The exstrophy-epispadias complex is a serious congenital abnormality with many variations. It is the condition that can be observed immediately after birth, where the bladder and related structures protrude through an opening in the abdominal wall instead of inside the body. It varies depending on which structures constitute the problem. The most severe is cloacal exstrophy, involving the urethra, bladder, and intestines. In classical bladder exstrophy, the abnormality is related to the abdominal wall, bladder, genitals, and urethra. The mildest is epispadias (external genital organ development disorder), which is an opening in the urinary tract.
The true cause of bladder exstrophy has not yet been clarified. A determining risk factor or the presence of agents to cause this condition could not be determined. The incidence 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%, he states that environmental factors are among the risk factors. Classical bladder exstrophy occurs in approximately 4 out of 100,000 live births. 1 in 117,000 cases of male epispadias,
The case of female epispadias is seen in 1 in 484,000. Cloacal exstrophy is a disease that occurs in every 200,000-400,000 births.
Classic cases of bladder exstrophy or epispadias rarely lead to death. Survival rates after surgical treatment are very high. However, while the urinary retention rate is 75-90% in single-stage treatment, this rate may be lower than 25% in several-stage surgical treatment. 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. After successful surgical operations in men, reproductive function can continue into adult life. However, the use of assisted reproductive techniques may be required. Women with exstrophy can also experience a normal pregnancy process. However, female patients should inform their doctors about exstrophy treatment and delivery should be by cesarean section in order to avoid pelvic floor traumas.
Bladder exstrophy can also be detected by prenatal ultrasonography. The bladder should be protected by using special materials at birth. Each time the child is changed, the bladder should be carefully rinsed with a special solution. And the protective material must be replaced again. A general examination should be performed to observe other congenital abnormalities. There may be undetected female epispadia. This condition can also manifest itself in the form of urinary incontinence during childhood.
Aims in the treatment of classical bladder exstrophy;
1) The bladder must be closed in a way that will protect kidney function and ensure urine retention.
2) To create a functional and cosmetically acceptable sexual organ.
The surgical treatment of classical bladder exstrophy can be performed with two 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 hold the urine. However, this procedure can be done 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 is 2 years old. The reconstruction of the bladder neck is performed around the age of 5 years. In this way, the child is provided with the opportunity to control the urine.
The second method is the restructuring of the bladder, urinary tract and genitals in the early period after the child is born.
all done at once. After these surgeries, kidney functions are usually restored. However, in some cases, kidney stone problems may occur in the long term. Kidney infections and varying degrees of urinary incontinence may also occur. Exstrophy-epispadias syndrome is a complex abnormality. While the treatment is performed with the surgical procedure, a lifelong follow-up process begins.
Patients and their relatives should be informed in detail about all stages of the disease, the treatment procedure and possible complications and success rates after treatment. Before the patient is taken into surgery, it is important that the relatives of the patient are aware of the urinary tract to be reconstructed, the risks and the benefits of the surgery. However, it is also necessary for the relatives of the patients to know and accept that they should take responsibility for the daily care of the patient.