Horseshoe Kidney

What is a horseshoe kidney?

A horseshoe kidney is a congenital urological anomaly where you have one larger, horseshoe-shaped kidney instead of two smaller kidneys. “Congenital” means that you have the condition at birth. During fetal development, the kidneys join to form a shape like a horseshoe or the letter U. Another name for horseshoe kidney is “renal fusion.”

In all fetuses, the kidneys first form in the lower belly. They then typically move up from the pelvic area and toward the back, on both sides of the spine. With a horseshoe kidney, instead of moving into the typical position around the spine, the kidneys attach at their lower end and sit lower in the pelvis, closer to the front of your body.

How serious is a horseshoe kidney?

A horseshoe kidney usually doesn’t cause serious health problems. If you have a horseshoe kidney, you can still live an active lifestyle.

Can you live long with a horseshoe kidney?

For most people, a horseshoe kidney won’t affect life expectancy. But you may be at a higher risk of developing kidney (renal) cancer.

In the U.S., healthcare providers diagnose more than 62,000 people each year with kidney cancer. With early diagnosis and treatment, the 5-year survival rate for kidney cancer is 77%.

How common is horseshoe kidney?

Horseshoe kidney affects about 1 in 500 people. People assigned male at birth (AMAB) are more likely to have a horseshoe kidney than people assigned female at birth (AFAB).

What other conditions are associated with horseshoe kidney?

A horseshoe kidney can develop on its own. But it may also often occur alongside other conditions, including:

Chromosomal conditions

• Down syndrome.
• Turner syndrome.
• Pautau syndrome (trisomy 13).
• Edwards syndrome (trisomy 18).

Nonchromosomal conditions

• Ellis-van Creveld (EVC) syndrome.
• Fanconi anemia (FA).
• Goltz syndrome.
• Kabuki syndrome.
• Pallister-Hall syndrome.
• VATER syndrome.

What are the symptoms of horseshoe kidney?

Most people have no symptoms of a horseshoe kidney. If you do, they may include:

• Nausea and vomiting.
• Frequent urinary tract infections (UTIs), which may also cause pain when you pee (dysuria) along with a fever.
• Kidney stones, which may cause abdominal (belly) pain, blood in your pee (hematuria) and pain when you pee.

What causes horseshoe kidney?

Healthcare providers and medical researchers aren’t sure what causes horseshoe kidney. It relates to a problem with how your genes tell your kidneys to form during fetal development.

What is the most frequent complication of horseshoe kidney?

The most frequent complication of horseshoe kidney is ureteropelvic junction (UPJ) obstruction. UPJ obstruction is a blockage in the upper part of your ureter (renal pelvis). Your ureters are tubes of muscle that connect your kidney to your urinary bladder. UPJ obstruction occurs in about 33% of people who have a horseshoe kidney.

Other horseshoe kidney complications include:

• Vesicoureteral reflux (VUR). 
• Hydronephrosis.
• Polycystic kidney disease (PKD).
• Renovascular hypertension.

You’re also more likely to develop kidney cancer. It’s a good idea to be aware of kidney tumor signs if you have a horseshoe kidney. These signs may include:

• Blood in your pee.
• A bulge or lump (mass) in your abdomen.
• Flank pain.

How is a horseshoe kidney diagnosed?

Many times, a healthcare provider will notice a horseshoe kidney while diagnosing or treating another condition (incidental finding). If you see a provider because of kidney-related symptoms, they’ll perform a physical exam. They may also order a pee test (urinalysis) to check for blood or other elements in your pee. They may order kidney function tests, as well, to see how well your kidneys are working. These may include:

• Estimated glomerular filtration rate (eGFR).
• Blood urea nitrogen (BUN) test.
• Creatine clearance test.

They may also order imaging tests, such as:

• Kidney ultrasound.
• Voiding cystourethrogram (VCUG).
• Radionuclide scan.
• X-rays.
• Magnetic resonance imaging (MRI).
• Computed tomography (CT) scan.

Does horseshoe kidney need treatment?

It depends. There’s no cure for horseshoe kidney. So, if you don’t have any symptoms, you may not need any treatment. But if you have symptoms, treatment can help. Options may include:

• Antibiotics to treat a bacterial infection such as a UTI.
• Surgery to remove kidney stones or make it easier to pee.

Can you remove a horseshoe kidney?

Healthcare providers typically don’t operate to remove (nephrectomy) or separate horseshoe kidneys. Horseshoe kidneys are benign (not cancer). Many people live long and healthy lives with them.

How can I prevent horseshoe kidney?

There’s no known way to prevent horseshoe kidney. But it’s important to remember that you did nothing wrong during your pregnancy to cause your child to develop a horseshoe kidney. It’s a condition that sometimes happens, and healthcare providers aren’t sure why.

What can I expect if I have a horseshoe kidney?

In most cases, the outlook for horseshoe kidney is good. You may need ongoing care to manage symptoms. But it doesn’t usually cause serious health problems, and it shouldn’t prevent you from doing the activities you wish to do.

Healthcare providers generally recommend regular checkups and ultrasounds to monitor your kidney health and check for signs of kidney cancer. You’ll work with a care team to learn about horseshoe kidney and the best ways to take care of yourself. A care team may include:

• A doctor who treats children (pediatrician).
• A kidney specialist (nephrologist).
• A urinary system specialist (urologist).
• Other specialists as necessary.

How do I take care of myself if I have horseshoe kidney?​

Because a horseshoe kidney is closer to the front of your body than a typical kidney, there’s a higher chance that you may damage it while playing sports or due to another physical injury. If you have a horseshoe kidney, it’s a good idea to:

• Wear a medical alert bracelet. A medical alert bracelet lets healthcare providers and emergency responders know to be aware of possible kidney damage in the event of an accident or other trauma.
• Avoid contact sports. This is especially important for children. Contact sports such as football, martial arts, lacrosse and baseball could injure your kidney. If you (or your child) play a sport or engage in an activity that may increase your risk of injury, it’s a good idea to wear special padding (a kidney guard or kidney shield) around your abdomen.

When should I see a healthcare provider?

Reach out to a healthcare provider if you or your child has symptoms that affect the kidneys, especially abdominal pain, kidney stones or frequent UTIs.

What questions should I ask a healthcare provider?

Questions you may want to ask your healthcare provider include:

• How do you know that I have a horseshoe kidney?
• What treatment do you recommend?
• Will I need surgery?
• Should I get screened for kidney cancer?
• Do I need other preventive tests?
• Should I get a medical alert bracelet?
• How else can I keep my kidney healthy?

Can you donate a kidney if you have a horseshoe kidney?

Yes, you can donate a horseshoe kidney. But it isn’t common. If a deceased organ donor has a horseshoe kidney, healthcare providers may divide the horseshoe kidney and transplant it into two separate recipients. They may also transplant a horseshoe kidney to one recipient, but it depends on the anatomy; that is, whether the horseshoe kidney and blood vessels “fit” in the donor.

A note from Cleveland Clinic

If your child has a horseshoe kidney, it’s important to remember that you’re not to blame. Healthcare providers don’t know what causes it. Many people who have a horseshoe kidney don’t experience symptoms. However, complications may occur, including frequent UTIs and kidney stones. There’s no cure for a horseshoe kidney. But it usually won’t affect your child’s life expectancy, and treatment can help relieve the symptoms of related complications. However, you may need to take some extra precautions to avoid damage or injury to the kidney.

Talk to a healthcare provider if you or your child has a horseshoe kidney and has frequent UTIs, urinary changes and/or abdominal pain. They can answer your questions and recommend the proper necessary treatment.