Closing Gastroschisis: eine Sonderform der Gastroschisis mit hoher Morbidität und Mortalität

Zusammenfassung

Ziel: Selten kommt es bei der Gastroschisis durch intrauterine Einengung der Bauchdeckenlücke um die hernierten extraabdominalen Darmschlingen zu schweren Darmschädigungen. Durch die Korrelation von pränatalen Ultraschallbefunden mit den postnatalen Verhältnissen sollen mögliche Frühzeichen dieser „Closing Gastroschisis“ erarbeitet werden. Nach pränataler Diagnosestellung kann durch rechtzeitige Entbindung ischämischer Darm gerettet und so ein Kurzdarmsyndrom vermieden werden.

Material und Methoden: Von den 18 Patienten mit Gastroschisis, die von 2007 – 2009 an unserer Institution behandelt wurden, zeigten 5 Kinder die charakteristischen Befunde einer Closing Gastroschisis. Die pränatalen Ultraschallbefunde wurden mit dem Ausmaß der postnatal erkennbaren Darmschädigung korreliert.

Ergebnisse: Wir konnten keine einheitlichen Ultraschallkriterien zur pränatalen Diagnose einer Closing Gastroschisis feststellen. 3 Kinder mit signifikanter pränataler Magendilatation zeigten aufgrund ausgedehnter Darmnekrosen sehr schwere postnatale Verläufe. In einem Fall konnte als Korrelat einer intrauterinen Darmschädigung aufgrund einer Einengung der Bauchdeckenlücke eine progrediente, intraabdominale Schlingendilatation bei gleichzeitiger Schrumpfung des extraabdominalen Darmkonvoluts beobachtet werden.

Schlussfolgerung: Die Closing Gastroschisis ist eine Sonderform der Gastroschisis. Aufgrund ausgedehnter Darmnekrosen entwickelt sich postnatal oft eine vitale Bedrohung für das Kind. Die pränatale Diagnose kann sich in Einzelfällen aus der Dynamik der fetalen Ultraschallveränderungen ergeben. Bei progredienter intraabdominaler Schlingendilatation besteht der dringende Verdacht auf eine Closing Gastroschisis.

Abstract

Purpose: We correlate severe bowel damage in gastroschisis to the rare intrauterine event of narrowing of the abdominal wall around the protruding intestines. We describe this “closing gastroschisis” as a distinct entity. Prenatal ultrasound findings as gastric or bowel dilation were compared to the postnatal findings in order to find markers for an early in utero diagnosis of closing gastroschisis. Early diagnosis could prompt timely delivery to save the compromised bowel and avoid short gut syndrome.

Materials and Methods: We documented the pre- and postnatal course of our patients with gastroschisis from 2007 to 2009. Closing gastroschisis was suspected antenatally and confirmed postnatally. We identified 5 out of 18 patients showing closure of the abdominal wall with varying degrees of bowel damage. Prenatal ultrasound findings were correlated to the postnatally confirmed extent of intestinal damage.

Results: We could not find consistent ultrasound markers for prenatal diagnosis of closing gastroschisis. In prenatal ultrasound three patients presented significant gastric dilation and then experienced severe courses postnatally due to segmental gut necrosis. One of these three died and the other two developed short gut syndrome. In one case progressive intraabdominal loop dilation with simultaneous shrinking of the extraabdominal loops occurred corresponding to closing gastroschisis with segmental midgut necrosis.

Conclusion: Closing gastroschisis must be seen as a special form of gastroschisis. Extended intestinal damage is often life-threatening. In longitudinal observation dynamics of fetal ultrasound findings can lead to the diagnosis of closing gastroschisis. Progressive intraabdominal loop dilation is always highly suspicious and must lead to close follow-up and timely delivery.

Key words

abdominal wall - fetus - ultrasound

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