Autosomal recessive polycystic kidney disease

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Large echogenic kidneys
Large echogenic kidneys
  • Inheritable cystic kidney disease with variable penetrance and severity
  • Enlarged hyperechoic kidneys
  • Bladder with little urine
  • Oligohydramnios
  • Liver typically involved


Nonfunctioning kidney replaced by cysts
2nd most common abdominal mass in neonate
Bilateral is fatal, but very rare
Unilateral a/w anomalies of contralateral kidney

  • Trisomy 13
Kidneys usually echogenic, enlarged, cysts may be visible
Facial anomalies

  • Meckel-Gruber syndrome
Cystic renal dysplasia

Scan parental kidneys
Asymmetric renal enlargement
Rarely presents in fetus
Renal echogenicity generally normal but hyperechoic kidneys have been described
Amniotic fluid normal

Also consider: medullary cystic kidney disease, aquired cystic disease of uremia, Von Hippel-Lindau syndrome, and Tuberous sclerosis