Treatment Of Vascular Anomalies Research Articles

Lymphangiomas in the head and neck region in children

The treatment of lymphangiomas in children is a challenge because of slow but insidious growth, as well as because of infiltrative growth. In the region of the head and neck in children the problem of solving is a very difficult moment. On the one hand, the damage to the organs located in this region, on the other hand, the cosmetic problem and the growth of the child. Surgical treatment is the mainstay in the treatment of lymphangiomas in children, but the only method does not solve the problem in question. The goal. Evaluation of methods of treatment of lymphangiomas in children. Materials and methods. The study was carried out on the bibliographic basis and in particular the treatment of lymphangiomas in children in the head and neck region with pharmaceutical preparations. The ones reported in the bibliography were Sirolimus and Bleomycin. Several parameters were examined in both groups - age, sex, location of neoplasm, treatment and complications following treatment Results. More severe adverse effects were found in the group of children who received Serolim compared to those who were treated by sclerotherapy with Blaomycin. Conclusion Safety is a major concern in the treatment of vascular anomalies in children. The use of Sirolimus is indicated in cases of danger to the child’s life. Further research with randomized controlled trials is needed to establish the benefits of off-label use of sirolimus in these complex pathological processes.

A 16-year retrospective study of vascular anomalies in the head and neck region

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

Sirolimus Early Treatment in Vascular Anomalies Leads to a Better Response

Objectives: Sirolimus has become a relevant drug in the treatment of vascular anomalies, initially relegated to rescue therapy for refractory lesions but more recently has become first line. Our objective is to determine when it is best to start sirolimus treatment. Methods: A retrospective review of patients with vascular anomalies treated with sirolimus during a 9-year period was performed. The variables analyzed included subtype of vascular anomaly, age at the start of treatment, sirolimus dosage and levels, response, and duration of treatment among others. Results: One hundred twenty-two patients were included and classified according to ISSVA in: tumor (7), lymphatic malformation (60), venous malformation (16), combined malformations (18), associated overgrowth (8), and others (15). Overall response was positive in 90.8%. Median duration of treatment was 32 months (0–116). Response was not influenced by dosage or levels, neither by the subtype of vascular anomaly, location, extension, symptoms, genetic testing nor previous treatments. However, the lower the age of starting sirolimus, the better the response, mainly under 5 years of age (P = 0.004). At 6 months, 67% of patients had responded while at 12 months >84% did. By age, patients <5 years showed positive response at a median time of 2 months, compared to >5 months from older patients. Time until a positive response was not influenced by subtype or severity. Conclusion: Overall response to sirolimus was good and most patients responded irrespective of their severity in location, extension or symptoms. Patients <5 years old respond better and faster making our goal to attempt for an early treatment.

Propranolol for Vascular Anomalies: Efficacy and Complications in Pediatric Patients.

Congenital vascular anomalies are classically subdivided into vascular tumors and vascular malformations. The role of propranolol in the regression of infantile hemangioma (IH), a vascular tumor, is well establishe. This study aimed to analyze the therapeutic efficacy and complications associated with oral propranolol and adjuvant therapy in the treatment of vascular anomalies. A prospective interventional study was undertaken over 10 years duration extending from 2012 to 2022 at a tertiary care teaching institute. All children with cutaneous hemangiomas and lymphatic and venous malformations under 12 years of age, except those with contraindications for administration of propranolol, were included in the study. Out of 382 patients, there were 159 males and 223 females (male: female = 1:1.4). The majority (53.66%) were between ≥3 months and 1 year. There were 481 lesions in 382 patients. There were 348 patients with IH, and 11 were congenital hemangiomas (CHs). There were 23 patients with vascular malformations; lymphatic malformation (n = 19) and venous malformation (n = 4) were present. The size of the lesions ranged from 5 mm to 20 cm; 50.73% were 2-5 cm in size. Ulceration (>5 mm) was the most common complication present in 20/382 (5.24%) patients. Complications related to oral propranolol were seen in 23 (6.02%) patients. Drugs were given for a mean period of 10 months (range from 5 months to 2 years). At the end of the study, 282 (81.03%) out of 348 patients with IH showed an excellent response; 4 (36.36%) patients in the case of CH (n = 11) and 5 (21.74%) patients with vascular malformation (n = 23) showed excellent response. The study validates the use of propranolol hydrochloride as the first-line agent for the treatment of IHs and congenital hemangiomas. It may have an additive role in lymphatic malformations, and venous malformations, as a part of a multimodality treatment approach for vascular malformations.

Sodium Phytate-Incorporated Gelatin-Silicate Nanoplatelet Composites for Enhanced Cohesion and Hemostatic Function of Shear-Thinning Biomaterials.

Shear-thinning biomaterials (STBs) based on gelatin-silicate nanoplatelets (SNs) are emerging as an alternative to conventional coiling and clipping techniques in the treatment of vascular anomalies. Improvements in the cohesion of STB hydrogels pave the way toward their translational application in minimally invasive therapies such as endovascular embolization repair. In the present study, sodium phytate (Phyt) additives are used to tune the electrostatic network of SNs-gelatin STBs, thereby promoting their mechanical integrity and facilitating injectability through standard catheters. We show that an optimized amount of Phyt enhances storage modulus by approximately one order of magnitude and reduces injection force by ≈58% without compromising biocompatibility and hydrogel wet stability. The Phyt additives are found to decrease the immune responses induced by SNs. In vitro embolization experiments suggest a significantly lower rate of failure in Phyt-incorporated STBs than in control groups. Furthermore, the addition of Phyt leads to accelerated blood coagulation (reduces clotting time by ≈45% compared to controls) due to the contributions of negatively charged phosphate groups, which aid in the prolonged durability of STB in coagulopathic patients. Therefore, the proposed approach is an effective method for the design of robust and injectable STBs for minimally invasive treatment of vascular malformations.

The Genetic Architecture of Vascular Anomalies: Current Data and Future Therapeutic Perspectives Correlated with Molecular Mechanisms.

Vascular anomalies (VAs) are morphogenesis defects of the vascular system (arteries, capillaries, veins, lymphatic vessels) singularly or in complex combinations, sometimes with a severe impact on the quality of life. The progress made in recent years with the identification of the key molecular pathways (PI3K/AKT/mTOR and RAS/BRAF/MAPK/ERK) and the gene mutations that lead to the appearance of VAs has allowed the deciphering of their complex genetic architecture. Understanding these mechanisms is critical both for the correct definition of the phenotype and classification of VAs, as well as for the initiation of an optimal therapy and the development of new targeted therapies. The purpose of this review is to present in synthesis the current data related to the genetic factors involved in the etiology of VAs, as well as the possible directions for future research. We analyzed the data from the literature related to VAs, using databases (Google Scholar, PubMed, MEDLINE, OMIM, MedGen, Orphanet) and ClinicalTrials.gov. The obtained results revealed that the phenotypic variability of VAs is correlated with genetic heterogeneity. The identification of new genetic factors and the molecular mechanisms in which they intervene, will allow the development of modern therapies that act targeted as a personalized therapy. We emphasize the importance of the geneticist in the diagnosis and treatment of VAs, as part of a multidisciplinary team involved in the management of VAs.

Medical management of vascular anomalies of the head and neck.

Depending on impairment, treatment of vascular anomalies is decided on a case-by-case basis in pluridisciplinary consultations. Interventional treatments, especially surgery and sclerotherapy, are usually partially efficient and management of patients with vascular anomalies increasingly involves the use of medical drugs. The most common vascular tumor is infantile hemangioma where first-line medical treatment, when necessary, is propranolol. Kasabach-Merritt phenomenon is now largely treated with sirolimus whereas first-line treatment of coagulation disorders associated with venous malformations is based on low-molecular-weight heparins or direct anticoagulants. Sirolimus is the standard treatment for painful inflammatory manifestations of low-flow vascular malformations such capillary, venous, and lymphatic malformations that can occur singly or in combination but PIK3CA inhibitors, originally developed in oncology, have shown promising results in patients with PIK3CA-related overgrowth spectrum. Currently, medical treatments are poorly developed for high-flow malformations such as arteriovenous malformations. However, new research aimed at delineating the different arteriovenous malformations based on molecular findings has given new hope for future development of targeted therapies.

[Interdisciplinary management of extracranial vascular anomalies].

Amultitude of vascular anomalies exist and can lead to severe complications. Treatment can be complex. This overview aims to provide important information for the management of vascular anomalies. In addition to current literature, experiences from the interdisciplinary Vascular Anomalies Center in Marburg were included in this review. Hemangiomas at critical sites, arteriovenous malformations, and vascular anomalies of uncertain etiology require particular attention. Self-help and support groups, specialized interdisciplinary centers, scientific medical societies, and networks can provide help for the treatment of vascular anomalies.

The role of sirolimus in the treatment of vascular anomalies

The role of sirolimus in the treatment of vascular anomalies

Systemic, local, and sclerotherapy drugs: What do we know about drug prescribing in vascular anomalies?

Off-label drug prescribing, frequent in the treatment of vascular anomalies (VA), relies on the quality of the literature reporting drug efficacy and safety. Our objective is to review the level of evidence (LOE) surrounding drug use in VA, which is more prevalent in pediatric care. A list of drugs used in VA was created with a literature review in July 2020. For each drug listed, the article displaying the highest LOE was determined and then compared between efficacy/safety data, routes of administration, pharmacological categories and a subset of VA. The influence of research quality on study results was also explored. The median LOE for the 74 drugs identified poor methodological quality, with a predominance of retrospective studies or case reports. Drug safety is currently inadequately reported. This is alarming as many treatments display significant safety concerns. Also, current literature displays major publication bias that probably leads to overestimation of drug efficacy in VA.

Clinical Uses and Short-Term Safety Profile of Ethiodized Poppy Seed Oil Contrast Agent in the Diagnosis and Treatment of Vascular Anomalies and Tumors.

Background: There is a sparsity of data on the use of ethiodized poppy seed oil (EPO) contrast agent (Lipiodol) in patients. We investigated the safety of EPO in children, adolescents, and some adults for diagnostic and therapeutic interventions. Methods: All patients who underwent procedures with EPO between 1995 and 2014 were retrospectively included. Demographic characteristics, diagnosis, dose, route of administration, preparation of EPO in combination with other agents, and complications were recorded. Results: In 1422 procedures, EPO was used for diagnostic or treatment purposes performed in 683 patients. The mean patient age was 13.4 years (range: 2 months–50 years); 58% of patients were female. Venous malformations (n = 402, 58.9%) and arteriovenous malformations (n = 60, 8.8%) were the most common diagnosis. Combined vascular anomalies included capillary–lymphatic–venous malformations, fibroadipose vascular anomalies (n = 54, 7.9%), central conducting lymphatic anomalies (n = 31, 4.5%), lymphatic malformations (n = 24, 3.5%), aneurysmal bone cysts (n = 22, 3.2%), and vascularized tumors (n = 11, 1.6%). In 1384 procedures (96%), EPO was used in various combinations with sclerosing and embolization agents, including sodium tetradecyl sulfate, ethanol, and glue. The mean volume of EPO used in interventions was 3.85 mL (range: 0.1–25 mL) per procedure with a mean patient weight of 45.9 kg (range: 3.7–122.6 kg) and a weight-adjusted dose of 0.12 mL/kg (range: 0.001–1.73 mL/kg). In 56 procedures (4%), EPO was used as a single agent for diagnostic lymphangiography. The mean volume was 4.8 mL (range: 0.3–13 mL) per procedure with a mean patient weight of 27.4 kg (range: 2.4–79.3 kg) and a weight-adjusted dose of 0.2 mL/kg (range: 0.04–0.54 mL/kg). Procedural-related complications occurred in 25 (1.8%) procedures. The 20 minor and 5 major complications were related to the primary treatment agents. None of them were directly related to EPO. No allergic reactions were noted. Conclusion: The use of an ethiodized poppy seed oil contrast agent in children, adolescents, and adults for diagnostic or therapeutic purposes is safe.

Percutaneous MR Imaging-Guided Laser Ablation and Cryoablation for the Treatment of Pediatric and Adult Symptomatic Peripheral Soft Tissue Vascular Anomalies

PurposeTo evaluate the safety and effectiveness of percutaneous magnetic resonance (MR) imaging-guided laser ablation and cryoablation for the treatment of symptomatic soft tissue vascular anomalies (VAs) of the trunk and extremities. Materials and MethodsAn institutional review board-approved retrospective review was undertaken of all pediatric and adult patients who underwent MR imaging-guided and monitored laser ablation and/or cryoablation for the treatment of symptomatic peripheral soft tissue VA. Preablation and postablation MR imaging was independently reviewed. Pain scores on the visual analog scale (0 to 10) and self-reported subjective symptomatic improvement were assessed. Change in VA size and pain scores were compared using a paired t test. ResultsThirty patients (24 females; age, 10–75 years) with 34 VAs were treated for moderate to severe pain (n = 27) or swelling/mass effect (n = 3) with 60 total ablation sessions. The baseline maximum VA diameter was 9.5 cm ± 8.6. At baseline, all VAs (100%) demonstrated variable T2-weighted signal hyperintensity and enhancement. The baseline pain score was 6.4 ± 1.6. Clinical follow-up was available for 23 patients. At a mean follow-up time of 12.2 months ± 10.1, 19 of 20 (95%) patients treated for pain and 2 of 3 (67%) patients treated for swelling/mass effect reported partial or complete symptomatic relief. There was a significant decrease in the postablation pain scores (−5.7 ± 1.0, P < .001) and maximum VA size (−2.3 cm ± 2.7, P = .004), with >50% reduction in VA T2 signal (59%) and enhancement (73%). Nine of 30 (30%) patients experienced minor complications. ConclusionsMR imaging-guided and monitored percutaneous laser ablation and cryoablation appear to be safe and effective for the treatment of symptomatic peripheral soft tissue VAs.

Cell Populations Expressing Stemness-Associated Markers in Vascular Anomalies.

Treatment of vascular anomalies (VAs) is mostly empirical and, in many instances unsatisfactory, as the pathogeneses of these heterogeneous conditions remain largely unknown. There is emerging evidence of the presence of cell populations expressing stemness-associated markers within many types of vascular tumors and vascular malformations. The presence of these populations in VAs is supported, in part, by the observed clinical effect of the mTOR inhibitor, sirolimus, that regulates differentiation of embryonic stem cells (ESCs). The discovery of the central role of the renin-angiotensin system (RAS) in regulating stem cells in infantile hemangioma (IH) provides a plausible explanation for its spontaneous and accelerated involution induced by β-blockers and ACE inhibitors. Recent work on targeting IH stem cells by inhibiting the transcription factor SOX18 using the stereoisomer R(+) propranolol, independent of β-adrenergic blockade, opens up exciting opportunities for novel treatment of IH without the β-adrenergic blockade-related side effects. Gene mutations have been identified in several VAs, involving mainly the PI3K/AKT/mTOR and/or the Ras/RAF/MEK/ERK pathways. Existing cancer therapies that target these pathways engenders the exciting possibility of repurposing these agents for challenging VAs, with early results demonstrating clinical efficacy. However, there are several shortcomings with this approach, including the treatment cost, side effects, emergence of treatment resistance and unknown long-term effects in young patients. The presence of populations expressing stemness-associated markers, including transcription factors involved in the generation of induced pluripotent stem cells (iPSCs), in different types of VAs, suggests the possible role of stem cell pathways in their pathogenesis. Components of the RAS are expressed by cell populations expressing stemness-associated markers in different types of VAs. The gene mutations affecting the PI3K/AKT/mTOR and/or the Ras/RAF/MEK/ERK pathways interact with different components of the RAS, which may influence cell populations expressing stemness-associated markers within VAs. The potential of targeting these populations by manipulating the RAS using repurposed, low-cost and commonly available oral medications, warrants further investigation. This review presents the accumulating evidence demonstrating the presence of stemness-associated markers in VAs, their expression of the RAS, and their interaction with gene mutations affecting the PI3K/AKT/mTOR and/or the Ras/RAF/MEK/ERK pathways, in the pathogenesis of VAs.

Managing Vascular Anomalies in the Era of Genetics and Precision Medicine: An Opportunity or a Challenge?

The era of genetics and precision medicine has been reforming this world. How will plastic surgeons in the field of vascular anomalies conform to the trend? This article systematically reviews the identification of serum biomarkers, risk factors, specific mutations in the angiogenesis-related genes such as GNAQ, RASA1, TEK, and their impact on the diagnosis and treatment of vascular anomalies with preliminary results that have been previously reported and leading the tide. Moreover, a new disease classification for complex vascular malformations based on PIK3CA genetic evidence and various treatment breakthroughs is briefly summarized. With gene sequencing, bioinformatics, and big data, we confront the challenges of research in the vascular anomalies domain and explore possibilities of precision medicine development.

A Primer on a Comprehensive Genetic Approach to Vascular Anomalies.

The field of vascular anomalies has grown tremendously in the last few decades with the identification of key molecular pathways and genetic mutations that drive the formation and progression of vascular anomalies. Understanding these pathways is critical for the classification of vascular anomalies, patient care, and development of novel therapeutics. The goal of this review is to provide a basic understanding of the classification of vascular anomalies and knowledge of their underlying molecular pathways. Here we provide an organizational framework for phenotype/genotype correlation and subsequent development of a diagnostic and treatment roadmap. With the increasing importance of genetics in the diagnosis and treatment of vascular anomalies, we highlight the importance of clinical geneticists as part of a comprehensive multidisciplinary vascular anomalies team.

Topical sirolimus for the treatment of cutaneous manifestations of vascular anomalies: A case series.

Vascular anomalies (VA), characterized by the abnormal development or growth of blood and/or lymphatic vessels, encompasses a spectrum of conditions with a range of symptoms and complications. VA are frequently associated with cutaneous complications that can cause significant morbidity. Systemic sirolimus has previously been shown to be effective in the treatment of complicated VA. There are limited studies to date on the use of topical sirolimus for the treatment of cutaneous manifestations of VA. Retrospective review of medical records of pediatric patients with VA treated with topical sirolimus at a single quaternary pediatric institution. Response was determined by clinical subjective and objective measures of improvement. Twenty-three patients with cutaneous VA manifestations were treated with topical sirolimus. Median age was 14 (range 4-27 years). The main indication for treatment was complication of lymphatic blebbing (82%, n=19) including lymphatic fluid leakage, bleeding, pain, pruritus, swelling, or recurrent infection. Treatment course ranged from 109 to 1424 days with median of 622 days. No major side effects were reported. Eighty-six percent of patients (n=20) had subjective or objective improvement of cutaneous lesions. Lymphatic blebbing complications improved in 90% (n=17) of individuals. Eighty-two percent (n=14) of patients not receiving concurrent systemic sirolimus demonstrated improvement with topical therapy. One patient electively stopped treatment due to pruritus and burning sensation. Topical sirolimus appears to be a beneficial therapy for lymphatic blebbing associated with lymphatic malformations or mixed malformations with a lymphatic component, although benefit in other VA remains unclear. Topical sirolimus was well-tolerated with minimal side effects.

Sirolimus in the treatment of vascular anomalies

Despite that the large, diversified group known as vascular anomaly represents vascular tumors and vascular malformations together, the authors successfully clarified different outcomes of sirolimus with these two entirely different groups of vascular disorders.1 Indeed, vascular tumors/hemangioma are the outcome of increased neoplastic endothelial cell proliferation, but vascular malformations are the outcome of the defective development of any part of the circulation system during embryogenesis.

Vascular anomalies: Presentation and response to medical and surgical management, our experiences in the last 5 years

Aim: Vascular anomalies, lesions of abnormal vascular development pose a significant challenge in some of the cases for diagnosis and management. The International Society for the Study of Vascular Anomalies has classified this condition into two broad types as vasoproliferative or vascular neoplasms such as hemangioma and vascular malformation. We have analyzed all the cases with vascular anomalies that have presented in outpatient department (OPD) in the past 5 years. Materials and Methods: All patients presented to vascular OPD of Dhulikhel Hospital in the year 2013–2017, if patients are diagnosed with vascular anomalies, are included in the study. Details on age, sex, site of the lesion, and medical or operative management performed are analyzed. Results: Of the total 51 vascular anomalies cases, the mean age of the patient was 22.4 years (standard division. 8.4, range 6 months to 51 years). Female-to-male ratio was 2.4:1. Forty cases (70.48%) belonged to vascular malformation while 11 cases (21.56%) were of hemangioma. In case of vascular malformation, 18 cases were of venous malformation while 8 cases were of capillary malformation. There were a total of 14 arteriovenous malformations. The highest number of anomalies was noticed in the lower limb (39.22%) followed by the head (25.49%). Regarding treatment modality opted, in five cases, medical management with oral steroids and propranolol was advised. In 35 cases, surgical resection was done. Conclusion: Vascular anomalies are detected in fairly young patients with preponderance in the female. Treatment of vascular anomalies is complex and is based on stage, type, and location of each anomaly.

Percutaneous Image-Guided Cryoablation in Vascular Anomalies.

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.

Advances in the Medical Management of Vascular Anomalies

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.