Bladder Exstrophy

Young women's version of this guide
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utiBladder exstrophy is a rare condition that affects males and females. Only one out of every 20,000 babies born will have bladder exstrophy, and there is no known cause. This guide is intended as an overview for teen guys and young men with who have been diagnosed with his condition.

What is bladder exstrophy?

Bladder exstrophy (also called “classic exstrophy”) happens while a baby is developing inside the mother’s womb. The bladder develops inside out and is visible when the baby is born. Since the bladder is exposed to the outside, urine constantly trickles onto the skin, causing irritation.

This condition affects the bladder and may affect other parts of the body, such as the:

  • Entire urinary tract
  • Muscles
  • Bones
  • Digestive system
  • External genitalia (private parts)

Epispadias and cloacal exstrophy are conditions that are closely related to bladder exstrophy.

What is epispadias?

Epispadias is almost always seen with bladder exstrophy. Some babies are born with epispadias without bladder exstrophy. Epispadias occurs when the urethra (tube that drains urine from the bladder to the outside of the body) is not fully closed. Because the tube is open where it should be closed, usrine can leak from wherever the opening occurs. In boys, the opening is usually on the top of the penis and not at the tip.

What is cloacal exstrophy?

Cloacal exstrophy happens when a baby is born with a portion of his/her intestines and the bladder on the outside of the abdomen (belly). The bladder is split in half with the two sides attached to the intestines.

What causes bladder exstrophy/epispadias?

Some studies have found that this condition may be inherited, but the gene has not been identified. A baby born to a parent who had exstrophy has a 1/77 chance of having exstrophy.

The urinary system: Knowing the basics of how the urinary system normally works can help you understand the treatments for bladder exstrophy.

Front View of Urinary Tract

Here are the parts of the urinary system and what they do:

Kidneys: A pair of purplish-brown organs located below the ribs toward the middle of the back.

Their function is to:

  • Remove liquid waste from the blood in the form of urine
  • Keep a balance of salts, water, and other substances in the blood
  • Make erythropoietin, a hormone that helps make red blood cells form

Ureters: Two narrow tubes that carry urine from the kidneys to the bladder. If urine backs up, or is allowed to stand still, bacteria can grow and a kidney infection can develop.

Bladder: A balloon-shaped organ in the lower abdomen (belly) that stores urine. The bladder muscle squeezes to empty the urine when a person pees.

Sphincter muscles: Two circular muscles that help keep urine from leaking by closing tightly like a rubber band around the opening of the bladder. The sphincter muscles relax when a person urinates (pees).

Nerves in the bladder: Signal the body when it’s time to urinate or empty the bladder

Urethra: A tube that is attached to the bladder and allows urine to exit the body

What are the treatments for bladder exstrophy?

Surgery: The first treatment for bladder exstrophy is surgery to place the bladder inside the body. The surgery is almost always done early in life, soon after a baby is born.

After surgery patients require ongoing care, which may include:

  • Medications
  • Catheterization (passing a tube to empty urine from the bladder)
  • More surgery is sometimes needed

The goals of surgical treatment for bladder exstrophy are:

  • Normal kidney function
  • Good bladder function
  • Urinary continence, meaning no leakage of urine
  • Making sure that the patient and their parents are satisfied with the appearance and function of their genitalia (private parts)

Medicine: Many people with bladder exstrophy need to take medicine to help the bladder work correctly. A person may also need to take medicine to prevent bladder and kidney infections.

If you have bladder exstrophy, it’s important to take your medications as prescribed by your health care provider, even if you’re feeling fine. This is because many of the medications prevent problems from happening.

Two types of medications used for teens and young adults with bladder exstrophy are:

  • Anticholinergics: These medicines relax the bladder, help the bladder hold urine longer, and take pressure off of the kidneys.
  • Antibiotics: These medicines treat and prevent infections of the urinary system.

Catheterization: Many patients with bladder exstrophy have trouble emptying their bladders completely and require help using catheterization.

Catheterization involves passing a tube through the urethra (or a surgically created opening in the abdomen) so that urine can be emptied from the bladder. It’s usually done on a set schedule, multiple times per day (similar to if you were to urinate on your own). It’s important to stay on the schedule your health care provider gives you, even if your bladder doesn’t feel full.

Penis Size/Shape: Most boys and men with exstrophy have a shortened penis. Some young men also have widening and/or curvature of the penis.

Going to the bathroom in public: Many guys with exstrophy prefer to use a stall instead of a urinal to “cath” (catheterize). If you have bladder exstrophy, don’t feel ashamed of using a stall – most guys in the same situation use one too.

If you use a catheter, one easy way to keep it private is to fold the tube and place it in a snack-sized plastic bag that can be sealed and carried in a backpack or pocket.

Sexual Activity for Guys with Bladder Exstrophy:

  • Most guys with bladder exstrophy are able to have a normal sex life and are able to have children.
  • Some guys prefer specific sexual positions and/or medicine to help them get a full erection.
  • Sometimes guys may have trouble using a condom due to the shape of the penis. If you’re having trouble, talk to your health care provider.
Remember: If you’re sexually active, it’s important to protect yourself and your partner from STIs. If you have a female partner, always use protection to prevent unwanted pregnancy.

Bladder Exstrophy Health Tips:

Following your treatment plan will:

  • Lessen your chances of getting a urinary infection and keep your kidneys working well
  • Prevent unscheduled clinic or ER visits and overnight hospital stays
  • Avoid having to take extra medicine
  • Lower the risk of bladder rupture
  • Help you feel well so that you can participate in fun activities

Ways to keep your bladder healthy:

  • Drink plenty of fluids
  • Catheterize and/or urinate at least 5 times per day
  • Empty your bladder before playing sports or going on a long car ride
  • Take medications as instructed, even if you feel fine
  • Flush your bladder with saline or antibiotic wash (only if your health care provider tells you to)

Call your health care provider right away if you have any of the following symptoms:

  • Fever greater than 101.5°F
  • You can’t empty your bladder by urinating or catheterizing
  • Severe pain in your abdomen (belly) or back
  • Nausea or vomiting that doesn’t go away
  • You’re unable to have a bowel movement (poop) for more than 2 days

If you have any of the symptoms listed above and you can’t reach your health care provider, go to the nearest emergency room.

Additional Resources: