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The diagnosis of vascular anomalies remains challenging due to significant clinical heterogeneity and uncertain etiology. Evaluation using biopsy and/or genetic testing for somatic variants is invasive, expensive, and prone to sampling error. There is great need for noninvasive and easily measured blood laboratory biomarkers that can aid not only in diagnosis, but also management of treatments for vascular anomalies. Angiopoietin-2, a circulating blood angiogenic factor, is highly elevated in patients with kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon and kaposiform lymphangiomatosis. Here, we describe our clinical experience using serum angiopoietin-2 as a biomarker for diagnosis and monitoring response to treatment., (© 2024 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

Introduction: Genetically targeted drugs in vascular anomalies (VA) are used despite the absence of a validated severity score. The aim of this study was to evaluate the feasibility of grouping phenotypic VA clinical characteristics into a single severity score., Methods: A systematic literature review including children treated with sirolimus accompanied by a detailed description of phenotype and management was conducted. Demographic data and clinical features were extracted to define distinct categories of phenotypes., Results: Children with VA display two main phenotypes regardless of VA subtype, which may overlap. A systemic phenotype results from direct invasion and compression of vital structures generally leading to hospitalization and aggressive management in infancy. A functional phenotype is associated with chronic pain and disability manifesting mainly during early adolescence and managed in the outpatient setting., Conclusion: The two distinct phenotypes described could be the basis for developing a unified scoring system for VA severity assessment., (© 2022 Wiley Periodicals LLC.)

Vascular anomalies, both vascular tumors and vascular malformations, can occur in isolation or as part of syndromes including those which feature phenotypic overgrowth. To update what is known about vascular anomalies associated with overgrowth, PubMed was searched for "overgrowth syndromes and vascular anomalies or malformations." PubMed, OMIM, and the Rare Disease Database also were searched for specific diagnoses. We review individual overgrowth syndromes, provide a case-based approach to the clinical, radiographic, pathologic, and genetic basis for diagnosis, to complications of both the vascular anomalies and the overgrowth, and emphasize the need for a multidisciplinary approach to care., (© 2021 Wiley Periodicals LLC.)

Vascular anomalies are a group of disorders divided into two distinct subtypes: vascular tumors and vascular malformations. Vascular tumors are proliferative in nature, while malformations are nonproliferative. Simple, localized vascular malformations refer to a group of malformations that are localized to a single area of involvement. These simple malformations include capillary, lymphatic, venous, and arteriovenous malformations. The pediatric hematologists and oncologists are becoming increasingly involved in the diagnosis and management of these disorders. This review presents four cases as a means to discuss the diagnosis, clinical and imaging features, and management strategies of simple, localized vascular malformations., (© 2021 Wiley Periodicals LLC.)

Vascular anomalies represent a diverse group of complex disorders that can cause significant complications, including coagulopathies, pain, and decreased function. The diagnosis of vascular anomalies is often challenging due to heterogeneity of presenting phenotypes and overlapping clinical features with other pediatric conditions. Pediatric hematologists/oncologists (PHO) are uniquely positioned for an essential role in diagnosing, managing, and coordinating the multidisciplinary care required to maximize the quality of life of these patients. Here, we review the diagnostic approach involved in patients with vascular anomalies and utilize cases to highlight the challenges involved, and how PHOs can play a vital part in the care of these patients., (© 2022 Wiley Periodicals LLC.)

The care of patients with vascular anomalies is quickly becoming a complex field requiring high-quality, coordinated multidisciplinary care. In this article, we review the history of multidisciplinary care in this field, discuss the benefits of this model of care, and outline some of the essential components and structure of a successful vascular anomalies team. We provide an overview of two example programs and a roadmap for other centers to develop their own multidisciplinary vascular anomalies teams., (© 2020 Wiley Periodicals LLC.)

Vascular anomalies are a heterogeneous group of disorders that are currently classified based on their clinical and histological characteristics. Over the past decade, there have been significant advances in molecular genetics that have led to identification of genetic alterations associated with vascular tumors, vascular malformations, and syndromes. Here, we describe known genetic alterations in vascular anomalies, discuss when and how to test, and examine how identification of causative genetic mutations provides for better management of these disorders through improved understanding of their pathogenesis and increasing use of targeted therapeutic agents in order to achieve better outcomes for our patients., (© 2021 Wiley Periodicals LLC.)

Some vascular anomalies can present with challenging hematologic aberrations. Kaposiform hemangioendothelioma (KHE) may be complicated with Kasabach-Merritt phenomenon (KMP) and stagnant blood flow in slow-flow malformations can promote activation and consumption of coagulation factors, which results in bleeding and clotting known as localized intravascular coagulopathy (LIC). These patients can experience significant morbidity secondary to pain due to thrombosis and are at higher risk of hematologic complications during surgical procedures. No standard of care has been established to prevent or manage these complications. This review focuses on the management of coagulopathy in children and adults with vascular anomalies., (© 2021 Wiley Periodicals LLC.)

Background: Congenital venous malformations (VMs) are low-flow vascular anomalies that can cause coagulation abnormalities. This phenomenon, referred to as localized intravascular coagulopathy (LIC), is characterized by elevated D-dimer, hypofibrinogenemia, and/or thrombocytopenia. Increased risk for LIC includes patients with an extensive VM, multifocal VM, and Klippel-Trenaunay and CLOVES syndromes. Peri-procedural anticoagulation has been given to prevent complications from LIC in patients undergoing surgical/interventional procedures; however, the rate of clinically relevant complications from sclerotherapy is largely unknown. The purpose of this study is to describe a single-institution's incidence of LIC in patients with VMs and coagulopathy-related complications following sclerotherapy., Design/methods: Retrospective chart review of patients, 0-21 years of age, with VM who underwent sclerotherapy without peri-procedural anticoagulation and had coagulation profiles evaluated within 1 month prior to sclerotherapy., Data Collected: diagnosis type (high vs. low risk for LIC), coagulation profile (including PT/PTT, D-dimer, fibrinogen, and platelet count), sclerosant used, and post-procedure outcomes. Coagulopathy-related complications included clinically relevant bleeding, deep vein thrombosis (DVT), and pulmonary embolism (PE)., Results: N = 138 patients; 59.4% were female. The most common location of VM was the lower extremity (47.8%; 66/138). Of patients with high-risk VMs (29/138 [21%]), 11/29 (37.9%) had laboratory values consistent with LIC, whereas 5% (5/109) of low-risk VMs had LIC. In sum, 492 sclerotherapy procedures were performed with no complications of bleeding, DVT, or PE., Conclusion: Patients undergoing sclerotherapy for VM with abnormal coagulation profiles may not require peri-procedural low molecular weight heparin (LMWH). Further studies are needed to precisely define which patients would benefit from anticoagulation., (© 2022 Wiley Periodicals LLC.)

Vascular anomalies comprise a spectrum of disorders characterized by the abnormal development or growth of blood and lymphatic vessels. These growths have unique features and diverse behaviors, mandating a multidisciplinary approach in their evaluation, diagnosis, and management. Here we describe the case of a male toddler presenting with an abdominal mass, originally treated as a metastatic retroperitoneal tumor, but subsequently felt to represent a vascular anomaly., (© 2021 Wiley Periodicals LLC.)

Background/objectives: Slow-flow vascular malformations are abnormal vessels that can lead to activation and consumption of coagulation factors and thrombosis, known as localized intravascular coagulopathy (LIC). Most clinical and research evidence of vascular malformation hemostasis relies on conventional coagulation studies, which may not provide a complete picture. Thromboelastograpy (TEG) is a tool that can provide real-time assessment of a patient's coagulation dynamics, and may allow for a more individualized treatment approach. We hypothesized that patients with slow-flow vascular malformations will have changes in TEG parameters peri-procedure that will help predict blood product or medication administration., Design/methods: Institutional Review Board approved prospective study of patients with slow-flow vascular malformations undergoing a sedated, minor procedure. TEG and conventional coagulation studies were obtained preprocedure, 15 min, and when possible, at 30 min after the start of the procedure., Results: Twenty-five patients were enrolled. Median age was 15 years (range 3-47 years). Procedures included laser and/or sclerotherapy. There were no changes in TEG parameters from baseline to 15 min or 30 min. The following decreased from baseline to 15 min: fibrinogen 313 to 287 mg/dL (P = .001), D-dimer 1.3 to 1.1 mg/L (P = .02), hemoglobin 12.8 to 11.8 g/dL (P = .001), and platelet count 272 000 to 256 000 (P = .006). No patient had a bleeding/thrombotic complication during or within 1 week postprocedure., Conclusion: We saw no change in TEG parameters or bleeding or clotting complications despite significant numerical changes in conventional coagulation profiles, suggesting that conventional studies may not be as useful in determining risks of bleeding or thrombotic complications peri-procedure for minor procedures., (© 2020 Wiley Periodicals LLC.)

Background: Slow-flow vascular malformations (SFVM) are associated with localized intravascular coagulopathy (LIC), which is characterized by elevated D-dimer and, when severe, hypofibrinogenemia. LIC results in intralesional clotting and hemorrhage and increases risk for significant thrombotic and bleeding complications. Sclerotherapy has been a suggested potential trigger for LIC worsening to disseminated intravascular coagulopathy. Hematologic complications of sclerotherapy in SFVM, along with low-molecular-weight heparin (LMWH) used to prevent worsening LIC, are largely unstudied., Procedure: Medical records of patients with SFVM and LIC who underwent sclerotherapy at Cincinnati Children's Hospital Medical Center from July 2008 to December 2016 were reviewed for periprocedural hematologic complications. LMWH dose, frequency, and course length were evaluated., Results: Fifty-nine patients with SFVM and LIC underwent 281 sclerotherapy procedures, of which 86% were in children. Eighty-five percent of patients received periprocedural LMWH, although at various doses and course lengths. No thrombotic complications occurred in children. One adult on LMWH developed pulmonary emboli after sclerotherapy. No major bleeding complications occurred postoperatively. In four patients, fibrinogen dropped below 100 mg/dL post-sclerotherapy, requiring cryoprecipitate. One patient required packed red blood cell (RBC) transfusion for sclerotherapy-induced hemolysis. No intraoperative bleeding or thrombotic events occurred., Conclusion: LMWH use, at subtherapeutic dosing, was common in this patient population and did not appear to increase risk of significant bleeding before, during, or after sclerotherapy. In children with SFVM, bleeding and thrombotic complications after sclerotherapy appear rare. Although safe, prospective studies are needed to evaluate the efficacy of LMWH to prevent worsening coagulopathy with procedures., (© 2020 Wiley Periodicals, Inc.)

Purpose: Sclerotherapy or surgical resection is options for symptomatic venous malformations (VM). Sclerotherapy may require repetitive intervention and resection is often avoided due to operative morbidity. The purpose of this study was to report use of single-stage n-butyl cyanoacrylate glue embolization and surgical resection of focal VM., Methods: A review of patients with focal VM who underwent glue embolization followed by resection at a single tertiary care vascular malformations center was performed. All embolizations were performed with ultrasound and fluoroscopy under the same anesthetic as resection. Patient characteristics and outcomes were evaluated., Results: Fifteen procedures were performed in 12 patients with a total of 20 VM addressed, as several patients had multiple VM. Mean age was 16 ± 9 years. Malformation locations included scalp, hip, gluteal, labial, toe, finger, face, lip, chest, and foot and size ranged from 1.0 to 10.5 cm. Median (range) of prior sclerotherapy treatments was 3 (0-5) and three patients previously underwent surgical resection. Median blood loss was zero (0-10) mL. Surgical complications occurred after five procedures (33%) including superficial wound dehiscence and cellulitis. No complications required readmission or reoperation. At a median follow up of 195 (103-266) days, no patients have required additional treatment., Conclusion: Glue embolization and resection of focal VM of variable size and location appears to have durable results and low surgical morbidity. This single-stage procedure, often performed as an outpatient, may be utilized as upfront treatment for symptomatic malformations or for VM refractory to other treatments., (© 2019 Wiley Periodicals, Inc.)

Background: Vascular malformations (VM) are congenital lesions that can be debilitating and cause significant aesthetic and functional limitations. The chemotherapeutic agent bleomycin has been utilized as a sclerosant, directly injected percutaneously into the VM. Unfortunately, little is known about the benefits and short-term side effects of bleomycin with intralesional injections., Procedure: An IRB approved, retrospective chart review was performed on patients with VM who had been treated with intralesional bleomycin. Data included type of VM, number of treatments, total bleomycin dose per m², and adverse effects. A questionnaire was administered to available patients to assess subjective outcomes and side effects., Results: Forty-six patients were treated with 141 procedures of bleomycin sclerotherapy for VM. Patient ages ranged from 1 to 20 years (median age 10 years). The median cumulative bleomycin dose was 16.3 units/m²/person (range of 1.7-97.0 units/m²/person). Sixty-three percent of patients were reached for a questionnaire to assess short-term side effects. Ninety percent of patients surveyed were satisfied to very satisfied with the results from the procedure. About 24% of patients experienced transient nausea, vomiting and/or local hyperpigmentation., Conclusion: Bleomycin sclerotherapy can be an effective treatment of VM with repeat exposure with minor risk of short-term side effects, however, long-term risks are of great concern. Further studies are required to assess systemic absorption and long-term risks., (© 2018 Wiley Periodicals, Inc.)

Vascular anomalies (VAs) are classified as tumors or malformations depending on their clinical characteristics, pathological diagnosis, and genomic information. Diagnosis can be challenging because of the heterogeneity of clinical presentation; thus, the best diagnosis and care are provided by an interdisciplinary team of specialists. Over the past 10 years, an increasing number of pediatric hematologist/oncologists are caring for patients with VAs secondary to new medical therapy options and clinical trials. This paper focuses on complicated VA issues often seen by the pediatric hematologist/oncologist. The paper reviews clinical pearls on diagnosis, histology, radiology, and treatment options., (© 2017 Wiley Periodicals, Inc.)