Kidney Dysplasia: Understanding Fetal Kidney Development
Updated: 2 days ago
Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: October 2026
Quick Answer: Key Facts About Kidney Dysplasia
Kidney dysplasia, also called renal dysplasia, is a condition in which a baby's kidney tissue develops abnormally, affecting an estimated 2 to 4 out of every 1,000 babies. Its cause is usually unknown, and it can affect one or both kidneys. It is found on prenatal ultrasound, and treatment manages complications such as UTIs and high blood pressure.
Feature | Details |
Common name | Kidney dysplasia, also called renal dysplasia or dysplastic kidneys. [1] |
Prevalence | An estimated 2 to 4 out of every 1,000 babies worldwide. [1] |
Causes | Usually unknown. Known causes are genetic or environmental (certain medicines or drugs taken during pregnancy). [1] |
Types | Unilateral (one kidney, about twice as common) or bilateral (both kidneys). [1] |
Diagnosis | Prenatal ultrasound, confirmed by an ultrasound after birth and kidney function tests. [1] |
Treatment focus | Managing complications such as UTIs, high blood pressure and chronic kidney disease. [1] |
Long-term goal | Monitoring and protecting the kidney tissue that works. [1] |
What the research says
Most children with a multicystic dysplastic kidney do well without surgery. A systematic review of 44 cohort studies (2,820 patients) found a 3.2% risk of hypertension associated with a multicystic dysplastic kidney (27 of 838 patients), a malignancy risk of 0.07% (2 of 2,820) and signs of involution (shrinking) of the dysplastic kidney in 53.3% (1,502 of 2,820). The authors concluded that conservative, non-operative management is appropriate. (Chang et al., J Pediatr Urol, 2018) [2]
It is one of the most common anomalies seen before birth. A review describes multicystic dysplastic kidney as the most common cause of cystic kidney disease in children, with an incidence of 1 in 1,000 to 4,300 live births and a benign course with few sequelae. The key clinical questions are whether the other kidney has anomalies and how to follow up with imaging. (Cardona-Grau and Kogan, Curr Urol Rep, 2015) [3]
Genes explain a minority of cases, so many causes are still unknown. A review of kidney and urinary tract malformations (CAKUT) reports that almost 50% of chronic kidney disease appearing in the first three decades of life is caused by CAKUT. Mutations in about 54 genes cause CAKUT and account for 12% to 20% of cases, and copy number variants are found in 4% to 11% of patients. Environmental and epigenetic factors can also raise risk. (Kolvenbach et al., Nat Rev Nephrol, 2023) [4]
What Is Kidney Dysplasia?
Kidney dysplasia is a condition in which one or both kidneys do not develop the way they should while a fetus grows in the uterus. It is one of several congenital anomalies of the kidney and urinary tract (CAKUT). If your child has kidney dysplasia, the normal structures in the kidney do not develop properly, are not organized in the usual way, contain fewer filtering units (nephrons) and may contain types of tissue that are not usually in the kidney, such as cartilage. [1]
The kidneys of a child with dysplasia are also usually smaller than normal (renal hypodysplasia). They may contain fluid-filled sacs called cysts, which can make them look larger than they should on an ultrasound. [1]
What are the types of kidney dysplasia?
The impact of kidney dysplasia depends largely on whether it affects one or both kidneys. [1]
Unilateral kidney dysplasia: this affects one kidney and is about twice as common. The remaining kidney usually gets bigger and stronger to make up for the dysplastic kidney, and many children never have health problems from it.
Bilateral kidney dysplasia: this affects both kidneys and is more serious. These children have a higher chance of chronic kidney disease, and kidney function can be very poor at birth. Severe cases in both kidneys can be fatal.
What causes kidney dysplasia and who is at risk?
In most cases, experts do not know what causes kidney dysplasia. There are two main categories of known causes: [1]
Genetic factors: a problem with how genes direct the kidneys to form. It can occur on its own or as part of a larger condition involving other organs.
Environmental factors: taking certain medicines or drugs during pregnancy, including antiseizure medicines, blood pressure medicines such as ACE inhibitors and ARBs, and cocaine.
A baby's risk is higher if there is a biological family history of CAKUT or if the mother has a history of kidney disease or diabetes. Kidney disease also affects males slightly more than females. It is a good idea to talk to a healthcare provider about any medicines you take if you are pregnant or planning to become pregnant. [1]
What are the signs and symptoms of kidney dysplasia?
Kidney dysplasia itself usually has no symptoms. If problems develop in the urinary system along with it, symptoms can include urinary tract infections (UTIs), blood in the urine (hematuria), fever and abdominal pain. [1]
If severe, untreated kidney failure leads to a buildup of waste products in the blood (uremia), symptoms can include fatigue, nausea and vomiting, loss of appetite, swelling (especially around the ankles, hands or face), changes in how often a child pees, and dry or itchy skin. [1]
How is kidney dysplasia diagnosed and monitored?
Healthcare providers most often diagnose kidney dysplasia during a prenatal ultrasound and confirm the diagnosis with an ultrasound after birth. They also order kidney function tests to see how well the kidneys work. [1]
Once kidney dysplasia is identified, providers may refer your child to a genetics team, especially if they find other abnormalities. The team decides whether genetic testing is indicated and explains what the results mean. [1]
How is kidney dysplasia managed and treated?
Kidney dysplasia cannot be treated or reversed by any current therapy, so care focuses on its complications. Treatments only address complications such as UTIs and chronic kidney disease (CKD). Your child may not need treatment if they have one dysplastic kidney and the other kidney is healthy. [1]
Unilateral dysplasia: if the other kidney is healthy, regular monitoring may be all that is needed.
Managing CKD complications: this can include managing high blood pressure, anemia (low levels of healthy red blood cells), poor growth and high protein levels in the urine (proteinuria).
Severe cases: severe kidney dysplasia that causes kidney failure requires dialysis or a kidney transplant.
Schedule regular appointments with a pediatric nephrologist (a kidney specialist for children) to monitor your child's kidney health. Your child should start seeing one shortly after birth, and how often depends on their kidney function and any other complications. [1]
What is the long-term outlook for kidney dysplasia?
The outlook depends on the case. If your child has mild dysplasia in one kidney, they can grow and develop with few or no problems, and the unaffected kidney grows bigger and stronger to compensate. Regular checkups with a pediatric nephrologist help make sure that kidney stays healthy. [1]
Fetuses with severe dysplasia in both kidneys may not live long after birth. If they do, they will likely develop kidney failure and need dialysis or a kidney transplant. To help protect a healthy kidney, work with the treatment team on regular appointments, giving medicines exactly as prescribed, monitoring blood pressure, explaining the dangers of tobacco and alcohol to the kidney as your child grows, and a healthy lifestyle with a healthy weight and a heart-healthy diet. [1]
Kidney dysplasia cannot currently be prevented. [1]
Conclusion
Learning that your child has a congenital condition can be frightening, but many children with dysplasia in only one kidney lead healthy lives. Early diagnosis, regular monitoring with a pediatric nephrologist and prompt treatment of complications protect the kidney tissue that works. Healthcare providers understand how you feel and can support you at every step. [1]
Learn more. For more evidence-based health guides, visit Rinnit.com.
Frequently Asked Questions
Can kidney dysplasia be prevented?
There is currently no way to prevent kidney dysplasia. Because some medicines taken during pregnancy are linked to it, talk to your provider about any medicine you take if you are pregnant or planning to become pregnant.
Does a dysplastic kidney always have cysts?
No. Some dysplastic kidneys contain fluid-filled cysts, and a multicystic dysplastic kidney is largely replaced by them, but others are simply small and disorganized.
Will my child need surgery?
Most children with a single dysplastic kidney do not need surgery, and a systematic review found that conservative management is appropriate. Your child's specialists may consider surgery if there are complications such as a mass effect on nearby organs or other problems in the urinary tract.
Can a child with one dysplastic kidney play sports?
Many children lead active lives. Ask your pediatric nephrologist about contact sports and any precautions to protect the working kidney.
Is kidney dysplasia the same as kidney cancer?
No. Kidney dysplasia is a developmental condition, not a cancer.
How often does my child need to see a specialist?
It depends on their kidney function and any other complications. Your pediatric nephrologist will set the schedule.
Can kidney dysplasia be seen before birth?
Yes. Healthcare providers most often diagnose it during a prenatal ultrasound and confirm it with an ultrasound after birth.
Will the dysplastic kidney eventually disappear?
Sometimes. In a systematic review of children with multicystic dysplastic kidney, about half showed involution (shrinking) of the dysplastic kidney.
Does kidney dysplasia cause pain?
The dysplasia itself usually has no symptoms. Pain or fever can signal a complication, such as a urinary tract infection, and should be checked by a provider.
Can my child eat a normal diet?
Many children with one dysplastic kidney can follow a healthy, heart-healthy diet. If kidney function is reduced, ask your nephrologist whether dietary changes are needed.
Is it safe to have more children if one has kidney dysplasia?
Many parents go on to have healthy children. If a genetic cause is suspected, a genetics team can help you understand the chances for future pregnancies.
What is the difference between dysplasia and hypoplasia?
Dysplasia means the internal structure of the kidney developed abnormally and is disorganized. Hypoplasia means the kidney is small. A dysplastic kidney is also often smaller than normal, which is called hypodysplasia.
Can a dysplastic kidney start working normally?
No. There is currently no therapy to treat or reverse kidney dysplasia, and the care team focuses on protecting the kidney tissue that works.
Should I monitor my child's blood pressure at home?
Your nephrologist will tell you whether home monitoring is needed. Monitoring blood pressure is an important part of protecting the healthy kidney.
What are the early signs of a UTI in a baby?
Signs can include unexplained fever, irritability, poor feeding or unusually foul-smelling urine. Contact your child's provider promptly, because UTIs can be a complication of kidney dysplasia.
References
Cleveland Clinic. Kidney Dysplasia. Cleveland Clinic. Last updated January 20, 2026. https://my.clevelandclinic.org/health/diseases/kidney-dysplasia
Chang A, et al. Evidence-based treatment of multicystic dysplastic kidney: a systematic review. J Pediatr Urol. 2018;14(6):510-519. PMID 30396841. https://doi.org/10.1016/j.jpurol.2018.09.018
Cardona-Grau D, et al. Update on multicystic dysplastic kidney. Curr Urol Rep. 2015;16(10):67. PMID 26255066. https://doi.org/10.1007/s11934-015-0541-7
Kolvenbach CM, et al. The genetics and pathogenesis of CAKUT. Nat Rev Nephrol. 2023;19(11):709-720. PMID 37524861. https://doi.org/10.1038/s41581-023-00742-9
Medical disclaimer
This article is for general educational purposes only and is not a substitute for professional medical advice. Always seek the advice of your child's pediatrician, pediatric nephrologist or other qualified health provider with any questions about a medical condition.
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