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Imaging of central lymphatic abnormalities in Noonan syndrome.

Authors :
Biko, David M.
Reisen, Breanne
Otero, Hansel J.
Ravishankar, Chitra
Victoria, Teresa
Glatz, Andrew C.
Rome, Jonathan J.
Dori, Yoav
Source :
Pediatric Radiology. May2019, Vol. 49 Issue 5, p586-592. 7p. 1 Color Photograph, 2 Black and White Photographs, 2 Charts.
Publication Year :
2019

Abstract

<bold>Background: </bold>Children with Noonan syndrome are known to have increased risk for lymphatic disorders, the extent and nature of which are poorly understood.<bold>Objective: </bold>Our objective was to describe the imaging findings of the central lymphatic abnormalities in children with Noonan syndrome who underwent central lymphatic imaging.<bold>Materials and Methods: </bold>We conducted a single-center retrospective review of all children with a confirmed history of Noonan syndrome who presented for lymphatic imaging over a 5-year period. Imaging evaluation was performed on unenhanced T2-weighted (T2-W) imaging, dynamic-contrast MR lymphangiography or conventional lymphangiography. Two readers evaluated the imaging in consensus for the distribution of fluid on T2-W imaging and for lymphatic flow of intranodal contrast agent and thoracic duct abnormalities on dynamic-contrast MR lymphangiography and conventional lymphangiography. We performed a chart review for clinical history and outcomes.<bold>Results: </bold>We identified a total of 10 children, all but one of whom had congenital heart disease. Presenting symptoms included chylothorax (n=9) and ascites (n=1). Nine had T2-W imaging, seven had dynamic-contrast MR lymphangiography, and seven had conventional lymphangiography. All with T2-W imaging had pleural effusions. On both dynamic-contrast MR lymphangiography and conventional lymphangiography, perfusion to the lung was seen (n=6), with intercostal flow also seen on dynamic-contrast MR lymphangiography (n=6). The thoracic duct was not present in three children and the central thoracic duct was not present in three. A double thoracic duct was seen in two children.<bold>Conclusion: </bold>Children with Noonan syndrome and clinical evidence of lymphatic dysfunction have central lymphatic abnormalities characterized by retrograde intercostal flow, pulmonary lymphatic perfusion, and thoracic duct abnormalities. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
03010449
Volume :
49
Issue :
5
Database :
Academic Search Index
Journal :
Pediatric Radiology
Publication Type :
Academic Journal
Accession number :
135841700
Full Text :
https://doi.org/10.1007/s00247-018-04337-6