The specific cause of this developmental anomaly has not been identified, the pathology is considered multifactorial. Proven genetic predisposition to some of the combined defects of the abdominal organs, among which there is extrophy of the bladder.
In addition, increases the risk of anomalies of exposure to various toxic and infectious agents during pregnancy, in particular, smoking, intrauterine infections, certain medications, etc. Fetal injuries can also contribute to the violation of proper embryogenesis with the development of defects.
The main pathogenetic moment of bladder exstrophy is a delay in the reverse development of the cloacal septum, which prevents the introduction of a mesodermal layer between the outer and inner embryonic layers. This leads to the fact that the process of formation of the walls of the bladder is incomplete, and the organ remains open and turned out.
The described processes normally take place at 4-6 weeks of embryonic development, therefore, exposure to teratogenic factors is most dangerous at the very beginning of pregnancy. However, this concerns not only the exstrophy of the bladder, but also the majority of malformations.
Symptoms of bladder exstrophy
Manifestations of blemish are visually noticeable immediately after birth. The pediatrician will pay attention to the bright red mucous membrane of the bladder, which replaces the missing part of the anterior abdominal wall. Mucous, as a rule, has the usual morphology, but pseudopapillomatous change is also possible. In the lower part of the visible holes of the ureters, of which constantly excreted urine.
Over time, the main problem in the exstrophy of the bladder becomes irritation of the surrounding tissues with urinary salts, constant maceration around the open area and in the thighs. Infection of the mucous membrane of the bladder is the cause of ascending infection of the ureters and kidneys.
Bladder exstrophy is often combined with splitting of the ureters. In addition, extrophy always accompanied by discrepancy of the pubic symphysis. This leads to the fact that anorectal muscles attached to it, which normally keep the external anal sphincter, begin to stretch it. In patients it is located in the transverse direction and closer to the urethra compared with the norm.
Therefore, one of the signs of exstrophy of the bladder is fecal incontinence. Due to anatomical differences in boys and girls, there are various changes in neighboring organs. In boys, there is splitting of the cavernous bodies or their reduction, in girls – splitting of the clitoris, anomalies of the development of the vagina.
Diagnosis and treatment of bladder exstrophy
In recent years, methods of antenatal diagnosis of this defect have been actively developed. Bladder ecstrophy may be noticeable already on the second ultrasound screening, but this does not always happen. Often an abnormal development is detected only after birth. Since the symptoms are specific, the diagnosis is beyond doubt.
It is necessary to conduct an x-ray of the abdominal cavity to detect combined defects, primarily in the urinary system, but also in the intestine and spinal cord (the most characteristic combinations with bladder exstrophy). With the same purpose is carried out ultrasound. Recommended excretory urography, which allows to ensure the integrity and proper development of the ureters and kidneys.
Treatment is prompt. The type of surgery depends on the degree of exstrophy of the bladder, namely, on the size of the inverted part of the organ and, accordingly, the missing segment of the anterior abdominal wall, as well as on concomitant malformations. If the size of the defect does not exceed 4 cm, reconstructive plastic surgery is performed, the purpose of which is to close the defect with the help of nearby tissues and restore the integrity of the bladder. This is sufficient, because histologically the organ is developed correctly, despite the pathology in the anatomy.
In the case of a large defect size, an artificial bladder is created, which can function as a reservoir and be emptied at will. Also, often the ureters are excreted in the sigmoid colon, it is a mandatory measure in case of their splitting. A major problem for surgeons is the divergence of the bones of the pelvis during bladder exstrophy.
This fact is the cause of most fatal complications, in particular, incontinence of urine and feces. The accumulated medical experience indicates a high frequency of unsatisfactory results of operations, which is associated with a large number of combined defects and a significant defect in the anterior abdominal wall.
Forecast and prevention of bladder exstrophy
The prognosis for recovery is dubious. Non-operated patients rarely live to 10 years. With regard to the success of surgical treatment, 20-80% of the interventions performed lead to only a slight improvement. Violations of urodynamics continue to progress, incontinence persists. The risk of developing an ascending infection remains high.
Prevention of bladder exstrophy is possible in the antenatal period, even better at the planning stage of pregnancy in order to maximally eliminate the impact of teratogenic factors, especially at the stage of laying out the main organs and systems, that is, in the first 4-8 weeks of intrauterine development.
Bladder exstrophy is a malformation of the urogenital system, in which the formation of the bladder and the anterior abdominal wall in its projection remains incomplete. From birth, there is an open and outward mucous membrane of the posterior wall of the bladder, as well as constant separation and drainage of urine from the ureters opening in the lower part of the organ.
Bladder exstrophy is often combined with defects of the external and internal genital organs, ureters and kidneys. Diagnosed by characteristic external signs, an additional X-ray and ultrasound examination. The treatment is operative, reconstructive plastic and replacement surgeries are performed.
Bladder exstrophy is one of the most common abnormalities of the urogenital system. The frequency in the population – 1 case for 30-50 thousand newborns, about 1/5 of the patients have combined malformations. Boys are more affected, according to different authors, the prevalence of pathology among them is 2-6 times higher than among girls.
Experts in the field of pediatrics and surgery face great difficulties in treating bladder exstrophy, although the first surgeries for correcting this anomaly were proposed as early as the 1960s. Reconstruction and plastic do not lead to a cure. Continuing incontinence of urine and feces significantly impairs the quality of life of the child, and frequent ascending infections cause chronic renal failure, which is a serious danger to life.