What is bladder exstrophy

The experts in the Bladder Exstrophy Program at Boston Children's Hospital are dedicated to providing exceptional pediatric care. Our team includes physician specialists in urology, nephrology, orthopedic surgery, anesthesiology, radiology, and nuclear medicine, as well as nurses, social workers , and child life specialists who will care and support you and your child at every step, from diagnosis through treatment , and even after your child returns home.

We are always here to answer any questions and to listen to your concerns. The MFCC provides support through prenatal counseling, treatment, and follow-up for families with a confirmed or suspected fetal congenital anomaly.

In addition, medical staff, social workers, child life specialists, and chaplaincy services are available to support the expectant mother and her family. For Patients. Contact the Department of Urology Fax Bladder Exstrophy and Epispadias. What is bladder exstrophy? There is no clear cause for this condition. It is thought to happen during the 11 th week of pregnancy, as the organs develop.

Some experts believe that the bladder defect occurs at the times the tissues in the lower wall of the belly or abdomen develop. At the same time, the developing muscles and pelvic bones are affected too.

See More See Less. A temporary tissue called the cloacal membrane covers the lower belly wall and is replaced by maturing and developing abdominal muscles. If the cloacal membrane bursts before the abdominal muscles fully form, this may result in an "exstrophied" bladder.

Another tissue called the urorectal septum helps to separate the developing bladder from the bowels or intestines. Whether the child is born with epispadias , classic bladder exstrophy, or cloacal exstrophy depends on when the cloacal membrane bursts and if the bladder and intestines are separated by the urorectal septum.

Mostly, bladder exstrophy occurs as the only birth defect. Spinal cord problems can also occur but are more common with cloacal exstrophy. Bladder exstrophy can often be found before birth during a routine sonogram.

In any case, this condition will be clear at birth. If this condition has been noticed before birth, plans are often made for immediate treatment at birth.

A pediatrician and surgeon can check the baby right away. The doctor will note the bladder size and quality, the shape of the pelvis, and the state of the outer sex organs.

A surgeon will be contacted for treatment. Bladder exstrophy is treated with surgery. The type of surgery used depends on how severe the defect is. It is critical to work with a surgeon who is experienced with treating exstrophy experience. Advances over the last 15 years have led to successful reconstruction. This includes reconstruction of the penis and bladder for a more "normal" and functional lifestyle.

One form of treatment is "staged reconstruction. When the bladder's quality is good, and the penis size is good at birth, closing the bladder and penile reconstruction can be done in a "single operation" at an early age.

Both early and staged reconstruction have good results. If the bladder has grown enough and the surgeon is skilled, continence control over urination is possible. Often, further operations are needed over time to improve the child's ability to urinate. A urethral stent will also have been inserted into the urethra to keep it open while the area heals.

After the first week, some of the tubes will be removed. The drip will be removed when the child starts feeding again. The epidural is usually removed three to five days after the operation. Seven days after the operation, the nurses will remove the ureteric stents if they were inserted.

This will be done on the ward — we will give the child pain relief beforehand, although it may still be uncomfortable. The child will be able to go home once they are recovering and have been reviewed by the doctors. Around three months later, the child will have a cystoscopy to check how the bladder is healing.

After the initial closure of the bladder exstrophy, there is no sphincter at the junction of the bladder and urethra. The Kelly procedure also called a soft tissue reconstruction of the bladder neck uses existing muscle and soft tissue to create a ring of muscle that acts like a sphincter. This holds urine in the bladder allowing it to stretch and gain more capacity and also helps form a strong stream of urine when weeing.

In boys, the Kelly procedure may also involve a reconstruction of the urethra and penis, or it may be done in a separate operation at a later stage. During the same operation, the ureters may be re-positioned within the bladder if they are not joining the bladder in the correct place. This can cause a condition called vesico-ureteric reflux VUR where the valves can fail, allowing urine to flow backwards from the bladder towards the kidney.

Depending on the severity of the VUR, sometimes the urine can flow backwards as far as the kidneys. This can damage the kidney and eventually lead to kidney failure. The ureteric re-implantation operation involves disconnecting the ureters and re-attaching them in the correct place. All surgery carries a small risk of bleeding during or after the operation. Every anaesthetic carries a risk of complications, but this is small.

There is a small risk of infection, but the child may be given antibiotics as a precaution. After the first operation to repair the bladder and abdominal wall, there is a risk that the wound will not heal properly and open up again. This can cause the bladder to move out of position. This happens more often if the area to be repaired is large, as the skin needs to stretch to cover it.

If the wound opens up again, your child will need another operation to repair the bladder and abdominal wall. The surgeons may correct the pelvic bones during this operation as well. There is also a risk of kidney damage in children with bladder exstrophy.

The abnormal join between the ureters and bladder allows urine to flow backwards towards the kidneys. This is called vesicoureteric reflux VUR. It can sometimes lead to a condition called hydronephrosis , where the kidneys become swollen. The outlook for babies born with bladder exstrophy is good, although around 20 per cent of all children born with bladder exstrophy need some further treatment later in childhood if they are having problems keeping dry. Following the Kelly procedure, if the ring of muscle around the base of the bladder is too weak, urine could dribble out all the time.

This also happens if the bladder cannot hold enough urine. This can be improved with an operation called bladder augmentation , which involves making the bladder larger, and therefore able to hold a larger volume of urine, using a section of intestine. These children then empty their bladder using a catheter to drain away the urine.