 1, and orbital lymphatic malformation n = 1) who were treated with peroral sirolimus. Three of the children initially presented with a Kasabach-Merrit phenomenon. Median duration of treatment was 10 months; two children are still on treatment. Three children each achieved complete and partial remission. Kasabach-Merrit phenomenon resolved within 1 month in all patients. Treatment with sirolimus was tolerated well; only mild reversible leukopenia was observed.Conclusion: Sirolimus proved to be effective in children with complicated lymphatic or lymphatico-venous malformations and kaposiform hemangioendotheliomas. Treatment was tolerated well with acceptable side effects. The optimum length of treatment and possible long-term side effects have to be evaluated.What Is Known: • Vascular anomalies including vascular tumors and vascular malformations may lead to life-threatening conditions.• Some patients are refractory to established treatment and/or are not available for local invasive procedures.What Is New: • We reviewed the literature focusing treatment of vascular anomalies inc hildren and adolescents.• Our data support recent studies that sirolimus is an effective treatment option in patients with complicated vascular tumors andmalformations [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]

Objective: To evaluate the effectiveness and safety of sirolimus therapy in a child with macroglossia due to lymphatic malformation. Methods: Sirolimus treatment was applied to the patient with an initial dosing of 0.8 mg/m2 per dose, administered orally, twice daily at approximately 12-hour intervals. Results: After 9 months of sirolimus therapy, there was a nearly complete resolution of lymphatic malformation. The last evaluation was performed 6 months after withdrawal of treatment, and the lesion had almost completely resolved. Conclusion: This article presents a novel approach to the treatment of lymphatic malformation of the tongue using sirolimus, which appears to be safe and effective for the management of complex cases. [ABSTRACT FROM AUTHOR]

Background: To evaluate the effectiveness of endolaser photocoagulation by a two-port pars plana nonvitrectomy approach for treating Coats' disease with shallow exudative retinal detachment. Methods: This study included 24 patients (23 boys with an age range of 2-17 years, and one girl, age 6 years) with stage 3 Coats' disease (25 eyes) from December 2012 and May 2014 at a single center. All of the 25 eyes were complicated with serous or total retinal detachment and received none-vitrectomized endolaser: two (23- or 25-gauge) incisions were routinely made 3 mm posterior to the corneal limbus and a laser was applied directly on the abnormal blood vessels. Additional treatments included subretinal fluid drainage (five eyes), intravitreal triamcinolone injection (seven eyes), and intravitreal anti-vascular endothelial growth factor (VEGF) injection (17 eyes). Best-corrected visual acuity, intraocular pressure, and fundus and abnormal vascular changes were recorded to determine therapeutic effects. Results: Twenty-four out of the 25 treated eyes (96 %) had retina reattached. The number of treatment sessions differed case by case (1-5 sessions, average 1.96) and the time to full treatment of retinal reattachment was 4 months in average. One patient (4 %) presented with retinal redetachment. Five (20 %) eyes received further laser treatment with indirect ophthalmoscope and four eyes (16 %) presented with total retinal detachment at their first visits received consecutive treatments. At the end of the follow-up period (mean, 10.08 months), telangiectasias of 24 (96 %) eyes were resolved and no severe complications occurred. Conclusions: Endolaser photocoagulation by a two-port pars plana nonvitrectomy approach is an effective treatment for advanced Coats' disease with serous retinal detachment. The long-term safety of the approach needs further investigation. [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]