Skeletal manifestations in lymphatic diseases: nonenhanced magnetic resonance lymphography

The diffuse lymphatic diseases-generalized lymphatic anomalies (GLA) and primary lymphangiectasia-can present with both visceral and skeletal involvement. These diseases are life-threatening and can lead to malnutrition and immunodeficiency as consequences of impaired lymphatic function. Skeletal involvement has specific complications, including deformities, pain, fractures, septic arthritis, and osteomyelitis. The presence of bone involvement with bone lysis and cortical destruction is a marker of disease extension and severity. Identification of skeletal involvement may orient patients toward targeted therapies. On standard CT and MRI, bone lesions of lymphatic origin usually show no specific features, and etiological diagnosis can be difficult. Nonenhanced magnetic resonance lymphography (NEMRL) is a noninvasive and non-ionizing technique based on T2-weighted sequences that enables the visualization of the lymphatic circulation. In the setting of undetermined bone lesions, NEMRL may suggest a lymphatic origin by identifying extraosseous lymphatic anomalies or a communication between the bone lesion and the lymphatic system. Furthermore, NEMRL may determine the cause of the lymphatic anomalies and guide therapeutic options. The technique of NEMRL, the different imaging patterns of bone lesions of lymphatic origin, and the associated lymphatic anomalies identified by NEMRL that can orient the diagnosis and help in classifying lymphatic diseases are discussed. CRITICAL RELEVANCE STATEMENT: Nonenhanced magnetic resonance lymphography is a non-ionizing, noninvasive tool to detect and characterize skeletal involvement in diffuse lymphatic diseases. KEY POINTS: Skeletal involvement in lymphatic disorders ranges from simple lymphangioma to bone destruction. NEMRL is noninvasive and allows the study of lymphatic system disorders. NERML evaluates lymphatic disease extension when assessing a lymphatic bone lesion. NEMRL can confirm the lymphatic origin of an undetermined bone lesion. NEMRL sometimes identifies communications between lymphatic bone lesions and extraosseous lymphatic anomalies.