Search Results (6)

Background/Objectives: Bleomycin electrosclerotherapy (BEST), which combines intralesional bleomycin administration with electroporation, enhances drug uptake and has shown efficacy in treating vascular malformations resistant to conventional therapies. While BEST is increasingly used in various anatomical sites, its application in the upper aerodigestive tract remains underexplored. This study evaluates the safety and effectiveness of BEST in managing slow-flow vascular malformations of the oral cavity, tongue, larynx, and hypopharynx. Methods: In this retrospective, multicenter study, 20 patients with symptomatic slow-flow vascular malformations of the upper aerodigestive tract were treated with BEST. Clinical and radiological assessments were used to evaluate the treatment response, categorized as “significantly reduced”, “reduced”, “stable disease”, or “lesion growth”. Postprocedural complications and functional outcomes were systematically recorded. Results: A total of 29 BEST sessions were performed. Lesions of the tongue (n = 8) and combined oral cavity and tongue (n = 6) showed the highest response rates, with significant symptom reduction in five out of eight and five out of six patients, respectively. Among isolated oral cavity lesions (n = 4), one out of four demonstrated a significant reduction. In contrast, laryngeal and hypopharyngeal lesions (n = 2) had limited response, with one case showing partial reduction and the other remaining stable. Severe complications, including bleeding and dyspnea requiring tracheostomy, limited further treatment in these locations. No systemic adverse events, such as pulmonary toxicity, were observed. Conclusions: BEST is effective for treating vascular malformations of the upper aerodigestive tract, particularly in the tongue and oral cavity, but presents significant risks in laryngeal and hypopharyngeal lesions. A multidisciplinary approach is required to optimize treatment protocols for these challenging locations. Full article

Background: Venous malformations (VMs) are congenital vascular abnormalities characterized by tortuosity, slow blood flow, and gradual growth. Intra-articular venous malformations (IAVMs) of the knee are rare and often present with symptoms similar to juvenile idiopathic arthritis (JIA) or late sequelae of trauma. VM in children is commonly misdiagnosed as hemangioma. This study aims to analyze the clinical and MRI features of IAVM in the knee joint. Methods: This retrospective study analyzed patients from a reference unit for the treatment of vascular malformations in the Pediatric Surgery Department. The group was collected starting from the year 2014 until the 100th patient was identified in the year 2018, all with MRI-confirmed VM based on a predefined protocol. From this group, 19 patients with lower limb symptoms were identified, and 9 patients with VM involving the knee joint were selected for further analysis. Results: The most common symptoms in IAVM patients were pain and swelling, chronic in five (55%) and intermittent in four (45%). Four (45%) reported worsening pain during or after physical activity. A history of intra-articular bleeding was noted in five (55%), leading to mild knee contracture (10° reduction in extension) and decreased mobility. Limb deformities were observed in eight (89%). Diffuse VMs, affecting both intra- and extra-articular tissues, were present in eight (89%), involving the thigh in seven (78%), crus in five (56%), gluteal muscles in three (33%), and foot tissues in one (11%). The suprapatellar recess and Hoffa’s fat pad were involved in all patients (100%). Conclusions: IAVMs are rare causes of knee dysfunction in children and young adults, particularly in cases of unexplained pain, swelling, or instability. They should be considered in the differential diagnosis of hemophilic arthropathy, JIA, or late post-traumatic sequelae. Untreated IAVMs can lead to intra-articular bleeding, cartilage degeneration, and disability. Early diagnosis via MRI and ultrasound is crucial to identifying IAVMs and preventing joint degeneration. Timely treatment helps avoid further damage and long-term disability. Full article

Congenital cervicofacial vascular anomalies are extremely rare and present many difficulties in diagnosis and treatment requiring a multidisciplinary approach. Firstly, there is little consensus on this subject among head and neck specialists. There are two main types of vascular anomalies: vascular tumors and vascular malformations. Vascular malformations are also divided into malformations with slow blood flow (veins, lymphatics, capillaries or combined) and malformations with a fast blood flow (arteriovenous malformations and fistula). Vascular tumors like hemangiomas are known for their spontaneous involution with aging, while vascular malformations grow in dimensions with age. It is very important to choose the correct differential diagnosis between cervicofacial hemangiomas and vascular malformations for proper therapy management. Anamnesis and clinical exams help in raising suspicions about the real nature of a cervico-vascular anomaly. Furthermore, imaging brings in-depth details of the anomaly, ranging from ultrasound and contrast CT to MRI scanning and minimally invasive angiography. Angiography with selective embolization is rarely a curative procedure for arteriovenous malformations, being more suitable as a preliminary step before attempted surgical removal. Surgery is clearly necessary when there are aesthetic and functional deficits. Slow-flow vascular malformations present a reduced morbidity, and in cases without involution, the surgical ablation is reserved for the cases with aesthetic dysfunctions or psychological trauma. Lymphatic malformations must undergo surgical ablation when they are associated with mass effects and compression of great vessels or aerial viscera. The prognosis after surgical removal is good, with a low rate of recurrence or morbidity. Fast-flow vascular malformations require a combined approach, with embolization and excision in the next 48 h for safety reasons. Removal may be followed by reconstructive surgery depending on the location and dimensions of the malformation, with a possible secondary recovery of the normal microscopic vessels. Some of the masses may hinder the normal airflow and swallowing. Pathology is the gold standard for confirming the clinical and imaging diagnosis. Full article

Prediction of response to percutaneous sclerotherapy in patients with venous malformations (VM) is currently not possible with baseline clinical or imaging characteristics. This prospective single-center study aimed to predict treatment outcome of percutaneous sclerotherapy as measured by quality of life (QoL) by using radiomic analysis of diffusion-weighted (dw) magnetic resonance imaging (MRI) before and after first percutaneous sclerotherapy. In all patients (n = 16) pre-interventional (PRE-) and delta (DELTA-) radiomic features (RF) were extracted from dw-MRI before and after first percutaneous sclerotherapy with ethanol gel or polidocanol foam, while QoL was assessed using the Toronto Extremity Salvage Score (TESS) and the 36-Item Short Form Survey (SF-36) health questionnaire. For selecting features that allow differentiation of clinical response, a stepwise dimension reduction was performed. Logistic regression models were fitted and selected PRE-/DELTA-RF were tested for their predictive value. QoL improved significantly after percutaneous sclerotherapy. While no common baseline patient characteristics were able to predict response to percutaneous sclerotherapy, the radiomics signature of VMs (independent PRE/DELTA-RF) revealed high potential for the prediction of clinical response after percutaneous sclerotherapy. This proof-of-concept study provides first evidence on the potential predictive value of (delta) radiomic analysis from diffusion-weighted MRI for Quality-of-Life outcome after percutaneous sclerotherapy in patients with venous malformations. Full article

Cardiovascular disease (CVD) is the leading cause of global mortality, which has caused a huge burden on the quality of human life. Therefore, experimental animal models of CVD have become essential tools for analyzing the pathogenesis, developing drug screening, and testing potential therapeutic strategies. In recent decades, zebrafish has entered the field of CVD as an important model organism. HEG1, a heart development protein with EGF like domains 1, plays important roles in the development of vertebrate cardiovascular system. Loss of HEG1 will affect the stabilization of vascular endothelial cell connection and eventually lead to dilated cardiomyopathy (DCM). Here, we generated a heg1-specific knockout zebrafish line using CRISPR/Cas9 technology. Zebrafish heg1 mutant demonstrated severe cardiovascular malformations, including atrial ventricular enlargement, heart rate slowing, venous thrombosis and slow blood flow, which were similar to human heart failure and thrombosis phenotype. In addition, the expression of zebrafish cardiac and vascular markers was abnormal in heg1 mutants. In order to apply zebrafish heg1 mutant in cardiovascular drug screening, four Traditional Chinese Medicine (TCM) herbs and three Chinese herbal monomers were used to treat heg1 mutant. The pericardial area, the distance between sinus venosus and bulbus arteriosus (SV-BA), heart rate, red blood cells (RBCs) accumulation in posterior cardinal vein (PCV), and blood circulation in the tail vein were measured to evaluate the therapeutic effects of those drugs on DCM and thrombosis. Here, a new zebrafish model of DCM and thrombosis was established, which was verified to be suitable for drug screening of cardiovascular diseases. It provided an alternative method for traditional in vitro screening, and produced potential clinical related drugs in a rapid and cost-effective way. Full article

Reduction of the wound healing time after surgical procedures along with good hemostasis, and the reduction of post-surgical edema, pain and infective complications are generally desirable to both clinicians and patients. Recently, a gel compound containing sodium hyaluronate and four synthetic aminoacids (glycine, leucine, proline, lysine) and marketed as Aminogam^® (Errekappa Euroterapici, Italy), has been proposed as a medical device promoting faster wound healing after oral surgery procedures. To assess its achievable clinical benefits, we studied retrospectively 580 cases (290 study cases and 290 control cases) undergoing oral surgery and receiving Aminogam ^® gel application. More precisely, cases were divided into 7 groups on the bases of the kind of surgery (teeth extraction, oral surgery in patients taking bisphosphonates, surgical treatment of jaw osteonecrosis related to bisphosphonates therapy, placement of endosseous implants, diode laser surgery of oral mucosa lesions with second intention healing without stitches, diode laser photocoagulation of slow flow vascular malformations and bone surgery). In all instances, Aminogam^® gel was applied at least five times a day until the wound healed completely. We compared the elapsed time between surgery and complete healing with Aminogam^® application compared to control cases receiving no other drug treatment. Our results confirmed that the overall time of healing is certainly reduced in cases receiving Aminogam^® gel regardless of the kind of oral surgery. Full article