Renal agenesis, bilateral
Bilateral Renal Agenesis is a rare medical condition characterized by the absence of both kidneys at birth. This condition is a type of renal agenesis, which can also occur unilaterally (affecting only one kidney). Bilateral renal agenesis is a critical condition, as the kidneys play a crucial role in filtering waste products and excess fluids from the blood, which are then excreted in the urine.
Causes and Pathophysiology
The exact cause of bilateral renal agenesis is not fully understood, but it is believed to involve genetic and environmental factors. During fetal development, the kidneys form from the intermediate mesoderm. Disruption in the signaling pathways or genetic mutations can prevent the kidneys from developing. This condition is often associated with a range of other congenital anomalies, including issues with the lungs, heart, and urinary tract.
Diagnosis
Bilateral renal agenesis is typically diagnosed through prenatal ultrasound. The absence of kidneys and amniotic fluid (oligohydramnios) can indicate the condition. Oligohydramnios occurs because the fetus plays a significant role in producing amniotic fluid, primarily through urination. The lack of amniotic fluid can lead to further complications, including lung hypoplasia (underdeveloped lungs).
Prognosis
The prognosis for infants born with bilateral renal agenesis is poor. The condition is usually fatal shortly after birth due to the inability to remove waste products from the body and severe lung underdevelopment. There is currently no cure for bilateral renal agenesis, and treatment options are limited to supportive care.
Epidemiology
Bilateral renal agenesis is a rare condition. The exact incidence is difficult to determine but is estimated to occur in 1 in 3,000 to 4,000 pregnancies. The condition appears to affect males more frequently than females.
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Contributors: Prab R. Tumpati, MD