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Pulmonary lymphatic flow disorders involve the abnormal lymphatic flow via lymphatic channels to the lungs and pleural space. Plastic bronchitis and chylothorax are the main complications of this abnormal lymphatic perfusion, which has been termed pulmonary lymphatic perfusion syndrome (PLPS). Following lymphatic access, dynamic contrast MR lymphangiography is the imaging modality of choice to diagnose these disorders. Management includes medical therapy, percutaneous interventions under fluoroscopy, and surgical interventions., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Lymphatic complications in patients with single ventricle include plastic bronchitis, protein-losing enteropathy, and chylous pleural effusion are a source of significant morbidity and mortality with historically limited therapeutic options. Novel lymphatic imaging techniques such as intranodal lymphangiography, dynamic contrast enhanced magnetic resonance lymphangiography and liver lymphangiography have allowed visualization of the lymphatic system and discovery of the pathophysiological mechanism of these conditions. This mechanism includes the combination of 2 factors: increased lymphatic flow in patients with elevated central venous pressure and presence of the lymphatic anatomical variant that allows the lymph to flow in close proximity to the serous (pleural space in chylothorax) or mucosal (plastic bronchitis and protein losing enteropathy) surfaces. Novel minimally invasive lymphatic interventional techniques, such as thoracic duct embolization, interstitial embolization and liver lymphatic embolization have allowed the obliteration of these abnormal lymphatic networks, resulting in resolution of the symptoms. Further refinement of the imaging techniques and interventional methods have subsequently allowed better patient selection and improved long term outcome of these procedures., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Vascular malformations are generally congenital benign lesions that have multiple variations in treatment algorithms. Surgery can be used as a single modality or as an adjunct in multimodal therapy to treat these lesions. Here we discuss surgical treatment of the major vascular malformations, including lymphatic, venous, and arteriovenous malformations. We explain some of the basic principles to resection of simple and complex lesions and adjunctive therapies. These adjunct therapies include chemotherapeutic injections, embolization, and laser therapy. Surgical resection of complex lesions should only be performed by an experienced vascular anomalies surgeon. A team approach is generally necessary to provide safe and effective treatment. While surgery for these complex lesions is an option, the most important principle to adhere to when treating any of these lesions is that the treatment should be no worse than the disease., (Copyright © 2019. Published by Elsevier Inc.)

Complex lymphatic anomalies include a variety of disorders with overlapping clinical, histological and imaging features. The often-confusing nomenclature used for lymphatic anomalies limits timely diagnosis and treatment. The updated 2018 classification of the International Society for the Study of Vascular Anomalies divides lymphatic anomalies into several subsets. 1 Newer imaging techniques including intranodal and magnetic resonance lymphangiography have improved our understanding of anatomy and function of the lymphatic system. Advances in medical, interventional, and surgical treatments have opened a realm of new therapeutic options for patients with complex lymphatic disorders., (Copyright © 2019 Elsevier Inc. All rights reserved.)

The approach to treating common (cystic) lymphatic malformations (LMs) has evolved significantly over the last decade due to clinical research and recent developments in molecular biology. Surgery, sclerosing agents, and medical drugs with specific targets for biological therapy have been reported for the management of LMs. We will discuss the importance to standardize the location and imaging characterization of LMs to improve the knowledge about the outcome of the different therapeutic options. Our goal is to help the reader understand the different options for the management of LMs with the balance between risk and benefit for the patients., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Lymphatic malformations are slow-flow vascular anomalies composed of dilated lymphatic channels and cysts of varying sizes. Percutaneous treatments, particularly sclerotherapy, play an important role in the treatment of these lesions, often obviating the need for surgical intervention. Owing to the complex nature of these lesions, a multidisciplinary approach should be used to guide diagnosis and management. This submission focuses on the workup and treatment of pediatric lymphatic malformations at our institution, with a focus on sclerotherapy. Therapeutic outcomes and the management of postprocedural complications are also discussed., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Pulmonary lymphatic diseases have been recognized for many years and have been referred as pulmonary lymphangiectasia, pulmonary lymphangiomatosis, plastic bronchitis, and idiopathic chylothorax or chylopericardium. The lymphatic etiology of these conditions has been determined by detection of cystic lymphatic structures on biopsy or postmortem examination. Development of new imaging techniques such as dynamic contrast-enhanced magnetic resonance lymphangiography has allowed better understanding of pathophysiology of these conditions. Dynamic contrast-enhanced magnetic resonance lymphangiography demonstrated that the common denominator of these disorders is an abnormal pulmonary lymphatic flow from the thoracic duct toward pulmonary parenchyma. This abnormal lymphatic flow propagates into mediastinum, lung parenchyma, pleural surfaces, and bronchial submucosa and has been termed as pulmonary lymphatic perfusion syndrome (PLPS). Known clinical presentation of PLPS includes spontaneous chylothorax or pericardium, neonatal chylous effusions, and plastic bronchitis. PLPS has been observed in all age groups and can be considered as a congenital anatomical lymphatic variant. The onset of the clinical symptoms can be provoked by increase of the lymphatic flow owing to elevated central venous pressure that results in lymphatic distention, trauma, and severe upper respiratory infection. Reported treatment of PLPS is obliteration of these abnormal lymphatic pathways by percutaneous embolization, a technique similar to thoracic duct embolization in chylothorax., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Vascular anomalies are comprised of either hemangiomas or vascular malformations.Low-flow vascular malformations can be divided into capillary, venous, and lymphatic types and are usually present at birth, undergo pari passu growth, and produce symptoms related to mass effect or stasis. High-flow malformations are comprised pre-dominantly of arteriovenous malformations that follow a more aggressive clinical course of hyperemia, adjacent mass effect, steal phenomenon, tissue destruction, and ultimately high output failure. Ultrasound, CT, nuclear medicine, angiography, and particularly MRI have greatly enhanced diagnostic accuracy and provide detailed information for percutaneous and surgical treatment planning and an objective means of following therapeutic efficacy. Interventional radiologic percutaneous sclerotherapy for low-flow lesions and embolosclerotherapy for high-flow lesions with or without adjunctive surgical intervention have become the mainstay of therapy.