PROF ANTONIO RUFFO
Specialist in Urology
Andrology Genital and Urethral Recostructive Surgery
mobile +39 3511718896
EDISPADIA
Epispadias is a rare congenital malformation (present from birth) of the external genitalia and lower urinary tract.
This condition is characterized by incomplete development of the dorsal portion of the penis or clitoris and the dorsal portion of the urethra (the tube from which urine exits the bladder).
In men, the urethra opens at the top of the penis instead of at the tip, like an open book. In women, the urethral opening faces the clitoris.
This results in an aesthetic and functional abnormality of the genitals. In more severe cases, the failure to close the urethral shower also affects the bladder wall, and in this case we will speak of the bladder estrophy-epispadias complex.
Male epispadias
Epispadias is a rather rare condition; in fact, it affects only 1 in 117,000 males born.
In patients with epispadias, the penis tends to be wide, short and curved ("dorsal cord"). The pubic bones are separated (diastasis) and as the penis is connected to them at the base (crura) they result in a retraction of the organ, which precisely appears shorter and wider.
Under conditions of normal genital development, the opening of the urethra (the "meatus") is at the tip of the penis. But in boys with epispadias, it is above the penis, on the dorsal face.
Epispadias is classified according to the position of the meatus on the penis
1) Epispadias glandulare or balanica: The opening of the urethra is on the dorsal aspect of the glans
2) Penile epispadias: The opening of the urethra is located on the dorsal part of the penis
3) Penile epispadias: The opening of the urethra is located at the base of the penis or near the pubic bone
The location of the urinary meatus can help predict normal bladder function. If the meatus is near the base of the penis (and abdominal wall), the bladder sphincter is likely to be poorly functioning, leading to incontinence problems. In most patients with penopubic epispadias, the pelvic bones are not joined at the front, leading to incomplete closure of the bladder sphincter. For this reason, patients are incontinent.
Most individuals with penopubic epispadias and about 2/3 of those with penile epispadias have Stress Incontinence, which is an involuntary loss of urine that occurs when pressure is exerted on the bladder : coughing, sneezing, jumping, laughing, heavy lifting.
In this case, the patient will require bladder neck reconstruction surgery to try to regain continence
Fortunately, almost all patients with glandular epispadias have a functioning bladder neck and can retain normally.
However, they will still require surgery to repair the abnormal penile opening (Urethroplasty) and straighten the corpora cavernosa (Corporoplasty)
Epispadias in women
Epispadias is much rarer in girls, 1 case in 484,000 births.
Affected girls have diastasis of the pubic bones of varying severity.
This results in altered development of the clitoris, which appears split in half. The bladder neck is almost always affected resulting in 'Stress Incontinence.
In most cases, timely surgical treatment can resolve these disorders.
What happens under normal conditions?
The urinary tract is like a hydraulic system. It has special "pipes'' that allow the passage of urine. The urinary tract consists of 2 kidneys, 2 ureters, the bladder and the urethra.
The kidneys are the body's filters : They remove waste substances from the blood and produce urine.
This is then carried to the bladder through two small tubes (Ureters).
The bladder stores urine until, as it relaxes, it feels the urge to urinate. A valve mechanism keeps the urine from flowing back into the ureters and kidneys.
The small tube that carries urine from the bladder to the outside is called the urethra.
Your urethra has muscles called sphincters. The sphincter complex (bladder neck) is a ring-shaped muscle wrapped around the urethra. It is used to keep the urethra closed so that urine does not leak out before the urinary urge. These sphincters open when the bladder contracts and urine is expelled outside.
Symptoms
In most cases epispadias is diagnosed at birth during the infant's objective examination. Sometimes in milder cases it may go unnoticed until parents notice small urine leakage after urination.
Causes
Only rarely do epispadias occur on their own.
The degree and severity of epispadias vary from patient to patient. In some boys, it may be only a small dimple at the tip of the glans above the normal urethral outlet.
In women, it may present colo with a double clitoris. When there is urethral or bladder involvement, epispadias is usually more severe. This condition is named estrophy-epispadias complex.
Diagnosis
Epispadias is most often noticed at birth. If the defect is mild, it may not be noticed at first. In some cases, it may not be noticed until the child has been toilet trained and has urine leakage. This is more common with girls.
Treatment
The main goal of surgical treatment is both cosmetic and functional.
The main goals of epispadias surgery in men are:
- Normal erectile function of the penis
- Correction of penile morphology (dorsal curve and chord)
- Lengthening of the penis (Elongation phalloplasty)
- Giving the penis a more normal appearance by placing the urethra ventrally and the urinary meatus at the tip.
If the bladder and bladder neck are affected surgery will be needed to restore urinary continence.
There are two popular surgical techniques and both give the penis a normal appearance. Surgery is often performed in pediatric age ( Mitchell technique and Cantwell-Ransley technique). The choice of surgical technique depends on:
The type of epispadias
Experience of the surgeon
Surgeon's preference
Often patients born with the estrophy-epispadias complex an underdeveloped penis. In these cases.
Surgical options in women
Fortunately, reconstruction in women is less complex than in men.
The internal genital system (uterus, fallopian tubes, and ovaries) are usually normal.
The urethra and vagina may be smaller in size, and The clitoris may be bifid (divided into two parts).
If an early diagnosis is made at birth, surgical correction can be made :
Reconstruction of the bladder neck
Placement of the urethra in the normal position
Reconstruction of the clitoris
More information
Children born with epispadias are often healthy and do not suffer from other diseases.
For this reason, these children do not need many diagnostic tests (Ultrasound, RX or CT scan).
Men with penopubic epispadias or the estrophy-epispadias complex may have fertility problems because of a defective bladder neck that may not close completely during ejaculation. This allows sperm to move backward in the bladder (retrograde ejaculation). This can cause problems when a couple is trying to have children. In addition, the size of the short, stubby penis can make it difficult to perform normal sexual activity. Proper surgical repair can reduce these problems.
Women are usually not at risk for infertility because their internal organs are in the normal range.
Do children with epispadias have more urinary infections ?
Yes. But in cases of complete estrophy-epispadias, the baby will be at greater risk. This is mainly due to vesicoureteral reflux.
Can epispadias be prevented?
There is no known prevention.
When is the best time to intervene surgically ?
It has been seen that it is best to correct episapdia as early as possible. If surgery is performed within the first few months of life, the child may have a better chance of having a normal bladder.
