Prognose und Therapie der angeborenen Zwerchfellhernie abhängig vom Zeitpunkt der klinischen Präsentation – Erfahrungen eines hochspezialisierten Zentrums

Zusammenfassung

Die Diagnose angeborene Zwerchfellhernie (CDH) hat viele verschiedene, meist unbekannte Ursachen, wodurch sich eine facettenreiche klinische Präsentation ergibt. Bei pränataler Diagnosestellung lässt sich die Prognose und Intensität der erforderlichen Therapiemaßnahmen gut vorhersagen. Die Behandlungsresultate in hochspezialisierten Zentren haben sich in den letzten Jahren kontinuierlich gebessert. Ohne pränatale Diagnose zeigen sich die Neugeborenen mit CDH mit einer schweren Atemstörung, nach der Neonatalzeit stehen häufiger gastrointestinale als respiratorische Symptome im Vordergrund. Weil die Symptomatik bei der späten Präsentation der von banalen Infekten ähnelt, wird die seltene Differenzialdiagnose CDH leichtt verpasst. Die Prognose dieser Fälle ist fast immer gut, kurativ ist die chirurgische Therapie.

Abstract

Congenital diaphragmatic hernia (CDH) is a diagnosis with multiple, mostly unknown origin. Therefore its clinical presentation is highly variable. In case of prenatal detection prognosis and amount of interventions in neonatal intensive care is predictable. The results reported by high volume centres for CDH improved continuously during the last years. In neonates suffering from CDH without prenatal diagnosis severe respiratory distress is the leading clinical sign. Presenting symptom of late-onset CDH can also be gastrointestinal problems. Due to more subtle symptoms late presenting CDH is more difficult to diagnose. Prognosis is good, but it still requires surgical intervention to alleviate the symptoms.

Schlüsselwörter

angeborene Zwerchfell­hernie - Prognose - Präsentationszeitpunkt - Symptome

Key words

congenital diaphragmatic hernia - prognosis - time of presentation - symptoms

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