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Zwerchfellhernie

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Kinderchirurgie

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Zusammenfassung

Die kongenitale Zwerchfellhernie besteht in der Regel aus einer angeborenen Zwerchfelllücke mit in den Thoraxraum prolabierten Bauchorganen und einer ipsilateral betonten Lungenhypoplasie. Diese ist in mehr als 80% der Fälle links lokalisiert. Aufgrund dieser Lungenhypoplasie besteht eine starke Einschränkung der Atemfläche. Bei großen Defekten kommen eine Hypoplasie des linken Ventrikels sowie eine persistierende pulmonale Hypertonie hinzu, der fetale Kreislauf bleibt erhalten. Trotz kleines kreislaufunterstützender Therapie mit Entlastung des Lungenkreislaufs ist eine Hypertrophie der glatten Muskelzellen nicht aufzuhalten, infolgedessen verschlechtert sich die pulmonale Hypertonie. Dies führt auch heute noch zu einer reduzierten Überlebenswahrscheinlichkeit der betroffenen Neugeborenen außerhalb spezialisierter Zentren, eine frühzeitige intrauterine Diagnosestellung ist daher sehr wichtig. Versuche einer fetalchirurgischen Korrektur des Zwerchfelldefektes blieben bisher ohne Erfolg. Der pränatal temporäre Verschluss der Trachea mittels eines intrauterin endoskopisch platzierten Ballons zur Anregung des Lungenwachstums wird in der Literatur kontrovers diskutiert. Um postnatal die bestmögliche Therapie und somit das bestmögliche Überleben anbieten zu können, soll die Geburt der Kinder an einem spezialisierten Zentrum geplant werden. Postnatal ist eine hoch differenzierte Beatmungstherapie der wichtigste Baustein der Therapie, nicht selten wird auch eine ECMO-Therapie (Extrakorporale Membranoxygenierung) notwendig. Nach Stabilisierung des Kindes erfolgt die chirurgische Korrektur mit Verschluss der Zwerchfelllücke meist über eine Laparotomie, in geeigneten Fällen auch über eine Thorakoskopie oder eine Thorakotomie. Leider wird das Überleben in vielen Fällen auf Kosten einer ausgeprägten Morbidität erreicht.

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  1. Kinderchirurgische Klinik, Universitätsklinikum Mannheim, Theodor-Kutzer-Ufer 1-3, 68167, Mannheim, Deutschland

    L.M. Wessel, B. Lange & K.L. Waag

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  1. L.M. Wessel
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  1. Kinderchirurgische Klinik, Universitätsklinikum München Dr. von Haunersches Kinderspital, München, Germany

    Dietrich von Schweinitz

  2. Abt. Kinderchirurgie, Medizinische Hochschule Hannover (MHH) Kinderklinik, Hannover, Germany

    Benno Ure

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Wessel, L., Lange, B., Waag, K. (2013). Zwerchfellhernie. In: von Schweinitz, D., Ure, B. (eds) Kinderchirurgie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-29779-3_21

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