Bladder Exstrophy

Bladder exstrophy is a rare congenital medical condition where the bladder and sometimes other organs such as the urethra, vagina, or intestines are exposed outside the abdominal wall due to an abnormal formation of the pelvic bones and muscles during fetal development.

Causes and Risk Factors

The exact cause of bladder exstrophy is not fully understood. However, it is believed to result from a combination of genetic and environmental factors that affect the normal development of the fetus during pregnancy. The condition occurs in approximately 1 in every 50,000 births.

Symptoms

The symptoms of bladder exstrophy can vary depending on the severity of the condition. Common symptoms include:

• A visible bladder and urethra outside the abdominal wall
• Abnormal appearance of the genital area
• Incontinence or inability to control urination
• Urinary tract infections (UTIs)
• Kidney damage due to UTIs or other complications

Diagnosis

Bladder exstrophy is typically diagnosed at birth, as the exposed bladder and urethra are visible. Further testing such as ultrasound, CT scans, or MRI may be performed to assess the extent of the condition and any associated abnormalities.

Treatment

The primary goal of treatment for bladder exstrophy is to repair the abnormal anatomy, achieve urinary continence, and prevent long-term complications. Treatment typically involves a series of surgeries, which may include:

• Initial surgery to close the bladder and abdominal wall, usually performed in the first few days or weeks of life
• Subsequent surgeries to repair the urethra, vagina, or other affected organs
• Bladder reconstruction or augmentation to improve urinary function and continence
• Epispadias repair to correct any abnormalities of the penis or urethra in males

Complications and Long-term Outlook

Individuals with bladder exstrophy are at risk for various complications, including UTIs, kidney damage, and infertility. With modern surgical techniques and ongoing medical care, many people with bladder exstrophy can achieve good urinary function, fertility, and overall quality of life.

Management and Follow-up

Lifetime follow-up with a urologist or other specialist is essential to monitor for potential complications and address any issues that may arise. Ongoing management may include:

• Regular check-ups to assess urinary function and overall health
• Preventative measures such as UTI prophylaxis or bowel management
• Counseling and support for individuals and families affected by bladder exstrophy