Pulmonary Lymphatic Disorders

Pulmonary vascular changes induced by congenital obstruction of pulmonary venous return

Masato Endo, Shigeo Yamaki, Mikio Ohmi, Koichi TabayashiTohoku University School of Medicine.Japan Annals of Thoracic SurgeryAnn Thorac Surg 2000; 69: 193-197DOI: 10.1016/s0003-4975(99)01079-6 AbstractBackground: Pulmonary venous obstruction (PVO) induces pulmonary arterial hypertension, as well as pulmonary venous hypertension, and jeopardizes the repair of cardiac lesions.Methods: Four cases of congenital mitral stenosis and 4 cases of cor triatriatum (Lucas type […]

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The Hamman-Rich syndrome in childhood; report of a case with unilateral pulmonary arterial and venous stenosis and atriovenous occlusion

Israel DiamondChildren’s Hospital and University of Louisville School of MedicineUnited States PediatricsPediatrics 1958; 22: 279–288https://doi.org/10.1542/peds.22.2.279 AbstractThe Hamman-Rich syndrome is described in a 4-year-old Negro male. The clinical picture was that of persistent cough and progressive dyspnea beginning at 4 months of age. Diagnosis was made ante mortem by lung biopsy. The fibrotic process and arteriolosclerosis

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Congenital pulmonary lymphangiectasis associated with a blind common pulmonary vein

Arkadi M. Rywlin, Rita M. FojacoUniversity of Miami.United States Pediatrics Pediatrics 1968; 41: 931–934https://doi.org/10.1542/peds.41.5.931 AbstractThree main theories have to be considered in the pathogenesis of congenital pulmonary lymphangectasis: obstruction of major pulmonary lymphatics, obstruction to pulmonary venous flow, and anomalous pulmonary development. The authors report an infant with congenital pulmonary lymphangiectasis associated with a blind common

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Unilateral congenital pulmonary lymphangiectasis presenting with pneumothorax and an NRAS variant

Majid Al Teneiji, Marie-Anne Brundler, Mary Noseworthy, Kyle C. KurekAlberta Children’s Hospital and University of Calgary. Canada Pediatric PulmonologyPediatr Pulmonol 2021; 56: 2374-2376DOI: 10.1002/ppul.25401 AbstractNo Abstract Available CategoryPulmonary Lymphatic DiseaseGenetic Factors Associated with Pulmonary Vascular DiseasePulmonary Vascular Pathology Age Focus: Pediatric Pulmonary Vascular Disease Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication

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A rare case of pulmonary lymphangiectasia associated with CHD

Claudio Henriques, Ana Lai, Helena Andrade, Raquel Pina, Antonio Marinho-da-Silva, Antonio Pires Paediatric Hospital and University Hospital.Portugal Cardiology in the YoungCardiol Young 2022; 32: 132-134DOI: 10.1017/S1047951121002328 AbstractCHD may, at times, occur in the framework of other rare pathologies. These, having similar clinical manifestations, present a diagnostic dilemma for the clinician.The authors present the case of an infant

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[Diffuse pulmonary lymphangiomatosis with pleural and pericardial involvement. Pediatric case report]

Rodolfo P. Moreno, Yanina Hernández, Patricia Garrido, Bethy Camargo Vargas, Alberto Hernández, Javier Faín, Cecilia Seligra, Patricia Topp, Alberto Maffey, Gustavo CardigniSanatorio de la Trinidad Palermo. Argintina Archives of Argentina PediatricsArch Argent Pediatr 2021; 119: e264-e268DOI: 10.5546/aap.2021.e264 AbstractDiffuse pulmonary lymphangiomatosis is a rare disease characterized by marked proliferation and dilation of lymphatic vessels in the lungs,

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MR lymphangiography of lymphatic abnormalities in children and adults with Noonan syndrome

C. C. Pieper, J. Wagenpfeil, A. Henkel, S. Geiger, T. Köster, K. Hoss, J. A. Luetkens, C. Hart, U. I. Attenberger, A. MüllerUniversity of Bonn and Children’s Hospital.Germany Scientific ReportsSci Rep 2022; 12: DOI: 10.1038/s41598-022-13806-w AbstractNoonan syndrome is associated with complex lymphatic abnormalities. We report dynamic-contrast enhanced MR lymphangiography (DCMRL) findings in children and adults with

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Midodrine, an Oral Alpha-1 Adrenoreceptor Agonist, Successfully Treated Refractory Congenital Chylous Pleural Effusion and Ascites in a Neonate

Satoshi Tamaoka, Asami Osada, Takane Kin, Takeshi Arimitsu, Mariko HidaKeio University School of Medicine.Japan ChestChest 2021; 159: e189-e191DOI: 10.1016/j.chest.2020.10.071 AbstractA trisomy 21 neonate presented with congenital chylous pleural effusion and ascites that was refractory to conventional pharmacotherapy. Midodrine, an oral alpha-1-adrenoreceptor agonist, achieved remission of chylous effusion without any adverse effects. To the best of our

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Non-immune hydrops fetalis secondary to congenital chylothorax with diffuse interstitial lung disease: a diagnostic conundrum

Alvin Jia Hao Ngeow, Mei Yoke Chan, Oon Hoe Teoh, Sarat Kumar Sanamandra, Daisy Kwai Lin ChanSingapore General Hospital. KK Women’s and Children’s Hospital. Singapore British Medical Journals Case ReportsBMJ Case Rep 2021; 14: DOI: 10.1136/bcr-2020-240688 AbstractA Chinese male infant was born at 35 weeks weighing 2935 g to a mother with polyhydramnios and prenatal hydrops

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Recurrent prenatal PIEZO1-related lymphatic dysplasia: Expanding molecular and ultrasound findings

Gioia Mastromoro, Daniele Guadagnolo, Antonella Giancotti, Maria Grazia Di Gregorio, Enrica Marchionni, Flaminia Vena, Francesca Romana Lepri, Lavinia Bargiacchi, Flavia Ventriglia, Cira Di Gioia, Antonio Novelli, Antonio PizzutiSapienza University of Rome. Ospedale San Pietro Fatebenefratelli. Bambino Gesù Children’s Hospital. University of Rome.Italy European Journal of Medical Genetics Eur J Med Genet 2021; 64: DOI: 10.1016/j.ejmg.2020.104106 AbstractGeneralized

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