Hospital Tunku Azizah, Kuala Lumpur, Malaysia
There is a wide spectrum of lymphatic disorders ranging from congenital malformations to other processes leading to lymph and/or chyle accumulation or lymphatic flow abnormalities. The lymphatic system performs multiple important functions in the human body, including maintenance of fluid balance, absorption of fat from the digestive tract and immune surveillance. Pulmonary lymphatic flow disorders involve the abnormal lymphatic flow via lymphatic channels to the lungs and pleural space.
The International Society for the Study of Vascular Anomalies (ISSVA) 2018 classifies the various lymphatic malformation (LM) into common cystic LM, generalized lymphatic anomaly, Gorham-Stout disease, channel-type LM, acquired progressive lymphatic anomaly and primary lymphedema. Given the heterogenous conditions, the precise workup and management depend on the underlying disorder and clinical context.