Eur J Pediatr Surg 2018; 28(02): 176-182
DOI: 10.1055/s-0036-1597946
Original Article
Georg Thieme Verlag KG Stuttgart · New York

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

Thomas Bogs
1  Department of Neonatology and Pediatric Intensive Care, Children's Hospital, University of Bonn, Bonn, Germany
,
Nadine Zwink
2  Division of Clinical Epidemiology and Aging Research, German Cancer Research Center, Heidelberg, Germany
,
Vera Chonitzki
3  Institute of Human Genetics, University of Bonn, Bonn, Germany
,
Alice Hölscher
4  Department of Pediatric Surgery and Pediatric Urology, Children's Hospital Amsterdamer Strasse, Municipal Hospital of the City of Cologne, Cologne, Germany
,
Thomas M. Boemers
4  Department of Pediatric Surgery and Pediatric Urology, Children's Hospital Amsterdamer Strasse, Municipal Hospital of the City of Cologne, Cologne, Germany
,
Oliver Münsterer
5  Department of Pediatric Surgery, University Hospital Mainz, Mainz, Germany
,
Ralf Kurz
6  Department of Pediatric Surgery, University Hospital Bonn, Bonn, Germany
,
Andreas Heydweiller
6  Department of Pediatric Surgery, University Hospital Bonn, Bonn, Germany
,
Marcus Pauly
7  Department of Pediatric Surgery, Asklepios Children's Hospital St. Augustin, St. Augustin, Germany
,
Andreas Leutner
8  Department of Pediatric Surgery, Medical Center Dortmund, Dortmund, Germany
,
Benno M. Ure
9  Center of Pediatric Surgery, Hannover Medical School, Hannover, Germany
,
Martin Lacher
10  Department of Pediatric Surgery, University of Leipzig, Leipzig, Germany
,
Oliver Johannes Deffaa
10  Department of Pediatric Surgery, University of Leipzig, Leipzig, Germany
,
Holger Thiele
11  Cologne Center for Genomics, University of Cologne, Cologne, Germany
,
Soyhan Bagci
1  Department of Neonatology and Pediatric Intensive Care, Children's Hospital, University of Bonn, Bonn, Germany
,
Ekkehart Jenetzky
2  Division of Clinical Epidemiology and Aging Research, German Cancer Research Center, Heidelberg, Germany
12  Department of Child and Adolescent Psychiatry and Psychotherapy, Johannes-Gutenberg University, Mainz, Germany
13  Child Center Maulbronn gGmbH, Hospital for Pediatric Neurology and Social Pediatrics, Maulbronn, Germany
,
Johannes Schumacher
3  Institute of Human Genetics, University of Bonn, Bonn, Germany
,
Heiko Reutter
1  Department of Neonatology and Pediatric Intensive Care, Children's Hospital, University of Bonn, Bonn, Germany
3  Institute of Human Genetics, University of Bonn, Bonn, Germany
› Author Affiliations
Further Information

Publication History

03 August 2016

30 November 2016

Publication Date:
06 January 2017 (eFirst)

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.

Materials and 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).

Conclusion 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.