A guide for parents of children born with an external bladder (bladder exstrophy)
What is bladder exstrophy?
Bladder exstrophy is a congenital structural defect. It is a disease characterized by the lack of development of the urinary bladder and the anterior abdominal wall in front of it, the appearance of the bladder when viewed from the outside, and the direct outflow of urine. The bladder cannot form its normal closed spherical shape. It is observed that the ureters open directly outward. Skeletal anomalies are common in these cases. Normally, the hip bones that meet at the front of the body are located separately from each other. Prostate and penis, which are organs of the lower urinary tract, may also show structural disorders. It is almost always associated with a penile anomaly called epispadias in boys. Undescended testis is very common. In girls, their clitoris is separated.
How often does it occur?
This disorder occurs in one in 10,000 to 50,000 live births. The probability of bladder exstrophy in the second child of a family with a child with exstrophy is 1/100, and if one of the parents has bladder exstrophy, the probability of the child having this disorder is 1/70. The male/female ratio is 2.3/1.
How is the diagnosis made, is it possible to diagnose before birth?
Continuously empty bladder in repeated prenatal ultrasonographic examinations creates suspicion of exstrophy. Again, in ultrasonography, a lower abdominal mass is observed that grows in the lower abdomen of the fetus as the pregnancy progresses. Rarely, on fetal ultrasonography, it may be noticed that the penis is small, the scrotum is displaced anteriorly, the umbilical cord is lower, and there is a gap in the iliac bones.
Usually, the diagnosis is made with the typical postnatal appearance. A film called IVP is taken to identify additional pathologies of the kidneys and ureters. On direct urinary system radiographs, it is observed that the hip bones are separated.
What are the goals in surgery?
The treatment for this congenital defect is surgery. This surgery is planned with objectives such as closure of the bladder and anterior abdominal wall, creation of a cosmetically acceptable and functional penis, preservation of kidney functions and prevention of urinary incontinence.
What are the surgical treatment options?
Today, there are two options for bladder exstrophy. The first is the classic 3-stage operation. First, the bladder, abdominal wall and posterior urethra are closed, in the second stage, the bladder neck is reshaped and antireflux operations are performed to prevent the backflow of urine, and in the last stage, the penis is reconstructed. However, this method has undergone significant modifications in recent years. These; In the neonatal period, closure of the bladder, posterior urethra and abdominal wall, if there is an indication, approximation of the iliac bones, reconstitution of the penis at the age of 6 months-1 and reshaping the bladder neck with antireflux operation between the ages of 4-5 if the child has sufficient bladder capacity.
The second option is total reconstruction. In recent years, encouraging reports on total reconstruction of this defect have also been published. Some authors suggest reconstructing the penis with the closure of the bladder in the neonatal period. Some apply bladder closure alone or in combination with bladder neck reshaping in the newborn and recommend reconstructing the penis at a later age. Some authors recommend closure of the bladder, ureteral reimplantation, which is to re-open the ureters to the bladder, reconstitution of the penis, and reshaping of the bladder neck, all in the neonatal period.This is the technique I also use, and I learned this technique from Prof. Bradley P. Kropp in USA. It works quite well in appropriate cases.
In both methods, if the child is not operated within the first 3-5 days after birth, an incision is made on the hip bones by the pediatric orthopedic surgeon to approximate the hip bones and give them a ring shape, and these bones are attached to an external stabilizer, and the screws are fixed at certain intervals to bring these bones closer. Other instruments can also be used to stabilize and hold the legs in tension.
What should these babies pay attention to after they are born?
The bladder surface should be protected from trauma and infection during the period until the operation. Connecting the umbilical cord with 2/0 silk instead of a plastic clamp after birth reduces the trauma that may occur in the bladder mucosa. The bladder should be covered with a plastic wrap to allow urine flow.
How is the preparation for the surgery done?
The surgery is scheduled shortly after the baby is born. The patient's blood count, kidney and liver function tests are checked. Chest X-ray is taken. The patient is hospitalized on the morning of the surgery or the night before. According to the planned operation time, the patient is asked to stay hungry and thirsty for 4-6 hours. During this period, the patient is given the necessary fluids by opening the vascular access. The anesthesiologist examines the patient before surgery, checks his/her examinations and confirms his/her suitability for general anesthesia. The antibiotic treatment given before the procedure is continued for a while after the operation.
How long does the surgical procedure take?
Surgery is performed under general anesthesia and takes 4-6 hours in total. The baby stays in the intensive care unit for at least 24-48 hours after the surgery. After intensive care, they are taken to their rooms and these children spend about 15 days in the hospital.
How is the follow-up in the early postoperative period?
The baby can be fed orally 6 hours after the operation. The drainage tube is removed in two to three days. The openings of the urinary catheter coming out of the abdomen and the silicone catheters in the urinary canals are checked with intermittent washings. The stitches are removed on the seventh day. Before discharge from the hospital, the upper urinary system is checked with ultrasonography.
How is the follow-up in the late postoperative period?
The child's bladder is checked at regular intervals with endoscopic and urodynamic tests. Additional medical and surgical interventions can be planned when necessary. Urine culture and urinary ultrasonography of the patient, who was called back for control 6 weeks after discharge, are performed.
What are its complications and how are they treated?
Urinary infection is a frequently encountered problem in the postoperative period. Febrile urinary tract infections may need to be treated by hospitalization with appropriate antibiotics. In the follow-up, suppressive low-dose antibiotic therapy is given to prevent the development of urinary infection.
It is recommended to insert a clean intermittent catheter in operated cases that cannot completely empty the bladder. The urine accumulating in the patient's bladder manifests itself as swelling in the lower abdomen. In this case, a healthcare provider should be consulted immediately. Urine is emptied by inserting a catheter.
Kidney functions and kidney enlargement should also be checked.
One of the important complications of bladder closure is herniation of the bladder and opening of the suture line. Such a situation is an urgent problem that negatively affects surgical success and threatens the health of the patient. The family should reach their doctor without delay. The patient will need to be re-operated.
Stone formation in the bladder and kidneys are other problems to be seen in exstrophy patients in the long term. Today, stone treatment is mostly possible with endoscopic methods.