Epispadia: Symptoms and Treatment

Epispadia — is one of the varieties of anomalies in the development of the urogenital system. With this ailment, the wall of the urethra is not enlarged. Despite the fact that the ailment is quite rare, it is in most cases diagnosed in boys, rather than in girls.



The causes and pathogenesis of the development of such a disease is currently unknown, but clinicians suggest that the main factor is either the complicated course of pregnancy, or the unhealthy lifestyle that the future mother led during the period of gestation.

Symptomatology will differ depending on the shape of a similar developmental anomaly. The main signs are considered to be soreness during urination, urinary incontinence, tenderness during intercourse.

The correct diagnosis can be made already during the initial examination, but a number of instrumental diagnostic measures are needed to confirm the theories. Treatment is carried out only surgically.

Etiology

To date, the reasons for the formation of this type of anomaly of the genitourinary system remain unknown. There are theories that such a pathology arises against the background of embryogenesis disturbance in the interval between the seventh and fourteenth weeks of intrauterine development of the fetus.

Nevertheless, clinicians identify a large number of predisposing factors causing the occurrence of such an ailment:


  • a wide range of viral infections that a woman can bear during the period of gestation;
  • addiction to bad habits — not all women consider it necessary to give up alcohol, nicotine and drugs just because they will soon become mothers
  • adverse environmental conditions
  • early or severe toxicosis
  • long-term effects of stressful situations and nerve stresses
  • injuring the abdomen;
  • effects on the body of ionizing radiation
  • the presence of endocrine disruption in the woman in the position
  • the effect on the fetus of injuries from the place of work of the future mother.

Other factors are not excluded.

Classification

It is worth noting that epispadia is divided into male and female. In addition, absolutely all forms of epispadias can be of two types:


  1. Only with the cleavage of the urethra
  2. with splitting of the sphincter of the bladder.

According to the degree of splitting of the urethra of epispadias, boys can:


  • capitate — while the urethra is divided up to the coronary groove of the penis
  • trunk — in such cases, the splitting is observed along the entire length of the penis
  • pubic-trunk — a distinctive feature is that the urethral fissure is split completely, and the sphincter of the bladder is not completely
  • total epispadia — not only the urethra but also the front wall or neck of the bladder has undergone separation

A similar deviation in girls happens:


  1. clitoral — the clitoris is split into two bodies, separated by a transversely located slit of the urethra. In this case, the clitoris is located below it;
  2. subtotal — the child is divided clitoris and labia;
  3. total — the pathology is subjected to such parts of the reproductive system as the clitoris, labia, lonnoe articulation and the sphincter of the bladder. The opening may look like a wide funnel, from which the shell of the last organ is often protruded.

The overwhelming majority diagnoses a total or subtotal form of pathology.

Symptoms

Epispadia in children has various clinical manifestations, which directly depend on the variety of the disease. Thus, the head type of such a disease can be completely asymptomatic, but only on the condition that the cleavage of the foreskin region does not occur. In such cases, the main signs are:


  • spattering of the urine stream, but the act of urination itself is not violated;
  • Minor deformation of the penis. Nevertheless, erection and other sexual functions remain normal.

Symptoms of the trunk form of congenital malformation:





  1. The urethral opening is located near the root of the penis
  2. significant spattering of urine, why the boy or man is forced to urinate while sitting
  3. urinary incontinence — observed in cases of strong physical exertion, intense laughter or cough
  4. pronounced curvature of the penis — its shortening and curvature towards the anterior wall of the abdominal cavity is observed
  5. difficulty in making sexual contact

The lobkovo-stem type of pathology is expressed in:


  • shortening and considerable deformation of the body
  • the location of the urethral opening directly under the lone articulation
  • the impossibility of having sex;
  • urinary incontinence

Total epispadia has the following symptoms:


  1. Underdevelopment of the penis and its shape in the form of a hook
  2. the constant outflow of urine outwards — this is due to the fact that the cavernous bodies and the neck of the bladder have undergone cleavage
  3. total absence of sexual functions
  4. curvature of the pubic bones, which is expressed in the «duck’s gait»
  5. the divergence of the abdominal muscles, which leads to the formation of a «frog» belly.

In addition, approximately every third patient has:


  • pyelonephritis;
  • hydronephrosis;
  • hypoplasia of the testicles, prostate or scrotum
  • cryptorchidism
  • vesicoureteral reflux.

As for the similar disease in girls, then:


  1. the clitoral version of the disease does not become a cause of the violation of the act of urination;
  2. The subtotal form is expressed in urinary incontinence when sneezing, coughing, or under heavy loads
  3. total type is characterized by the fact that there is a constant leakage of urine, which is why maceration of the skin in the area of ​​the outer genital and hips from the inside is observed. In addition, there may be an infection in the urinary tract, diarrhea, vulvovaginitis, vaginal doubling, clitoral hypoplasia, or labia.

Diagnostics

The pronounced varieties of such an anomaly are recognized immediately after the infant appears on the light, and the less obvious ones are detected only when urine incontinence is noted in a child older than a year.

The primary examination is as follows:


  • conducting a detailed interview of the patient or his parents for the severity of symptomatology
  • studying the anamnesis of the life of the patient and his parents
  • conducting an objective examination — to assess the size of the pathology. This will enable the doctor to determine the form of the disease and the tactics of treatment.

A primary inspection can be carried out:


  1. pediatrician
  2. as a child surgeon
  3. child urologist or gynecologist

Using laboratory methods of examination, it is possible to identify the attachment of infection of the genitourinary system.

For complex assessment of the condition and functioning of the urethra and bladder, it is necessary to conduct instrumental examinations, among which are:


  • Ultrasound of organs such as the kidneys and bladder
  • radiography
  • urethrography
  • urography
  • profilometry
  • cystometry
  • electromyography
  • cystography
  • pelvic ultrasound for girls
  • Scrotal ultrasound for boys.

Treatment

Such a congenital defect is eliminated only by surgical means. It is worth noting that the maternal form of the disease in boys and the clitoral form in girls do not require any specific tactics of therapy.

Indications for the operation can serve as:


  1. urinary incontinence
  2. defects of a cosmetic nature;
  3. a psychophysical disorder of a small patient

The best age for the operation is five years.

In the treatment of pathology in boys, the doctor sets himself the solution of several problems:


  • Elimination of penile deformities
  • formation of a complete canal of urination
  • Sphincter recovery.

To achieve these effects:


  1. correction of the curvature of the genital organ
  2. urethroplasty;
  3. Sphincteroplasty.

Girls have plastic surgery of the clitoris or the neck of the bladder.

Regardless of the treatment tactics, the postoperative period should include washing and bladder catheterization.

Possible complications

The operational tactics of therapy can cause development:


  • fistulas in the bladder or canal of urination
  • strictures in the urethra
  • urethritis and cystitis.

In addition, male members can be observed:


  1. erectile dysfunction
  2. infertility;
  3. the curvature of the penis.

Prevention and Forecast

Since the reasons for the development of such an anomaly remain unknown, there are no specific preventive measures. However, female representatives during pregnancy need:


  • completely abandon bad habits;
  • Properly and nutritionally balanced
  • Avoid stress as much as possible
  • observe precautionary measures, and it is best to completely abandon work that involves occupational hazards
  • Seek qualified help in case of a small change in your health.

Owing to the possibility of early detection and operation, it is often possible to achieve a favorable prognosis, which consists in the restoration and control of urination, as well as the prevention of psychological disorders.




It should be noted that those patients who have had an operation to eliminate epispadias in their childhood should be constantly observed in the urologist (for males) or the gynecologist (for the female sex).