Congenital Vascular Malformations in Children: From Historical Perspective to a Multidisciplinary Approach in the Modern Era-A Comprehensive Review.

Congenital vascular malformations (CVMs) are the result of an aberrant development during embryogenesis. Although these lesions are present at birth, they are not always visible yet. Once symptomatic, patients suffer from pain, bleeding, ulcers, infections or lymphatic leakage, depending on the subtype of vessels involved.

Health-related quality of life in children with congenital vascular malformations.

Unlabelled: A cross-sectional study was performed to evaluate health-related quality of life (HRQOL) in children with congenital vascular malformations (CVM) and to investigate factors associated with an impaired HRQOL. Children (2-17 years) with CVMs who visited the HECOVAN expertise center between 2016-2018 were included. The PedsQL 4.

Magnitude and relevance of change in health-related quality of life in patients with vascular malformations treated with sirolimus.

Introduction: Vascular malformations are rare congenital anomalies of the vascular system, which can involve the capillaries, veins, arteries, lymphatics, or a combination of vessel types. Patients with vascular malformations experience an impaired health-related quality of life (HRQoL) because of their symptoms (e.g.

Clinical differences in sirolimus treatment with low target levels between children and adults with vascular malformations - A nationwide trial.

The clinical presentation of patients with slow-flow vascular malformations is very heterogeneous. High clinical burden and subsequent reduced health-related quality of life is something they have in common. There is an unmet medical need for these patients for whom regular treatments like surgery and embolization are either insufficient or technically impossible.

[Disorders of the central lymphatic system].

The central lymphatic system consists of the thoracic duct, cisterna chyli and the retroperitoneal lymphatics through which lymph and chyle flows. Disorders of the central lymphatic system can for instance lead to leakage (chylothorax), accumulation of fluid (lymphedema) and retrograde flow (protein losing enteropathy). Abnormalities in the central lymphatic system were overlooked for years, followed by lack of diagnostic and therapeutic options.

The lymphatic system throughout history: From hieroglyphic translations to state of the art radiological techniques.

A comprehensive lymphatic system is indispensable for a well-functioning body; it is integral to the immune system and is also interrelated with the digestive system and fluid homeostasis. The main difficulty in examining the lymphatic system is its fine-meshed structure. This remains a challenge, leaving patients with uninterpreted symptoms and a dearth of potential therapies.

Semantic modelling of common data elements for rare disease registries, and a prototype workflow for their deployment over registry data.

Background: The European Platform on Rare Disease Registration (EU RD Platform) aims to address the fragmentation of European rare disease (RD) patient data, scattered among hundreds of independent and non-coordinating registries, by establishing standards for integration and interoperability. The first practical output of this effort was a set of 16 Common Data Elements (CDEs) that should be implemented by all RD registries. Interoperability, however, requires decisions beyond data elements - including data models, formats, and semantics.

Predicting surgical outcome in posterior retroperitoneoscopic adrenalectomy with the aid of a preoperative nomogram.

Background: Posterior retroperitoneoscopic adrenalectomy (PRA) has several advantages over transperitoneal laparoscopic adrenalectomy (TLA) regarding operative time, blood loss, postoperative pain, and recovery. However, it can be a technically challenging procedure. To improve patient selection for PRA, we developed a preoperative nomogram to predict operative time.

Effective low-dose sirolimus regimen for kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon in young infants.

Aims: Management of kaposiform haemangioendotheliomas (KHE) with Kasabach-Merritt phenomenon is challenging in young infants who are subjected to developmental pharmacokinetic changes. Sirolimus, sometimes combined with corticosteroids, can be used as an effective treatment of KHE. Simultaneously, toxicities such as interstitial pneumonitis related to the use of sirolimus may be fatal.

Patients with Congenital Low-Flow Vascular Malformation Treated with Low Dose Sirolimus.

Introduction: Patients with congenital vascular malformations often suffer from an impaired quality of life (QoL) because of pain and functional disabilities. Previous studies have shown that the mTOR inhibitor sirolimus can reduce complaints and improve QoL in some patients. High target levels of sirolimus of 10-15 ng/ml were well tolerated; however, in a relative high percentage of patients sirolimus caused serious adverse events (AEs).

Lymphatic malformations in children: treatment outcomes of sclerotherapy in a large cohort.

This retrospective study examines the outcomes of sclerotherapy in children with (veno)lymphatic malformations who received sclerotherapy between 2011 and 2016 (116 children, 234 procedures). Complication severity was classified using the Society of Interventional Radiology classification. Clinical response was rated on a scale of 0 (no change) to 3 (good improvement).

Treatment Outcomes of Embolization for Peripheral Arteriovenous Malformations.

Purpose: To evaluate treatment outcomes of embolization for peripheral arteriovenous malformations (AVMs) in a tertiary referral center where ethanol is the primary agent of choice.

Methods: A retrospective study was performed of 93 patients (median age, 31 years; range, 2-66 years) with peripheral AVMs treated with embolization (n = 442; median, 2 per patient; range, 1-82) between January 2010 and July 2016. Ethanol was used in most cases (n = 428; 97%).

A Clinical Feasibility Study to Image Angiogenesis in Patients with Arteriovenous Malformations Using Ga-RGD PET/CT.

Arteriovenous malformations (AVMs) have an inherent capacity to form new blood vessels, resulting in excessive lesion growth, and this process is further triggered by the release of angiogenic factors. Ga-labeled arginine-glycine-aspartate tripeptide sequence (RGD) PET/CT imaging may provide insight into the angiogenic status and treatment response of AVMs. This clinical feasibility study was performed to demonstrate that Ga-RGD PET/CT imaging can be used to quantitatively assess angiogenesis in peripheral AVMs.

A New and Innovative Method of Preoperatively Planning and Projecting Vascular Anatomy in DIEP Flap Breast Reconstruction: A Randomized Controlled Trial.

Background: In deep inferior epigastric perforator (DIEP) flap breast reconstructions, a free tissue flap from the abdomen is shaped into a breast and transferred to the thorax. Survival of this free flap relies on minuscule blood vessels, so-called perforators, providing blood supply to this newly molded breast. Preoperative mapping of these randomly distributed blood vessels is essential to avoid complications.

Comprehensive molecular and clinicopathological analysis of vascular malformations: A study of 319 cases.

Vascular malformations are part of overgrowth syndromes characterized by somatic mosaic mutations or rarely by germline mutations. Due to their similarities and diversity, clinicopathological classification can be challenging. A comprehensive targeted Next Generation Sequencing screen using Unique Molecular Identifiers with a technical sensitivity of 1% mutant alleles was performed for frequently mutated positions in ≥21 genes on 319 formalin-fixed paraffin-embedded samples.

Innate glycosidic activity in metallic implants for localized synthesis of antibacterial drugs.

Iron-containing metallic implants are shown herein to mediate hydrolysis of glycosidic linkages. Using glucuronide prodrugs for broad-spectrum fluoroquinolone antibacterial agents, we capitalize on this behaviour and perform localized synthesis of antimicrobials which affords a significant zone of inhibition of bacterial growth around the metallic material.

Self-expandable tubular collagen implants.

Collagen has been extensively used as a biomaterial, yet for tubular organ repair, synthetic polymers or metals (e.g., stents) are typically used.

Enzyme Prodrug Therapy Achieves Site-Specific, Personalized Physiological Responses to the Locally Produced Nitric Oxide.

Nitric oxide (NO) is a highly potent but short-lived endogenous radical with a wide spectrum of physiological activities. In this work, we developed an enzymatic approach to the site-specific synthesis of NO mediated by biocatalytic surface coatings. Multilayered polyelectrolyte films were optimized as host compartments for the immobilized β-galactosidase (β-Gal) enzyme through a screen of eight polycations and eight polyanions.

Noninvasive diagnostic tools for pelvic congestion syndrome: a systematic review.

Introduction: In the work-up of patients with suspected pelvic congestion syndrome, venography is currently the gold standard. Yet if non-invasive diagnostic tools are found to be accurate, invasive venography might no longer be indicated as necessary.

Material And Methods: A literature search in Pubmed and EMBASE was performed from inception until 6 May 2017.

Pasireotide does not improve efficacy of aspiration sclerotherapy in patients with large hepatic cysts, a randomized controlled trial.

Objectives: We tested whether complementary use of the somatostatin analogue pasireotide would augment efficacy of aspiration sclerotherapy of hepatic cysts.

Methods: We conducted a double-blind, placebo-controlled trial in patients who underwent aspiration sclerotherapy of a large (>5 cm) symptomatic hepatic cyst. Patients were randomized to either intramuscular injections of pasireotide 60 mg long-acting release (n = 17) or placebo (sodium chloride 0.

Embolization of the Geniculate Arteries Is an Effective Treatment of Recurrent Hemarthrosis Following Total Knee Arthroplasty That Can Be Safely Repeated.

Background: Recurrent hemarthrosis is a late complication in up to 1.6% of patients following total knee arthroplasty (TKA). In the absence of intrinsic coagulopathy, one etiology is bleeding of hypertrophic vascular synovium.

An innovative method of planning and displaying flap volume in DIEP flap breast reconstructions.

Background: Determining the ideal volume of the harvested flap to achieve symmetry in deep inferior epigastric artery perforator (DIEP) flap breast reconstructions is complex. With preoperative imaging techniques such as 3D stereophotogrammetry and computed tomography angiography (CTA) available nowadays, we can combine information to preoperatively plan the optimal flap volume to be harvested. In this proof-of-concept, we investigated whether projection of a virtual flap planning onto the patient's abdomen using a projection method could result in harvesting the correct flap volume.

Continuously Grooved Stent Struts for Enhanced Endothelial Cell Seeding.

Purpose: Implantation of pre-endothelialized stents could enhance cellular recovery of a damaged vessel wall provided attached cells remain viable, functional and are present in sufficient numbers after deployment. The purpose of this study was to evaluate the feasibility of grooved stainless steel (SS) stents as a primary endothelial cell (EC) carrier with potentially enhanced EC protection upon stent deployment.

Materials And Methods: Attachment and behavior of enzymatically harvested human adult venous ECs seeded onto gelatin-coated vascular stents were evaluated in an in vitro setting.

Shared Decision-Making in the Management of Congenital Vascular Malformations.

Background: In shared decision-making, clinicians and patients arrive at a joint treatment decision, by incorporating best available evidence and the patients' personal values and preferences. Little is known about the role of shared decision-making in managing patients with congenital vascular malformations, for which preference-sensitive decision-making seems obvious. The authors investigated preferences regarding decision-making and current shared decision-making behavior during physician-patient encounters.

Laser Guidance in C-Arm Cone-Beam CT-Guided Radiofrequency Ablation of Osteoid Osteoma Reduces Fluoroscopy Time.

Purpose: To assess whether laser guidance can reduce fluoroscopy and procedure time of cone-beam computed tomography (CBCT)-guided radiofrequency (RF) ablations of osteoid osteoma compared to freehand CBCT guidance.

Materials And Methods: 32 RF ablations were retrospectively analyzed, 17 laser-guided and 15 procedures using the freehand technique. Subgroup selection of 18 ablations in the hip-pelvic region with a similar degree of difficulty was used for a direct comparison.