A bladder exstrophy in newborns is to treat emergency medical normally. Despite successful therapeutic steps, symptoms can occur for a lifetime.

What is a bladder exstrophy?

According to sciencedict, bladder exstrophy is an already congenital malformation that occurs comparatively rarely. Bladder exstrophy occurs in approximately one in 10,000 to 50,000 newborns. As a rule, boys are more often affected by the malformation than girls.

One of the main visible symptoms of bladder exstrophy is a urinary bladder that is open to the outside of the body. In those affected by bladder exstrophy, the mucous membrane of the urinary bladder merges with the skin of the anterior abdominal wall. In both girls and boys, bladder exstrophy is usually accompanied by a split in the urethra.

In addition to the urethra and bladder, in most cases the external genitalia (sexual organs) and the pelvis are also impaired by the developmental disorder. In urology (a medical specialty that deals with the urinary tract, among other things), bladder exstrophy is a serious defect.

Causes

Symptoms of bladder exstrophy are usually caused by impaired development of the lower abdominal wall of an affected fetus. As part of this undesirable development, for example, associations of abdominal muscles or the bones of the pelvis are impaired.

As a result, a rupture (a breakthrough) occurs in the abdominal wall, through which the urinary bladder penetrates to the outside. The frequent consistent dripping of urine from the urinary bladder, which is impaired by bladder exstrophy, is usually caused by a lack of contact between the bladder neck (the transition between the urinary bladder and urethra) and the urinary bladder sphincter.

This lack of attachment is also a consequence of the embryonic developmental disorder. The causes of bladder exstrophy are still largely unknown – but both environmental and genetic factors probably play a role.

Symptoms, ailments & signs

Bladder exstrophy is primarily noticeable through the urinary bladder that is visible from the outside. Most of the time, part of the split urethra is also exposed. This leads to leakage of urine and occasionally infections.

A malformation of the urinary bladder can be diagnosed quickly based on the clear external signs and the symptoms mentioned and usually treated directly. If this happens early enough, there will be no further complaints. If left untreated, bladder exstrophy can cause a number of other symptoms. These include urinary incontinence, infections of the bladder and genital organs as well as chronic pain in the area of ​​the malformation.

In some patients, the urine backs up, which can cause kidney damage. Such a back pressure is initially expressed by increasing pressure pain and urinary retention. As the disease progresses, cramps and fever may occur. Bladder exstrophy can also affect sexual functions.

This manifests itself, for example, erectile dysfunction through to erectile dysfunction. In the course of an untreated bladder exstrophy, there is a risk that emotional complaints will develop. Problems such as depression or social anxiety often arise in childhood and represent a significant burden for both those affected and their relatives.

Diagnosis & course

The suspected diagnosis of bladder exstrophy can occasionally be made during prenatal (prenatal) examinations using ultrasound. Such a suspicion is based, for example, on the fact that an insufficiently filled bladder of the fetus has repeatedly been detected.

If the prenatal diagnosis is not made, bladder exstrophy is usually recognized at birth based on the characteristic symptoms. The individual course of a bladder exstrophy is influenced, among other things, by the success of the therapeutic measures that take place.

Failure to properly treat the developmental disorder can result in complications such as urinary incontinence (lack of control over the retention of urine), backlogs of urine in the kidneys, frequent inflammation of the urinary and reproductive system or sexual dysfunction. Corresponding consequential damage is occasionally also possible in successfully treated patients.

Complications

Bladder exstrophy in newborns is a urological emergency. The longer it takes until the first surgical reconstruction, the greater the risk of later complications. Due to the defect of the anterior abdominal wall, an infection with pathogenic microorganisms can occur in bladder exstrophy. In the worst case, there is a risk of sepsis (blood poisoning) immediately after the birth. Prophylaxis with antibiotics is therefore mandatory from the first day of life.

Surgical restoration of urinary continence is in the foreground in the further treatment of bladder exstrophy. If the incontinence is not adequately corrected, chronic skin irritation can occur. These often lead to superinfections with Candida albicans and other fungi.

In addition to sexual dysfunction, the long-term consequences of successful operations are above all recurrent inflammation in the urogenital area and a backlog of urine in the kidneys. Regular checks ensure an early diagnosis of metabolic diseases and serve to identify the development of cancer.

Because of the anatomical relationships between the vagina and uterus, women born with bladder exstrophy are more likely to become pregnant. Due to the loosening (diastasis) of the pubic symphysis and the changed pelvic floor muscles, there is a risk of uterine prolapse. In order not to jeopardize the results of previous operations, doctors always recommend delivery by caesarean section (elective cesarean section).

When should you go to the doctor?

Usually, bladder exstrophy is diagnosed during an ultrasound scan during pregnancy or immediately after giving birth. The malformation must be treated immediately, otherwise the child may die. After the operation, further surgical interventions and visits to the doctor are usually required. Parents should consult their family doctor or pediatric urologist on a regular basis in order to be able to react quickly to any complications.

Further medical examinations are necessary if the incontinence leads to infections and other complaints. If you have psychological complaints, you should go to a doctor with the affected child. The quality of life can often be improved through further surgical measures and cosmetic interventions.

At the same time, the doctor will refer the person concerned to a therapist or a self-help group. The detailed measures to be taken depend on the severity of the bladder exstrophy and the physical and mental effects. Early consultation, ideally during pregnancy, can optimize treatment and largely rule out complications.

Treatment & Therapy

Promising medical treatments for bladder exstrophy usually take place surgically. Bladder exstrophy is an emergency in urology.

According to international guidelines, closure of the urinary bladder with accompanying stabilization of the abdominal wall in the affected child must initially be performed surgically within 24 to 72 hours after birth. As a rule, further operations then follow in the further years of a child suffering from bladder exstrophy; The goals of such interventions include regaining voluntary control over the function of the bladder (urinary continence) and maintaining healthy kidney functions.

Since the genital organs are usually also impaired by a bladder exstrophy, further possible interventions aim to restore the corresponding organs; these restoration measures can be carried out on both a functional and a cosmetic level.

In most cases, bladder exstrophy ultimately requires lifelong, regular check-ups. Above all, these examinations serve to identify possible secondary diseases of a bladder exstrophy at an early stage. These secondary diseases include, for example, metabolic disorders or the development of carcinomas (malignant tissue growth) on the mucous membranes of the lower abdomen.

Outlook & forecast

The chances of recovery from bladder exstrophy depend on the severity of the disease, the start of treatment and the general health of the patient.

If there are no other disorders or diseases, the newborn will normally undergo an operation within the first two days of life. The malformation of the bladder is corrected as much as possible. Only in a few patients is a single corrective intervention sufficient to heal or alleviate the symptoms. In most cases, further operations follow as the growth and development process progresses. In these, an attempt is made to create the physical prerequisites for voluntary bladder control.

Since the genital organs are often also damaged in bladder exstrophy, these too are subjected to corrective treatment in the first years of life up to adulthood. Any surgery comes with the usual risks and side effects. As a result, the patient is exposed to multiple heavy loads over the first 20 years of his life, 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 goes without the corrections, he will suffer from urination problems and sexual dysfunction for life. If the interventions take place with optimal results, you can largely achieve freedom from symptoms.

Prevention

Since medicine has so far not had much knowledge about the causes of the formation of a bladder exstrophy, the disease can hardly be prevented.

The severity of complaints, complications and possible secondary diseases in connection with a bladder exstrophy can, however, be positively influenced by early and consistent treatment steps. Logically, women should completely refrain from smoking, alcohol and drugs during pregnancy in order to avoid malformations of the child.

Aftercare

After the bladder exstrophy has been surgically corrected, various follow-up measures apply. The patient must first spend a few hours in the recovery room in order to identify and treat complications in good time. The doctor will check your blood pressure and pulse regularly and will also make sure that the sutures are healing optimally. If there are no abnormalities in this phase, the patient can be discharged.

First of all, however, the person concerned receives medical recommendations for taking painkillers and sedatives. Bladder exstrophy can lead to complications for some time after the operation, which must be clarified by a doctor. A visit to the doctor is recommended, for example, if there is inflammation, itching or bleeding in the area of ​​the surgical scar.

In addition, follow-up appointments agreed with the operating doctor must be observed. General measures such as drinking enough (especially mineral water and tea), avoiding alcohol and nicotine and avoiding strong sunlight in the area of ​​the scars are essential.

With a fresh seam, you should also avoid the shower for seven to nine days. If there are no complications, no further follow-up is required. However, patients with bladder exstrophy often suffer from other diseases, which is why a regular visit to the urologist is recommended.

You can do that yourself

The malformations in the area of ​​the urinary bladder that exist from birth as part of bladder exstrophy can only be treated by medical interventions, so that no direct measures for self-help can be applied. However, patients and their guardians support the medical treatment by adopting behavior that is usually adapted to surgical interventions.

Usually, the newborn patient undergoes an operation within the first few days of life to compensate for the deformity. Constant medical monitoring of the newborn is essential, whereby parents generally adhere to the instructions of specialists and clinic staff.

In many cases, further surgical interventions are required by the time patients with bladder exstrophy reach adulthood. The aim of these operations is to ensure urinary continence and, if necessary, to reconstruct the genital organs. With regard to continence, the patients support the success of the therapy through physiotherapy, which strengthens the corresponding muscles in the lower abdominal area. Such exercises can be performed at home.

As long as the urinary continence is not given, the patients are often dependent on diapers. Suitable models are discreet so that they are hardly noticeable from the outside and do not disturb those affected in their normal everyday life as much as possible. Thus, despite bladder exstrophy, patients are able to take part in social life and minimize restrictions caused by incontinence.

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