How are Renal Dysplasia and Cystic Disease Treated?

Most of these conditions have no single treatment, and the focus is on managing the symptoms. You should talk about choices with your health care provider.

Renal Agenesis

  • For infants and children, there is no treatment when only one kidney is affected. Treatment will depend on other issues present.
  • Dietary changes can sometimes help. A low blood-glucose or a low-sodium diet may be recommended. Your health care team and/or a dietitian trained in kidney disease would offer guidance.
  • Restricting contact sports is recommended, along with avoiding activity that places you at risk for injury (e.g., motorcycle riding, and use of all-terrain vehicles and trampolines).
  • Regular monitoring should be done to look for changes in the kidneys.

Renal Dysplasia

  • Treatment may only include symptom management.
  • Monitoring should include blood pressure checks, kidney function tests, and urine testing for protein.
  • Periodic ultrasound can be used to make sure the other kidney continues to grow normally and no other problems develop.
  • Antibiotics may be needed for urinary tract infections.
  • The kidney should be removed only if it causes pain or high blood pressure, or ultrasound is abnormal.

Renal Hypoplasia

  • Any related ureteral problems should be managed to lower the risk of infection and the rapid loss of kidney function. Unfortunately, this will not undo the process of hypoplasia.
  • Treatment involves keeping up fluid and electrolyte balance, and correcting acidity levels in the blood. Your health care team or a trained dietitian can help.

Autosomal Recessive Polycystic Kidney Disease (ARPK)

  • Management focuses on supportive measures, such as dialysis for kidney failure.
  • If the organs are enlarged, some children benefit from removing them. This is done mostly if the size causes trouble with breathing, feeding or dialysis.

Autosomal Dominant Polycystic Kidney Disease (ADPK)

  • High blood pressure occurs in about 60% of patients with this disease. Blood pressure should be managed to prevent heart disease and brain hemorrhages or stroke.
  • More than half of patients with ADPK experience pain, which can be intense, sudden and last a long time. The pain can be from kidney stones, blood clots, or an infection. Treating the pain depends on its cause.
  • Infections are often difficult to treat due to the limited strength of antibiotics in the cyst fluid. Women with ADPK are at higher risk for urinary tract infections. If the infection does not respond to drugs, your doctor may suggest draining the cyst.
  • Needle aspiration, laparoscopy or open surgery can drain cysts. While draining the cysts can be helpful, fluid will likely collect again. In any case, patients are followed closely since the disease can get worse and renal failure can occur.

Juvenile Nephronophthisis and Medullary Cystic Disease Complex

  • Because these patients are very thirsty and have water loss, they will need to replace the salt in their diets.
  • Kidney failure will likely occur, which will call for supportive measures (like dialysis).

Renal Cystic Disease Syndromes

Tuberous Sclerosis

  • Some symptoms can be treated and you should work with your doctor to manage them in the best way.
  • Angiomyolipomas may need to be treated when they get larger to reduce the risk of spontaneous bleeding.
  • Drugs may be used to control seizures and help with behavior problems.

Von Hippel-Lindau Disease

  • When solid lesions are larger than 4 cm, surgery is usually recommended to remove the tumors in the kidney (partial nepohrectomy).

Multicystic Dysplastic Kidney (MCDK)

  • The majority of these kidney shrink in size over time until they are no longer seen on ultrasound. Some are big enough and can push on the stomach and diaphragm and cause vomiting and difficulty breathing, respectively. In these cases removal of the kidney surgically may be required.

Simple Renal Cyst

  • The vast majority of simple renal cysts are asymptomatic and are thus observed.
  • If you have symptoms your urologist may drain or remove the cyst.

Medullary Sponge Kidney

  • The focus is on avoiding the development of kidney stones and urinary infections.
  • Increase water intake may help lower calcium output and limit stone formation.
  • If you have urinary infections that keep coming back, long-term, low dose antibiotics may help.

Acquired Renal Cystic Disease

  • Surgery is usually only required if there is a solid renal tumor suspicious for a renal cancer.