Acinar dysplasia congenital alveolar dysplasia and alveolar capillary dysplasia with misalignment of pulmonary veins belong to the diffuse developmental disorders (congenital lung dysplasia), very rare fatal disorders of infancy that occur early in lung development. A case of quickly fatal congenital lung dysplasia in a full-term infant is presented and underlines the necessity to suspect this disease in a newborn suffering from severe and refractory respiratory distress.
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http://dx.doi.org/10.3109/15513815.2013.878009 | DOI Listing |
Stem Cells Transl Med
January 2025
Developmental and Stem Cell Biology Program, Peter Gilgan Centre for Research and Learning, The Hospital for Sick Children, Toronto, ON, Canada M5G 0A4.
Disruption of developmental processes affecting the fetal lung leads to pulmonary hypoplasia. Pulmonary hypoplasia results from several conditions including congenital diaphragmatic hernia (CDH) and oligohydramnios. Both entities have high morbidity and mortality, and no effective therapy that fully restores normal lung development.
View Article and Find Full Text PDFPort J Card Thorac Vasc Surg
October 2024
Thoracic Surgery Department - Hospital de Santa Marta, Centro Hospitalar Universitário Lisboa Central, Portugal.
Kartagener syndrome (KS) is a rare congenital disorder, characterized by sinusitis, bronchiectasis and situs inversus. Lung transplantation is an effective treatment for end-stage lung failure, but dextrocardia and differences between hilar structures and pulmonary lobes require adjustments to conventional surgical technique. We present a case of a double-lung transplant without extracorporeal oxygenation in a 48-year-old male patient with KS.
View Article and Find Full Text PDFPediatr Surg Int
January 2025
Division of Neonatology, The Hospital for Sick Children, Toronto, ON, Canada.
Introduction: Congenital diaphragmatic hernia (CDH) in the preterm population is increasingly common in the current era of fetal endoluminal tracheal occlusion (FETO) therapy. There remains a lack of clinical guidance for clinicians and surgeons regarding optimal management strategies for such infants. We aimed to describe our experience in managing preterm CDH in a single quaternary neonatal intensive care unit (NICU).
View Article and Find Full Text PDFJ Cardiothorac Surg
January 2025
Department of Thoracic and Vascular Surgery, and Lung Transplantation, Marie-Lannelongue Hospital, Le Plessis-Robinson, France.
Background: Post-pneumonectomy bronchopleural fistula (BPF) is a life-threatening event whose treatment is not standardized.
Case Presentation: We report the management of a 28-year-old patient with a 3-year history of BPF complicating right pneumonectomy for congenital emphysema. Despite closure by an Amplatzer device, the patient had chronic pyothorax and severely deteriorated general health and quality of life.
Background: Pulmonary vein stenosis in children is associated with a poor prognosis. However, the cause and risk factors for mortality remain uncertain.
Methods: This retrospective, single-center study identified children with primary and secondary pulmonary vein stenosis through a cardiac catheterization database.
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