Introduction:-
Bladder exstrophy is a rare birth defect that develops within the mother's body while the fetus develops. During the development of the bladder, the skin of the abdominal wall and pelvis does not form/join completely, exposing the bladder outside the body through an opening in the lower abdominal wall. Instead of the ideal round shape, the exstrophy bladder is flat. The bladder cannot function normally or store urine, resulting in leakage.
Bladder exstrophy is a rare condition occurring in approximately one in every fifty thousand births. It is more common in males than females. The disorder may also involve other organs, like the bowel, reproductive organs, and pelvic bones. Family history plays an important role in the development of bladder exstrophy. Kids born to a parent with bladder exstrophy have a one in seventy chance of having the disorder. If one child is born with this condition, then there is a one in one hundred probability of having the other child born with bladder exstrophy. Fertility treatments might play a role as well. Some studies have suggested that kids born with assisted fertility treatments are seven times more likely to have the condition.
What Causes Bladder Exstrophy?
There is no definite cause of bladder exstrophy. This condition is thought to occur during the eleventh week of pregnancy. The defect in the bladder happens at the time when the tissues in the lower abdominal wall develop. A temporary tissue known as the cloacal membrane covers the lower abdominal wall, which is replaced by the matured and developed abdominal muscles.
During this time, if the cloacal membrane tears before the complete formation of the abdominal muscles, it results in an exstrophy bladder.
What Are the Symptoms of Bladder Exstrophy?
The sign and symptoms of bladder exstrophy depend upon the severity of the condition and the involvement of other organs.
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Small Bladder- The development of the bladder is not proper; hence a small bladder is formed. It cannot hold a lot of urine. Therefore there is urine leakage or frequent urination.
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Incomplete Formation of the Bladder Neck and Sphincter- The bladder neck is the lowermost part of the bladder. The sphincter is like a band of muscles around the neck of the bladder. They regulate the urine flow out of the bladder. The child cannot hold the urine when they are not formed fully.
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Urine Backflow- The urine flow is from the kidneys through the ureters (tubes connecting the kidneys to the urinary bladder) into the bladder. During backflow, the urine flows from the bladder back toward the kidneys.
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Epispadias- This is a condition in which the urethra (a tube through which the urine passes out of the body) is short and has a spit. In girls, the urethra opens between the clitoris and the labia minora (inner skin flaps around the vaginal opening). In boys, it begins on the outer surface of the penis.
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Chordee- In this condition, the penis of the boy curves upwards.
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Split Pubic Bone- Either side of the pubic bone in the pelvis does not meet in the front. This leads to a wide split in the pubic bone. This condition is called diastasis.
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Spinal Lipoma- Few kids develop a severe form of exstrophy wherein there is a fatty outgrowth or lipoma on their spinal cord. This causes problems with the legs, bladder, and rectum (the end part of the large intestine). It might also affect the genitals and bowels of the kids.
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Kidney Problems- Few kids might have more than two kidneys or displaced kidneys.
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Bowel Problems- Some kids may have large bowels that are underdeveloped and without a rectum. Surgery is usually needed to make an opening from the intestine to outside the abdominal wall for stool to come out. This surgical process is known as colostomy.
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Umbilical Hernia- This is the displacement of the belly button or umbilicus.
How Is Bladder Exstrophy Diagnosed?
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Fetal Ultrasound- Bladder exstrophy is diagnosed during ultrasound of a pregnant woman. The condition often goes unnoticed since the bladder is hard to see. This is the reason why many babies are born with the condition. A specialist usually confirms the sex determination of the newborn baby.
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Amniocentesis- This can be performed for gender identification.
How Is Bladder Exstrophy Treated?
Bladder exstrophy is treated with surgery. The severity of the defect determines the type of surgery.
The surgery aims to:
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Close the bladder, the back of the urethra, and the pelvis.
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Make the bladder hold urine till it is time to urinate without harming the kidney function.
During the early childhood years, staged reconstruction surgeries are performed-
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The first surgery is done at birth to close the bladder and the pelvis.
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The subsequent surgery is done after six months to rebuild the urethra and the penis.
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When the child is around four or five years old, and the bladder has grown large enough, the surgery is performed at the neck of the bladder to achieve continence.
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Suppose the bladder structure is good, and the penis size is also acceptable. The surgery involving the closure of the bladder and penis reconstruction can be done in a single surgery at an early age.
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Surgeries involving both early and staged reconstruction have a good prognosis. If the bladder has good growth, then surgery can achieve continence.
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Future surgeries are required from time to time to improve the kids' ability to pass urine.
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Genital reconstruction surgeries are often needed to enhance the external reproductive organs.
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Long-term management is required after completing the surgeries.
Conclusion:-
Bladder exstrophy occurs when the baby's urinary bladder develops inside the body. Such babies have a small bladder, split pelvic bones, or not well-developed genitals. They need surgeries to correct the position of the bladder and close the abdominal openings. Depending upon the situation, the babies might need surgery immediately after birth and a couple more in due course of time. The kids can experience a good life and carry out all activities with prompt treatment. If left untreated, functions like urine continence and sexual functions are compromised.
