Neonatal lymphatic flow disorders: impact of lymphatic imaging and interventions on outcomes

How We Approach: Pediatric Congenital Chylous Effusions and Ascites

“…The illustration depicts select lymphatics of the left lung and small intestine in a healthy neonate (1) thoracic duct (2) left brachiocephalic vein and branches (3) inferior vena cava (4) cysterna chyli. 1b: The illustration depicts select abnormalities of the lymphatic system (5) idiopathic stenosis of the thoracic duct; results in impaired lymphatic drainage, increased trans-pleural filtration pressure (lymphatic hypertension), retrograde lymphatic flow and increased permeability that results in lymphatic leak (6) large region of pulmonary lymphangiomatosis (over proliferation of lymphatic vessels) and lymphangiectasia (inappropriately dilated lymphatic vessels) enveloping the ventral surface of the lung; results in failure of appropriate lymphatic drainage (7) chylothorax collecting in the left pleural cavity (8) chylous ascites collecting in peritoneum causing a distended abdomen. 1.pdf available at https://authorea.com/users/410125/articles/519608-how-we-approachpediatric-congenital-chylous-effusions-and-ascites Hosted file Table 2.pdf available at https://authorea.com/users/410125/articles/519608-how-we-approachpediatric-congenital-chylous-effusions-and-ascites Hosted file Table 3.pdf available at https://authorea.com/users/410125/articles/519608-how-we-approachpediatric-congenital-chylous-effusions-and-ascites…”

“…[5,6] New imaging, surgical and interventional techniques have reduced mortality significantly in certain populations. [7]…”

“…[34] In patients with neonatal chylothorax, Lipiodol ® embolization and low-fat diet has recently been shown as effective treatment strategy. [7] In neonates with multicompartment disorders care should be taken not to occlude the central lymphatic ducts as this can lead to adverse outcomes. More selective and conservative management is favored in the neonatal population with diet and diuretics.…”

Novel Use of a Synovis™ Coupler device for a Surgical Lymphovenous Anastomosis for the treatment of refractory Chylothorax in a patient with Central Lymphatic Flow Disorder.

“…1 Additionally, techniques such as intra-nodal lymphangiography and TD embolization using ethiodized oil (Lipiodol) have further advanced this emerging field but again their utilization, timing, and outcomes remain limited in literature in the management of infants with complex lymphatic disease processes. 1,5,7 Of particular interest are patient's categorized with central lymphatic flow disorder (CLFD) as in the case above. In previous case series, CLFD has a mortality rate greater than 50%.…”

“…In previous case series, CLFD has a mortality rate greater than 50%. 1,5 CLFD has been shown to be difficult to treat with catheter based lymphatic interventions and there are mixed outcomes in improving lymph burden without significant trend towards survival. 1,5 Of the very limited literature and data available it appears that LVA gives these vulnerable patients the best chance of resolution of chylothorax and survival to discharge.…”