Exstrophy-epispadias complex is a serious congenital abnormality that has many varieties. It is a condition that can be observed immediately after birth, in which the bladder and related structures protrude through an opening in the abdominal wall instead of inside the body. It varies depending on which structures cause the problem, and the most severe is cloacal exstrophy, which involves the urethra, bladder and intestines. In classical bladder exstrophy, the abnormality is related to the abdominal wall, bladder, genitals and urinary tract. The mildest one is epispadias, which is an opening in the urinary tract.
The real cause of bladder exstrophy has not yet been elucidated. The presence of a determining risk factor or agents that may cause this condition has not been identified. The frequency of this problem in children of patients with exstrophy-epispadias complex is 1 in 70. Observation of exstrophy in twins is accepted as evidence of genetic effect.
However, since the rate of co-occurrence in identical twins is less than 100%, it indicates that environmental factors are also among the risk factors.
Classical. bladder exstrophy occurs in approximately 4 out of 100,000 live births. Male epispadias cases occur in 1 in 117,000, and female epispadias cases occur in 1 in 484,000. Cloacal exstrophy is a disease that occurs once in 200,000-400,000 births.
Classic cases of bladder exstrophy or epispadias rarely lead to death. More than one procedure may be required to improve the urinary retention function of these patients. Many patients may feel the need to perform clean intermittent catheterization. However, the use of assisted reproductive techniques may be necessary. Women with exstrophy can also experience normal pregnancy. However, female patients should inform their doctors about exstrophy treatment and birth should be by cesarean section to avoid pelvic floor trauma.
Bladder exstrophy can also be detected by prenatal ultrasonography. At birth, the bladder should be protected using special materials. Every time the child's diaper is changed, the bladder should be carefully washed with a special solution and the protective material should be changed again. A general examination should be performed to observe other congenital abnormalities. not noticed Female epispadia may occur, and this condition may also manifest itself as urinary incontinence in childhood.
Aims in the treatment of classical bladder exstrophy:
1) Closing the bladder in a way that protects kidney function and ensures urine retention
2) To create a functionally and cosmetically acceptable sexual organ.
Surgical treatment of classical bladder exstrophy can be performed by 2 different methods. The first method consists of multiple surgical procedures spread over several years. In the first surgery, the bladder is closed to allow it to retain urine. However, this procedure can be performed if the bladder is large enough. The bladder is placed in the pelvis and the abdominal wall is closed. Then, a series of surgeries are required to reconstruct the urinary tract and genitals. These surgeries are usually performed before the child turns 2. Reconstruction of the bladder neck is performed around the age of 5. In this way, the child can be given the opportunity to control his/her urine.
The second method is the surgical treatment in which bladder, urinary tract and genital restructuring procedures are performed all at once in the early period after the child is born.
One of these surgeries. Afterwards, kidney functions are usually restored. However, in some cases, kidney stones, kidney infection and different degrees of urinary incontinence problems may occur in the long term.
Exstrophy-epispaias syndrome is a complex abnormality and while treatment is carried out with surgery, a lifelong follow-up process begins. The patient and his/her relatives should be informed in detail about all stages of the disease, the treatment procedure and possible complications after treatment. However, it is also necessary for the patient's relatives to know and accept that they must take responsibility for the daily care of the patient.
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