Pathology | Renal Cystic Diseases - [click on image(s) below]
Infantile Polycystic Kidney Disease
Clinical: most cases result in stillbirth or early neonatal birth; often associated with Potter’s facies when the kidneys are totally nonfunctional
Gross: bilaterally enlarged kidneys with “rays” of cysts oriented perpendicular to the cortex; reniform shape is generally maintained; ureters are normal
Histology:
cystic dilatation of the ampullary collecting ducts +/- involvement of the PCT and Bowman’s capsule
nephrons between the dilated ducts are normal (vs. renal dysplasia)
Syndrome: autosomal recessive inheritance; hepatic lesions mimicking congenital hepatic fibrosis may also be present (older patients develop hepatosplenomegaly and portal hypertension)
Potter’s classification: Potter type I (perinatal and neonatal) or Potter type III (infantile and juvenile)
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