Bladder exstrophy is a rare birth irregularity where the bladder muscles don’t develop properly.
With birth irregularities in general only affecting about
This article will offer you more information about bladder exstrophy, including symptoms, treatment options, and how children can outgrow this health issue in their earliest days.
Exstrophy of the bladder is a birth irregularity in which the abdominal wall doesn’t fully form as the bladder is developing. This leaves the pubic bones separated and the bladder exposed to the outside skin surface.
In addition to the bladder, this birth irregularity can impact the formation and function of other organs, including the bowels and external genitalia. Left untreated, the bladder won’t be able to hold urine normally and sexual function may be compromised.
Bladder exstrophy is extremely rare, occurring in only approximately
Signs and symptoms of bladder exstrophy can include:
- Abnormal bladder development: Because the bladder is exposed, the bladder neck may not develop properly and the bladder may be smaller. Even after corrective surgery, the bladder will likely require time to grow and develop before it can properly store urine.
- Widened pubic bones: The pelvic bones typically join to protect and support the bladder, urethra, and abdominal muscles. The pelvic bones won’t normally join in a child with bladder exstrophy. This can cause the hips to be rotated outwardly.
- Epispadias: This is a condition where the urethra isn’t fully formed. In males, it may be opened on the top of the penis and not the tip. In females, it may be positioned farther up between the divided clitoris and labia minora.
- Vesicoureteral reflux: This is a condition where urine travels back up the ureters toward the kidneys instead of into the bladder.
- Umbilical hernia: This is the displacement of the belly button, which happens in 10 to 25% of newborns. This type of hernia is more common in babies who are born prematurely or with a low birth weight.
- Abnormal development of genitalia: In males, the penis may appear shorter and curved in an upright direction. Additionally, the testicles may not be normally placed in the scrotum and a hernia may be seen. In females, the clitoris and labia minora are typically separated and spread apart. The vagina and urethra are also shorter.
Bladder exstrophy may be diagnosed in pregnancy during a routine ultrasound. If it’s suspected but not clearly diagnosed through that ultrasound, it can be more definitively diagnosed by an MRI.
If not already identified during pregnancy, bladder exstrophy can be visually observed by the doctor right after a baby is born.
Although bladder exstrophy won’t heal with age, it can be completely fixed through surgery.
The severity of the symptoms may vary, but without complete repair surgery, the bladder will never be able to properly function.
Initially, the bladder is covered with a clear plastic dressing after birth to protect it. This will help prevent trauma and the formation of polyps.
However, this isn’t a long-term solution. Surgery is required to treat bladder exstrophy.
- moving the bladder inside the body
- reconstructing genitalia and addressing
epispadias - reconstructing the bladder neck
- closing the pelvic bones
Surgery to treat bladder exstrophy can also be performed in stages:
- within 3 days after birth
- between 6 and 9 months
- anywhere from shortly after birth, to around 4 or 5 years (
modern staged repair of bladder exstrophy [MSRE] )
In MSRE, there are three surgeries that aim to accomplish different tasks:
- Follows birth and is used to close the bladder and abdomen.
- Repairs the urethra and sex organs.
- Bladder neck reconstruction is performed by a surgeon.
Reports from several medical groups indicate that after surgery more than
However, hydronephrosis (the swelling of the kidneys because of the buildup of urine) materialized in
Following MSRE, recurrent urinary tract infections occurred in approximately
After surgery, a baby will typically spend several weeks in the hospital. During this time, they’ll be immobilized. A thin tube may be placed into the spinal canal during surgery to deliver pain medication directly to the area it’s needed.
Long-term follow-up medical care will be required to make sure that the bladder and kidneys are developing in a healthy way. Ultrasounds and medical visits will be required as the child grows up.
Individual and family therapy may be necessary to address the emotions and concerns that can come with bladder exstrophy. This birth irregularity is so rare that it may be difficult to find a local support group, but online communities can offer connections for parents and children with bladder exstrophy as they age.
With early surgery, doctors can optimize urinary control, preserve kidney function, and improve the appearance and function of external genitalia. Some individuals may struggle with incontinence even after surgery, though. Additional surgeries may be necessary as that person gets older to help with incontinence or to complete genital reconstruction.
With medical care, those born with bladder exstrophy can have typical life expectancies and full, productive lives.
As they age, they can have a typical sex life. They can even become pregnant. However, a pregnancy will be considered high risk for both the birthing parent and baby if that adult has bladder exstrophy. A planned cesarean (C-section) delivery may be necessary.
Bladder exstrophy is a rare birth irregularity where the bladder develops outside of the developing baby. Left untreated, the bladder won’t be able to store urine or function properly.
Symptoms of bladder exstrophy can vary in severity, but the only permanent cure is repair surgery. A doctor or healthcare professional will discuss surgical options with you if your baby is born with bladder exstrophy. If your child is diagnosed with bladder exstrophy, it may be comforting to know that many individuals with this birth irregularity go on to have normal lives following surgery.