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Giant choledochal cyst and infantile polycystic kidneys as prenatal sonographic features of Caroli syndrome
Authors:Kuntharee Traisrisilp MD  Fuanglada Tongprasert MD  Komson Wannasai MD  Theera Tongsong MD
Affiliation:1. Department of Obstetrics and Gynecology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand;2. Department of Pathology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
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.
Keywords:Caroli syndrome  choledochal cyst  fetus  prenatal diagnosis  ultrasound
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