Lymphoscintigraphy in the diagnosis of lymphangiomatosis

Lymphangiomatosis is a rare condition characterized by multiple abnormalities of the lymphatic system. Diagnosis is often difficult, as chronic, intermittent, or acute pain; edema; and other symptoms may affect the respiratory, gastrointestinal, renal, hepatic, skeletal, and other organ systems. We report the case of a patient who first presented with lymphedema in childhood and was treated intermittently for related symptoms before diagnosis was achieved 36 years later. Plain film radiography, bone scanning, computed tomography, magnetic resonance imaging, and lymphoscintigraphy were used to arrive at a diagnosis. Information derived from all scan types was combined to derive a diagnosis of lymphangiomatosis. Lymphoscintigraphy provided direct evidence of the abnormal lymphatic flows associated with lymphangiomatosis. Lymphangiomatosis presents a diagnostic challenge; information from several scan types, including lymphoscintigraphy, is useful in deriving this diagnosis.