What is meant by Bladder exstrophy?
Bladder exstrophy symptoms are a relatively uncommon birth defect in which the bladder develops outside of the foetus. Urine leakage occurs because the exposed bladder is unable to store urine or function properly (incontinence).
The effect of problems caused by exstrophy of the bladder varies. They often include bladder, genitourinary, and pelvic bone deformities, as well as the intestine and reproductive organ deformities.
During pregnancy, a routine ultrasound may reveal exstrophy of the bladder. However, the deformity may not be visible until the infant is delivered. The deformities in infants born with exstrophy of the bladder will require reconstructive surgery.
Causes of bladder exstrophy
Exstrophy of the bladder is unrelated to what a parent did or did not do during pregnancy. There is no known cause for this disease.
It is believed to occur during the 11th week of pregnancy when the organs are still developing. The bladder deformity, according to a few specialists, occurs when the tissues in the lower wall of the tummy or abdomen develop. The developing muscles and pelvic bones are also affected at the same time.
The lower tummy wall is covered by a temporary tissue called the cloacal membrane, which is replaced by evolving and developing core muscles. If the cloacal membrane emerges before the pelvic floor muscles have fully formed, the bladder may become “exstrophy.” The urorectal septum is a tissue that assists in the separation of the developing bladder from the bowels or digestive tract.
When the cloacal membrane booms and the bladder and intestines are separated by the urorectal septum, the child is born with epispadias, classic bladder exstrophy, or cloacal exstrophy.
Symptoms
Depending on the severity of the developmental abnormality that causes it, the bladder-exstrophy-epispadias-cloacal exstrophy complex can take many different forms. When there is an opening in the urethra, the condition is the mildest (epispadias). When there is an opening in the urethra, bladder, or bowel, it is the most severe form (cloacal exstrophy).
Classic exstrophy of the bladder is the most common type, in which the bladder and associated structures are turned inside out via a hole in the abdominal wall. The bladder is open from the top of the bladder through the urethra and to the tip of the penis in classic exstrophy of the bladder, which is of intermediate severity. The urethra of a boy with epispadias is extremely short and split, with the opening on the upper surface of the penis. The urethral opening in epispadias girls is located between the split clitoris and the labia minor.
Cloacal exstrophy is a serious rare complication in which the abdominal contents are usually contained by a membrane-covered area on the abdominal wall (omphalocele). Males have a split penis and a divided bladder. Females have two vaginal openings and a clitoris that is divided into two halves. The rectum’s opening to the outer body is frequently absent or abnormally tiny.
Factors that are at risk to develop exstrophy of the bladder:
- Exstrophy of the bladder can be caused by several factors, including:
- Having a Family History: Children born first, children of a parent with bladder exstrophy, and siblings of a child with exstrophy of the bladder have a higher risk of developing the disease.
- Race: White people are more likely than other races to suffer from exstrophy of the bladder.
- Sex: Exstrophy of the bladder affects more boys than girls.
- Assisted reproduction: Exstrophy of the bladder is more common in children born through assisted reproductive technology, such as IVF.
Diagnosis of exstrophy bladder
A foetus with a complex situation may have no bladder filling, a low-set umbilical cord, pubic bone separation, small genitals, and an abdominal mass that grows in size as the foetus grows, according to prenatal ultrasound.
Treatment and Preventions
Surgery is used to treat exstrophy of the bladder. The type of surgery required is determined by the severity of the defect. It’s critical to work with a surgeon who has dealt with exstrophy cases before.
Over the last 15 years, technological advancements have resulted in successful reconstruction. This includes penis and bladder reconstruction for a more “normal” and fully functioning routine.
The treatment’s main objectives are to:
Shut the bladder, the urethra’s back, and the pelvis.
In boys, rebuild a normal-looking and functioning penis; in girls, rebuild the outer sex organs.
Fix the bladder so it can hold urine until it’s time to urinate without affecting kidney function or urine incontinence.
“Staged reconstruction” is one type of treatment. Parts of the above surgeries were performed during the early childhood years:
The bladder and pelvis are closed in the first surgery. This procedure is carried out shortly after the baby is born.
The epispadias urethra and penis are rebuilt during surgery when the child is about 6 months old.
When the bladder has grown large enough and the child is ready for potty training (usually around the age of 4 or 5 years old), bladder neck surgery is performed to achieve continence.
Closing the bladder and reconstructing the penis can be done in a “single operation” at a young age if the bladder quality is good and the penis size is good at birth.
Early and staged reconstruction are both possible.
The results of both early and staged reconstruction are positive. Continence (control over urination) is possible if the bladder has grown sufficiently and the surgeon is skilled. To improve the child’s ability to urinate, additional operations are frequently required over time. Additional surgery may be required to rebuild and/or improve the outer sex organs.
Longer-term management is required in more difficult situations. With modern reconstructive surgery, a baby can still reach his or her late teens and have a successful outcome.
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