Kongenitale Gastroschisis – pränatale Diagnose und perinatales Management

Zusammenfassung

Die Geburtsprävalenz der Gastroschisis nimmt weltweit zu. Diese Feststellung trifft gerade auf jüngere schlanke Frauen mit Nikotinkonsum zu. Auf den ersten Blick ein Paradoxon, angesichts des immer älter werdenden Schwangerenkollektives, innerhalb dessen immer weniger Raucherinnen zu finden sind. In zahlreichen Studien konnten sowohl (nutritive) Teratogene und Umwelteinflüsse als auch epidemiologische Ursachen für dieses Phänomen glaubhaft gemacht werden. Heutzutage werden bis zu 90% der Feten mit Gastroschisis pränatal erkannt. Vorteil einer pränatalen Diagnose sind u. a. die Erkennung assoziierter Störungen und die Feststellung einer high-risk-Konstellation (IUGR, intraabdominale Darmdilatation o. ,vanishing gut’). Nur so wird eine adäquate interdisziplinäre Beratung der betroffenen Eltern gemeinsam mit Geburtshelfern, Kinderchirurgen und Neonatologen möglich. Die Wirksamkeit serieller Amnionaustauschinfusionen hinsichtlich eines besseren neonatalen Outcomes konnte bislang noch nicht eindeutig geklärt werden. Die Möglichkeiten eines fetalchirurgischen Vorgehens sind limitiert und derzeit nur in experimentellen Ansätzen im Tiermodell denkbar. Aus geburtshilflicher Sicht scheinen der in utero Transport und die elektive Sectio vor der vollendeten 36. SSW in einem tertiären Zentrum mit entsprechender Logistik (Kinderchirurgie, Neonatologie) auch trotz nicht schlüssiger Datenlage derzeit die von den meisten Autoren empfohlene Vorgehensweise zu sein. Die Überlebensraten von Feten mit einer Gastroschisis nach operativer Therapie (primärer Defektverschluss, Silotechnik) sind insgesamt hoch (>90%).

Abstract

The birth prevalence of gastroschisis is increasing world-wide. This situation applies particularly to young, slim women who smoke. At a first glance this is a paradox in light of the ever-increasing age of pregnant women among whom there are fewer and fewer smokers. In numerous studies it has been clearly demonstrated that not only (nutritional) teratogenic substances and environmental factors but also epidemiological causes can be held responsible for this phenomenon. Nowadays gastroschisis is detected prenatally in up to 90% of all foetuses. Advantages of a prenatal diagnosis include the identification of associated disorders and the determination of a high-risk constellation (IUGR, intraabdominal bowel dilatation or vanishing gut). This is essential for an adequate interdisciplinary counseling for the afflicted parents together with obstetricians, paediatric surgeons and neonatalogists. The efficacy of serial amnioexchanges with regard to a better neonatal outcome has as yet not been unambiguously clarified. The possibilities for surgical procedures on the foetus are limited and can at present only be considered as experimental attempts in animal models. From an obstetrical perspective the in utero transport and elective Caesarean section before completion of the 36^th week of gestation in a tertiary centre with appropriate facilities (paediatric surgery, neonatalogy) seem to be the course recommended by most authors in spite of inconclusive data. The survival rates for babies with gastroschisis after operative treatment (primary defect closure, silotechnique) are considerably high (>90%).

Schlüsselwörter

Gastroschisis - pränatal - perinatal - Ultraschall - Darm - Management

Key words

Gastroschisis - prenatal - perinatal - ultrasound - bowel - management

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