Neonatal Lymphatic (Chylous) Disorders
- 1 December 2013
- journal article
- Published by American Academy of Pediatrics (AAP) in NeoReviews
- Vol. 14 (12), e600-e612
- https://doi.org/10.1542/neo.14-12-e600
Abstract
Lymphatic (chylous) disorders are rare in neonates, and most clinicians will only experience a handful of cases during their careers. Chylous effusions of the pleura, peritoneum and pericardium, and lymphedema are not diagnoses but rather symptoms of an underlying disease of the lymphatics. Although it is relatively easy to establish whether an effusion is of chylous origin or not, expertise in different imaging techniques of the lymphatic system does not appear to be universally available. This makes it difficult to determine the cause of the effusion and thereby arrive at a prognosis, and whether and when to recommend surgical treatment. The goal of current medical management is to treat symptoms by reducing overall lymphatic flow and to allow enough time to resolve the lymphatic leakage (whatever the underlying cause) without simultaneously risking infectious complications. Most current therapies are not evidence-based. A prospective registry of patients who have chylous effusions and a multicenter randomized controlled trial to evaluate different treatment options are needed.Keywords
This publication has 41 references indexed in Scilit:
- Role of immunoglobulin supplementation for secondary immunodeficiency associated with chylothorax after pediatric cardiothoracic surgery*Pediatric Critical Care Medicine, 2012
- Thoracic Duct Embolization for Chylous LeaksSeminars in Interventional Radiology, 2011
- Octreotide for the treatment of chylothorax in neonatesEmergencias, 2010
- Congenital chylothorax: from foetal life to adolescenceActa Paediatrica, 2010
- Congenital chylothorax: Clinical course and prognostic significancePediatric Pulmonology, 2009
- Primary intestinal lymphangiectasia (Waldmann's disease)Orphanet Journal of Rare Diseases, 2008
- Nonimmune idiopathic hydrops fetalis and congenital lymphatic dysplasiaAmerican Journal of Medical Genetics Part A, 2006
- Chylothorax after repair of congenital diaphragmatic hernia—Risk factors and morbidityJournal of Pediatric Surgery, 1998
- Management of chylopericardiumJournal of Pediatric Surgery, 1990
- Pleuroperitoneal shunts in the management of neonatal chylothoraxJournal of Pediatric Surgery, 1983