What is exstrophy of urinary bladder?
What is Bladder Exstrophy? An abnormality of formation of the bladder and the bony pelvis. The bladder does not form into its normal round shape but instead is flattened and exposed on the abdominal wall. The pelvic bones are also widely separated.
What are the types of bladder exstrophy?
Symptoms
- Epispadias. This is the least severe form of BEEC , in which the tube to expel urine (urethra) doesn’t fully develop.
- Bladder exstrophy. This defect causes the bladder to form on the outside of the body.
- Cloacal exstrophy. Cloacal exstrophy (kloe-A-kul EK-stroh-fee) is the most serious form of BEEC .
What is associated with exstrophy of the bladder?
Bladder exstrophy is a congenital anomaly that exists along the spectrum of the exstrophy-epispadias complex, and most notably involves protrusion of the urinary bladder through a defect in the abdominal wall. Its presentation is variable, often including abnormalities of the bony pelvis, pelvic floor, and genitalia.
What are the signs and symptoms of Epispadias?
Symptoms
- Abnormal opening from the joint between the pubic bones to the area above the tip of the penis.
- Backward flow of urine into the kidney (reflux nephropathy)
- Short, widened penis with an abnormal curvature.
- Urinary tract infections.
- Widened pubic bone.
Can bladder exstrophy be cured?
Complete repair. This procedure is called complete primary repair of bladder exstrophy. Complete repair surgery is performed in a single procedure that closes the bladder and the abdomen and repairs the urethra and outer sex organs. This can be done soon after birth, or when the baby is around two to three months old.
How many people have bladder exstrophy?
How Often does Bladder Exstrophy Occur? Bladder exstrophy is rare. On average, it occurs in about 1 out of every 50,000 live births. It is slightly more common in males than females.
How common is cloacal exstrophy?
Cloacal exstrophy (OEIS Syndrome) is a rare and complicated condition that affects the lower abdominal wall structures of infants In utero, occurring in 1 out of 200,000 pregnancies and 1 in 400,000 live births. It is often diagnosed prenatally (before birth) by a fetal ultrasound and confirmed at the time of birth.
Is bladder exstrophy a rare disease?
Bladder exstrophy is a rare developmental abnormality that is present at birth (congenital) in which the bladder and related structures are turned inside out.
What is the treatment for epispadias?
Surgery can correct epispadias to improve the look and function of the genitals. Your pediatric urologist will discuss the surgical options with you. Healthcare providers recommend doing epispadias repair as early as possible. Early surgery helps achieve better results, especially with bladder control.
How do you fix epispadias?
Patients who have epispadias limited to the genitalia may be treated with one surgical procedure, but patients with other issues may have a treatment plan that includes multiple stages of surgical procedures. The genitalia repair may be done as early as six months of age, and is typically completed by a year.
How is bladder exstrophy diagnosed?
How is bladder exstrophy diagnosed? Bladder exstrophy may be diagnosed during a routine fetal ultrasound or other indicated imaging techniques such as magnetic resonance imaging (MRI). Often it is diagnosed immediately after birth during initial physical examination.
What causes cloacal exstrophy?
The bladder exstrophy-epispadias-cloacal exstrophy complex is caused by a developmental abnormality that occurs 4-5 weeks after conception in which the cloacal membrane is not replaced by tissue that will form the abdominal muscles. The underlying cause of this error in development is not known.
Who is more likely to have bladder exstrophy?
Family history. Firstborn children, children of a parent with bladder exstrophy or siblings of a child with bladder exstrophy have an increased chance of being born with the condition. Race. Bladder exstrophy is more common in whites than in other races. Sex. More boys than girls are born with bladder exstrophy. Use of assisted reproduction.
What to do if your child has bladder exstrophy?
Children sometimes need to have a tube inserted into their bladders to drain urine (catheterization). Additional surgeries may be needed as your child grows. Having a baby with a significant and rare birth defect such as bladder exstrophy can be extremely stressful.
Can a guy with bladder exstrophy have sex?
Most guys with bladder exstrophy are able to have a pleasurable 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 their penis.
Which is the last resort for bladder exstrophy?
Surgical management of bladder exstrophy demands patience and perseverance. It is possible to provide all patients with socially acceptable continence with bladder neck division and catheterizable continent stoma as the last resort. Urodynamic assessment has emerged as an essential tool in the follow-up evaluation of these patients.