Renal dysgenesis and hypoplasia
What is it?
Renal dysgenesis include renal agenesis and renal hypoplasia. Renal agenesis is a condition in which a baby is missing one or two kidneys, as the kidney bud fails to develop in early stages of pregnancy. Bilateral kidney agenesis is much rarer than unilateral kidney agenesis. Babies with bilateral kidney agenesis usually have a syndrome called Potter Syndrome and do not survive. They could also have a low set of ears, widely separated eyes, small chin, and defects of the arms and legs. Risk factors include maternal diabetes, young maternal eye, pregnancy obesity and alcohol use during pregnancy. Some cases are due to genetic mutations which can be tested prenatally. Renal hypoplasia is a condition in which only a part of the kidney fails to develop, causing small kidneys with reduced number of nephrons. Kidney hypoplasia may also be a result of chronic pyelonephritis, chronic glomerulonephritis, renovascular accident or nephrolithiasis, genetic mutations, and radiation therapy.
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Additional names
This group contains additional names:
- Congenital hypoplasia of kidney
- Potter syndrome
- Renal agenesis
- Single kidney
Signs & symptoms
Renal agenesis symptoms may include problems in the baby’s lungs, genitals, urinary tracts, stomach, intestines, heart, muscles, bones, eyes and ears. High blood pressure, protein in urine or blood, edema of the legs, face and hands. Renal hypoplasia can also be associated with abnormalities in the ureter, chronic urinary infection, nephrolithiasis, chills and fever.
Diagnosis
Renal agenesis or hypoplasia is conclusively diagnosed only through direct assessment by abdominal ultrasound, CT or MRI scan, surgery, or autopsy. Bilateral renal agenesis should be considered in an infant with features of Potter sequence. Also, during pregnancy, the mother can be diagnosed with oligohydramnios, due to lack of fetus urine.
Treatment
Treatment depends on the severity of renal agenesis. Babies with bilateral renal agenesis usually die within the first few days but if they survive, dialysis is required. For unilateral renal agenesis, or hypoplasia recommendations include eating a healthy diet, salt intake restriction, staying hydrated, blood pressure control and avoiding medications such as NSAIDS.
☝️ This is not a substitute for professional medical advice. Please consult with your physician before making any medical decision.
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