title: Direct Tracheobronchopexy and Posterior Descending Aortopexy for Severe Left Mainstem Bronchomalacia Associated With Congenital Pulmonary Airway Malformation and Left Circumflex Aortic Arch.
abstract: Complex tracheobronchial obstruction and malacia can be associated with conotruncal and aortic arch anomalies. A circumflex aortic arch composed of a left aortic arch and right descending aorta is an extremely rare anomaly that can severely affect the distal trachea and mainstem bronchi, resulting in severe respiratory symptoms. We report the case of a patient with circumflex aortic arch and severe left mainstem bronchial obstruction and malacia in which the external aortic compression and residual bronchomalacia were addressed with direct bronchial and tracheal intervention.
authors.affiliation: Department of Cardiac Surgery, Boston Children's Hospital & Harvard Medical School, Boston, Massachusetts. Electronic address: christopher.baird@cardio.chboston.org.
authors.forename: Christopher W
authors.initials: CW
authors.lastname: Baird
authors.affiliation: Department of Radiology, Boston Children's Hospital & Harvard Medical School, Boston, Massachusetts.
authors.forename: Sanjay
authors.initials: S
authors.lastname: Prabhu
authors.affiliation: Department of General Surgery, Boston Children's Hospital & Harvard Medical School, Boston, Massachusetts.
authors.forename: Terry L
authors.initials: TL
authors.lastname: Buchmiller
authors.affiliation: Department of General Surgery, Boston Children's Hospital & Harvard Medical School, Boston, Massachusetts.
authors.forename: Charles
authors.initials: C
authors.lastname: Smithers
authors.affiliation: Department of General Surgery, Boston Children's Hospital & Harvard Medical School, Boston, Massachusetts.
authors.forename: Russell
authors.initials: R
authors.lastname: Jennings
mesh_terms: D001013:Aorta, Thoracic / Q000002:abnormalities* / Q000601:surgery*; D001980:Bronchi / Q000002:abnormalities / Q000601:surgery*; D001982:Bronchial Diseases / Q000601:surgery; D055091:Bronchomalacia / Q000601:surgery*; D001999:Bronchoscopy; D003251:Constriction, Pathologic / Q000601:surgery; D005260:Female; D006801:Humans; D007223:Infant; D013902:Radiography, Thoracic; D014057:Tomography, X-Ray Computed; D014132:Trachea / Q000002:abnormalities / Q000601:surgery*; D014135:Tracheal Stenosis / Q000601:surgery