IMAGING OF LYMPHATIC DYSPLASIA IN NOONAN SYNDROME: CASE STUDIES AND HISTORICAL ATLAS

Abstract

To determine the historical use and
utility of various lymphatic imaging
modalities in Noonan syndrome (NS) patients,
we performed a comprehensive literature
review by collecting the published medical
imaging of NS lymphatic dysplasias. We
correlated imaging findings with clinical
phenotypes and treatment. Our analysis of
lymphatic imaging modalities provides an
algorithmic approach to imaging and patient
care across the spectrum of NS developmental
defects. A total of 54 NS cases have been
published since 1975. Using the observations
reported in 15 reviewed publications, an
association was made between disruptions in
central lymphatic flow and poor clinical
presentations/outcomes in NS patients.