Bilateral renal agenesis

Bilateral renal agenesis is the congenital absence of both kidneys and ureters. Also known as Potter syndrome, it is a lethal anomaly in which absence of the kidneys results in lack of amniotic fluid and pulmonary hypoplasia, along with characteristic facies and positional extremity abnormalities. Fetal urine production is the major source of amniotic fluid production after 18 weeks’ gestation, and a lack of amniotic fluid after this gestational age supports the diagnosis. When the kidneys are absent, the adrenal glands fill the renal fossae bilaterally, and color Doppler imaging of the descending aorta fails to demonstrate either renal artery. Associated anomalies and aneuploidy are common. Bilateral renal agenesis is also a component of several genetic syndromes. First-degree relatives may be at increased risk for other renal anomalies.