Pulmonary Lymphatic Disorders Archives

Trametinib as a targeted treatment in cardiac and lymphatic presentations of Noonan syndrome

Isabel De Brouchoven, Juan Lorand, Léon Bofferding, Arthur Sorlin, An Van Damme, Olivier DanhaiveSaint-Luc University Hospital,. Luxembourg Hospital Center. Laboratoire National de Santé. University of California San Francisco.Belgium, Luxembourg and United States Frontiers in PediatricsFront Pediatr 2025; 13: DOI: 10.3389/fped.2025.1475143 AbstractIntroduction: Rare pathogenic variants in the PTPN11, KRAS, SOS1 and RAF1 genes are the main molecular causes of Noonan syndrome (NS). […]

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The Usefulness of Intraoperative Indocyanine Green Fluorescence Imaging in Surgical Treatment of Refractory Chylothorax in Pediatric Patients: A Case Report

Kanji Ishizu, Kanta Araki, Koji Kagisaki, Hideto OzawaOsaka City General Hospital.Japan Surgical Case Reports Surg Case Rep 2025; DOI: 10.70352/scrj.cr.24-0112 AbstractIntroduction: Chylothorax is one of the complications in cardiovascular surgery. Although prolonged chylothorax leads to critical status and is associated with high mortality, its treatment has not been well established. We present a successful case of surgical

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Refractory Chylothorax and Ventricular Hypertrophy Treated with Trametinib in a Patient with Noonan Syndrome: 18-Month Follow-Up

Antonia Pascarella, Giuseppe Limongelli, Alessandro De Falco, Elia Marco Paolo Minale, Giangiacomo Di Nardo, Giovanni Maria Di Marco, Geremia Zito Marinosci, Giorgia Olimpico, Paolo Siani, Daniele De BrasiSantobono-Pausilipon Children’s Hospital. University of Campania “Luigi Vanvitelli” and Monaldi Hospital. University “Federico II”. Italy ChildrenChildren 2024; 11: DOI: 10.3390/children11111342 AbstractRASopathies are a group of genetic syndromes caused by

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MR Lymphangiography: Congenital Lymphatic Flow Disorders

Seunghyun Lee, Saebeom Hur, Young Hun Choi, Jae-Yeon Hwang, Jung-Eun CheonSeoul National University Hospital and College of Medicine.Republic of Korea Investigative RadiologyInvest Radiol 2024; DOI: 10.1097/RLI.0000000000001112 AbstractCongenital lymphatic flow disorders collectively refer to a heterogeneous group of diseases that manifest as chylothorax, chylous ascites, intestinal lymphangiectasia, protein-losing enteropathy, and peripheral extremity or genital lymphedema, all in

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Lymphatic Imaging and Intervention in Congenital Heart Disease

Christoph Bauer, Mario Scala, Jonathan J. Rome, Gerald Tulzer, Yoav DoriKepler University Hospital and Johannes Kepler University Linz. Children’s Hospital of Philadelphia.Austria and United States Journal of the Society for Cardiovascular Angiography and InterventionsJ Soc Cardiovasc Angiogr Interv 2024; 3:DOI: 10.1016/j.jscai.2023.101174 AbstractThe lymphatic system plays a central role in some of the most devastating complications associated

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Pediatric Chylothorax Treated With Lymphangiography and Thoracic Duct Embolization: A Case Report

Harneet Sangha, Nathan G. Savolainen, Douglas A. Murrey, Jason S. VergnaniElson S. Floyd College of Medicine. Inland Imaging.United States CureusCureus 2024; 16: DOI: 10.7759/cureus.63981 AbstractA chylothorax, the accumulation of lymphatic fluid in the pleural space, may occur for a variety of reasons. It is commonly seen in adults post-thoracic surgery. We present the case of a

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Neonatal neuroblastoma presenting as recurrent chylothorax in a hydropic baby: A case report

Daniah S. Alhazmi, Maha BamehrezKing Faisal Specialist Hospital and Research Center.Saudi Arabia Journal of Family Medicine and Primary CareJ Family Med Prim Care 2024; 13: 2780-2783DOI: 10.4103/jfmpc.jfmpc_992_23 AbstractWe report here a rare case of neuroblastoma in a neonate who presented as a hydropic baby with respiratory distress due to recurrent chylothorax. The neuroblastoma regressed after six

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Trametinib restores the central conducting lymphatic flow in a premature infant with Noonan syndrome

Erika K. S. M. Leenders, Lotte E. R. Kleimeier, Lauren C. Weeke, Catelijne H. Coppens, Willemijn M. Klein, Jos M.T. DraaismaRadboud University Medical Center. Amalia Children’s Hospital.Netherlands Clinical Case ReportsClin Case Rep 2024; 12: DOI: 10.1002/ccr3.9164 AbstractWe describe a premature hydropic infant with Noonan syndrome and a therapy refractory chylothorax. This was shown to be due to a central conducting lymphatic anomaly.

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Successful Treatment of Congenital Chylothorax by Early Use of Prednisolone: A Case Report

Kyoka Hirano, Koji Nakae, Manaka Matsunaga, Kentaro Ueno, Yasuhiro OkamotoKagoshima University Hospital.Japan CureusCureus 2024; 16:DOI: 10.7759/cureus.60628 AbstractCongenital chylothorax is the most common form of pleural effusion during the neonatal period; however, no treatment strategy exists. The pathogenesis and etiology of this disease are not fully understood; hence, several cases are difficult to treat. Some patients with

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Neonatal perspective on central lymphatic disorders

Brian M. Dulmovits, Dalal K. TahaChildren’s Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania.United States Seminars in Pediatric SurgerySemin Pediatr Surg 2024; 33:DOI: 10.1016/j.sempedsurg.2024.151424 AbstractLymphatic disorders presenting in the first year of life are difficult to identify and manage given the broad range of underlying etiologies. Neonatal lymphatic disease arising from

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