MR Lymphangiography: Congenital Lymphatic Flow Disorders.

Abstract: Congenital lymphatic flow disorders collectively refer to a heterogeneous group of diseases that manifest as chylothorax, chylous ascites, intestinal lymphangiectasia, protein-losing enteropathy, and peripheral extremity or genital lymphedema, all in the absence of identifiable injury to the lymphatic system. We have only recently begun to understand congenital lymphatic flow disorders through the ability to image lymph flow dynamically. Intranodal dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) is a crucial technique for imaging lymphatic flow in pediatric patients with congenital lymphatic flow disorders. However, as lymphatic imaging is still a nascent discipline with many uncertainties regarding optimal imaging and treatment, effective patient management requires a comprehensive understanding of imaging techniques, disease pathophysiology, and multidisciplinary treatment approaches. Above all, a fundamental understanding of the physiological lymphatic flow of the central conducting lymphatics is essential for the correct interpretation of DCMRL images. This knowledge helps to avoid unnecessary examinations, erroneous diagnoses, and potentially harmful treatment approaches. This review provides an overview of the methods, advantages, and precautions for interpreting the DCMRL examination, a state-of-the-art lymphatic system imaging technique, and shares various case studies.