Prenatal MR imaging features of Caroli syndrome in association with autosomal recessive polycystic kidney disease

Caroli syndrome, which is characterized by saccular and fusiform dilatation of the biliary ducts, is usually observed in association with autosomal recessive polycystic kidney disease (ARPKD). Although the diagnosis of ARPKD is generally easy to make in postnatal ultrasound, the diagnosis of Caroli...

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Main Authors: Amanda Rivas, Monica Epelman, MD, Enrico Danzer, MD, N. Scott Adzick, MD, Teresa Victoria, MD, PhD
Format: Article
Language:English
Published: Elsevier 2019-02-01
Series:Radiology Case Reports
Online Access:http://www.sciencedirect.com/science/article/pii/S1930043318304618
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author Amanda Rivas
Monica Epelman, MD
Enrico Danzer, MD
N. Scott Adzick, MD
Teresa Victoria, MD, PhD
author_facet Amanda Rivas
Monica Epelman, MD
Enrico Danzer, MD
N. Scott Adzick, MD
Teresa Victoria, MD, PhD
author_sort Amanda Rivas
collection DOAJ
description Caroli syndrome, which is characterized by saccular and fusiform dilatation of the biliary ducts, is usually observed in association with autosomal recessive polycystic kidney disease (ARPKD). Although the diagnosis of ARPKD is generally easy to make in postnatal ultrasound, the diagnosis of Caroli syndrome may be challenging in prenatal ultrasound. Herein, we present a case of a 29-week fetus with ARPKD associated with Caroli syndrome in whom fetal magnetic resonance imaging was essential to identify the “central dot sign” within the dilated biliary ducts to confirm the prenatal diagnosis of Caroli syndrome and to increase our level of confidence in this diagnosis. Keywords: Fetal MRI, Caroli syndrome, Autosomal recessive polycystic kidney disease
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spelling doaj.art-07d5122be15b41ab97b3205aeebc22202022-12-21T23:42:44ZengElsevierRadiology Case Reports1930-04332019-02-01142265268Prenatal MR imaging features of Caroli syndrome in association with autosomal recessive polycystic kidney diseaseAmanda Rivas0Monica Epelman, MD1Enrico Danzer, MD2N. Scott Adzick, MD3Teresa Victoria, MD, PhD4Emory University, 201 Dowman Dr, Atlanta, GA 30322, USA; Department of Medical Imaging/Radiology, Nemours Children's Health System/Nemours Children's Hospital, 13535 Nemours Parkway, Orlando, FL 32827, USADepartment of Medical Imaging/Radiology, Nemours Children's Health System/Nemours Children's Hospital, 13535 Nemours Parkway, Orlando, FL 32827, USA; University of Central Florida, College of Medicine, 6850 Lake Nona Blvd, Orlando, FL 32827, USA; Corresponding author.Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 10104, USACenter for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 10104, USADepartment of Radiology, The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 10104, USACaroli syndrome, which is characterized by saccular and fusiform dilatation of the biliary ducts, is usually observed in association with autosomal recessive polycystic kidney disease (ARPKD). Although the diagnosis of ARPKD is generally easy to make in postnatal ultrasound, the diagnosis of Caroli syndrome may be challenging in prenatal ultrasound. Herein, we present a case of a 29-week fetus with ARPKD associated with Caroli syndrome in whom fetal magnetic resonance imaging was essential to identify the “central dot sign” within the dilated biliary ducts to confirm the prenatal diagnosis of Caroli syndrome and to increase our level of confidence in this diagnosis. Keywords: Fetal MRI, Caroli syndrome, Autosomal recessive polycystic kidney diseasehttp://www.sciencedirect.com/science/article/pii/S1930043318304618
spellingShingle Amanda Rivas
Monica Epelman, MD
Enrico Danzer, MD
N. Scott Adzick, MD
Teresa Victoria, MD, PhD
Prenatal MR imaging features of Caroli syndrome in association with autosomal recessive polycystic kidney disease
Radiology Case Reports
title Prenatal MR imaging features of Caroli syndrome in association with autosomal recessive polycystic kidney disease
title_full Prenatal MR imaging features of Caroli syndrome in association with autosomal recessive polycystic kidney disease
title_fullStr Prenatal MR imaging features of Caroli syndrome in association with autosomal recessive polycystic kidney disease
title_full_unstemmed Prenatal MR imaging features of Caroli syndrome in association with autosomal recessive polycystic kidney disease
title_short Prenatal MR imaging features of Caroli syndrome in association with autosomal recessive polycystic kidney disease
title_sort prenatal mr imaging features of caroli syndrome in association with autosomal recessive polycystic kidney disease
url http://www.sciencedirect.com/science/article/pii/S1930043318304618
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