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Primary intestinal lymphangiectasia (PIL) is a rare disease that was discovered in 1961 by Waldmann et al., who first described the concept of intestinal lymphangiectasia. PIL is manifested by large and dilated intestinal lymphatic vessels that lead to the loss of lymphatic fluid to the intestinal lumen, including severe hypoalbuminemia and, consequently, generalized edema. The etiology of PIL is unknown; however, several genes are involved in the development of the lymphatic system. The case of a two-month-old patient with a prenatal diagnosis of ascites on third-trimester ultrasound was presented. The patient was born with rapidly progressive ascites and showed an endoscopic study with evidence of snowflake images and histology compatible with intestinal lymphangiectasia. The patient remained on octreotide infusion at baseline and subsequently with refractory ascites the dose was increased. Several evacuatory paracentesis were performed and he remained on enteral nutrition based on an extensively hydrolyzed formula with a 54% contribution of medium chain triglycerides (MCTs) and parenteral nutrition (PN). Subsequently, the patient was switched to a subcutaneous infusion of octreotide, which allowed hospital discharge and adequate outpatient follow-up with a favorable evolution. [ABSTRACT FROM AUTHOR]

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Patients with chylothorax present a high risk for malnourishment since continuous loss of chylo leads to a significant impairment of their nutritional status. Chylothorax treatment, which initially is conservative, includes dietary measures and medications such as octreotide that decreases chylothorax flow. In this paper we present the case of a patient with chylothorax treated by means of pleural drainage, parenteral nutrition, and octreotide, and we review the most appropriate nutritional support as well as the efficacy and safety of octreotide in chylothorax therapy. The types of nutritional intervention that may be done are a low-fat diet supplemented with intermediate-chain triglycerides (ICT), fat-free enteral nutrition or EN with a high percentage of ICT, and parenteral nutrition. There is no consensus on which is the most appropriate measure. We found very few comparative studies, and the literature is based on single cases or case series. Some authors consider parenteral nutrition as the first choice, whereas others recommend starting with a specific diet and using parenteral nutrition only in specific cases. Parenteral nutrition must cover the patient's demands together with compensating the protein and energy losses due to chylothorax. The use of lipid emulsions is no contraindicated since they do not reach the lymphatic system. With regards to EN, the formulations may be lipid-free or with low lipid content. There is no agreement on when to start them once the drainage of chylo decreases. There are cases and case series indicating that octreotide use in chylothorax seems to be safe and effective. There is no consensus on when to start the therapy, the most appropriate dose, or the time to withdraw the treatment. [ABSTRACT FROM AUTHOR]