Ekstrofiya of a bladder – a malformation of urinogenital system at which formation of a bladder and a forward belly wall in its projection remains incomplete. Since the birth the mucous membrane of a back wall of a bladder opened and twisted outside, and also constant office and running off of urine from the channels of mochetochnik opening in the lower part of body is observed. Ekstrofiya of a bladder is often combined with defects of external and internal genitals, mochetochnik and kidneys. It is diagnosed on characteristic external signs, also ultrasonography inspection is in addition carried out radiological. Treatment is quick, reconstructive and plastic and replaceable operations are carried out.
Ekstrofiya of a bladder
Ekstrofiya of a bladder – one of the most often met anomalies of development of urinogenital system. Frequency in population – 1 case on 30-50 thousand newborns, about 1/5 part of patients has the combined malformations. More boys suffer, according to different authors prevalence of pathology among them is 2-6 times higher, than among girls. Experts in the sphere of pediatrics and surgery meet great difficulties in treatment of an ekstrofiya of a bladder though the first operations for correction of this anomaly were offered in the 1960th years. Reconstruction and plasticity do not lead to treatment. The remaining incontience of urine and a calla considerably worsens quality of life of the child, and the frequent ascending infections become the reason of the chronic renal failure posing serious hazard to life.
Bladder ekstrofiya reasons
The concrete reason of this anomaly of development is not established, pathology is considered multifactorial. Genetic predisposition to some combined defects of abdominal organs among which there is also a bladder ekstrofiya is proved. Besides, influence of various toxic and infectious agents increases risk of development of anomaly during pregnancy, in particular, smoking, pre-natal infections, some medicines etc. Fruit injuries can also promote violation of the correct embryogenesis with development of defects.
The main pathogenetic issue of an ekstrofiya of a bladder – a delay of the return development of a kloakalny partition which interferes with introduction of a mesodermal leaf between external and internal embryonic layers. It leads to the fact that process of formation of walls of a bladder is incomplete, and the body remains opened and turned out outside. The described processes normal proceed on 4-6 week of an embryonal development therefore influence of teratogenny factors is most dangerous at the very beginning of pregnancy. However, it concerns not only ekstrofiya of a bladder, but also the majority of malformations.
Bladder ekstrofiya symptoms
Manifestations of defect are visually noticeable right after the birth. The pediatrician will pay attention to a bright red mucous membrane of a bladder which replaces the absent part of a forward belly wall. Mucous, as a rule, has usual morphology, but also psevdopapillomatozny change is possible. In the lower part openings of mochetochnik from which urine is constantly emitted are noticeable. Eventually the irritation of surrounding fabrics uric salts, continuous maceration around the open site and in hips becomes the main problem at an ekstrofiya of a bladder. Infection of a mucous membrane of a bladder is the reason of the ascending infection of mochetochnik and kidneys.
Ekstrofiya of a bladder is often combined with splitting of mochetochnik. Besides, the ekstrofiya is always accompanied by a divergence of a pubic simfiz. It leads to the fact that the anorektalny muscles which are attached to it which normal hold an external anal sphincter begin to stretch it. At patients it settles down in the cross direction and is closer to an urethra in comparison with norm. Therefore one of signs of an ekstrofiya of a bladder is the incontience a calla. Owing to anatomic distinctions at boys and girls different changes in the next bodies are observed. Splitting of cavernous bodies or their reduction occurs at boys, girls have a splitting of a clitoris, anomaly of development of a vagina.
Diagnostics and treatment of an ekstrofiya of a bladder
In recent years actively methods of antenatalny diagnostics of this defect develop. Ekstrofiya of a bladder can be noticeable already on the second ultrasonography screening, however it happens not always. Often anomaly of development is found only after the birth. As the symptomatology is specific, the diagnosis does not raise doubts. Carrying out a X-ray analysis of an abdominal cavity for detection of the combined defects, first of all, in an urinary system, but also in intestines and a spinal cord (the most characteristic combinations to a bladder ekstrofiya) is obligatory. With the same purpose ultrasonography is carried out. The excretory urography which allows to be convinced of integrity and the correct development of mochetochnik and kidneys is recommended.
Treatment is quick. The type of operation depends on bladder ekstrofiya degree, namely – on the sizes of the turned-out part of body and, respectively, the absent segment of a forward belly wall, and also on the accompanying malformations. If the extent of defect does not exceed 4 cm, reconstructive and plastic intervention which purpose – closing of defect by means of nearby fabrics and restoration of integrity of a bladder is carried out. It is enough as histologically the body is developed correctly, despite pathology in anatomy.
In case of the big extent of defect the artificial bladder which can perform function of the tank is created and be emptied at will. Also often mochetochnik are removed in a sigmovidny gut, it is an obligatory measure in case of their splitting. The big problem for surgeons is represented by a divergence of bones of a basin at a bladder ekstrofiya. This fact is the reason of the majority of ineradicable complications, in particular, incontiences of urine and a calla. The accumulated medical experience says about the high frequency of unsatisfactory results of operations that it is connected with a large amount of the combined defects and a major defect of a forward belly wall.
Forecast and prevention of an ekstrofiya of a bladder
The forecast for recovery doubtful. Not operated patients seldom live up to 10 years. As for success of expeditious treatment, 20-80% of the carried-out interventions lead only to minor improvement. Violations of an urodinamika continue to progress, the incontience remains. The risk of development of the ascending infection remains high. Prevention of an ekstrofiya of a bladder is possible in the antenatalny period, it is even better – at a pregnancy planning stage as much as possible to exclude influence of teratogenny factors, especially at a stage of laying of the main bodies and systems, that is in the first 4-8 weeks of pre-natal development.