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Bladder Exstrophy – Definition, Causes and Complications

Definition of Bladder Exstrophy

Bladder exstrophy is a rare congenital condition that affects the development of the urinary system. In individuals with bladder exstrophy, the bladder fails to properly form and close during fetal development, leading to a malformation where the bladder is exposed and located outside the abdominal wall. This condition is present at birth and can have significant implications for both physical and psychological well-being. The severity can vary among individuals, and it often requires surgical intervention to reconstruct the affected area and improve bladder function.

The physical manifestations of bladder exstrophy include a visible and open bladder on the lower abdomen, as well as associated anomalies such as pelvic bone abnormalities. Additionally, affected individuals may experience urinary incontinence and face challenges related to bladder and pelvic floor function. Treatment typically involves a series of surgical procedures aimed at reconstructing the bladder and surrounding structures, often starting in infancy. Long-term management may involve ongoing medical care and support to address associated complications and promote overall quality of life for individuals with bladder exstrophy.

What are the types of bladder exstrophy?

Bladder exstrophy is one condition in a group of birth defects called bladder exstrophy-epispadias complex (BEEC). Some babies are born with one type of BEEC. Others have multiple BEEC conditions.

The other types of BEEC include:

Epidemiology

Occurring at a rate between 1 in 10,000 to 1 in 50,000 with a male-to-female ratio of 2.3–6:1, bladder exstrophy is relatively rare. For those individuals with bladder exstrophy who maintain their ability to reproduce, the risk of bladder exstrophy in their children is approximately 500-fold greater than the general population.

Pathophysiology of Bladder Exstrophy

Bladder-exstrophy-epispadias-complex (BEEC) depicts a spectrum of birth defects, ranging from epispadias to cloacal exstrophy at the milder and the most severe ends of the spectrum. The classical bladder exstrophy is characterized by abnormalities involving the lower urinary tract, abdominal wall, bony pelvis, genitalia, pelvic floor, spine, and the anus.

As a general rule, all the structures, including the bladder, urethra, vagina, and rectum, are displaced anteriorly in bladder exstrophy. The distorted anatomy of the bony pelvis in bladder exstrophy needs a special mention. The ‘open book configuration’ of the pelvis is an appropriate term for its description. The main abnormalities in the bony pelvis include pubic diastasis, shorter anterior segments, and outward rotation of the anterior and posterior segments.

In children with exstrophy, the pubic diastasis is around 4 cm at birth and increases to around 8 cm by the age of 10 years. Contrary to this, in a normal human, the distance is less than 1 cm at all ages. It has been postulated that a disruption in the symphyseal ligaments might be responsible for this. Secondly, the anterior segments of the bony pelvis are approximately 30% shorter. However, the dimensions of the posterior segments of the bony pelvis are within normal limits. Both, the anterior and posterior segments of the bony pelvis, are also externally rotated.

Causes

We don’t know what causes conditions in the epispadias-exstrophy complex. Boys are slightly more likely to develop one of these conditions.

The symptoms may include:

What are the symptoms of bladder exstrophy?

The signs and symptoms vary depending on the severity of the defect, but they may include:

Complications

It can lead to several complications, affecting both physical and psychological aspects of an individual’s well-being. Some of the common complications associated with bladder exstrophy include:

  1. Urinary Incontinence: Individuals with bladder exstrophy often experience difficulties controlling urination, leading to urinary incontinence. This can impact daily life and may require ongoing management strategies.
  2. Pelvic Floor Dysfunction: The malformation can affect the pelvic floor muscles and structures, leading to issues with bowel function and sexual function.
  3. Orthopedic Problems: Bladder exstrophy is often associated with abnormalities in the pelvic bones and musculoskeletal system, which may result in orthopedic complications and gait abnormalities.
  4. Recurrent Urinary Tract Infections (UTIs): The exposed bladder and altered anatomy may increase the risk of recurrent UTIs, requiring regular monitoring and management with antibiotics.
  5. Psychosocial Challenges: Living with a visible malformation can lead to psychosocial challenges, including self-esteem issues, social stigma, and difficulties with body image. Psychological support and counseling may be beneficial.
  6. Surgical Complications: The surgical interventions required to reconstruct the bladder and surrounding structures may have associated complications, including infection, bleeding, and the need for additional surgeries over time.
  7. Fertility Issues: In some cases, individuals with bladder exstrophy may face challenges with fertility and reproductive health.

Risk factors of Bladder Exstrophy

Factors that increase the risk include:

Diagnosis and tests

Often, bladder exstrophy is diagnosed prenatally via sonogram. However, sometimes this condition is not discovered until a baby is born.

Your doctor will order several tests to better understand your baby’s specific type of malformation and develop a plan.

These tests may include:

Treatment

Surgery is the primary treatment for bladder exstrophy. Some children will require many surgeries over several years as they grow and develop. Most children will eventually have fully functioning bladders and normal-looking genitals with appropriate treatment.

There are several approaches a specialist known as a pediatric surgeon may take.

Modern Staged Repair of Exstrophy (MSRE)

Modern staged repair of exstrophy (MSRE) is a series of surgeries done in stages between the ages of three days and four to five years. The steps are broken down as follows:

Complete Primary Repair of Exstrophy (CPRE)

Complete primary repair of exstrophy (CPRE) was created to reduce the number of surgeries a child with BE has to undergo. Ideally, the repair is done in one sitting. It is a complex surgery done when the child is a few months old and can take around 10 hours to complete.

After completing CPRE, the child will need to be immobilized with a pelvic cast.

Other Surgeries

In addition to MSRE or CPRE, other surgeries may be needed to repair other complications of bladder exstrophy. Some of the options include:

Living & managing with bladder exstrophy

Your child may need a range of surgical procedures and tests. In many cases, additional surgeries are required during childhood, adolescence and young adulthood. Your child may require great emotional and psychological support. 

Even with successful surgery, patients may have long-term problems with the following:

Fortunately, sexual function and libido remain normal in bladder exstrophy patients. Successful pregnancies and delivery are possible.

Prevention

It is generally not preventable through lifestyle choices or interventions during pregnancy.

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