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Background:
Autosomal recessive polycystic disease (ARPKD) is a heritable but phenotypically variable disorder characterized by varying degrees of nonobstructive renal collecting duct ectasia, hepatic biliary duct ectasia and fibrosis of both liver and kidney. The phenotypes of the ARPKD are distinguished by the age of the diagnosis at presentation and the predominance of renal over hepatic manifestations or vice versa. Sonography is currently the imaging technique of choice in children with renal cystic disease. Classic ARPKD is evident at birth with symmetrically enlarged kidneys with increased Parenchymal echogenicity at ultrasound with using a curved array transducer. In previous reports, nephromegaly is a constant imaging finding at the time of diagnosis. Asymmetrical enlargement has only been reported in one case report and the current case report is the second observation in this regard.
