In epispadias there is a gap in the urethra. Boys are significantly more likely to be affected by the phenomenon than girls. Epispadias can be corrected surgically, and the procedure should be performed before puberty.
What is epispadias?
Epispadias is a malformation of the urethra. This malformation mainly affects the male sex. The term Epispadie comes from the Greek and translates as: “Column above”. Aptly, the disease is a congenital split in the urethra that lies on the back of the penis. This is to be distinguished from hypospadias.
This is also a split urethra, which, unlike epispadias, lies on the underside of the penis. The prevalence of epispadias in boys is around one in 300,000. In girls, the malformation is significantly less common. Medicine assumes one out of 400,000 people 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 genital cusps of the fetus do not completely fuse in the area of the cloacal membrane. There is a gap due to the painting position. Medicine assumes a hereditary factor, as the malformation occurs more frequently in some families.
General growth delays in pregnancy are now considered risk factors. In addition, taking medication during pregnancy can promote the malformation. In the case of epispadias as part of a bladder exstrophy, the malformation of the cloacal membrane is even more pronounced than in individual epispadias.
Symptoms, ailments & signs
In boys, epispadias is either a gap on the back of the penis or an enlargement of the external urethral opening. In addition to epispadias, the limb can be shortened. Typically there is also too much foreskin. Usually the bladder is also split, causing urinary incontinence. Usually there is no erectile dysfunction. In girls, epispadias usually appear as a complete gap in the clitoris and bladder. The mound of venus is flat.
The urethra is wide and shortened. For gradual differentiation of epispadias, medicine uses the additions gladis, pubis, coronaria, glandis and penis. With gladis a urethral opening on the glans penis is meant. The word “coronaria” refers to the “Corona glandis”. Penis means a localization on the penis shaft and “pubis” stands for a position above the penis root. A “totalis” epispadia, on the other hand, is associated with an open bladder.
Diagnosis
The doctor makes the diagnosis of epispadias via eye diagnosis. To determine the extent, he also performs ultrasound examinations or creates an elimination program. Due to incontinence, those affected by epispadias have to struggle with psychological problems from a certain age.
In order to avoid psychological complications, the malformation should be corrected as soon as possible. Correction is recommended before puberty so that the genital organs can develop normally during this time.
When should you go to the doctor?
Epispadias should be corrected as soon as possible. The malformation is usually diagnosed by a doctor right after the birth, who will then initiate the treatment as a matter of routine. If the symptoms are less pronounced, the epispadias is often only noticed after days or weeks. Parents in their child abnormalities in the genital area, or even find a pronounced urinary incontinence should, with the children – or family doctor speak. If treatment is not received, those affected often develop psychological problems.
Then therapeutic advice is definitely indicated. At the same time, of course, the malformation must also be corrected. If the diagnosis is made after puberty, a specialist in genital plastic surgery may need to be called in. Regular check-ups should be carried out after the procedure in order to identify any complications at an early stage. Women who took medication during pregnancy are more likely to have a child with epispadias. If medication cannot be avoided, the growth of the fetus should be monitored particularly closely.
Treatment & Therapy
Like hypospadias, epispadias can only be treated surgically. Surgical treatment aims to produce a functional and optically largely normal penis or, in girls, a largely normal clitoris. In boys, the surgeon usually performs urethral surgery for this purpose. This procedure is possible from the first year of life. An age between one and four years is recommended for the procedure.
In girls, on the other hand, the surgeon reconstructs the mons pubis and unites the two halves of the clitoris that have been 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 relieved of the malformation by puberty, psychological problems often arise. In boys around the age of three, attempts are therefore made to reconstruct the non-existent sphincter muscle.
If this attempt is unsuccessful, the urine must be diverted from the urinary bladder in some other way. How many interventions are required for correction depends primarily on the wound healing after the first operation. For fistulas, scarred constrictions of the urethra or bulging of the urethral mucosa, for example, follow-up operations are due. Those affected must therefore appear for regular check-ups after an operation, during which on the one hand the wound healing and on the other hand the success of the operation are checked.
These check-ups can take several years under certain circumstances. Usually, after a correction, the penis or clitoris will 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. Secondary diseases can occur if the deformity is particularly severe or is located in a location that is difficult to reach. Epispadias in the area of the penis base can lead to problems with ejaculation.
The ejaculation problems cannot be completely resolved even with comprehensive treatment. In female patients, epispadias can be associated with stress incontinence. In addition, there can be problems with sexual intercourse. Other organs are usually not affected in either sex.
The prospect of recovery therefore depends on the gender of the patient and the severity of the epispadias. Apart from the defect, the patients are considered healthy, although psychological problems can occur in addition to the subsequent symptoms mentioned. If the epispadias is diagnosed and treated immediately after birth, surgical treatment usually improves any accompanying symptoms. If there are no further symptoms in the first few months after the procedure, a cure can be assumed.
The prognosis is worse if the epispadias is not detected because it occurs, for example, in the urethra. Then incontinence, infections and the resulting complications can occur in early childhood. In this case, too, an operation brings 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 waiver 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 has only very limited options for follow-up care. The affected person is primarily dependent on a medical examination and treatment by a doctor in order to permanently alleviate the symptoms of epispadias. If the disease is not treated in time, it can lead to serious complications and complaints that can make the patient’s life significantly more difficult.
First and foremost, an early diagnosis should therefore be made. The treatment of epispadias is usually supported by a surgical procedure. This does not result in any particular complications, and the person affected should rest after the procedure and take care of their body. Complete recovery can only occur through strict bed rest.
As a rule, strenuous and stressful activities should also not be performed. In many cases, even after successful treatment, those affected are dependent on regular examinations by a doctor, above all 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 the person affected is usually not restricted 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 insufficient to alleviate the malformation.
Despite the disease, it is important to ensure that you drink enough fluids. This should not be neglected or restricted, as otherwise there is a risk of an undersupply of the organism.
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 advice. It is advisable to gain your own intimate experiences before sexual contact with another person takes place. If this is not enough, professional help should be sought.
Sex therapists help the patient to optimize their attitudes or behavior. In addition, an exchange with other patients can be perceived as helpful. Experiences can be discussed together and inhibitions can be broken down.
Many patients manage to talk about their everyday challenges in a trusting atmosphere. This allows mutual tips and hints to be exchanged. In addition, relaxation techniques are recommended to reduce stress. These strengthen the mental strength and lead to the build-up of emotional resources.
