David M Biko1, Breanne Reisen2, Hansel J Otero3, Chitra Ravishankar2, Teresa Victoria3, Andrew C Glatz2, Jonathan J Rome2, Yoav Dori2. 1. Department of Radiology, Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, 3401 Civic Center Blvd., Philadelphia, PA, USA. bikod@email.chop.edu. 2. Division of Cardiology, Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA. 3. Department of Radiology, Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, 3401 Civic Center Blvd., Philadelphia, PA, USA.
Abstract
BACKGROUND: Children with Noonan syndrome are known to have increased risk for lymphatic disorders, the extent and nature of which are poorly understood. OBJECTIVE: Our objective was to describe the imaging findings of the central lymphatic abnormalities in children with Noonan syndrome who underwent central lymphatic imaging. MATERIALS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.
BACKGROUND:Children with Noonan syndrome are known to have increased risk for lymphatic disorders, the extent and nature of which are poorly understood. OBJECTIVE: Our objective was to describe the imaging findings of the central lymphatic abnormalities in children with Noonan syndrome who underwent central lymphatic imaging. MATERIALS AND METHODS: 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. RESULTS: 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. CONCLUSION: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.
Authors: Christian A Barrera; Teresa Victoria; Fernando A Escobar; Ganesh Krishnamurthy; Christopher L Smith; Julie S Moldenhauer; David M Biko Journal: Pediatr Radiol Date: 2020-11-30
Authors: Karen I Ramirez-Suarez; Luis Octavio Tierradentro-García; David M Biko; Hansel J Otero; Ammie M White; Yoav Dori; Christopher L Smith; Seth Vatsky; Jordan B Rapp Journal: Pediatr Radiol Date: 2022-07-16
Authors: Taylor Paul Cox; Christopher James Vance; Sarah K Daley; Cristobal Papendieck; Hugh McGregor; Philip Kuo; Marlys H Witte Journal: J Vasc Surg Venous Lymphat Disord Date: 2022-05-10
Authors: Mandi Liu; Christopher L Smith; David M Biko; Dong Li; Erin Pinto; Nora O'Connor; Cara Skraban; Elaine H Zackai; Hakon Hakonarson; Yoav Dori; Sarah E Sheppard Journal: Eur J Hum Genet Date: 2022-05-24 Impact factor: 5.351
Authors: C C Pieper; J Wagenpfeil; A Henkel; S Geiger; T Köster; K Hoss; J A Luetkens; C Hart; U I Attenberger; A Müller Journal: Sci Rep Date: 2022-07-01 Impact factor: 4.996