Epispadias is a malformation of the urethra. This malformation mainly affects the male sex. The term epispadia comes from the Greek and means “cleft above”. Aptly enough, the condition is a congenital splitting of the urethra, which lies at the back of the penis. This is to be distinguished from hypospadias .

This is also a urethral cleft, which, unlike epispadias, is on the underside of the penis. The prevalence of epispadias is about one in 300,000 boys. The malformation is even less common in girls. Medicine assumes one in 400,000 people will be affected. There is a connection between the more common bladder exstrophy and epispadias. Bladder exstrophy is often associated with epispadias.

causes

Epispadias is a developmental disorder of the fetus during pregnancy. This disorder occurs in the third week of pregnancy and affects the cloacal membrane. The fetal genital protuberances do not fully fuse in the area of ​​the cloacal membrane. There will be gaping due to the painting position. Medicine assumes a hereditary factor, since the malformation occurs more frequently in some families.

General growth delays during pregnancy are now considered risk factors. In addition, taking medication during pregnancy can promote the malformation. In the case of epispadias associated with bladder exstrophy, the malformation of the cloacal membrane is even more pronounced than in the case of isolated epispadias.

Symptoms, Ailments & Signs

In boys, epispadias is either a cleft on the back of the penis or a widening of the external urethral meatus. In addition to epispadias, the limb may be shortened. Typically, there is also too much foreskin. Usually, the bladder is also split, resulting in urinary incontinence . There is usually no erectile dysfunction. In girls, epispadias usually presents as a complete cleft in the clitoris and bladder. The mons pubis is flat.

The urethra is wide and shortened. Medicine uses the suffixes gladis, pubis, coronaria, glandis and penis to differentiate between epispadias. Glans means a urethral opening on the glans penis. The word “coronaria” refers to the “corona glandis”. Penis means a location on the shaft of the penis and “pubis” means a location above the base of the penis. Totalis epispadias, on the other hand, is associated with an open bladder.

Diagnose

The doctor makes the diagnosis of epispadias via visual diagnosis. To determine the extent, he also carries out ultrasound examinations or draws up an elimination program. As a result of incontinence, those affected by epispadias have to struggle with psychological problems from a certain age .

In order to avoid psychological sequelae, the malformation should be corrected as quickly as possible. Correction before puberty is recommended so that the sexual organs can develop normally during this time.

When should you go to the doctor?

Epispadias should be corrected as soon as possible. In most cases, the malformation is diagnosed by a doctor immediately after birth, who will then initiate treatment as a matter of routine. With less pronounced symptoms, epispadias is often only noticed after days or weeks. Parents who notice abnormalities in their child’s genitals or even pronounced urinary incontinence should speak to their child ‘s doctor or family doctor . If treatment is not given, those affected often develop psychological problems.

Then a therapeutic consultation is definitely indicated. Of course, the malformation must also be corrected at the same time. If the diagnosis is made after puberty, a specialist in genital plastic surgery may need to be consulted. Regular check-ups should be carried out after the operation in order to detect any complications at an early stage. Women who took medication during pregnancy are more likely to give birth to a child with epispadias. If the medication cannot be avoided, the growth of the fetus should be monitored particularly closely.

Treatment & Therapy

Like hypospadias, epispadias can only be treated surgically. The aim of the surgical treatment is to produce a functional and optically largely normal penis or, in the case of girls, a largely normal clitoris. In boys, the surgeon usually performs a urethral plastic surgery for this purpose. This operation is possible from the first year of life. Ages between one and four years are recommended for the procedure.

In girls, on the other hand, the surgeon reconstructs the mons pubis, uniting the two halves of the clitoris that were separated by the cleft. The reconstructive operation should therefore take place at the youngest possible age so that social urinary continence can be achieved before puberty. If those affected cannot be freed from the malformation by puberty, psychological problems often arise. When boys are about three years old, attempts are therefore made to reconstruct the missing sphincter muscle.

If this attempt is unsuccessful, the urine must be drained from the bladder in some other way. How many operations are required for correction depends primarily on the wound healing after the first operation. In the case of fistulas , scarred constrictions of the urethra or bulges in the urethral mucosa, for example, follow-up operations are required. Those affected must therefore appear for regular check-ups after an operation, in which the wound healing and the success of the operation are checked on the one hand.

These check-ups can sometimes take several years. Normally, after correction, the penis or clitoris continue to develop normally, even during puberty. Only in rare cases is another intervention necessary to enable conventional development.

Outlook & Forecast

Isolated epispadias is not associated with an increased risk of urinary tract infections. Complications can occur if the deformity is particularly severe or is localized in an area that is difficult to reach. Epispadias in the area of ​​the base of the penis can lead to problems with ejaculation.

Ejaculation problems cannot be completely eliminated even with comprehensive treatment. In women affected, epispadias can be accompanied by stress incontinence. There can also be problems with sexual intercourse. Other organs are usually not affected in either sex.

The prospect of recovery therefore depends on the sex of the patient and the severity of the epispadias. Apart from the defect, the patients are considered to be healthy, although psychological problems can also occur in addition to the subsequent symptoms mentioned. If epispadias is diagnosed and treated immediately after birth, surgical treatment usually brings an improvement in any accompanying symptoms. If no further symptoms occur in the first few months after the procedure, a cure can be assumed.

The prognosis is worse if the epispadias are not detected, since they occur, for example, in the urethra. This can lead to incontinence, infections and the resulting complications in early childhood. In this case, too, an operation brings about an improvement in the symptoms.

prevention

So far it is largely unclear what causes epispadias. Since medication intake is likely to play a role during pregnancy, not taking medication is already a preventive measure. If this renunciation is not possible for health reasons, epispadias can hardly be prevented according to the current state of medicine.

aftercare

In the case of epispadias, the patient usually only has very limited options for aftercare. The affected person is primarily dependent on the medical examination and treatment by a doctor in order to permanently relieve the symptoms of epispadias. If the disease is not treated in time, it can lead to serious complications and ailments that can significantly complicate the patient’s life.

An early diagnosis should therefore be made in the first place. The treatment of epispadias is usually supported by an operative intervention. There are no particular complications, although the person concerned should rest after the procedure and take care of their body. Only strict bed rest can make a full recovery.

As a rule, strenuous and stressful activities should also not be carried out. In many cases, those affected are dependent on regular examinations by a doctor, even after successful treatment, in order to guarantee proper wound healing.

Care and support from one’s own family or friends also has a positive effect on the course of the epispadias and can prevent psychological upsets. The life expectancy of those affected is usually not reduced by this disease.

You can do that yourself

Patients with epispadias have no means of curing the disease. The body’s self-healing powers or alternative methods are not sufficient to alleviate the deformity.

Despite the illness, it is important to ensure adequate fluid intake . This should not be neglected or restricted, otherwise there is a risk of the organism being undersupplied.

In order to avoid sexual disorders after a correction of the urethra, this topic should be discussed intensively. The patient can inform himself comprehensively about the disease and also take advantage of a consultation. It is advisable to gain your own intimate experiences before having sexual contact with another person. If this is not sufficient, professional help should be sought.

Sex therapists help the patient to optimize their attitudes or their own behavior. In addition, an exchange with other patients can be perceived as helpful. Experiences can be discussed together and inhibitions can be reduced.

In a trusting atmosphere, many patients are able to talk about their everyday challenges. This allows mutual tips and hints to be exchanged. In addition, relaxation techniques are recommended to reduce stress. These strengthen mental strength and lead to a build-up of emotional resources.