title: Surgical Reconstruction for Severe Tracheal Obstruction in Morquio A Syndrome.
abstract: Progressive tracheal obstruction is commonly seen in Morquio A syndrome and can lead to life-threatening complications. Although tracheostomy can address severe upper airway obstruction, lower airway obstruction, commonly associated with a narrow thoracic inlet and vascular compression, requires an alternative approach. We describe the case of a 16-year-old patient with Morquio A syndrome whose near-fatal tracheal obstruction was relieved by timely surgical tracheal vascular reconstruction with dramatic resolution of his respiratory symptoms.
authors.affiliation: Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware. Electronic address: christian.pizarro@nemours.org.
authors.forename: Christian
authors.initials: C
authors.lastname: Pizarro
authors.affiliation: Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware.
authors.forename: Ryan R
authors.initials: RR
authors.lastname: Davies
authors.affiliation: Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware.
authors.forename: Mary
authors.initials: M
authors.lastname: Theroux
authors.affiliation: Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware.
authors.forename: Ellen A
authors.initials: EA
authors.lastname: Spurrier
authors.affiliation: Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware.
authors.forename: Lauren W
authors.initials: LW
authors.lastname: Averill
authors.affiliation: Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware.
authors.forename: Shunji
authors.initials: S
authors.lastname: Tomatsu
mesh_terms: D000293:Adolescent; D000072226:Computed Tomography Angiography / Q000379:methods; D017548:Echocardiography, Transesophageal / Q000379:methods; D015199:Extracorporeal Membrane Oxygenation / Q000379:methods; D005500:Follow-Up Studies; D006801:Humans; D021621:Imaging, Three-Dimensional; D008297:Male; D009085:Mucopolysaccharidosis IV / Q000150:complications* / Q000000981:diagnostic imaging; D035583:Rare Diseases; D019651:Plastic Surgery Procedures / Q000379:methods*; D018570:Risk Assessment; D012720:Severity of Illness Index; D056346:Sternotomy / Q000379:methods; D013524:Surgical Flaps; D014135:Tracheal Stenosis / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery*; D016896:Treatment Outcome