Autosomal Recessive Polycystic Kidney Disease : Potter's Facies

Autosomal Recessive Polycystic Kidney Disease (ARPKD) - Clinical Features: (continued from the previous two images). ARPKD kidneys are massively enlarged but often non-functioning with low urine output leading to oligohydroamnios. The pressure of the uterus on the developing fetus leads to a peculiar physical characteristics called Potter's sequence consisting of limb anomalies (contracted limbs, clubbed feet), pulmonary hypoplasia, and Potter's facies (low-set ears, flattened nose, receding chin, prominent epicanthal folds, widely separated eyes). Potter's sequence is not limited to ARPKD and can be seen with oligohydroamnios associated with bilateral renal agenesis, renal hypoplasia, amniotic rupture and many other conditions.