Seminars in Interventional Radiology最新文献

Vascular malformations (VMs) are a heterogeneous group of arterial, venous, capillary, and/or lymphatic networks that typically are present at birth and grow with the patient. Signs and symptoms associated with VM range from absent to severe, with a broad spectrum of pain, cosmetic disfigurement, and local tissue destruction. Treatment options for these malformations extend from observation to surgical reconstruction. Each treatment modality has a role in the management of vascular anomalies, but also has specific challenges. Observation is not always sufficient, medical therapies may only be available for a subset of patients and are ultimately not curative, and surgical excision may itself be disfiguring or incomplete/not definitive. Sclerotherapy and/or embolotherapy have become a cornerstone of treatment for many types of VMs. These techniques are applicable to a wide range of VMs, are less invasive, have a shorter recovery period, and are repeatable. Although some VMs may not be amenable to percutaneous or endovascular treatment given their location, and multiple sessions may be required for select large and complex VMs, these minimally invasive treatments are often first line and may be definitive in treating VMs. Various agents may be used in sclerotherapy and embolotherapy, each with their own risks and benefits. Although there is a paucity of Level 1 evidence supporting the superiority of one agent over another, longstanding case series and expert experience exist, informing our knowledge of the safety and efficacy of sclerotherapy and embolotherapy. A brief overview of the most used sclerotherapy and embolotherapy agents and their uses is provided.

Recent advancements in endovascular techniques have transformed the management of vascular malformations, shifting away from traditional surgical resections to less invasive methods like embolization and sclerotherapy. Vascular malformations are congenital anomalies resulting from embryonic angiogenesis failures, often misdiagnosed but manageable through a multidisciplinary approach. These malformations range from high-flow arteriovenous connections to low-flow venous, lymphatic, or mixed lesions, each requiring specific diagnostic and treatment strategies. Specific syndromes like Klippel-Trenaunay and Parkes-Weber require tailored approaches due to their unique presentations. Diagnosis relies on a combination of physical examination and advanced imaging techniques, including ultrasound and MRI. Treatment is focused on symptomatic relief and improving the patient's quality of life. Interventional treatment is indicated when conservative and/or medical management is insufficient. Surgical interventions remain relevant for select cases, often in a multidisciplinary context. Newer therapies, such as sirolimus, are emerging for complex, treatment-resistant cases.

High-flow arteriovenous malformations (AVMs) are among the rarest and most recalcitrant lesions in medicine. Patients with these anomalies are best managed longitudinally in tertiary referral centers by dedicated multidisciplinary teams that include surgeons, dermatologists, hematologists, geneticists, and interventional radiologists. A significant percentage of these lesions cannot be cured per se and intervention can worsen symptoms or preclude effective future embolization. Existing classification systems help guide treatment, but in nearly all cases, therapy must be individualized based on patient, symptoms, and lesion angioarchitecture. For various reasons, referral to tertiary centers may be impossible for some patients. The purpose of this article is to provide an approach to the evaluation and treatment of these abnormalities.

Venous malformations (VMs) represent the most common type of congenital vascular anomalies, characterized by slow-flow lesions arising from disorganized angiogenesis. These malformations can vary widely in size, location, and clinical impact, presenting significant challenges in diagnosis and management. A multidisciplinary approach is essential for optimizing care, with goals centered on symptom relief and functional preservation. Diagnostic evaluation typically involves clinical examination, imaging, and, in complex cases, genetic testing. Interventional radiology, particularly sclerotherapy and endovascular embolization, has become the first-line treatment, complemented by surgical and emerging molecular therapies, particularly targeting the mTOR/PI3K/AKT pathway. This review provides a comprehensive examination of the genetics, clinical presentation, and key diagnostic imaging aspects of VMs, along with a detailed discussion of current treatment modalities, emerging therapeutic techniques, and future directions for improving patient care.

The purpose of this article is to familiarize the reader with the basic genetics and vascular biology behind the array of vascular anomalies they may encounter in their practice. Individuals with vascular malformations are often referred to multidisciplinary clinics composed of diverse specialists all with the same goal: how to provide the best care possible. The team is typically composed of physicians, nurses, social workers, and technical staff from multiple specialties including diagnostic and interventional radiology, dermatology, hematology/oncology, otolaryngology, plastic surgery, and several additional subspecialties. Imaging plays a crucial role in diagnosis and treatment planning, but increasingly biopsies are needed for more accurate histopathological and genetic information to inform the plan of treatment, as well as for counseling patients and their families on the natural history, heritability, and long-term prognosis of the condition. Understanding the molecular mechanism that gives rise to vascular anomalies is crucial for arriving at the proper diagnosis and choosing among treatment options. As oncological medications are being increasingly repurposed to treat vascular malformations, it is vital for those caring for patients with vascular anomalies to understand how these anomalies develop, and which drug may be appropriate to repurpose for this benign disease.

Lymphatic malformations (LMs) arise from errors in lymphatic vascular development during embryogenesis and encompass an array of conditions that span from common cystic LMs to complex lymphatic anomalies (CLAs). Manifestations of LMs are wide-ranging, from clinically inconsequential to life-threatening. Proper diagnosis and management can be challenging and often benefit from an experienced multidisciplinary team. Cystic LMs are localized entities for which percutaneous sclerotherapy is the mainstay treatment. CLAs, on the other hand, are more diffuse in involvement and typically require multimodal therapy. With advances in the genetic understanding of LMs, targeted systemic therapies have been increasingly utilized with promising results. Thoracic duct interventions, both surgical and percutaneous, have a limited role in CLAs and should be approached cautiously to avoid significant complications. In this review, we discuss the genetic basis, imaging findings, and management options for LMs, with a particular focus on relevant interventional radiology techniques.

Vascular anomalies (VAs) are a group of rare diseases affecting 0.01 to 0.02% of the population. VA may be associated with complex disorders affecting multiple organ systems, requiring a multidisciplinary approach to treatment. Multidisciplinary clinics are most frequently affiliated with pediatric centers. Multidisciplinary clinics in the adult setting are rare. Many adult VA patients find it difficult to establish care with individual providers, let alone a multidisciplinary clinic, leading to barriers to lifelong comprehensive care.

Vascular anomalies (VAs) are a heterogeneous group of vascular malformations and tumors that are frequently encountered in daily practice. While most are benign, malignant vascular tumors exist, as do other nonvascular soft-tissue malignancies that may masquerade as a benign VA. The subtleties of arriving at an accurate diagnosis can be intimidating, as new entities are described and names of conditions have been modified. Therefore, the goal of this article is to describe the classic imaging findings of VAs so that the interventional radiologist can confidently and competently contribute to management decisions, with special attention to ominous imaging features that should prompt biopsy or further imaging workup.

Historically, the care for patients with vascular anomalies has been challenging due to the complex nature and diversity of these anomalies with a wide array of symptomatology. In the recent past, most therapies for vascular anomalies focused on surgical, procedural, and supportive care measures to treat local symptoms, but many patients still experienced significant disease with excess morbidity and mortality. Today, the pharmacotherapeutic options available for treating vascular anomalies have greatly expanded due to the increased understanding of the genetic and molecular pathways causing these anomalies, with the subsequent development of more targeted pharmacotherapies. In addition to the growth in targeted medications available to treat patients with vascular anomalies, there has been an improved understanding of the hematologic abnormalities related to these diseases and how to manage them. While interventional radiologists do not typically primarily manage systemic medications to treat vascular anomalies, a baseline understanding of the medical management of these diseases is essential to ensuring that a contemporary, multidisciplinary, multimodal approach to treatment is pursued when appropriate. Ultimately, patients are now benefitting from having multiple modalities of treatments available to them and are experiencing improved quality of life and less morbidity.