Hepatoomphalozelen – eine interdisziplinäre Herausforderung

Ein Plädoyer für die primär konservative Therapie

Giant omphaloceles—an interdisciplinary challenge

A plea for the primarily conservative treatment

Zusammenfassung

Hintergrund

Die Therapie von Hepatoomphalozelen („Riesenomphalozelen“) stellt eine Herausforderung für alle behandelnden Fachdisziplinen dar. Eine respiratorische Insuffizienz nach der Geburt ist häufig. Es gibt zwei grundsätzliche chirurgische Behandlungskonzepte: den „staged repair“, der einen Bauchwandverschluss kurz nach Geburt anstrebt, und den „delayed repair“, der eine primär konservative Therapie mit einem Bauchwandverschluss erst nach der Epithelialisierung der Omphalozele beinhaltet.

Ziel der Arbeit

In diesem Artikel sollen beide Therapiemethoden erläutert und verglichen werden, zudem wird eine Fallserie von primär konservativ behandelten Hepatoomphalozelen präsentiert.

Material und Methoden

Die Therapiemodalitäten und Ergebnisse von 16 primär konservativ behandelten Patienten mit Hepatoomphalozelen wurden retrospektiv ausgewertet, diskutiert und mit der Literatur verglichen.

Ergebnisse

Bei 7 von 16 Patienten bestand postpartal eine respiratorische Insuffizienz (43,7 %), die eine invasive Beatmung erforderlich machte. Bei 11 von 16 Patienten (69 %) konnte der Bauchwandverschluss in einer einzigen Operation erfolgen. Das mittlere Alter hierbei betrug 9,3 Monate (Range 3 bis 49 Monate). Ein Patient verstarb während des Follow-up, dies war jedoch nicht therapieassoziiert.

Diskussion

Die hohe Inzidenz von postpartaler respiratorischer Insuffizienz bei Patienten mit Hepatoomphalozelen sollte die Wahl der Therapiemethode zu einer interdisziplinären Entscheidung machen, da sie Einfluss auf die respiratorische Situation hat. Die regelmäßigen Verbandwechsel bis zur vollständigen Epithelialisierung der Omphalozele können ambulant durch den niedergelassenen Pädiater erfolgen, daher ist eine Kenntnis dieser Therapiemethode empfehlenswert.

Abstract

Background

The treatment of giant omphaloceles is challenging. There are two main treatment strategies: the primarily operative staged repair and the primarily nonoperative delayed repair, also known as “paint and wait”. In many other countries the delayed repair is the current principal treatment strategy, whereas the standard method in Germany seems to be the staged repair.

Objective

We describe and compare both methods and present a case series of 16 patients with giant omphaloceles that were treated with a delayed repair management in our institution between 2008 and 2015.

Material and methods

We retrospectively describe the treatment modalities of 16 patients with giant omphaloceles, including conservative treatment and operative procedures and evaluate the results.

Results

Of the 16 patients with giant omphalocele 7 were respiratory insufficient at birth (43.7%) and required mechanical ventilation. In 69% of cases the abdominal wall closure could be carried out in one single operation. The mean age at closure of the abdominal wall was 9.3 months (range 3–49 months). Of the 16 patients four developed a local infection of the omphalocele, four patients developed latent hypothyroidism during povidone iodine treatment and one patient died during follow-up due to an incident unrelated to treatment.

Conclusion

We consider the delayed repair a safe and feasible method, particularly regarding the high incidence of other malformations in patients with a giant omphalocele and specifically respiratory insufficiency at birth. This has been shown to be an independent predictor of mortality. Early surgical intervention to attempt closure of the abdominal wall can aggravate respiratory insufficiency after birth. Therefore, the choice of therapeutic strategy and its implications should be made after an interdisciplinary discussion. The regular changes of wound dressings during the epithelialization period of the omphalocele sac can be carried out by the practising pediatrician.

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Correspondence to Dr. R. Pohle.

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R. Pohle, N. Marathovouniotis, T. Klein und T.M. Boemers geben an, dass kein Interessenkonflikt besteht.

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Berthold Koletzko, München

Thomas Lücke, Bochum

Ertan Mayatepek, Düsseldorf

Norbert Wagner, Aachen

Stefan Wirth, Wuppertal

Fred Zepp, Mainz

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Pohle, R., Marathovouniotis, N., Klein, T. et al. Hepatoomphalozelen – eine interdisziplinäre Herausforderung. Monatsschr Kinderheilkd (2021). https://doi.org/10.1007/s00112-021-01129-5

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Schlüsselwörter

  • Exomphalos major
  • Konservative Therapie
  • Bauchwanddefekt
  • Respiratorische Insuffizienz
  • Epithelialisierung

Keywords

  • Exomphalos major
  • Paint and wait
  • Delayed repair
  • Abdominal wall defect
  • Respiratory insufficiency