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2006-02-13-11 Recessive polycystic kidney  © Werner www.thefetus.net/


Recessive polycystic kidney disease

Heron Werner, MD

Clínica de Diagnóstico por Imagem (CDPI) & Instituto Fernandes Figueira (IFF) – FIOCRUZ - Rio de Janeiro – Brazil

The polycystic renal disease displays a recessive autosomal inheritance with an early intrauterine manifestation. It is the most common heritable cystic renal disease occurring in infancy. The prenatal diagnosis is done by ultrasound but in difficult cases, such as maternal obesity, MRI can offer additional images of the fetal anatomy.

This is a 24-year-old primigravida at 29 weeks referred to our unit for a routine antenatal ultrasound. We diagnosed the presence of enlarged echogenic fetal kidneys, an empty bladder, ascites, small thorax and severe oligohydramnios. In cases like this, the prognosis is very poor. The pregnancy was interrupted at 34 weeks and the diagnosis was confirmed at autopsy.

View at 19 weeks with and without Doppler of the right enlarged echogenic kidney (red arrow). Note the liver (blue arrow) and the presence of ascites (green arrow)

Axial (left image) and sagittal (right image) view showing the enlarged kidneys (red arrows). Note the presence of ascites (blue arrow)

3D view

3D view of the right kidney (black arrow) and the liver (white arrow)

3D view of the right kidney and the liver (left image) and the left kidney (right image)

Cephalic pole and the small thorax with color Doppler. Note the severe oligohydramnios.

 

References
• Ling YP, Cheng SJ, Huang JK. Autosomal recessive polycystic kidney disease: appearance on fetal MRI. Pediat Radiol 2006, 36:169.
• Werner www.thefetus.net/. Recessive polycystic renal disease.

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