title: Histologic identification of prominent intrapulmonary anastomotic vessels in severe congenital diaphragmatic hernia.
abstract: OBJECTIVE
  To determine whether prominent intrapulmonary anastomotic vessels (IPAVs) or bronchopulmonary "shunt" vessels can be identified in lungs from infants with fatal congenital diaphragmatic hernia (CDH).
  
  
  METHODS
  We performed histology with immunostaining for CD31 (endothelium) and D2-40 (lymphatics), along with high-precision 3-dimensional (3D) reconstruction on lung tissue from 9 patients who died with CDH.
  
  
  RESULTS
  Each patient with CDH required mechanical ventilation, cardiotonic support, and pulmonary hypertension (PH)-targeted drug therapy. All patients were diagnosed with severe PH by echocardiography, and 5 received extracorporeal membrane oxygenation therapy. Death occurred at a median age of 24 days (range, 10-150 days) from refractory hypoxemia with severe PH, pneumonia, or tension pneumothorax. Histology showed decreased alveolarization with pulmonary vascular disease. In each patient, prominent IPAVs were identified as engorged, thin-walled vessels that connected pulmonary veins with microvessels surrounding pulmonary arteries and airways in lungs ipsilateral and contralateral to the CDH. Prominent anastomoses between pulmonary arteries and bronchial arteries were noted as well. The 3D reconstruction studies demonstrated that IPAVs connect pulmonary vasculature to systemic (bronchial) vessels both at the arterial and venous side.
  
  
  CONCLUSIONS
  Histology and 3D reconstruction identified prominent bronchopulmonary vascular anastamoses in the lungs of infants who died with severe CDH. We speculate that IPAVs connecting pulmonary and bronchial arteries contribute to refractory hypoxemia in severe CDH.
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO; Pediatric Heart-Lung Center, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO. Electronic address: Shannon.acker@ucdenver.edu.
authors.forename: Shannon N
authors.initials: SN
authors.lastname: Acker
authors.affiliation: Pediatric Heart-Lung Center, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO; Section of Neonatology, Department of Pediatrics, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO.
authors.forename: Erica W
authors.initials: EW
authors.lastname: Mandell
authors.affiliation: Division of Nephrology, Department of Pediatrics, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA.
authors.forename: Sunder
authors.initials: S
authors.lastname: Sims-Lucas
authors.affiliation: Pediatric Heart-Lung Center, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO; Section of Neonatology, Department of Pediatrics, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO.
authors.forename: Jason
authors.initials: J
authors.lastname: Gien
authors.affiliation: Pediatric Heart-Lung Center, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO; Section of Pulmonary Medicine, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO.
authors.forename: Steven H
authors.initials: SH
authors.lastname: Abman
authors.affiliation: Pediatric Heart-Lung Center, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO; Section of Pathology, Department of Pediatrics, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO.
authors.forename: Csaba
authors.initials: C
authors.lastname: Galambos
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000378:metabolism*; D001164:Arteriovenous Fistula / Q000175:diagnosis* / Q000378:metabolism; D005260:Female; D065630:Hernias, Diaphragmatic, Congenital / Q000175:diagnosis* / Q000378:metabolism / Q000401:mortality; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis; D007223:Infant; D007231:Infant, Newborn; D008168:Lung / Q000098:blood supply*; D008297:Male; D019408:Platelet Endothelial Cell Adhesion Molecule-1 / Q000378:metabolism*; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000473:pathology; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000473:pathology
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D019408:Platelet Endothelial Cell Adhesion Molecule-1; C500987:monoclonal antibody D2-40