BLADDER EXSTROPHY
Bladder exstrophy is a rare birth defect in which the bladder develops outside the fetus. The exposed bladder can’t store urine or function normally, resulting in urine leakage (incontinence). Problems caused by bladder exstrophy vary in severity.
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Bladder Exstrophy
Bladder exstrophy-epispadias-cloacal exstrophy complex (BEEC) is a range of abnormalities related to the urinary tract, genital tract, musculoskeletal system, intestinal tract, etc. In general bladder exstrophy, most abnormalities are related to the faults of the genitalia, pelvic bones, abdominal wall, bladder, rectum, and anus.
Bladder exstrophy is a rare developmental congenital abnormality in which the bladder and its attached structures are turned inside out. The rear portion of the bladder wall aka the posterior vesical wall turns outward (exstrophy) through an opening in the abdominal wall. And through this opening, the urine excretes.
In most cases, the treatment of BEEC is surgical, and doctors usually do this in steps. There are several long-term outcomes of this difficult pediatric urological condition. That is why it necessitates an all-inclusive approach to therapy.
Types
The bladder-exstrophy-epispadias-cloacal exstrophy complex shows up in various forms. And the form depends on the severity of the developmental abnormality responsible for it. In the mildest form, there is an opening in the urethra (epispadias). And in the most severe form, there is an opening in the urethra, bladder, and bowel. (cloacal exstrophy).
Classic bladder exstrophy – It is the most common form in which the bladder and the associated structures turn inside out through an opening in the abdominal wall. Classic bladder exstrophy is medium in severity and the bladder is open right from the top of the bladder through the urethra to the tip of the penis.
Epispadias – Boys with this condition have a very short and split urethra. Here the opening is on the upper surface of the penis. On the other hand, girls with epispadias have a urethral opening in between a split clitoris and labia minora.
QUESTIONS AND ANSWERS
What is Bladder Exstrophy?
Bladder exstrophy is a congenital condition in which the bladder forms outside the body. It occurs during fetal development when the abdominal wall and the front part of the bladder do not close properly. This results in the exposed bladder and associated pelvic structures.
What are the symptoms of Bladder Exstrophy?
The most noticeable symptom of bladder exstrophy is the exposed bladder on the outside of the body, often accompanied by an open abdominal wall and other abnormalities in the pelvic region. Other symptoms may include urinary incontinence, urinary tract infections, and difficulty controlling urine flow. In boys, the condition may affect the penis and urethra as well.
How is Bladder Exstrophy Treated?
Treatment of bladder exstrophy typically involves surgical intervention. The primary goal of surgery is to reconstruct the bladder, abdominal wall, and associated structures to create a functional and cosmetically acceptable urinary system. Multiple surgeries are often required over the course of the individual’s childhood and adolescence to achieve optimal results. After surgery, ongoing medical care and follow-up are essential to monitor bladder function, prevent complications, and address any urinary issues. A multidisciplinary team of urologists, pediatric surgeons, and other specialists work together to provide comprehensive care for individuals with bladder exstrophy.
The Medical City Children’s Orthopedics and Spine Specialists doctors treat children with Bladder Exstrophy
Cloacal exstrophy – It is a severe congenital defect in which there is generally a membrane-covered area on the abdominal wall. And this area contains all the abdominal contents (omphalocele). The bladder is split into two halves. Males with this have a penis divided into two halves. Females with this condition have a clitoris split in two halves and some may even have two vaginal openings. The opening of the rectum outside of the body is generally missing or small.
Other abnormalities may also include a separation of the pubic bones, a missing lower portion of the bladder which leads to a lack of bladder control (incontinence), etc. Also, there might be an abnormal position of the tubes that carry urine from the kidneys to the bladder (ureters). And it might cause a backup or flux of urine in the kidneys.
Causes
In normal cases, the cloacal membrane temporarily divides the urogenital and anal structures. Then, they break when tissue that will later develop abdominal muscles starts to grow in its place. The bladder exstrophy-epispadias-cloacal exstrophy complex is the result of a developmental abnormality that occurs 4-5 weeks after conception. In this condition, the tissue that will form the abdominal muscles does not replace the cloacal membrane. However, the reason behind this flaw is not known.
The outcomes are an open bladder plate, a low-placed umbilicus, and a separation of the pubic bones. Right above the genital tubercle, the cloacal membrane breaks. It results in a penis that comes with an open dorsal surface which continues with the bladder plate. There are various types of this defect and the type is determined by when the rupture takes place. Thus, it results in different forms of BEEC.
Also, a combination of genetic and environmental factors plays a crucial role in the etiology of the disease. Parental age, male sex, race, certain drugs, and preconception mother exposure to smoking, and alcohol are associated with an increased incidence of BEEC in studies.
Symptoms
When the bladder gets exposed, babies with bladder exstrophy develop other related problems that affect their urinary system and pelvic bones. These problems have different levels of severity and do not affect every baby. Doctors confirm these by using ultrasound scans and x-rays. Also, these problems may be corrected through a series of operations. The symptoms include:
- Issues with the neck of the bladder and the ring of muscle (sphincter) that squeezes and relaxes to let urine flow from the bladder.
- The bladder has a smaller capacity than normal. That is why it cannot hold much urine.
- The ureters attach to the bladder in a different place than normal
- The middle part of the pelvic bones is split
On the other hand, Bladder exstrophy shows up with other symptoms. However, the doctor will closely examine the child to see what is the condition. Some may need correction with an operation. The more common symptoms are:
- The anus is more forward than usual
- The belly button is lower than usual
- Umbilical and inguinal hernia in which part of the abdominal lining or a part of the intestine comes out through the weak area in the abdominal wall muscles
- Undescended testes. Here the testicles are not in their normal place.
Diagnosis
A prenatal ultrasound examination of a fetus may help discover the low-set umbilical cord, absence of bladder filling, separation of pubic bones, small genitals, and abdominal mass that increases in size with the progression of the pregnancy.
Treatment
The treatment of bladder exstrophy is mostly surgical. It needs a series of corrective surgeries that doctors perform over several years. In the first surgery, surgeons focus on the closure of the bladder so it can hold urine, placement of the bladder inside the pelvis, and closing of the abdominal wall. In certain cases, children with bladder exstrophy may also need a series of surgical procedures to reconstruct the external genitalia.
Doctors usually perform these surgeries before the age of 2 years. Also, they perform the reconstruction of the Bladder neck at approximately 5 years of age. It allows better control of urine and also helps reposition ureters in a better way to prevent urine from backing up into the kidneys.
Thus, after surgical correction and reconstruction, normal kidney function is good. However, some children with this disorder may have to suffer from long-term urinary problems such as kidney infections, kidney stones, and differing levels of urinary incontinence. Other treatments are supportive and symptomatic.
Our surgeons will explain the customized treatment plan before surgery. Before every operation, the surgeon will discuss the operation with you in detail. Also, they will let you know about any worries.
Post Operative Care
After the surgery, urine will drain from the bladder through catheters. Initially, the patient will have an intravenous infusion supplying fluids and medications until the bladder starts to recover. The child will have regular pain relief through an epidural after the operation.
The child also may have ureteric stents in place. These are thin tubes that drain away urine while the bladder is recovering from surgery.
After the first week, doctors will remove some of the tubes. And they will also remove the drip once the child starts feeding again. Surgeons usually remove the epidural three to five days after the operation. And after seven days, the nurses will remove the ureteric stents (if inserted).
Before removing ureteric stents, we give the child pain relief. However, it may still be uncomfortable. Once your child starts recovering and the doctors review them, he/she will be able to go home. Roughly three months later, the patient will have a cystoscopy to check how the bladder is recovering.
Final Words
The child will need regular checkups to make sure that everything is alright and that the child is healing without further complications. Even when they grow up, they will still require regular follow-up appointments but maybe less frequently than before.
The Medical City Children’s Orthopedics and Spine Specialists has offices in Arlington, Dallas, Flower Mound, Frisco, and McKinney, TX, and specializes in Children. As specialists, Doctors Richard Hostin, MD, Shyam Kishan, MD, and Kathryn Wiesman, MD treat children for minor and major medical problems. We invite new patients to call and make an appointment. We will see your child quickly and find a solution to get your child back to being the kid he or she is.
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Footnote:
Bladder Exstrophy: National Library of Medicine
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