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Kongenitale Zwerchfellhernie

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Kinderchirurgie

Part of the book series: Springer Reference Medizin ((SRM))

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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. Aufgrund der unterschiedlich stark ausgeprägten Lungenhypoplasie ist die Atemaustauschfläche eingeschränkt. Zusätzlich besteht eine pulmonale Hypertonie. Ist diese ausgeprägt, kommt es konsekutiv zu einer Hyperplasie des rechten Ventrikels und einer relativen Hypoplasie des linken Ventrikels, woraus postnatal ein Linksherzversagen resultieren kann. Insbesondere bei suboptimaler postnataler Versorgung aggraviert sich die pulmonale Hypertonie, was auch heute noch zu einer reduzierten Überlebenswahrscheinlichkeit der betroffenen Neugeborenen außerhalb spezialisierter Zentren führt und die Bedeutung einer frühzeitigen intrauterinen Diagnosestellung hervorhebt. Versuche einer fetalchirurgischen Korrektur des Zwerchfelldefekts blieben bisher ohne Erfolg; auch der pränatal temporäre Verschluss der Trachea mittels eines intrauterin endoskopisch platzierten Ballons (fetoscopic tracheal occlusion therapy, FETO) zur Anregung des Lungenwachstums wird in der Literatur kontrovers diskutiert. Nach der Geburt an einem spezialisierten Zentrum sollte postnatal nach Stabilisierung des Kindes die chirurgische Korrektur mit Verschluss der Zwerchfelllücke erfolgen. Die Standardisierung der pränatalen Diagnostik und postnatalen Versorgung haben zu einer besseren Überlebensrate geführt; dadurch rückt die Langzeitmorbidität dieser Kinder immer mehr in den Fokus.

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Authors and Affiliations

  1. Kinderchirurgische Klinik, Universitätsmedizin Mannheim GmbH, Mannheim, Deutschland

    Lucas M. Wessel & Katrin Zahn

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  1. Lucas M. Wessel
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  2. Katrin Zahn
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Correspondence to Lucas M. Wessel .

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Editors and Affiliations

  1. Kinderchirurgische Klinik und Poliklinik, Klinikum der Universität München Kinderchirurgische Klinik und Poliklinik, München, Bayern, Germany

    Dietrich von Schweinitz

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

    Benno Ure

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Wessel, L.M., Zahn, K. (2018). Kongenitale Zwerchfellhernie. In: von Schweinitz, D., Ure, B. (eds) Kinderchirurgie. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-53390-1_34-1

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  • DOI: https://doi.org/10.1007/978-3-662-53390-1_34-1

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  • Print ISBN: 978-3-662-53390-1

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