ArticleScientific reports2022
MR lymphangiography of lymphatic abnormalities in children and adults with Noonan syndrome.
Article in Scientific reports, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 33 citations in OpenAlex.
- Lymphatic Imaging and Interventions in Children: Current Practice.Cardiovascular and interventional radiology · 2026Review
- Impact of fat content of oral nutritional intake on visibility and diameter of the thoracic duct: a prospective study.Scientific reports · 2026Article
- SHOC2 Is a Novel Cause of Central Conducting Lymphatic Anomaly.American journal of medical genetics. Part A · 2026Article
- Lymphedema imaging and AI: A review of diagnostic modalities, biomarkers, and clinical integration.Biomedical journal · 2026Review
- Imaging of complex lymphatic anomalies in children.Pediatric radiology · 2026Review
- The Nutmeg Lung Pattern in a Fetus with Hypoplastic Left Heart Syndrome and Turner Syndrome.Pediatric cardiology · 2026Article
- Article
- Effects of MEK-inhibitor treatment in infants with lymphatic abnormalities in noonan syndrome.Frontiers in pediatrics · 2026Article
- Cardiovascular aspects of Noonan syndrome and related disorders.Medizinische Genetik : Mitteilungsblatt des Berufsverbandes Medizinische Genetik e.V · 2025Article
- MR Lymphangiography in Pediatrics: Indications, Technique, Findings, and Management.Seminars in interventional radiology · 2025Review
- The application of non-enhanced magnetic resonance thoracic ductography combined with magnetic resonance abdominopelvic scanning in the diagnosis of chylous leakage of the female reproductive system.BMC medical imaging · 2025Article
- [The lymphatic system-an interdisciplinary challenge].Radiologie (Heidelberg, Germany) · 2025Review
- [Imaging of the lymphatic vascular system-techniques].Radiologie (Heidelberg, Germany) · 2025Article
- [Nodal x-ray lymphangiography and therapy of central lymphatic leakage].Radiologie (Heidelberg, Germany) · 2025Article
- [Lymphatic malformations in childhood and adolescence].Radiologie (Heidelberg, Germany) · 2025Article
- [Lymphatic vessel imaging-Indications and clinical applications].Radiologie (Heidelberg, Germany) · 2025Review
- Health outcomes and drug utilisation in children with Noonan syndrome: a European cohort study.Orphanet journal of rare diseases · 2025Article
- Lymphatic System Development and Function.Current cardiology reports · 2024Review
- The utility of dynamic contrast-enhanced intranodal magnetic resonance lymphangiography (MRL) in the investigation of primary lymphatic anomalies.Clinical radiology · 2024Article
- Lymphatic failure and lymphatic interventions: Knowledge gaps and future directions for a new frontier in congenital heart disease.Seminars in pediatric surgery · 2024Review
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Authors and funding
10 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Noonan syndrome is associated with complex lymphatic abnormalities. We report dynamic-contrast enhanced MR lymphangiography (DCMRL) findings in children and adults with Noonan syndrome to further elucidate this complex disease spectrum. A retrospective evaluation of patients with confirmed Noonan syndrome and clinical signs of lymphatic dysfunction undergoing DCMRL between 01/2019 and 04/2021 was performed. MRL included T2-weighted imaging (T2w) and DCMRL. Clinical history/presentation and genetic variants were recorded. T2w-imaging was evaluated for central lymphatic abnormalities and edema distribution. DCMRL was evaluated regarding the presence of cisterna chyli/thoracic duct, lymphatic leakages, pathological lymphatic reflux and abnormal lymphatic perfusion. The time from start of contrast-injection to initial enhancement of the thoracic duct venous junction was measured to calculate the speed of contrast propagation. Eleven patients with Noonan syndrome with lymphatic abnormalities (5 female, 6 male; 7 infants, 4 adults; mean age 10.8 ± 16.4 years) were identified (PTPN11 n = 5/11 [45.5%], RIT1 n = 5/11 [45.5%], KRAS n = 1/11 [9%]). Patients had a chylothorax (n = 10/11 [91%]) and/or pulmonary lymphangiectasia [dilated pulmonary lymph vessels] (n = 9/11 [82%]). Mediastinal/pulmonary edema was depicted in 9/11 (82%) patients. The thoracic duct (TD) was (partially) absent in 10/11 (91%) cases. DCMRL showed lymphatic reflux into intercostal (n = 11/11 [100%]), mediastinal (n = 9/11 [82%]), peribronchial (n = 8/11 [73%]), peripheral (n = 5/11 [45.5%]) and genital lymphatics (n = 4/11 [36%]). Abnormal pulmonary/pleural lymphatic perfusion was seen in 8/11 patients (73%). At infancy peripheral/genital edema was more prevalent in patients with RIT1 than PTPN11 (n = 3/5 vs. n = 0/5). Compared to patients with PTPN11 who had fast lymphatic enhancement in 4/5 patients, enhancement took markedly longer in 4/5 patients with RIT1-mutations. Thoracic duct dysplasia, intercostal reflux and pulmonary/pleural lymphatic perfusion are characteristic findings in patients with Noonan syndrome presenting with chylothorax and/or pulmonary lymphangiectasia. Central lymphatic flow abnormalities show possible phenotypical differences between PTPN11 and RIT1-mutations.
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