Caroli's syndrome with autosomal recessive polycystic kidney disease on fetal MRI: A case report

Abstract

A 21-year-old pregnant woman (gravida 1, abortion 0) with a his-tory of acrocyanosis and raised AST/ALT was referred for ultrasound assessment. The gestational age was 32 weeks according to LMP and 31 weeks 2 days as per biometry. USG revealed severe oligohydramnios with enlarged and echogenic bilateral kidneys (Figure 1A). The urinary bladder was not visualized. Fetal liver showed a single small cystic structure in the right lobe of the liver (Figure 1B). The fetal MRI was done after 2 days of ultrasound examination on 3T system to further evaluate. Sequences used were ultrafast T1 and T2 (VIBE and HASTE, respectively). MRI dem-onstrated single intrauterine fetus in breech presentation with severe oligohydramnios. Bilateral kidneys were grossly enlarged and showed increased T2 signal intensity with few cysts (Figure 1C). The urinary bladder was not seen. The liver was normal in size and showed tubular and cystic dilatation of intrahepatic biliary channels with most of them showing “ central dot sign ” (Figure 1D). Both fetal lungs showed low signal intensity (Figure 1D). The fetal spleen was normal in size. The fetal MRI findings were suggestive of Caroli's syndrome with ARPKD and bilateral pulmonary hypoplasia. The baby