Autosomal recessive polycystic kidney disease

Case contributed by Dr Hidayatullah Hamidi

Presentation

A cesarean-born 3-hour neonate with abdominal distension. The mother was oligohydramniotic.

Patient Data

Age: Neonate
Gender: Male

Both kidneys are enlarged in size (right: 10 cm and left 9 cm in bipolar diameter) showing increased echogenicity (due to acoustic enhancement of the tiny cysts) and altered corticomedullary differentiation. No hydronephrosis is noted. No focal mass lesion is seen. 

With high-frequency linear probe, numerous tiny cylindrical cysts are visualized representing ectatic collecting ducts. One of the cysts measures 1.6 mm.

Liver shows course echogenic texture (? hepatic fibrosis) with evidence of cystic dilatation in the right lobe of the liver (? biliary tract cystic change). The gallbladder is normal.

Case Discussion

The overall features are representing a typical case of Autosomal recessive polycystic kidney disease.

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