BLADDER EXSTROPHY

TYPES

In the unusual congenital disorder known as bladder exstrophy, the bladder is external to the body. A developmental abnormality during fetal development is the cause of its occurrence. Classic bladder exstrophy and cloacal bladder exstrophy are the two basic kinds of exstrophy.

• Classic Bladder Exstrophy: This is the most common type of bladder exstrophy. In classic bladder exstrophy, the bladder is exposed and open, with the inner lining visible on the outside. The pelvic bones may also be separated, and the pubic bone is often widened. Additionally, the urethra may be shortened or absent, and the external genitalia can be affected. Children with classic bladder exstrophy may also experience associated conditions such as urinary incontinence, urinary tract infections, and kidney abnormalities.
• Cloacal Exstrophy: Cloacal exstrophy is a more severe and less common form of bladder exstrophy. In this type, not only is the bladder exposed and open, but the intestines and sometimes the reproductive organs are also involved. The pelvis is usually split, and the pubic bone is separated even more than in classic bladder exstrophy. Cloacal exstrophy may also present with other abnormalities, such as spinal defects, abnormal genitalia, and gastrointestinal malformations.

SYMPTOMS

Bladder exstrophy is a congenital condition, meaning it is present at birth. The most noticeable symptom of bladder exstrophy is the exposed bladder and associated structures on the lower abdomen. This can be quite apparent and may vary in severity from case to case. Other symptoms that may be present include:

• Urinary Incontinence: Children with bladder exstrophy often experience difficulty controlling their urine. This can range from mild leakage to complete inability to hold urine.
• Abnormal Urine Flow: The positioning of the bladder and associated structures in bladder exstrophy can affect the normal flow of urine. This may result in a weak stream, dribbling, or spraying of urine.
• Urinary Tract Infections (UTIs): The abnormal positioning of the bladder can make it more difficult to fully empty the bladder, increasing the risk of urinary tract infections. Recurrent UTIs may be a symptom of bladder exstrophy.
• Difficulty Emptying the Bladder Completely: Children with bladder exstrophy may have problems completely emptying their bladders, leading to residual urine. This can contribute to urinary tract infections and other complications.

It’s important to note that the severity of symptoms can vary from person to person, and some individuals may have additional associated abnormalities or complications. If you suspect your child may have bladder exstrophy or if you have concerns about their urinary health, it is important to consult with a healthcare professional specializing in pediatric urology or bladder exstrophy. They will be able to provide a comprehensive evaluation, diagnose the condition, and guide you through the appropriate treatment options.

DIAGNOSIS

Bladder exstrophy is typically diagnosed shortly after birth based on a physical examination. The exposed bladder and associated structures on the lower abdomen are usually apparent and can be readily observed by healthcare professionals. However, additional diagnostic tests may be conducted to assess the extent of the condition and identify any associated abnormalities. Here are some common diagnostic procedures used for bladder exstrophy:

• Imaging Studies: To see the bladder and related tissues in more detail, imaging methods like ultrasound, X-rays, or MRI (Magnetic Resonance Imaging) may be employed. The results of these imaging tests may be used to assess the severity of bladder exstrophy and spot any associated abnormalities.
• Genetic testing: This may be done in certain circumstances to check for particular genetic disorders or anomalies that are linked to bladder exstrophy. This may provide crucial details regarding the underlying causes and possible health effects.
• Urodynamic Testing: Urodynamic testing involves assessing the function and behavior of the urinary system. This may include tests such as measuring urine flow rate, bladder pressure, and assessing how well the bladder empties. Urodynamic testing can help evaluate bladder function and guide treatment decisions.
• Physical Examination: In addition to the visual examination of the exposed bladder, a thorough physical examination may be conducted to assess the overall health and development of the child. This may include evaluating the abdominal wall, pelvic organs, and other associated structures.

TREATMENT

The treatment for bladder exstrophy typically involves a multidisciplinary approach and may vary depending on the individual’s specific needs. The primary goals of treatment are to achieve urinary continence, preserve kidney function, and restore the normal appearance and function of the urinary and genital organs. Here are some common treatment options for bladder exstrophy:

• Surgical Repair: The mainstay of treatment for bladder exstrophy is surgical repair. The surgery aims to bring the exposed bladder back into the body and reconstruct the pelvic area. This involves closing the abdominal wall, repairing the bladder, and creating a new urinary opening (urethra). The timing and specific techniques used for surgical repair may vary depending on the patient’s age, overall health, and individual circumstances.
• Continence Management: Achieving urinary continence is an important aspect of treatment. This may involve a combination of techniques such as intermittent catheterization, which involves emptying the bladder at regular intervals using a catheter. Bladder training exercises and medications may also be used to help control urinary function and improve continence.
• Follow-up Care: Regular follow-up care is crucial for monitoring the progress of bladder exstrophy treatment. This may involve routine check-ups, imaging studies, and urodynamic testing to assess bladder function and overall health. Regular follow-up care ensures that any potential complications or issues are addressed promptly.
• Supportive Care: Psychological support and counseling are essential for individuals and families dealing with bladder exstrophy. Support groups and resources can provide emotional support, education, and practical advice to help manage the condition and its impact on daily life.