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Giant choledochal cyst and infantile polycystic kidneys as prenatal sonographic features of Caroli syndrome

Giant choledochal cyst and infantile polycystic kidneys as prenatal sonographic features of... Caroli syndrome is a developmental disorder caused by complete or partial arrest of ductal plate remodeling, leading to dilated bile ducts along with fibrosis surrounding the portal tracts. It is most commonly associated with autosomal recessive polycystic kidney (ARPKD). We report a unique case of Caroli syndrome, diagnosed prenatally at 24 weeks of gestation in a 29‐year‐old Thai woman. Ultrasound findings revealed the association of a fetal giant choledochal cyst with ARPKD. Autopsy findings showed ductal plate malformation, typical of Caroli syndrome, associated with giant choledocal cyst and ARPKD. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Clinical Ultrasound Wiley

Giant choledochal cyst and infantile polycystic kidneys as prenatal sonographic features of Caroli syndrome

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References (9)

Publisher
Wiley
Copyright
© 2020 Wiley Periodicals, Inc.
ISSN
0091-2751
eISSN
1097-0096
DOI
10.1002/jcu.22778
Publisher site
See Article on Publisher Site

Abstract

Caroli syndrome is a developmental disorder caused by complete or partial arrest of ductal plate remodeling, leading to dilated bile ducts along with fibrosis surrounding the portal tracts. It is most commonly associated with autosomal recessive polycystic kidney (ARPKD). We report a unique case of Caroli syndrome, diagnosed prenatally at 24 weeks of gestation in a 29‐year‐old Thai woman. Ultrasound findings revealed the association of a fetal giant choledochal cyst with ARPKD. Autopsy findings showed ductal plate malformation, typical of Caroli syndrome, associated with giant choledocal cyst and ARPKD.

Journal

Journal of Clinical UltrasoundWiley

Published: Jan 1, 2020

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