Autosomal recessive polycystic kidney disease (ARPKD) in fetus: Autopsy based approach

Polycystic kidney disease is a rare developmental anomaly inherited as Autosomal dominant or recessive. Autosomal recessive polycystic kidney disease (ARPKD) is an intractable cystic renal disease that results in chronic renal failure. It has a profound effect on growing fetus and result in serious implications if pregnancy is continued in the long run after being detected on sonography. Although prenatal imaging studies and clinical findings are suggestive of ARPKD it can be accurately diagnosed by histopathology if an autopsy is performed in cases of infant death. In this article we here in present the features of ARPKD diagnosed antenatally by USG in a 22 yr female, and was confirmed further by fetal autopsy.