Primary Pulmonary Lymphatic Disease

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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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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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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Case report of generalized lymphatic dysplasia with PIEZO1 mutation and review of the literature

Wedad Alhazmia, Afnan Qurbana, Essa AlrashidiMaternity and Children Hospital in Makkah. King Saud Medical City in Riyadh.Saudi Arabia Respiratory Medicine Case ReportsRespir Med Case Rep 2023; 44:DOI: 10.1016/j.rmcr.2023.101872 AbstractBackground: The newborn malformation of primary lymphatic dysplasia (PLD), developed primarily due to PIEZO1 gene autosomal recessive mutation, is known to hinder with the lymphatic system action, causing chyle or lymph

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Successful thoracic duct embolisation in a child with recurrent massive pericardial effusion diagnosed as a lymphatic anomaly

Jue Seong Lee, Mi Kyoung Song, Saebeom HurKorea University College of Medicine and Korea University Medical Center. Seoul National University Children’s Hospital and Seoul National University Hospital.Republic of Korea Cardiology in the YoungCardiol Young 2020; 30: 571-573DOI: 10.1017/S1047951120000323 AbstractA 29-month-old girl had idiopathic massive pericardial effusion for over 6 months. Lymphangiography was performed for chronic and

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A familial case of alveolar capillary dysplasia with misalignment of the pulmonary veins: the clinicopathological features and unusual glomeruloid endothelial proliferation

Akiko Kitano, Masato Nakaguro, Seiichi Tomotaki, Shintaro Hanaoka, Masahiko Kawai, Akiko Saito, Masahiro Hayakawa, Yoshiyuki Takahashi, Hidenori Kawasaki, Takahiro Yamada, Masahiko Ikeda, Tetsuo Onda, Kazutoshi Cho, Hironori Haga, Atsuko Nakazawa, Sachiko MinamiguchiKyoto University and Kyoto University Hospital. Nagoya University Graduate School of Medicine and Nagoya University Hospital. Hokkaido University Hospital. Saitama Children’s Medical Center.Japan Diagnostic

A familial case of alveolar capillary dysplasia with misalignment of the pulmonary veins: the clinicopathological features and unusual glomeruloid endothelial proliferation Read More »

A homozygous variant in growth and differentiation factor 2 (GDF2) may cause lymphatic dysplasia with hydrothorax and nonimmune hydrops fetalis

Sietse M. Aukema, Gerdien A. ten Brinke, Wim Timens, Yvonne J. Vos, Ryan E. Accord, Karianne E. Kraft, Michiel J. Santing, Leonard P. Morssink, Esther Streefland, Cleo C. van Diemen, Elianne JLE Vrijlandt, Christian V. Hulzebos, Wilhelmina S. Kerstjens-FrederikseUniversity of Groningen, University Medical Center Groningen and Beatrix Children’s Hospital. Medical Center Leeuwarden. Netherlands American Journal

A homozygous variant in growth and differentiation factor 2 (GDF2) may cause lymphatic dysplasia with hydrothorax and nonimmune hydrops fetalis Read More »

Abnormal pulmonary lymphatic flow in patients with paediatric pulmonary lymphatic disorders: Diagnosis and treatment

Maxim Itkin, Aaron Chidekel, Kelly A. Ryan, Deborah RabinowitzPerelman School of Medicine at the University of Pennsylvania. Nemours/duPont Hospital for Children. Sidney Kimmel Medical College at Thomas Jefferson University. United States Paediatric Respiratory ReviewsPaediatr Respir Rev 2020; 36: 15-24 DOI: 10.1016/j.prrv.2020.07.001 AbstractPulmonary lymphatic disorders are characterized by the presence of the abnormal lymphatic tissues in the

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A Four-Week-Old Infant With Respiratory Distress: An Emergency Department Case Presentation of Congenital Lobar Emphysema

Kimberly L. Moulton, Andrea FangStanford University.United States CureusCureus 2021; 13: DOI: 10.7759/cureus.13814 AbstractCongenital lobar emphysema (CLE) and congenital pulmonary lymphangiectasis (CPL) are rare conditions that are most often identified with prenatal ultrasonography. Occasionally, this disease process is first identified in the emergency department (ED), where the physician should avoid common pitfalls in order to prevent acute

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Case Report: A Relatively Mild Presentation of Unilateral Congenital Pulmonary Lymphangiectasia

Dionne Adair, Raja Rabah, Maria Ladino-Torres, Thomas G. SabaCS Mott Children’s Hospital and Michigan Medicine.United States Frontiers in PediatricsFront Pediatr 2021; 9: DOI: 10.3389/fped.2021.657473 AbstractPulmonary lymphangiectasia (PL) is a rare congenital disorder of pulmonary lymphatic development. Although it was traditionally a fatal disorder of infancy, some cases in later childhood have been reported, suggesting a spectrum

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