The disorders of the central lymphatic conduction very commonly result in the development of chronic pleural effusions, which can be chylous or non-chylous in nature, and can be unilateral or bilateral. While the fluid can be drained with chest tubes of different types, the chronic loss of lymphatic fluid has profound negative nutritional and immunological effects on the patients and is not a sustainable long-term management strategy. There are in the marked several pleural drains that can be tunneled under the skin and can be used in the ambulatory setting and managed by the patients at home, but in our experience their life span is too short because they quickly become occluded by proteinaceous deposits. Most patients are managed with intermittent outpatient thoracentesis. Patients extend the time in between the thoracenteses until the volume is such that the loss of lung capacity causes shortness of breath at rest. The high concentration of proteins of the lymphatic fluid progressively leads to the development of septations in the pleural space, and a thick pleural rim. This phenomenon makes thoracentesis less and less effective as time goes by and results in the gradual chronic entrapment of the lungs, gradually decreasing the patients’ quality of life (Figure 1). Not uncommonly the chest wall undergoes a retraction that makes the re-recruitment of the trapped lungs very difficult. The classic management strategies for pleural effusions, such as a simple pleurodesis or a non-fat diet, hardly ever work in cases of chronic lymphatic effusions secondary to lymphatic conduction disorders. The current state-of-the-art management strategy is the percutaneous glue embolization of the anomalous lymphatic channels, and when that fails, intraoperative glue embolization is the last resource.

Similarly to what happens in the pleural space, central lymphatic conduction disorders can lead to chronic lymphatic ascites, chylous or non-chylous. The compliant nature of the abdominal cavity allows patients to accumulate large amounts of fluid before they become symptomatic. On one hand this allows for longer periods of time without the need for a paracentesis, but on the other hand, as it happens in the pleural space, when the lymphatic fluid remains undrained for long periods of time it develops septations that make the paracentesis less and less effective. Eventually, if it is not resolved, the ascites becomes multiloculated (Figure 2). The tunneled catheters that can be used for patients with ascites of oncologic origin (e.g., Denver® shunt, BD, NJ, USA) do not work well in cases of lymphatic ascites because the draining pumps frequently clog with proteinaceous debris. There are several percutaneous and surgical interventions that can be used for the management of chronic lymphatic ascites, and each patient requires a tailored approach.