Bladder exstrophy is one of the most severe congenital malformations in urology. Children with such a defect do not have a front wall of the urethra and an abdominal wall on it, so all the urine pours directly out.
In children with such a defect, the mucous membrane of the bladder literally "sticks out", irritates, bleeds and becomes inflamed, so the belly looks shocking to an unprepared person.
There is such a pathology infrequent - an average of 1: 10,000 to 1: 50,000 babies. But this often breaks the development of the urethra, urethra, and genitals. Other congenital malformations are also observed.
Causes of exstrophy
This defect is formed at about 4-5 weeks of embryo development. It is known that it is during this period that the organs and tissues of the body are laid, while some formations are divided, others are united. If something is broken in this process, the organ starts to form incorrectly. According to one version, at that moment the cloacal membrane does not close and therefore the bladder is behind the abdominal cavity, on the other - the layer of cells forming the wall of the abdomen is very thin and tears under the weight of the urea, releasing it outward.
What is the impetus for the beginning of the formation of exstrophy is not yet known. The most significant factors are:
- Hereditary predisposition. Children with such a defect often have a disruption in the structure of some genes. It is also noted that in some families this pathology is more common.
- Unhealthy lifestyle of the future mother - smoking, drinking alcohol and drugs, especially at the stage when laying the organs of the fetus.
- The impact of an unfavorable environment - polluted air, radiation, etc.
- Take drugs with a teratogenic effect.
- X-ray exposure.
- Taking too large doses of progesterone in the first weeks of pregnancy.
In addition, scientists suggest that there may be a link between the use of some reproductive technologies and exstrophy, but there is no proof of this.
Manifestations of vice
Exstrophy of the bladder implies an abnormal arrangement of the organ, because of which its inner wall is visible through the hole in the abdomen. In this case, urinary incontinence is observed - it simply oozes from the ureters, as well as its return to the kidneys. In addition, this pathology is often accompanied by other malformations of the pelvic organs. The most common are:
- Epispadia is a splitting of the head of the penis in toddlers of a male with its shortening.
- Violation of the structure of the urethra, vagina and labia in girls.
- The discrepancy of the muscles in the lower part of the abdominal wall.
- Wrong position of the navel, usually it is too low, near the frontal articulation.
- Umbilical hernia.
- An abnormal position of the anal opening. It can be near the vagina in girls and in the scrotum of male children.
- Divergence and deformation of the bones.
- Violation of innervation of the bladder, which affects its functioning. Often, problems remain after a successful operation.
Urogen exstrophy is a dangerous pathology that often occurs in combination with other developmental malformations. The most common among them are:
- Inguinal hernia;
- Congenital disorders of kidney development;
- Weakness of the anal sphincter, leading to prolapse of the rectum;
- Undeveloped coccyx and sacrum;
- No anal opening with blindly ending rectum.
Diagnosis and treatment of exstrophy
To detect such a serious pathology can be long before the birth of a child. To suspect an exstrophy it is possible, if on US:
- The urea is visualized throughout the study.
- The defect of the abdominal wall was noticed.
Given the early detection of the defect and its combination with other developmental disorders, doctors can recommend abortion.
If pathology has not been detected at the stage of intrauterine development, the diagnosis is made immediately after birth. This is not difficult because of the characteristic appearance of the child. But to determine the characteristics of violations, more detailed examinations are needed. First, it is important to make sure that urine is excreted from the hole in the abdominal wall, rather than serous exudate or peritoneal fluid, as is the case with other pathologies.
Each child is given an ultrasound of the abdominal cavity to determine the presence of other abnormalities. In addition, a computer tomogram and endoscopy examination may be required. It is very important to do a genetic examination, since the bladder exstrophy may be a manifestation of one of the trisomy or other abnormalities.
Eliminate such a serious pathology can only be through a complex surgical procedure, which is often threatened with death. Usually the operation takes place in several stages. Usually, the patient's own tissues are not enough to cover the entire defect, therefore, first (on the 10th day after birth), the hole in the bladder is eliminated, and the gap in the abdominal wall is covered with sterile polyethylene. When the baby grows up, the synthetic material is removed, and the defect is covered with your own skin and tissues.
In most cases, the correction can not be completed in two steps, they need much more. They make the genital organs, the neck of the bladder and the sphincters. The last operations are carried out approximately in 5 years.
After the operation and the entire period of life with a polyethylene implant, the child takes three groups of antibiotics to prevent secondary infection. After the end of the surgical correction treatment does not stop, patients need to regularly undergo examinations with the urologist and preventive treatment in the sanatoriums to prevent long-term complications.
Prognosis for exstrophy
The prognosis depends on the complexity of the pathology and the presence of associated vices. If the violations are not strong and the operation is carried out on time and correctly, the forecast is favorable. But the patient should regularly consult with the nephrologist, as the probability of pyelonephritis, renal failure, urolithiasis and urinary incontinence is high.