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Published on: March 8, 2013
Imaging of central lymphatic abnormalities in Noonan syndrome
David M Biko1, Breanne Reisen2, Hansel J Otero3
1Department 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.
Insights
Children with Noonan syndrome often have lymphatic disorders. Imaging reveals central lymphatic abnormalities including abnormal thoracic ducts and retrograde flow, impacting lung perfusion.
Area of Science:
- Pediatric Cardiology
- Radiology
- Genetics
Background:
- Noonan syndrome is associated with an increased risk of lymphatic disorders.
- The specific nature and extent of these lymphatic issues in children are not well understood.
Purpose of the Study:
- To characterize the central lymphatic abnormalities in children with Noonan syndrome using advanced imaging techniques.
- To correlate imaging findings with clinical presentation and outcomes.
Main Methods:
- Retrospective review of 10 children with Noonan syndrome undergoing lymphatic imaging over 5 years.
- Utilized T2-weighted imaging, dynamic-contrast MR lymphangiography, and conventional lymphangiography.
- Consensus review by two readers for fluid distribution, lymphatic flow, and thoracic duct abnormalities.
Main Results:
- All patients with T2-W imaging showed pleural effusions.
- Imaging demonstrated pulmonary lymphatic perfusion and retrograde intercostal flow in most patients.
- Thoracic duct abnormalities were common, including absence or duplication.
Conclusions:
- Children with Noonan syndrome and lymphatic dysfunction exhibit distinct central lymphatic abnormalities.
- Findings include retrograde intercostal flow, pulmonary lymphatic perfusion, and thoracic duct anomalies.
- These abnormalities are key to understanding lymphatic dysfunction in Noonan syndrome.
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.
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