What is a bladder ecstrophy?Neonatal bladder necrosis represents a urological emergency. The more time that elapses before the first operative reconstruction, the greater the risk of later complications.
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Bladder ecstrophy is an inherited malformation that is relatively rare. Bladder ecstasy occurs in approximately one in every 10, 000 to 50, 000 newborns. As a rule, boys are more frequently affected by the malformation than girls.
One of the main symptoms of bladder ecstrophy is a bladder that is exposed in the direction of the body. In those affected by the bladder ecstrophy, the mucous membrane of the bladder passes into the skin of the anterior abdominal wall. In addition, in both girls and boys, bladder ecstrophy is usually associated with splitting of the urethra.
In addition to the urethra and urinary bladder, in most cases external genitalia (genitals) and pelvis are also affected by the developmental disorder. In urology (a medical specialty that deals with, among other things, the urinary tract), the bladder ecstrophy is a serious defect.
Symptoms of bladder ecstrophy are usually caused by a disturbed development of the lower abdominal wall of an affected fetus. For example, associations of abdominal muscles or bones of the pelvis are impaired as part of this maldevelopment.
As a result, a rupture (breakthrough) occurs at the abdominal wall, through which the bladder penetrates to the outside. A common consistent trickle of urine from the bladder ecstasy-affected bladder is most often caused by a lack of attachment of the bladder neck (the bladder-urethra transition) and urinary bladder sphincter.
This lack of attachment is also a consequence of embryonic developmental disorder. The causes of bladder ecstrophy itself are still largely unknown - but probably both environmental factors and genetic factors play a role.
Symptoms, complaints & signs
Bladder ecstasy is primarily noticeable by the bladder visible from the outside. Most of the time part of the split urethra is also exposed. This leads to a flow of urine and occasionally to infections.
A malformation of the bladder can be quickly diagnosed based on the clear external signs and the symptoms mentioned and usually treated directly. If this happens early, there are no further complaints. In the absence of treatment, bladder ecstrophy can produce a number of other symptoms. These include urinary incontinence, bladder and genital infections as well as chronic malformations.
In some patients, urine builds up, causing kidney damage. Such a backlog is initially expressed by increasing pressure pain and urinary retention. In the further course cramps and fever can occur. Bladder ecstrophy can also affect sexual functions.
This manifests itself for example by potency disorders up to erectile dysfunction. In the course of an untreated bladder ecstrophy, there is a risk that mental discomfort will develop. Problems such as depression or social anxiety often occur as early as childhood and are a significant burden for those affected and their relatives.
Diagnosis & History
The suspected diagnosis of bladder ecstrophy can occasionally be carried out as part of prenatal (antenatal) examinations using ultrasound. Such a suspicion is based, for example, on the fact that repeatedly a defective bladder of the fetus is found.
If the prenatal diagnosis is not made, bladder ecstrophy is usually recognized at birth due to the characteristic symptoms. The individual course of bladder ecstrophy is influenced, among other things, by the success of therapeutic measures.
Failure to properly treat the developmental disorder may result in complications such as urinary incontinence (lack of urine retention control), urinary urinary reflux, frequent inflammation of the urinary and genital tract, or sexual dysfunction. However, consequential damage is occasionally possible even in successfully treated patients.
Neonatal bladder necrosis represents a urological emergency. The more time that elapses before the first operative reconstruction, the greater the risk of later complications. Due to the defect of the anterior abdominal wall, bladder ecstrophy can lead to an infection with pathogenic microorganisms. In the worst case, sepsis (blood poisoning) threatens immediately after birth. A prophylaxis with antibiotics is therefore absolutely necessary from the first day of life.
The surgical restoration of urinary incontinence is at the forefront of further treatment of bladder ecstasy. If the incontinence is not sufficiently resolved, chronic skin irritations can occur. These often result in superinfections with Candida albicans and other fungi.
In addition to sexual dysfunction, recurrent inflammations in the genitourinary tract as well as a backflow of urine into the kidneys are threatened as a long-term consequence of successful operations. Regular checks ensure an early diagnosis of metabolic diseases and detect carcinomas.
Due to the anatomical conditions of the vagina and uterus, women born with bladder ecstrophy are more likely to become pregnant. Due to the loosening (diastasis) of the pubic symphysis and the altered pelvic floor muscles, there is a risk of uterine prolapse. In order not to jeopardize the results of previous surgeries, physicians recommend in each case a delivery by caesarean section (elective cesarean section).
When should you go to the doctor?
As a rule, bladder ecstrophy is diagnosed during an ultrasound examination during pregnancy or immediately after birth. The malformation must be treated promptly, otherwise it may lead to the death of the child. After the surgery, more surgical procedures and visits to the doctor are usually required. Parents should consult with the family doctor or pediatric urologist regularly to be able to respond quickly to any complications.
Further medical examinations are necessary if incontinence causes infections and other complaints. Even with mental illness should be gone with the affected child to a doctor. Often the quality of life can be improved by further surgical measures and cosmetic interventions.
Accompanying this, the doctor will refer the affected person to a therapist or self-help group. The measures to be taken in detail depend on the severity of bladder ecstrophy and the physical and psychological effects. By early consultation, ideally during pregnancy, the treatment can be optimized and complications are largely excluded.
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Treatment & Therapy
Promising medical treatments for bladder ecstrophy are usually performed by surgery. The urinary bladder ecstrophy is an emergency.
According to international guidelines, closure of the bladder with concomitant stabilization of the abdominal wall in the affected child must first be performed surgically within 24 to 72 hours after birth. As a rule, in subsequent years of life of a child suffering from bladder eczema, further operations then follow; The objectives of such interventions include, for example, regaining voluntary control of urinary bladder function (urinary continence) and maintaining healthy kidney function.
Since most of the sex organs are affected by a bladder ecstrophy, further possible interventions also aim at a restoration of the corresponding organs; These recovery measures can be done both at the functional and the cosmetic level.
In most cases, those affected by a bladder ecstrophy eventually need lifelong, regular follow-up examinations. Above all, these examinations serve the early detection of possible sequelae of bladder ecstrophy. These sequelae include, for example, metabolic disorders or the development of carcinomas (malignant tissue neoplasm) on the mucous membranes of the lower abdomen.
Outlook & Forecast
The cure for bladder ecstrophy depends on the severity of the disease, the start of treatment and the general health of the patient.
If there are no other disorders or illnesses, the newborn will usually undergo surgery within the first two days of life. The malformation of the bladder is corrected as far as possible. Only a few patients need only one corrective intervention to cure or relieve the symptoms. In most cases, further operations follow during the growth and development process. In these attempts are made to create the physical conditions for a voluntary bladder control.
Since often the genitals are also damaged in a bladder ecstrophy, these are also subjected to a correction treatment in the first years of life into adulthood. Every surgery comes with the usual risks and side effects. As a result, over the first 20 years of life, the patient is repeatedly exposed to severe stress from which he has to recover.
The more stable the health and the stronger the immune system, the better and faster the individual interventions can heal. If the patient does not resort to corrections, he will suffer from problems of urination and sexual dysfunction for life. If the interventions take place with optimal results, it is possible to achieve a degree of freedom from symptoms as far as possible.
Since the medicine so far has little knowledge about the causes of the formation of bladder ecstrophy, the disease can hardly be prevented.
However, the symptoms, complications and possible sequelae associated with bladder ecstrophy can be positively influenced by early and consistent treatment steps. Logically, women should abstain from smoking, alcohol and drugs altogether during pregnancy in order to avoid malformations of the child.
After the bladder ecstrophy has been surgically corrected, various aftercare measures apply. The patient first has to spend several hours in the recovery room to detect and treat complications in good time. The doctor will regularly check blood pressure and heart rate, and also make sure that the sutures heal optimally. If no abnormalities occur during this phase, the patient can be discharged.
First, however, the person concerned contains medical recommendations for the use of painkillers and sedatives. The bladder ecstrophy can lead to complications for some time after the procedure, which must be clarified by a doctor. A visit to a doctor is recommended, for example, for inflammation, itching or bleeding in the area of the surgical scar.
In addition, follow-up appointments agreed with the operating physician must be observed. General measures such as sufficient drinking (especially mineral water and tea), the renunciation of alcohol and nicotine as well as the avoidance of strong sunlight in the area of the scars are essential.
With a fresh seam should also be waived seven to nine days on the shower. If no complications occur, no further follow-up is needed. Patients with bladder eczema, however, often suffer from other diseases, which is why a regular visit to the doctor is recommended.
You can do that yourself
The malformations in the area of the urinary bladder, which are part of bladder ecstrophy from birth, can only be treated by medical interventions, so that no direct measures for self-help are applicable. However, patients and their caregivers support the medical treatment by a behavior adapted to the most surgical procedures.
Usually, the newborn undergoes surgery within the first days of life to compensate for the malformation. Constant medical monitoring of the newborn is essential, whereby parents in principle follow the instructions of the specialists and the hospital staff.
Until the patients with bladder ecstrophy reach adulthood, in many cases further surgical procedures are necessary. The aim of these operations is to ensure urinary continence and, if necessary, to reconstruct the sexual organs. With regard to continence, patients support the therapeutic success of physiotherapy, which strengthens appropriate muscle areas in the lower abdominal area. Such exercises are feasible at home.
As long as the urinary incontinence is not given, the patients are often dependent on diapers. Suitable models are discreet, so that they hardly attract attention externally and do not disturb those affected in their normal everyday life. Thus, despite bladder ecstrophy, patients are able to participate in social life and minimize incontinence limitations.