How we approach pediatric congenital chylous effusions and ascites

Taizo A. Nakano, Yoav Dori, Lindsey Gumer, Deborah R. Liptzin, Lauren R.S. Hill, Ann M. Kulungowski

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

Congenital lymphatic leak may develop in patients with maldeveloped lymphatics and result in life-threatening fluid and electrolyte imbalance, protein deficiency, and immunodeficiency. 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.

Original languageEnglish
Article numbere29246
JournalPediatric Blood and Cancer
Volume69
Issue numberS3
DOIs
StatePublished - Aug 2022

Keywords

  • chylothorax
  • chylous ascites
  • chylous effusion

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