Studies have proven that unilateral renal agenesis and bilateral renal agenesis are genetically related.

Bilateral renal agenesis (BRA) was first recognized as a defect of human fetal development in 1671 by Wolfstrigel.

Classic Potter sequence occurs when the developing fetus has bilateral renal agenesis, which also presents with agenesis of the ureters.

Bilateral renal agenesis is the uncommon and serious failure of both a fetus' kidneys to develop during gestation, and is one causative agent of Potter sequence.

It is typically caused by fetal urinary tract abnormalities such as bilateral renal agenesis (Potter's syndrome), fetal polycystic kidneys, or genitourinary obstruction.

Kidney Agenesis Renal Agenesis None Bilateral Renal Agenesis is the absence of both kidneys at birth.

It can be caused by renal diseases such as bilateral renal agenesis (BRA), atresia of the ureter or urethra causing obstruction of the urinary tract, polycystic or multicystic kidney diseases, renal hypoplasia, amniotic rupture, uteroplacental insufficiency from maternal hypertension or toxemia.

In a female fetus with a normal karyotype and cryptophthalmos, ambiguous external genitalia, syndactyly, bilobed lungs, bilateral renal agenesis, hypoplastic bladder, and agenesis of internal genitalia with streak ovaries, Shafeghati et al. (2008) identified homozygosity for a splice site mutation in the FREM2 gene (608945.0002).