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Agenesis of the venous duct: two cases of extrahepatic drainage of the umbilical vein and extrahepatic portosystemic shunt with a review of the literature

Published online by Cambridge University Press:  22 May 2014

Rohit S. Loomba ,
Michele Frommelt ,
David Moe  and
Amanda J. Shillingford
Rohit S. Loomba*
Affiliation:
Department of Pediatric Cardiology, Children’s Hospital of Wisconsin/Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America
Michele Frommelt
Affiliation:
Department of Pediatric Cardiology, Children’s Hospital of Wisconsin/Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America
David Moe
Affiliation:
Department of Pediatric Cardiology, Children’s Hospital of Wisconsin/Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America
Amanda J. Shillingford
Affiliation:
Department of Pediatric Cardiology, Children’s Hospital of Wisconsin/Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America
*
Correspondence: R.S. Loomba, Department of Pediatric Cardiology, Children’s Hospital of Wisconsin, 9000 Wisconsin Avenue, Milwaukee 53226, United States of America. Tel: 414-266-2380; Fax: 414-266-2381; E-mail: rloomba@mcw.edu
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Abstract

Agenesis of the venous duct is a rare congenital anomaly resulting in abnormal drainage of the umbilical vein into the foetal venous circulation. The clinical presentation and prognosis is variable, and may depend on the specific drainage pathways of the umbilical vein. We present two foetuses with agenesis of the venous duct, both associated with a postnatal portosystemic shunt, but with markedly different postnatal clinical courses. We also review all previously reported cases to better characterise this foetal disorder and the prognosis.

Keywords

Venous ductabsenceagenesisumbilical veinAbernethy malformationportosystemic shuntductus venosus
Type
Review Articles
Information
Cardiology in the Young , Volume 25 , Issue 2 , February 2015 , pp. 208 - 217
Copyright
© Cambridge University Press 2014 

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