Die Ösophagusatresie – von der Herausforderung in der Pränatalmedizin bis zur chirurgischen Versorgung

Stanisław Jurk; Martin Lacher; Carsten Springer

doi:10.1055/a-0807-7963

Zeitschrift für Geburtshilfe und Neonatologie, Table of Contents

Z Geburtshilfe Neonatol 2019; 223(01): 15-25
DOI: 10.1055/a-0807-7963

Übersicht

Die Ösophagusatresie – von der Herausforderung in der Pränatalmedizin bis zur chirurgischen Versorgung

Oesophageal Atresia – from the Challenge in Prenatal Medicine until Surgical Care

Authors

Stanisław Jurk

¹Geburtshilfe und Pränatale Diagnostik, St. Elisabeth-Krankenhaus Leipzig, Leipzig
Martin Lacher

²Klinik für Kinderchirurgie, Uniklinik Leipzig, Leipzig
Carsten Springer

¹Geburtshilfe und Pränatale Diagnostik, St. Elisabeth-Krankenhaus Leipzig, Leipzig

Abstract

Zusammenfassung

Bei der Ösophagusatresie kommt es zu einer Hemmungsfehlbildung der Speiseröhre mit oder ohne Verbindung zur angrenzenden Trachea. Eine pränatale Diagnostik mittels Ultraschalluntersuchungen ist durch die indirekten Hinweiszeichen relativ unspezifisch und nur als eine Verdachtsdiagnose möglich. Neben dem gleichzeitigen Vorliegen eines Polyhydramnions sowie der wiederholt nicht möglichen Darstellung einer Magenblase, bzw. zu kleiner Magenblase, bestärkt das Vorliegen eines sog. „Pouch-sign“ die Verdachtsdiagnose. Eine MRT erhöht die pränatale Detektionsrate. Aufgrund der fehlenden sicheren sonografischen Marker, neben der möglichen direkten Darstellung des Ösophagus, sollten Ultraschallkontrollen in der Schwangerschaft durchgeführt werden. Insbesondere sollten kategorisch weitere mögliche Ursachen eine Polyhydramnions ausgeschlossen werden. Postnatal fallen die Kinder mit der klassischen Symptomatik auf. Die operative Versorgung ermöglicht den Betroffenen eine sehr gute Lebensqualität und Prognose. Eine lebenslange Observation und Begleitung ist notwendig.

Abstract

Oesophageal atresia causes a dysplasia of the oesophagus with or without a connection to the adjoining trachea. Prenatal ultrasound results are not specific enough to confirm a suspected diagnosis. In addition to polyhydramnios and a small or absent stomach, the so-called “pouch sign” reinforces the suspected diagnosis. An MRI increases the prenatal detection rate. Due to the lack of reliable sonografic markers, ultrasonic testing is advised during pregnancy. Particularly, further causes for the polyhydramnios should be categorically excluded. Postnatally, children present with classic symptoms. Surgical treatment results in a very high quality of life and a very good prognosis. Nevertheless lifelong monitoring and follow-up of the patient is required.

Schlüsselwörter

Ösophagusatresie - Polyhydramnion - Pouch-sign - Magenblase - tracheoösophageale Fistel - angeborene Fehlbildung

Key words

Esophageal Atresia - Polyhydramnios - Stomach - Prenatal Ultrasound - Congenital Malformation - Laparoscopy

Full Text

References

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