title: Esophageal Atresia with or without Tracheoesophageal Fistula (EA/TEF): Association of Different EA/TEF Subtypes with Specific Co-occurring Congenital Anomalies and Implications for Diagnostic Workup.
abstract: BACKGROUND
  Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) represents the most common developmental malformation of the upper digestive tract. It is classified into six subtypes according to the classification of Vogt, depending on anatomical variation of this malformation. Around 50% of the patients with EA/TEF present additional anomalies, which often influence, next to the EA/TEF subtype, the overall prognosis of EA/TEF newborns. Here, we investigated the association of the different EA/TEF subtypes with co-occurring congenital anomalies in EA/TEF patients and demonstrate their implications for postnatal diagnostic workup.
  
  
  METHODS
  We investigated 333 patients of a large German multicenter study born between 1980 and 2012. After evaluation of all available clinical records, 235 patients were included in our analysis. We compared our results with existing data.
  
  
  RESULTS
  The highest risk for co-occurring anomalies was seen in patients with most common Vogt 3b (p= 0.024), especially for additional gastrointestinal anomalies (p= 0.04). Co-occurring anomalies of the skin were significantly more common in patients with subtype Vogt 2 (p= 0.024). A significant correlation was observed for an impaired neurodevelopmental outcome and EA/TEF Vogt 3a (p= 0.041). Patients with EA/TEF showed a higher risk to present with any additional congenital anomaly compared with the general population (p< 0.001).
  
  
  CONCLUSIONS
  Our results warrant thorough clinical workup for gastrointestinal anomalies especially in patients with Vogt 3b. Moreover, it might be necessary to focus on a thorough aftercare for neurocognitive development in patients with Vogt 3a. The here presented observations need to be confirmed by future studies.
authors.affiliation: Department of Neonatology and Pediatric Intensive Care, Children's Hospital, University of Bonn, Bonn, Germany.
authors.forename: Thomas
authors.initials: T
authors.lastname: Bogs
authors.affiliation: Division of Clinical Epidemiology and Aging Research, German Cancer Research Center, Heidelberg, Germany.
authors.forename: Nadine
authors.initials: N
authors.lastname: Zwink
authors.affiliation: Institute of Human Genetics, University of Bonn, Bonn, Germany.
authors.forename: Vera
authors.initials: V
authors.lastname: Chonitzki
authors.affiliation: Department of Pediatric Surgery and Pediatric Urology, Children's Hospital Amsterdamer Strasse, Municipal Hospital of the City of Cologne, Cologne, Germany.
authors.forename: Alice
authors.initials: A
authors.lastname: Hölscher
authors.affiliation: Department of Pediatric Surgery and Pediatric Urology, Children's Hospital Amsterdamer Strasse, Municipal Hospital of the City of Cologne, Cologne, Germany.
authors.forename: Thomas M
authors.initials: TM
authors.lastname: Boemers
authors.affiliation: Department of Pediatric Surgery, University Hospital Mainz, Mainz, Germany.
authors.forename: Oliver
authors.initials: O
authors.lastname: Münsterer
authors.affiliation: Department of Pediatric Surgery, University Hospital Bonn, Bonn, Germany.
authors.forename: Ralf
authors.initials: R
authors.lastname: Kurz
authors.affiliation: Department of Pediatric Surgery, University Hospital Bonn, Bonn, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: Heydweiller
authors.affiliation: Department of Pediatric Surgery, Asklepios Children's Hospital St. Augustin, St. Augustin, Germany.
authors.forename: Marcus
authors.initials: M
authors.lastname: Pauly
authors.affiliation: Department of Pediatric Surgery, Medical Center Dortmund, Dortmund, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: Leutner
authors.affiliation: Center of Pediatric Surgery, Hannover Medical School, Hannover, Germany.
authors.forename: Benno M
authors.initials: BM
authors.lastname: Ure
authors.affiliation: Department of Pediatric Surgery, University of Leipzig, Leipzig, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Lacher
authors.affiliation: Department of Pediatric Surgery, University of Leipzig, Leipzig, Germany.
authors.forename: Oliver Johannes
authors.initials: OJ
authors.lastname: Deffaa
authors.affiliation: Cologne Center for Genomics, University of Cologne, Cologne, Germany.
authors.forename: Holger
authors.initials: H
authors.lastname: Thiele
authors.affiliation: Department of Neonatology and Pediatric Intensive Care, Children's Hospital, University of Bonn, Bonn, Germany.
authors.forename: Soyhan
authors.initials: S
authors.lastname: Bagci
authors.affiliation: Division of Clinical Epidemiology and Aging Research, German Cancer Research Center, Heidelberg, Germany.|Department of Child and Adolescent Psychiatry and Psychotherapy, Johannes-Gutenberg University, Mainz, Germany.|Child Center Maulbronn gGmbH, Hospital for Pediatric Neurology and Social Pediatrics, Maulbronn, Germany.
authors.forename: Ekkehart
authors.initials: E
authors.lastname: Jenetzky
authors.affiliation: Institute of Human Genetics, University of Bonn, Bonn, Germany.
authors.forename: Johannes
authors.initials: J
authors.lastname: Schumacher
authors.affiliation: Department of Neonatology and Pediatric Intensive Care, Children's Hospital, University of Bonn, Bonn, Germany.|Institute of Human Genetics, University of Bonn, Bonn, Germany.
authors.forename: Heiko
authors.initials: H
authors.lastname: Reutter
mesh_terms: D000015:Abnormalities, Multiple / Q000453:epidemiology* / Q000209:etiology; D000293:Adolescent; D000328:Adult; D018376:Cardiovascular Abnormalities / Q000453:epidemiology / Q000209:etiology; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D004065:Digestive System Abnormalities / Q000453:epidemiology / Q000209:etiology; D004933:Esophageal Atresia / Q000145:classification / Q000150:complications / Q000453:epidemiology; D005260:Female; D006801:Humans; D008297:Male; D015995:Prevalence; D012042:Registries; D012189:Retrospective Studies; D014138:Tracheoesophageal Fistula / Q000145:classification / Q000150:complications / Q000453:epidemiology; D014564:Urogenital Abnormalities / Q000453:epidemiology / Q000209:etiology; D055815:Young Adult