Klin Padiatr 2020; 232(04): 178-186
DOI: 10.1055/a-1149-9483
Diagnostic and Treatment Recommendation

Behandlung der Ösophagusatresie mit unterer tracheoösophagealer Fistel – Zusammenfassung der aktuellen S2K-Leitlinie der DGKCH

Current Treatment of Esophageal Atresia with Tracheoesophageal Fistula – Updated Guidelines of the German Society of Pediatric Surgery
Steffi Mayer
1   Department of Pediatric Surgery, University Hospital Leipzig, Leipzig
,
Heidrun Gitter
2   Department of Pediatric Surgery and Urology, Klinikum Bremen-Mitte, Children's Hospital, Bremen
,
Peter Göbel
3   Department of Pediatric Surgery and Pediatric Urology, Elisatbeth Hospital, Halle (Saale)
,
Franz Wolfgang Hirsch
4   Department of Pediatric Radiology, University Hospital Leipzig, Leipzig
,
Claudia Höhne
5   Department of Anesthesiology, Intensive Medicine and Pain Medicine, DRK Hospitals Berlin Koepenick, Berlin
,
Stuart Hosie
6   Department of Pediatric Surgery, München Klinik gGmbH, Munich
,
Jochen Hubertus
7   Department of Pediatric Surgery, Munich University Hospital Dr von Hauner Children’s Hospital, Munich
,
Andreas Leutner
8   Department of Pediatric Surgery, Medical Center Dortmund, Dortmund
,
Oliver Muensterer
9   Department of Pediatric Surgery, Johannes Gutenberg University Hospital Mainz, Mainz
,
Peter Schmittenbecher
10   Department of Pediatric Surgery, Klinikum Karlsruhe, Karlsruhe
,
Elias Seidl
11   Department of Pediatric Pneumology, Dr von Hauner Children's Hospital, Ludwig Maximilians University Munich, Munich
,
Holger Stepan
12   Department of Obstetrics, University Hospital Leipzig, Leipzig
,
Ulrich Thome
13   Divsion of Neonatology, University Hospital Leipzig, Leipzig
,
Holger Till
14   Department of Pediatric and Adolescent Surgery, Medical University of Graz, Graz, Austria
,
Anke Widenmann-Grolig
15   The Federation of Esophageal Atresia and Tracheo-Esophageal Fistula Support Groups, KEKS e.V., Stuttgart
,
Martin Lacher
1   Department of Pediatric Surgery, University Hospital Leipzig, Leipzig
› Author Affiliations

Zusammenfassung

Die Ösophagusatresie (ÖA) ist eine angeborene Kontinuitätsunterbrechung der Speiseröhre mit oder ohne Fistel zur Trachea (Tracheoösophageale Fistel, TÖF). Je nach Abstand zwischen den beiden Ösophagusenden unterscheidet man eine „kurzstreckige“ von einer „langstreckigen“ Form. Bis zu 50% der Neugeborenen weisen weitere Anomalien auf. Eine pränatale Diagnose der ÖA gelingt in 32–63% der Fälle. Die interdisziplinäre Betreuung dieser Kinder hat sich in den letzten Jahren gewandelt. In der folgenden Arbeit wird die aktuelle S2K-Leitlinie der Deutschen Gesellschaft für Kinderchirurgie zur Behandlung der ÖA mit unterer TÖF, die etwa 90% aller Fälle ausmacht, zusammengefasst. Hierzu gehören das präoperative Management sowie operative und anästhesiologische Aspekte (u. a. Thorakoskopie vs. Thorakotomie, präoperative Tracheobronchoskopie, intraoperative Hyperkapnie und Azidose). Ferner wird das postoperative Management insbesondere von relevanten Komplikationen wie der Anastomosenstenose beschrieben. Trotz Fortschritten in der Behandlung der ÖA ist die Langzeitmorbidität mit Motilitätsstörungen des Ösophagus, gastroösophagealer Refluxkrankheit, rezidivierenden Infektionen der oberen und unteren Atemwege, Tracheomalazie, mangelhaftem Gedeihen sowie orthopädischen Problemen nach Thorakotomie weiterhin hoch. Zum Erreichen einer guten Lebensqualität ist daher eine gute interdisziplinäre Nachsorge wichtig.

Abstract

Esophageal atresia (EA) is a congenital anomaly that entails an interrupted esophagus with or without tracheoesophageal fistula (TEF). Depending on the distance of the two esophageal pouches a “short-gap” is distinguished from a „long-gap“ variant. Up to 50% of newborns have additional anomalies. EA is prenatally diagnosed in 32–63% of cases. Recently, the interdisciplinary care in these children underwent substantial changes. Therefore, we summarize the current guideline of the German society of pediatric surgery for the treatment of patients with EA and distal TEF (Gross Type C). Controversies regarding the perioperative management include surgical-technical aspects, such as the thoracoscopic approach to EA, as well as general anesthesia (preoperative tracheobronchoscopy, intraoperative hypercapnia and acidosis). Moreover, postoperative complications and their management like anastomotic stricture are outlined. Despite significant improvements in the treatment of EA, there is still a relevant amount of long-term morbidity after surgical correction. This includes dysmotility of the esophagus, gastroesophageal reflux disease, recurrent respiratory infections, tracheomalacia, failure to thrive, and orthopedic complications following thoracotomy in the neonatal age. Therefore, close follow-up is mandatory to attain optimal quality of life.



Publication History

Article published online:
26 June 2020

© Georg Thieme Verlag KG
Stuttgart · New York

 
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