Epispadias is a cleft formation of the urethra. Boys are significantly more often affected by the phenomenon than girls. Epispadias can be corrected surgically, although the procedure should be performed before puberty.
What is an epispadias?
Epispadias is a malformation of the urethra. This malformation mainly affects the male sex. The term epispadias comes from the Greek and translates as “cleft above”. More accurately, the condition is a congenital cleft of the urethra, which is located at the back of the penis. Hypospadias is to be distinguished from this. This is also a urethral cleft, but unlike epispadias, it is located on the underside of the penis. The prevalence of epispadias in boys is about one in 300,000, while in girls the malformation is even rarer. Medical science assumes one affected person in 400 000. There is a connection between the more common bladder exstrophy and epispadias. Thus, bladder exstrophy is often associated with epispadias.
Causes
Epispadias is a developmental disorder of the fetus during pregnancy. This disorder occurs during the third week of pregnancy and affects the cloacal membrane. The genital cusps of the fetus do not fuse completely in the area of the cloacal membrane. There is a gap formation due to the malposition. Medical science assumes a hereditary factor, since the malformation occurs more frequently in some families. General growth retardation during pregnancy is now considered a risk factor. In addition, taking medications during pregnancy may promote the malformation. When epispadias occur as part of bladder exstrophy, the malformation of the cloacal membrane is even more extreme than in single epispadias.
Symptoms, complaints, and signs
In boys, epispadias are either a cleft on the back of the penis or an enlargement of the external urethral orifice. In addition to epispadias, the member may be shortened. Typically, there is also too much foreskin. Typically, the bladder is also split, resulting in urinary incontinence. Erectile dysfunction is usually not present. In girls, epispadias usually impress as complete clefting of the clitoris and bladder. The mons veneris is flat. The urethra is wide and shortened. For gradual differentiation of epispadias, medicine uses the additions gladis, pubis, coronaria, glandis and penis. Gladis refers to a urethral orifice 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 root of the penis. An epispadias “totalis”, on the other hand, is associated with an open bladder.
Diagnosis
The physician makes the diagnosis of epispadias by visual diagnosis. He also performs ultrasound examinations or establishes an elimination program to determine the extent. Due to incontinence, sufferers of epispadias have to deal with psychological problems after a certain age. To avoid psychological sequelae, the malformation should be corrected as soon as possible. It is advisable to correct it before puberty 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. Most often, the malformation is detected immediately after birth by a doctor, who will then routinely initiate treatment. If the symptoms are less pronounced, epispadias are often not noticed until days or weeks later. Parents who notice abnormalities in their child’s genital area or even marked urinary incontinence should talk to their pediatrician or family doctor. If treatment fails to take place, those affected often develop psychological problems. Therapeutic counseling is then indicated in any case. Accompanying this, of course, the malformation must be corrected. If the diagnosis is made after puberty, a specialist in genital plastic surgery may have to be consulted. After the procedure, regular check-ups should be performed to detect any complications at an early stage. Women who have taken medication during pregnancy are more likely to give birth to a child with epispadias. If medication cannot be avoided, the growth of the fetus should be monitored particularly closely.
Treatment and therapy
Like hypospadias, epispadias can only be treated surgically. Surgical treatment aims to produce a functional and visually largely normal penis or, in girls, a largely normal clitoris. In boys, the surgeon usually performs urethroplasty for this purpose. This procedure is possible from the age of one. The recommended age for the procedure is between one and four years. In girls, on the other hand, the surgeon reconstructs the mons veneris and unites the two halves of the clitoris that were separated by the cleft. Therefore, reconstructive surgery should be performed at the youngest possible age so that social urinary continence can be achieved before puberty. If the affected person cannot be freed from the deformity by puberty, psychological problems often set in. In boys, therefore, an attempt is made around the third year of life to reconstruct the non-existent sphincter. If this attempt is unsuccessful, the urine must be drained from the bladder in another way. The number of operations required for correction depends mainly on the wound healing after the first operation. In the case of fistulas, scarred narrowing of the urethra or bulging of the urethral mucosa, for example, follow-up operations are required. Patients must therefore attend regular check-ups after an operation to monitor wound healing and the success of the operation. These check-ups may extend over several years. Normally, the penis or clitoris develop normally after correction, even during puberty. Only in rare cases will a repeat operation be necessary to allow conventional development.
Outlook and prognosis
Isolated epispadias are not associated with an increased risk of urinary tract infections. Consequential disease may occur if the malformation is particularly severe or localized to a site that is difficult to reach. For example, epispadias in the region of the base of the penis can lead to problems with ejaculation. Ejaculation problems cannot be completely resolved even with extensive treatment. In female sufferers, epispadias may be associated with stress incontinence. In addition, there may be problems with sexual intercourse. Other organs are not usually affected in either sex. Thus, the prospect of recovery depends on the sex of the patient and the severity of the epispadias. Away from the defect, patients are considered healthy, although psychological problems may occur in addition to mentioned secondary symptoms. If epispadias are detected and treated immediately after birth, surgical treatment usually brings improvement of any accompanying symptoms. If no further complaints occur in the first few months after the operation, 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 resulting complications can occur in early childhood. In this case, surgery also brings an improvement of the symptoms.
Prevention
To date, it is largely unclear what causes epispadias. Since medication use during pregnancy probably plays a role, abstaining from medication is already a preventive measure. If this abstention 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 very limited options for aftercare. The affected person is primarily dependent on medical examination and treatment by a physician to permanently alleviate the symptoms of epispadias. If the condition is not treated in time, it can lead to serious complications and discomfort that can significantly complicate the patient’s life. Therefore, in the first place, an early diagnosis should be made. The treatment of epispadias is usually supported by surgical intervention. There are no particular complications, although the patient should rest and take care of his or her body after the procedure. Complete recovery can only be achieved through strict bed rest. As a rule, strenuous and stressful activities should also be avoided.In many cases, even after successful treatment, those affected are dependent on regular examinations by a doctor, above all to guarantee proper wound healing. In this context, care and support from one’s own family or friends also have a positive effect on the course of epispadias and can prevent psychological upsets. The life expectancy of the affected person is usually not limited by this disease.
What you can do yourself
Patients with epispadias have no options to bring about a cure for the condition. The body’s self-healing powers or alternative methods are not sufficient to achieve relief from the deformity. Despite the condition, adequate hydration should be ensured. This should not be neglected or restricted, otherwise there is a risk of undersupply of the organism. In order to avoid sexual disorders after correction of the urethra, this topic should be discussed intensively. The patient can inform himself comprehensively about the disease and additionally take advantage of a consultation. It is recommended to gain own intimate experience before sexual contact with another person takes place. If this is not sufficient, professional help should be sought. Sex therapists help patients to optimize their attitudes or their own behavior. In addition, an exchange with other patients may be found helpful. Experiences can be discussed together and inhibitions can be reduced. In a trusting atmosphere, many patients succeed in talking about their everyday challenges. This allows mutual tips and advice to be exchanged. In addition, relaxation techniques are recommended to reduce stress. These strengthen mental power and lead to a build-up of emotional resources.
