title: [XYY male with right aortic arch and ligamentum arteriosum: a fortuitus association?].
abstract: BACKGROUND
  The XYY male has characteristicaly tall stature, behavior problems and speech delay. There may may be an association with nephro-urologic malformations but cardiovascular anomalies are usually not present.
  
  
  METHODS
  It is reported a case of a boy with a 47 XYY karyotype with persistent respiratory distress and swallowing difficulties since two months old. On diagnosis workup the fiberoptic bronchoscopy showed a significant pulsatil tracheal obstruction at its right lateral wall and the magnetic resonance imaging of the mediastinum comproved the presence of a vascular ring (right aortic arch with left ligamentum arteriosum). The patient was submitted to surgery with improvment in the respiratory symptoms.
  
  
  CONCLUSIONS
  The XYY male is a sex chromosomal abnormality that usually is not associated with cardiovascular malformations. A vascular ring presents with persistent respiratory distress and feeding difficulties; the begining and severity of the symptoms varies with the type of vascular ring. The main procedure to be requested is the fiberoptic bronchoscopy and the best image study is the magnetic resonance imaging of the mediastinum.
authors.affiliation: Serviço de Pediatria, Hospital Dr. Manoel Constâncio, Abrantes.
authors.forename: António
authors.initials: A
authors.lastname: Amador
authors.forename: Isabel
authors.initials: I
authors.lastname: Martins
authors.forename: Rosário
authors.initials: R
authors.lastname: Massa
authors.forename: J
authors.initials: J
authors.lastname: Oliveira Santos
mesh_terms: D000015:Abnormalities, Multiple; D001013:Aorta, Thoracic / Q000002:abnormalities*; D002675:Child, Preschool; D006801:Humans; D008297:Male; D013577:Syndrome; D014997:XYY Karyotype