Leakage of lymph from the lymphatic ducts causes chylothorax (CT) or chylous ascitis (CA). This may happen for unknown reasons during fetal life or after birth and may also be caused by trauma after thoracic surgery or by other conditions. Fetal CT and CA may be lethal particularly in cases with fetal hydrops that sometimes benefit of intra-uterine instrumentation. After birth, symptoms are related to the amount of accumulated fluid. Sometimes, severe cardio-respiratory compromise prompts active therapy. Most patients with CT or CA benefit from observation, rest, and supportive measures alone. Drainage of the fluid may be necessary, but then loss of protein, fat, and lymphoid cells introduce new risks and require careful replacement. Low-fat diets with MCT and parenteral nutrition decrease fluid production while allowing adequate nutritional input. If lymph leakage does not stop, secretion inhibitors like somatostatin or octreotide are prescribed, although there is only weak evidence of their benefits. Imaging of the lymphatic system is indicated when the leaks persist, but this is technically demanding in children. Shunting of the lymph from one body space to another by means of valved catheters, embolization of the thoracic duct, and/or ligation of the major lymphatics may occasionally be indicated in cases refractory to all other treatments.
Keywords: Chyloperitoneum; Chylothorax; Chylous ascites; Fetal ascites; Fetal chylothorax; Fetal hydrops; Lymph; MCT; Parenteral nutrition; Shunt.
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