Bilateral Renal Agenesis is the absence of both kidneys at birth. It is a genetic disorder characterized by a failure of the kidneys to develop in a fetus. This absence of kidneys causes a deficiency of amniotic fluid (Oligohydramnios) in a pregnant woman. Normally, the amniotic fluid acts as a cushion for the developing fetus. When there is an insufficient amount of this fluid, compression of the fetus may occur resulting in further malformations of the baby. This disorder is more common in infants born of a parent who has a kidney malformation, particularly the absence of one kidney (unilateral renal agenesis).
HOW IS RENAL AGENESIS DIAGNOSED?
Renal agenesis is typically diagnosed during routine prenatal ultrasounds. When BRA is identified, prenatal MRI can be used to confirm the complete absence of both kidneys.
HOW IS RENAL AGENESIS TREATED?
Most individuals with unilateral renal agenesis lead normal lives although there is an increased risk of renal infections, nephrolithiasis, hypertension and/or renal failure. No specific treatment is necessary. Bilateral agenesis is lethal with pulmonary hypoplasia being the most common cause of death. Bilateral renal agenesis is said to carry a recurrence risk of 3% for sporadic cases.
DID YOU KNOW?
Newborns affected with BRA typically have distinct features that include:
• widely separated eyes with skin folds over the upper eyelids
• ears that are set low
• a nose that is pressed flat and broad
• a receding chin
• limb defects