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Z Geburtshilfe Neonatol 2001; 205(2): 65-70
DOI: 10.1055/s-2001-14823
KASUISTIK

Georg Thieme Verlag Stuttgart · New York

Pränatale Diagnose und Management bei VACTERL-Assoziation[1]

Prenatal diagnosis and management of VACTERL-AssociationS. Tercanli1 , C. Troeger1 , H. Fahnenstich2 , I. Hösli1 , W. Holzgreve1
  • 1 Universitäts-Frauenklinik Basel
  • 2 Universitäts-Kinderklinik Basel
Further Information

Publication History

Publication Date:
31 December 2001 (online)

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Zusammenfassung

Wir berichten von drei Fällen einer VACTERL-Assoziation mit unterschiedlicher Manifestation, die pränatal anhand der charakteristischen Befunde entdeckt wurden. Die sonographischen und klinischen Befunde sowie die Autopsie werden dargestellt und die Pathogenese der VACTERL-Assoziation diskutiert. Da die meisten Fälle dieser seltenen kongenitalen Erkrankung sporadisch auftreten, ist es wichtig die typischen Symptome sowie die Differenzialdiagnostik möglichst genau zu klassifizieren, um eine adäquate Beratung der betroffenen Paare anbieten zu können.

In terms of the VACTERL-Association we are dealing with a non-random association of malformations following a defect during mesodermal development of embryogenesis due to a variety of causes. We report on three cases with VACTERL-type malformations diagnosed by prenatal ultrasound presenting cardial defects, renal abnormalities, single umbilical arteries and esophageal stenosis. We present sonographical, clinical and autopsy findings and discuss the pathogenesis of VACTERL-Association as a defect of mesenchymal development in early embryogenesis.

Schlüsselwörter

VATER-Assoziation - VACTERL-Assoziation - Singuläre Nabelschnurarterie - Nierenfehlbildungen pränatal

Key words

VATER-Association - VACTERL-Association - single umbilical artery - renal malformation

01 Eingang: 27. 3. 2000
Angenommen nach Revision: 19. 6. 2000

Literatur

01 Eingang: 27. 3. 2000
Angenommen nach Revision: 19. 6. 2000

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Dr. med. S. Tercanli

Universitäts-Frauenklinik

Schanzenstrasse 46

4031 Basel

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