A guide for parents of children with epispadias
What is epispadias?
It is the opening of the urethra in any part of the penis facing the human face, from the pubis bone to the glans penis, the fleshy part at the tip of the penis. Thus, the urethra is in a position closer to the pubis bone than it should be in girls or boys. Epispadias is often observed with bladder exstrophy. This condition is called the exstrophy and epispadias complex. Epispadias alone is extremely rare (1/120,000 in boys, 1/450,000 in girls) and almost always presents with urinary incontinence, apart from cosmetic appearance. The male/female ratio is 5/1.
In mild cases, the urethra may be close to the glans penis, and these cases usually do not have any complaints except for the cosmetic appearance. Urine control is complete, urinary incontinence is rarely seen. As the severity of epispadias increases, the urinary hole approaches the pubis bone and the probability of urinary incontinence increases. In some cases, the urinary canal is open in the form of a groove and the pubis bones are split in the middle, as in bladder exstrophy, which is called complete epispadias or penopubic epispadias. In almost all cases (95%) there is urinary incontinence. In these, the bladder may sometimes be underdeveloped and the leakage of urine from the bladder to the kidneys (vesicoureteral reflux) is common.
In addition to the upper urinary opening and urinary incontinence, the penis is curved upwards in the form of a hook and is shorter and thicker than normal in boys. This curvature of the penis may cause difficulties in practicing sexual intercourse in advanced ages, as well as a cosmetic problem. In girls, it is remarkable that the clitoris is divided into two (bifid clitoris) and the lips (labia minor and labia major) are cosmetically distorted and separated.
When should epispadias surgery be performed?
In mild cases without urinary retention problems, the operation can be performed at the age of 6-18 months. In those with complete epispadias, it can be expected up to 3-4 years of age.
What are the aims of epispadias surgery?
Epispadias surgery has 4 purposes:
- Extension of the urethra and urethral meatus to the tip of the penis in boys. In girls, the urinary opening is moved behind the clitoris and between the labia minora of the vagina.
2. Correction of the size and structure of the penis in such a way as to be considered cosmetic. Correction of the clitoris in one piece and lips in a way that does not force sexual intercourse in girls.
- Correction of the urinary retention mechanism.
- Protection of the kidneys
How is the preparation for surgery?
Before the repair, the condition of the kidneys should be checked with ultrasound, and the presence of vesicoureteral reflux should be checked with voiding cystourethrography. Urinary tract infections are common, especially in severe epispadias, and it is necessary to clean the urinary tract from bacteria with appropriate treatments before surgery.
How is the procedure done?
Basically, 3 surgical procedures are performed: lengthening and transport of the urinary opening and duct (urethroplasty), reshaping of the bladder neck to assist the urinary retention mechanism (continence surgery), and surgery to correct vesicoureteral reflux (ureteral reimplantation).
In patients with complete epispadias, if the bladder capacity is sufficient, epispadias and bladder neck reshaping operations can be performed in the same session. However, it is not possible to reshape the bladder neck and ureteral reimplantation at the same time in small, incontinuous bladders with vesicoureteral reflux. In these, it is more appropriate to perform penile reconstruction and urethroplasty first, and to perform ureteral reimplantation together with bladder neck reconstruction when the bladder reaches sufficient capacity.
The urethroplasty technique varies according to the severity of epispadias. In mild cases, the urethra groove can be folded on itself and made into a tube shape and lengthened. The two cylinders (corpus cavernosum) that provide the hardening of the penis are separated from the pubic bones and rotated around themselves and brought closer to the newly formed urethral tube.
In continence surgery, an incision is made in the lower abdomen. To form the bladder neck, either a part of the bladder is taken and extended in the form of a tube, or the bladder neck can be narrowed in itself. In some cases, a muscle fascia taken from the child's own body in the form of a hammock can be placed under the bladder neck and the bladder can be compressed towards the pubis bone. In some cases, the bladder neck can be completely narrowed by turning this muscle fascia around the bladder neck several times.
Anti-reflux surgery is performed together with continence surgery. While the bladder is opened, the ureter coming from both kidneys to the bladder is passed through a new tunnel in the bladder.
How is the follow-up done in the early postoperative period?
Children who have only had urethroplasty can go home the same day. Usually, the urinary catheter should be removed within 7-14 days.
In children who have undergone continence surgery or reflux correction surgery, there may also be a catheter in the urinary tract and sometimes a catheter sent from the abdomen into the bladder, as well as a drain catheter to collect the urine accumulated around the bladder. The child starts to take food by mouth the next day of the operation.
Usually, the drain catheter is removed on the second or third day, and the child can be sent home with the catheter in the urinary tract or abdomen. The urinary catheter and the urinary catheter in the abdomen can be removed in 7-21 days, respectively. When families go home, they can use an antibiotic and pain relievers to prevent urinary tract infection.
How is follow-up done in the late postoperative period?
Epispadias surgery is a type of surgery with high complications. Continence surgery can fail 20-50% even in the best hands. Urinary hole may not fit properly, or holes called fistulas may form in the urinary tract. 6-12 months should be waited for the repair of the formed fistulas or the correction of other cosmetic problems. It may be necessary to wait 2-3 years for urinary retention to achieve maximum success. In some cases, the urinary retention mechanism may be over tightened. In this case, it may be necessary for the child to urinate by self-intermittent catheterization 6-8 times a day. Even in successful cases, annual controls and checking whether the kidneys are affected, and the condition of the bladder should be examined.