Synopsis
bilateral nephromegaly 9symetrically enlarged kidneys)
progressive dilatation of medullary collecting ducts (medullary cysts formnation) (early) then dilatation of cortical collecting ducts
normal nephronogenesis
no hydronephrosis
preservation of calyces
no significant interstitial fibrosis, inflammation and obstruction
conservation of renal shape
normal glomeruli and tubules between the cysts
loss of corticomedullary differenciation
oligohydramnios sequence (Potter sequence)
NB: All forms of renal ARPKD are associated with hepatic ARPKD (congenital hepatic fibrosis and ductal plate malformations).
See also:
multicystic kidneys/polycystic kidneys
ductal plate malformations (DPM)