Fetale Chirurgie für schwere kongenitale Fehlbildungen

E. Danzer; F. Schier; B. Paek; M. R. Harrison; C. T. Albanese

doi:10.1055/s-2001-18503

Zeitschrift für Geburtshilfe und Neonatologie, Inhaltsverzeichnis

Z Geburtshilfe Neonatol 2001; 205(5): 174-188
DOI: 10.1055/s-2001-18503

ORIGINALARBEIT

Georg Thieme Verlag Stuttgart · New York

Fetale Chirurgie für schwere kongenitale Fehlbildungen[1]

Fetal surgical therapy for severe congenital malformationsE. Danzer^{1, 2} , F. Schier¹ , B. Paek² , M. R. Harrison² , C. T. Albanese²

¹ Abteilung für Kinderchirurgie, Friedrich Schiller Universität Jena
² The Fetal Treatment Center, Department of Surgery, Division of Pediatric Surgery, University of California, San Francisco

Abstract

Zusammenfassung

Hintergrund und Fragestellung Mit Hilfe modernster Diagnosetechniken, der molekularen Zytogenetik und der Verbesserungen der bildgebenden Verfahren während der letzten 20 Jahre ist es immer öfter möglich geworden, Fehlbildungen und Anomalien des Feten bereits im Mutterleib zu diagnostizieren. Eine frühe Diagnose und fundiertes Wissen über den natürlichen Verlauf einer Fehlbildung gestatten dem Perinatalmediziner eine realistische Einschätzung der kindlichen Prognose und die Definition geeigneter therapeutischer Strategien. Die fetale Chirurgie ist eine logische Konsequenz der Entwicklungen in der pränatalen Diagnostik. Ziel dieser Arbeit ist es, einen Überblick über die aktuellen Entwicklungen und Möglichkeiten in der pränatalen chirurgischen Therapie zu geben sowie die Risiken und ethischen Probleme zu diskutieren.Material und Methodik Die gegenwärtig zur Verfügung stehende Literatur sowie die zur Zeit laufenden experimentellen und klinischen Studien wurden wissenschaftlich aufgearbeitet und die Ergebnisse zusammengetragen.Ergebnisse Wenn auch die meisten in utero festgestellten Fehlbildungen am besten nach der Geburt medikamentös und/oder chirurgisch behandelt werden, führen einige Anomalien bereits während der Schwangerschaft zu einer nicht mit dem postnatalen Leben des Fetus zu vereinbarenden Organschädigung. Insbesondere diese Feten profitieren von einer intrauterinen Korrektur. Um einen pränatalen Eingriff mit entsprechenden Risiken zu rechtfertigen, müssen verschiedene Selektionskriterien formuliert werden: die Diagnose muss in utero sicher zu stellen sein, assoziierte Anomalien müssen ausgeschlossen werden, die intrauterine Organschädigung muss nach dem Eingriff reversibel sein und die fetale Operation muss die Prognose des Feten signifikant verbessern.Es wurden drei Zugangswege zum Feten entwickelt: perkutan, fetoskopisch und die offene fetale Chirurgie. Derzeit werden folgende Eingriffe durchgeführt: temporäre Trachealokklusion bei der kongenitalen Zwerchfellhernie, Dekompression der abführenden Harnwege bei der obstruktiven Uropathie, intrauterine Lasertherapie bei dem Zwillings-Transfusion-Syndrom, Tumorresektion bei der zystisch-adenomatoiden Lungenmalformation und dem Steißbeinteratom, sowie die intrauterine Deckung einer Myelomeningocele. Die Hauptkomplikationen eines intrauterinen Eingriffes sind vorzeitige Wehen und vorzeitiger Blasensprung. Um die mütterliche Morbidität und das Risiko der Frühgeburtlichkeit zu senken, wurde die endoskopische fetale Chirurgie entwickelt (FETENDO).Schlussfolgerung Bei ausgewählten Patientinnen kann durch einen fetalchirurgischen Eingriff die Mortalität und die postnatale Morbidität des Feten bzw. des Neugeborenen verringert werden. Durch den Erfolg der minimal-invasiven fetalen Chirurgie wird die offene fetale Chirurgie mit all ihren Komplikationen in Zukunft nur eine untergeordnete Rolle einnehmen.

Fetal surgical therapy for severe congenital malformations

Background Over the past two decades the diagnosis of life threatening congenital malformations has evolved rapidly. Sophisticated and powerful new imaging and sampling techniques have stripped the veil of mystery from the once secretive fetus. Early detection and close follow-up of the fetus with congenital malformations have allowed us to define their natural history, determine the clinical features that affect clinical outcome, and plan management approaches to improve prognosis. Fetal surgical intervention is the logical culmination of the progress in fetal diagnosis. The purpose of this article is to describe the current techniques and recent advances in prenatal diagnosis and fetal intervention of severe congenital malformation.Material and Methods A complete review of the literature and our own experience concerning fetal surgery was performed.Results Although most prenatally diagnosed malformations are best managed by appropriate medical and surgical therapy after maternal transport and planned delivery at a tertiary care center, an expanding number of simple anatomical abnormalities with predictable, lethal consequences have been successfully corrected before birth. A malformation amenable to prenatal surgical intervention must fulfill a number of conditions. It must be severe enough to warrant the risks associated with in utero treatment and must be reliably detectable before birth. Additionally, the pathophysiology must be reversible by fetal surgery, significantly improving the prognosis over postnatal treatment.Many technical intricacies of open fetal surgery have been solved, but pre-term labor and premature rupture of membranes remain a omnipresent risks to both the mother and the fetus. To reduce maternal morbidity and the risk of prematurity we developed minimally invasive techniques to treat the fetus prenatally. Current indications of fetal surgery include the treatment of congenital diaphragmatic hernia, cystic adenomatoid malformation of the lung, sacrococcygeal teratoma, obstructive uropathy, twin-to-twin-transfusion-syndrome and myelomeningocele. Minimally invasive surgical techniques (FETENDO) have significantly lessened the incidence of preterm labor and promise to extend the indications for fetal surgical intervention.Conclusions Fetal surgical therapy for severe congenital malformations may improve the outcome of selected patients. The development of FETENDO will in all probability reduce the importance of open fetal surgery in the future.

Schlüsselwörter

Pränatale Diagnose - angeborene Fehlbildungen - Fetus - fetale Chirurgie

Key words

Prenatal diagnosis - Congenital malformation - Fetus - Fetal surgical intervention

Volltext

Referenzen

Literatur

1 Eingang: 18. 8. 2000
Angenommen nach Revision: 13. 11. 2000

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Dr. Enrico Danzer

Abteilung für Kinderchirurgie
Friedrich-Schiller-Universität

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