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Rapid diagnosis and therapy are necessary to prevent these complications; however, the field lacks clinical trials to support standardized diagnostic treatment guidelines. We present our current multidisciplinary approach to the diagnosis and management of congenital lymphatic leak including chylous pleural effusions and ascites. Depending on the rate of lymphatic leak, therapy can range from observation with nutritional modifications to surgical and interventional procedures aimed to reduce lymphatic drainage. Modalities to image central and peripheral lymphatics have advanced considerably. Genetic variants and subsequent targets that drive lymphatic maldevelopment have expanded the repertoire of possible pharmacotherapeutic options.<\/jats:p>","DOI":"10.1002\/pbc.29246","type":"journal-article","created":{"date-parts":[[2022,9,7]],"date-time":"2022-09-07T16:46:01Z","timestamp":1662569161000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["How we approach pediatric congenital chylous effusions and ascites"],"prefix":"10.1002","volume":"69","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0389-2297","authenticated-orcid":false,"given":"Taizo A.","family":"Nakano","sequence":"first","affiliation":[{"name":"Vascular Anomalies Center Children's Hospital Colorado  Aurora Colorado USA"},{"name":"Center for Cancer and Blood Disorders, Children's Hospital Colorado University of Colorado School of Medicine  Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoav","family":"Dori","sequence":"additional","affiliation":[{"name":"Division of Cariology, Children's Hospital of Philadelphia University of Pennsylvania School of Medicine  Philadelphia Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lindsey","family":"Gumer","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Children's Hospital Colorado University of Colorado School of Medicine  Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deborah R.","family":"Liptzin","sequence":"additional","affiliation":[{"name":"Breathing Institute, Children's Hospital Colorado University of Colorado School of Medicine  Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lauren R. 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