Recessive polycystic kidney disease

José Luis Bartha Rasero, MD , José Arrabal Velazquez, MD


Cádiz, Spain

This fetus presented a large amount of ascites and the initial diagnosis had been omphalocele with intrabdominal mass. On further examination, the ascites had been confused with amniotic fluid and the mass was a large left kidney with Infantile Polycystic Kidney Disease. The left kidney was larger but at pathology there was bilateral involvement with marked asymmetry of size of both kidneys.

Note:

  • The large echogenic left kidney
  • The ascites
  • The anhydramnios
pckd-2

pckd-1

pckd-3

The cord insertion helps assess where the abdominal wall truly is.

kidney2

The scaphocephaly resulting from the oligohydramnios.

kidney3

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