title: Vascular Anomalies Associated with Esophageal Atresia and Tracheoesophageal Fistula.
abstract: OBJECTIVE
  To report the incidence of congenital vascular anomalies in a cohort of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) while describing the clinical presentation, diagnosis, and consequences, and to evaluate the diagnostic value of esophagram in diagnosing an aberrant right subclavian artery (ARSA).
  
  
  METHODS
  All patients born with EA/TEF between 2005 and 2013 were studied. Preoperative echocardiography reports, surgical descriptions of primary esophageal repair, and esophagrams were reviewed retrospectively.
  
  
  RESULTS
  Of the 76 children born with EA/TEF included in this study, 14 (18%) had a vascular malformation. The incidence of a right aortic arch (RAA) was 6% (5 of 76), and that of an aberrant right subclavian artery (ARSA) was 12% (9 of 76). RAA was diagnosed in the neonatal period by echocardiography (4 of 5) or surgery (1 of 5), and ARSA was diagnosed by echocardiography (7 of 9) or later on the esophagram (2 of 9). Respiratory and/or digestive symptoms occurred in 9 of the 14 patients with vascular malformation. Both long-gap EA and severe cardiac malformations necessitating surgery were significantly associated with vascular anomalies (P<.05). The sensitivity of the esophagram for diagnosing ARSA was 66%, the specificity was 98%, the negative predictive value was 95%, and the positive predictive value was 85%.
  
  
  CONCLUSIONS
  ARSA and RAA have an incidence of 12% and 6% respectively, in patients with EA/TEF. A computed tomography angioscan is recommended to rule out such malformations when stenting of the esophagus is indicated, before esophageal replacement surgery, and when prolonged (>2 weeks) use of a nasogastric tube is considered.
authors.affiliation: Division of Pediatric Gastroenterology Hepatology and Nutrition, Sainte-Justine University Health Centre, Montreal, Québec, Canada.
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Berthet
authors.affiliation: Division of Pediatric Radiology, Sainte-Justine University Health Centre, Montreal, Québec, Canada.
authors.forename: Estelle
authors.initials: E
authors.lastname: Tenisch
authors.affiliation: Division of Pediatric Radiology, Sainte-Justine University Health Centre, Montreal, Québec, Canada.
authors.forename: Marie Claude
authors.initials: MC
authors.lastname: Miron
authors.affiliation: Division of Pediatric Cardiology, Sainte-Justine University Health Centre, Montreal, Québec, Canada.
authors.forename: Nassiba
authors.initials: N
authors.lastname: Alami
authors.affiliation: Division of Surgery, Sainte-Justine University Health Centre, Montreal, Québec, Canada.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Timmons
authors.affiliation: Division of Surgery, Sainte-Justine University Health Centre, Montreal, Québec, Canada.
authors.forename: Ann
authors.initials: A
authors.lastname: Aspirot
authors.affiliation: Division of Pediatric Gastroenterology Hepatology and Nutrition, Sainte-Justine University Health Centre, Montreal, Québec, Canada. Electronic address: christophe.faure@umontreal.ca.
authors.forename: Christophe
authors.initials: C
authors.lastname: Faure
mesh_terms: D001013:Aorta, Thoracic / Q000002:abnormalities; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D004933:Esophageal Atresia / Q000150:complications*; D004947:Esophagus / Q000002:abnormalities; D005260:Female; D006801:Humans; D008297:Male; D011237:Predictive Value of Tests; D015203:Reproducibility of Results; D012189:Retrospective Studies; D012680:Sensitivity and Specificity; D013348:Subclavian Artery / Q000473:pathology; D014138:Tracheoesophageal Fistula / Q000150:complications*; D054079:Vascular Malformations / Q000150:complications*