Intrahepatic bile ductal ectasia in autosomal recessive polycystic kidney disease evaluated by fetal magnetic resonance imaging: a more frequent complication

Abstract

Objective

This study aimed to evaluate liver malformations and intrahepatic bile ductal ectasia and dilatation (IBDED) in cases of prenatal diagnosis of autosomal recessive polycystic kidney disease (ARPKD) using magnetic resonance imaging (MRI).

Methods

This retrospective study involved 209 cases referred for fetal MRI studies (f-MRI) from March 2004 and December 2019, suspicious of congenital renal disease. Fetuses that met the criteria for ARPKD were selected.

Results

Six cases were diagnosed as ARPKD (2.8%). The median gestational age at MRI examination was 28 weeks (24–36 weeks). IBDED was observed in 84% of cases. Moreover, 66% presented multilobar liver lesions, and 33% exhibited monolobar lesions. The “central dot sign” (CDS) was found in half of the cases.

Conclusion

In this case series of prenatal diagnosis of ARPKD using f-MRI, IBDED was present in the majority of the cases, and the CDS was noted in half of the cases.

Keywords:

• Autosomal recessive polycystic kidney disease
• intrahepatic bile ductal ectasia and dilatation
• prenatal diagnosis
• central dot sign
• magnetic resonance imaging

Disclosure statement

No potential conflict of interest was reported by the author(s).