title: A novel treatment approach to H-type esophageal lung in presence of pulmonary artery sling with pneumonectomy and intrathoracic tissue-expander placement.
abstract: Congenital tracheal anomalies occur in 1:10,000 births and can be associated with congenital cardiac disease. This patient presented with right mainstem atresia, right bronchoesophageal fistula without esophageal atresia (H-type esophageal lung), and left pulmonary arterial (PA) sling. Taking this into consideration, surgical management included right pneumonectomy and placement of expandable prosthesis into the thoracic cavity to buttress the mediastinum and prevent tracheal deviation-induced kinking of the PA sling, which provided pulmonary blood flow to the remaining functional lung. To our knowledge, this is the first reported case of esophageal lung in conjunction with a PA sling, as well as the first documented use of an expandable prosthesis to prevent acute postpneumonectomy syndrome. This case can provide clinicians with increased clinical knowledge for treatment of this rare and potentially lethal combination of congenital anomalies.
authors.affiliation: Division of Pediatric Surgery, North Carolina Children's Hospital, The University of North Carolina at Chapel Hill, Chapel Hill, NC.
authors.forename: Michael R
authors.initials: MR
authors.lastname: Phillips
authors.affiliation: Division of Pediatric Surgery, North Carolina Children's Hospital, The University of North Carolina at Chapel Hill, Chapel Hill, NC.
authors.forename: Jeff J
authors.initials: JJ
authors.lastname: Dehmer
authors.affiliation: Division of Pediatric Surgery, North Carolina Children's Hospital, The University of North Carolina at Chapel Hill, Chapel Hill, NC.
authors.forename: Timothy M
authors.initials: TM
authors.lastname: Weiner
authors.affiliation: Division of Pediatric Surgery, North Carolina Children's Hospital, The University of North Carolina at Chapel Hill, Chapel Hill, NC. Electronic address: sang_lee@med.unc.edu.
authors.forename: Sang
authors.initials: S
authors.lastname: Lee
mesh_terms: D001983:Bronchial Fistula / Q000601:surgery*; D004937:Esophageal Fistula / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D011013:Pneumonectomy / Q000379:methods*; D019736:Prostheses and Implants; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000601:surgery; D035423:Thoracic Cavity / Q000601:surgery*; D019616:Thoracic Surgical Procedures / Q000295:instrumentation / Q000379:methods*; D016896:Treatment Outcome