Epispadia and bladder exstrophy in children

The most severe congenital disorders of the urinary system — bladder exstrophy. This rare disease is found in infants immediately after birth and requires surgical intervention. Attempts of conservative treatment aggravate the situation, the time the surgery fixes the defect. Unfortunately, it is not always possible to live a full life in the future.

Description of pathology

Bladder extrophy is a congenital anomaly in which the bladder falls out through the abdomen. In this case the front wall of the peritoneum and the bladder are absent. The body is not formed and fed into it by the ureters, the urine spills out. As a result, affected tissue surrounding the cavity, irritated the mucous membrane of the open bladder. Pathology is rare, 40 thousand kids have 1 case, while in boys it is found in 2 times more often.

Epispadia — underdevelopment of the external genitalia. The outer opening of the urethra (the urethra) is higher than it should be. Urethra split. For example, in boys it is on the pubis or on the surface of the penis. Bladder exstrophy and epispadia considered as a group of congenital abnormalities that are combined such phenomena is underdeveloped urethra and bladder.

The degree of extrophy bladder

According to the severity of the bladder extrophy divided into 3 degrees:

  1. Pathology assigned to the first degree, if the lesion of the abdominal wall not more than 4 cm of the Pubic bone should not diverge more than 4 cm Other pathologies is not available or is insignificant. Timely treatment eliminates the threat of human life.
  2. The second degree of gravity is characterized by a hole in the abdominal wall with a diameter of 5-7 cm Dice odds up to 8 cm are Observed comorbidities.
  3. To the third degree are the anomalies of the divergence of the bones of more than 9 cm Diameter holes on the abdominal wall more than 8 cm Condition is complicated by pathologies of other organs.

Classification of epispadias

If epispadias is only possible defect of the urethra or defect affecting the urethra, and a challenge to its connection with the bladder. Features of the disease differ depending on the sex of the patient. In boys, the disease can be:

  • full — struck the whole urethra and the bladder;
  • head — splitting of the urethra the glans penis;
  • stem — the urethra is affected along the entire penis;
  • pubic-stem — struck by the whole urethra to its inner hole.

Women’s epispadia found in such forms as:

  • total — affection spreads from the labia to the bladder;
  • Subtotal — the only defect of the clitoris and of the labia;
  • clitoria — the separation of the clitoris in half.

Head form

This is the least severe form of the disease, allowing you to live a full life. Observed deformation of tissue from the urethra to the coronary sulcus of the penis. The result is the penis pulled to the stomach, expressed its deformation. This type of disease does not interfere with the urination. There is a possibility of a normal sex life and conceive a child.

Epispadia penis (stem)

In stem epispadias urethra is split along the entire penis to dick pubic area. Penis shorter than the norm and somewhat biased upwards. This form of the disease may be accompanied by disruption to the structure of the muscles of the abdominal wall and the pubic bone. Pathology not allow to live normally. During urination, the urine splatters on the sides, which causes inconvenience. In the absence of necessary treatment in the future the conception will be impossible, because an erection will be accompanied by a deformation of the penis.

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Total (complete) epispadia

In this case affected the whole urethra to the place of its connection with the bladder. The pathology affects the muscle that opens/closes the urethra. Because of this, there is a constant release of urine, urge to urination. The muscles in the abdomen undeveloped. Urethral opening in boys is located at the base of the penis. Besides the main problems, irritation due to constant urine output. Dermatitis affects the scrotum, perineum, inner thighs. This is complicated by maceration, i.e. the stimulus literally corrodes the skin. As comorbidity is found cryptorchidism — absence of one or both testicles in the scrotum.

Epispadia negative impact on the development of the child. Discomfort and bad odor do not allow your child to attend kindergarten and live a full life.

The reasons for the development of pathology in children

The study of the factors in the emergence of pathology continues. But we already know that kids suffer from bladder exstrophy for the following reasons:

  • Burdened heredity. Scientists have found genetic abnormalities in some babies with this disease.
  • Maternal Smoking and the abuse of alcohol, intake of drugs before conception and during pregnancy. As a result there are serious violations in the process of formation of the fetus.
  • Bad ecology. Pollution of air and water, high radioactivity, etc. have a negative impact not only on the health of the young mother but also the fetus. During pregnancy, at least the first 3 months, you cannot take x-rays.
  • The use of a large amount of progesterone during the first trimester of pregnancy is able to provoke the disease.
  • The bladder extrophy can cause some medications, such as antibiotics. It is believed that the disease occurs due to the use of parents of reproductive technologies.

Clinical picture: boys and girls

The presence of pathology is determined during the first examination of the child immediately after its birth. In the abdomen there is some defect — the absence of the front walls of the peritoneum and bladder. The lesion has a diameter of 2-8 cm Under the action of intra-abdominal pressure on the surface is the rear wall of the bladder. From the ureter urine oozes. This pathology provokes inflammation of the skin, redness near the affected area, itching. The baby cries, refuses feeding, grows poorly.

If epispadias in boys, the opening of the urethra is found on the body of the penis. When the urine jet is aimed up, not down. If the external opening of the urethra is on the pubis, urine without the participation of the penis and flowing from the stomach at the crotch and legs of the baby. It causes skin irritation, rash. Special children powders without addressing the causes ineffective.

If you have the baby any abnormalities, consult a physician for diagnosis and necessary treatment.

A girl can suffer full or partial epispadia. In the first case, there is a lack or splitting of the clitoris, the labia majora from the top is not connected, the urinary hole located under the pubic articulation, Squirting out urine. In complete epispadias abdominal wall is underdeveloped, large labia very diluted in the pubic area. In the area of the symphysis is the entrance into the bladder, from which urine is constantly flowing. In both cases, the pathology is accompanied by severe skin irritation, because of what the baby can not eat normally, frequent waking.

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Diagnosis

Pathology is determined even before the birth of the baby by ultrasound (21 weeks gestation). Besides the main defect, it is possible to detect comorbidities. After the birth of an infant with this anomaly requires further investigation, check the status of other organ systems:

  • If there is a bladder exstrophy, conducted laboratory analysis of urine to exclude possible discharge from the cavity of the peritoneal fluid.
  • Cystoscopy helps to know the extent of destruction, to establish a relationship of pathology to other organs and its location relative to the urethra. During the procedure the probe is not injected into the urethra, and the bladder itself through the hole in the peritoneum.
  • Using ultrasound to reveal associated diseases of the kidneys and liver. In addition, it is recommended that endoscopy and computed tomography.
  • Conducted genetic research. It is possible that this pathology developed as a result of genetic diseases such as down syndrome. It is recommended to be screened during pregnancy.
  • If epispadias is carried out contrasting urethrography. By means of a catheter introduced into the bladder contrast agent. When urinating do radiology the. It shows the bladder and urethra, which can have blind offshoots.

Treatment of extrophy and epispadias

To eliminate such defects is possible only by surgery. If extropia operation is particularly dangerous, a great risk of death of the patient. To remedy the defect own tissues is not enough, because it is used several techniques. A common method to repair the hole in the bladder at the expense of its own tissues, and the defect of the peritoneum, when it is impossible enough to pull the fabric temporarily closed by a film. When tissue starts to cover the film, the operation is repeated. The synthetic insert is removed, the hole closes. In two steps the problem is solved rarely, surgery carried out more. During application of the implant and after the operation the doctor prescribes 3 groups of antibiotics to prevent infection.

Epispadia removed through surgery. Form of the disease determines the extent of the surgery. If the boy’s urethral opening is on the body of the penis, the correction is possible at the expense of its own tissues. If the external opening of the urethra is at the level of the pubis, is plastic surgery with the use of synthetic implants. Women’s epispadia requires surgery for urinary incontinence. Before surgery it is necessary to eliminate skin irritation. Otherwise, surgical intervention is postponed.

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Eating habits and lifestyle

People who have had similar disease, it is recommended to follow a diet. Do not eat salty, spicy, spicy food. The use of liquid in large quantity promotes the development of complications in the kidneys. Requires lifetime prevention of diseases of the genitourinary system. Periodically, it is recommended to visit the mineral resort «Morshyn», «Polyana».

When using implantov need to be careful to avoid injuries, to avoid excessive physical exertion. Parents should teach kids to change the catheter and to introduce a disinfectant. If extropia recommended daily inspection of the peritoneum. At the slightest suspicion on the leakage of the should consult a doctor. This may be the reason for the next operation.

Complications and prognosis

Allocate 2 main complications of epispadias:

  1. Inflammation of the skin. Due to constant contact of urine on the skin epidermis is destroyed, inflammation occurs. Affects deeper layers of the skin. Poor hygiene leads to infection and suppuration of the inflamed area. If you do not start treatment of the skin, the situation threatens to sepsis and death.
  2. Mental disorders. If the anomalies were not corrected in infancy, with time, the baby will begin to consider themselves inferior. In the future, even after a successful surgery, a psychological block can become an obstacle to the beginning of sexual life and a family.

If extropia are possible complications:

  • Hypothermia. The baby loses heat, since broken the natural barrier of the skin is missing. To avoid this, the baby kept in Cubase — a special device in the form of a glass cap in which the required temperature and humidity.
  • Bladder exstrophy complicated by sepsis and peritonitis because the body has a «gate» through which can pass the infection. Newborns with this anomaly is prescribed a course of antibiotics.
  • After the surgery may develop adhesive disease, which causes severe pain, intestinal obstruction.

Severe cases of extropia accompanied by abnormalities of the spine, digestive system and kidneys.

Prevention

Preventive measures taken before conception and during pregnancy. Recommended medical-genetic consultation, which reveals the pathology of the chromosomes. The expectant mother should be screened to detect infections that may harm the embryo: herpes, rubella, syphilis, toxoplasmosis. When detecting infection the woman undergoing treatment. During pregnancy, do not smoke, drink alcoholic beverages and drugs.

If the bladder extrophy define newborn at once, epispadia found in a form which is manifested later. So the baby had later mental health problems, this disease should be identified as early as possible. Parents should be careful. If the condition of the baby deteriorates, and in the genital area irritation and you need to consult a pediatric urologist.