September 8th, 2016
Epispadias is the development of abnormal urethral opening due to which the urethra and external genitalia are not able to function properly. It is a congenital disorder that can occur in both boys and girls, though incidence in general population is rare. Boys with epispadias have urethral opening at the top of penis instead of its tip. Likewise females born with epispadias have urethral opening more anterior (i.e. towards the clitoris or belly area).
According to latest estimates, epispadias affects 1 in 484,000 female livebirths and about 1 in 117,000 male livebirths (1).
The human urinary tract comprises of a pair of kidneys, two ureters, a urethra and bladder. The human kidneys serve as a filtration system for our body and are responsible for removing the hazardous toxins from the blood, while retaining useful substances such as, salt, minerals, protein and sugar. The resulting nitrogenous waste produced is referred to as urine which is carried by a pair of 10-12 inches long and a quarter-inch wide tubes called ureters. The ureters carry urine and transfer it into the bladder where it is stored until there is a need to urinate. It also prevents the backward flow of urine towards the kidney with the help of strong valves. The stored urine is carried out of the body via an opening known as urethra. Males have longer urethra than females. In females, the urethra starts from the bladder, passes in front of vagina and opens outside the body. While in males it runs all the way through the prostate gland, perineum (space in between the scrotum and anus) and ultimately into the penis.
Urethra possess strong involuntary muscles called sphincters which controls the opening and closing of urethra. The urethra is wrapped with a ring shaped muscle called sphincter complex or bladder neck which keeps the urethra closed; thereby preventing urinary leakage.
When a person is ready to urinate, bladder contracts and this contraction leads to the opening of sphincter; thereby promoting the expulsion of urine out of the body.
The penis of boys who are born with epispadias is short and curved upwards. The opening of urethra is called meatus and depending upon the location of meatus on the penis, epispadias can be classified as:
Meatus helps in determining the efficiency and storage capacity of bladder. If meatus point is located near the base of penis, it means that the bladder sphincters are affected and can’t hold the urine efficiently.
Urinary leakage is more likely to occur in the penopubic epispadias because the pelvic bones does not come close together in this case due to which the sphincter appears to be horseshoe shaped instead of a ring, resulting in improper closing of sphincters and leaking of urine. Most men with penile and penopubic epispadias require surgical correction of sphincter complex.
In glanular epispadias, the sphincter complex is not affected therefore, most men do not experience problems in holding urine. But the curved penis with abnormal opening will require a surgery to minimize the risk of complications. The surgical options depends largely on patient overall health, severity of symptoms and associated abnormalities (such as bladder exstrophy). According to a new study, the risk of developing bladder exstrophy and epispadias is 2.15 per 1,00,000 livebirths.
Epispadias in girls is much rarer than boys. Affected girl’s pubic bones are separated from each other by various degrees, which in turn prevents the adjoining of clitoris in the middle. As a result the clitoris divides into two halves (giving a bifid appearance). In most cases, the bladder neck is affected and leakage of urine occurs in states of high intra-abdominal pressure such as coughing, excessive laughing, strenuous activities (also known as stress incontinence). Usually surgeries can help in restoring normal urological functions.
1. Cervellione, R. M., Mantovani, A., Gearhart, J., Bogaert, G., Gobet, R., Caione, P., & Dickson, A. P. (2015). Prospective study on the incidence of bladder/cloacal exstrophy and epispadias in Europe. Journal of pediatric urology, 11(6), 337-e1.
2. Valerio, E., Vanzo, V., Zaramella, P., Salvadori, S., Castagnetti, M., & Baraldi, E. (2015). Exstrophy–Epispadias Complex in a Newborn: Case Report and Review of the Literature. American Journal of Perinatology Reports, 5(02), e183-e187.
3. Mahfuz, I., Darling, T., Wilkins, S., White, S., & Cheng, W. (2013). New insights into the pathogenesis of bladder exstrophy–epispadias complex. Journal of pediatric urology, 9(6), 996-1005.
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