Categories
Ailmemts & Remedies Pediatric

Epispadias

Definition:
An epispadias is a rare type of malformation of the penis in which the urethra ends in an opening on the upper aspect (the dorsum) of the penis. It can also develop in females when the urethra develops too far anteriorly. It occurs in around 1 in 120,000 male and 1 in 500,000 female births.

An epispadia occurs when the urethra opening is abnormally placed. In a male infant with epispadias, the urethra will be generally open on the top or side of the penis.

Click to see the picture…>…...(01).…..(2).…..(1)

Boys will suffer from a short, wide penis and widened pubic bone. In a female infant with epispadias, the urethra will generally be located between the clitoris and the labia or in the abdominal area. Girls will suffer from a widened pubic bone and an abnormal clitoris and labia. In both males and females, urine will flow into the kidney and urinary tract infections are common. It is also common for the child to have urinary incontinence, kidney damage and often infertility issues as an adult.

A doctor will perform a series of tests to diagnose epispadias, which may include blood tests, x-rays and ultrasounds. Treatment involves surgery to help with urine control and appearance.

It is also called bladder exstrophy

Symptoms:

In males:
*Abnormal opening from the joint between the pubic bones to the area above the tip of the penis
*Backward flow of urine into the kidney (reflux nephropathy)
*Short, widened penis with an abnormal curvature
*Urinary tract infections
*Widened pubic bone

In females:……..Picture
*Abnormal clitoris and labia
*Abnormal opening where the from the bladder neck to the area above the normal urethral opening
*Backward flow of urine into the kidney (reflux nephropathy)
*Widened pubic bone
*Urinary incontinence
*Urinary tract infections

Causes:
The causes of epispadias are unknown at this time. It may be related to improper development of the pubic bone.

In boys with epispadias, the urethra generally opens on the top or side of the penis rather than the tip. However, it is possible for the urethra to be open along the entire length of the penis.

In girls, the opening is usually between the clitoris and the labia, but may be in the belly area.

Epispadias can be associated with bladder exstrophy, an uncommon birth defect in which the bladder is inside out, and sticks through the abdominal wall. However, epispadias can also occur with other defects.

Epispadias is an uncommon and partial form of a spectrum of failures of abdominal and pelvic fusion in the first months of embryogenesis known as the exstrophy – epispadias complex. While epispadias is inherent in all cases of exstrophy it can also, much less frequently, appear in isolation as the least severe form of the complex spectrum. It occurs as a result of defective migration of the genital tubercle primordii to the cloacal membrane, and so malformation of the genital tubercle, at about the 5th week of gestation.

Presentation:
Most cases involve a small and bifid penis, which requires surgical closure soon after birth, often including a reconstruction of the urethra. Where it is part of a larger Exstrophy, not only the urethra but also the bladder (bladder exstrophy) or the entire perineum (cloacal exstrophy) are open and exposed on birth, requiring closure.

Relationship to other conditions:
Despite the similarity of name, an epispadias is not a type of hypospadias, and involves a problem with a different set of embryologic processes.

In women:
Women can also have this type of congenital malformation. Epispadias of the female may occur when the urethra develops too far anteriorly, exiting in the clitoris or even more forward. For females, this may not cause difficulty in urination but may cause problems with sexual satisfaction. Frequently, the clitoris is bifurcated at the site of urethral exit, and therefore clitoral sensation is less intense during sexual intercourse due to frequent stimulation during urination. However, with proper stimulation, using either manual or positional techniques, clitoral orgasm is definitely possible

Diagnosis:
•Blood test to check electrolyte levels
•Intravenous pyelogram (IVP), a special x-ray of the kidneys, bladder, and ureters
•MRI and CT scans, depending on the condition
•Pelvic x-ray
•Ultrasound of the urogenital system

Treatment:
The main treatment for isolated epispadias is a comprehensive surgical repair of the genito-urinary area usually during the first 7 years of life, including reconstruction of the urethra, closure of the penile shaft and mobilisation of the corpora. The most popular and successful technique is known as the modified Cantwell-Ransley approach. In recent decades however increasing success has been achieved with the complete penile disassembly technique despite its association with greater and more serious risk of damage

Prognosis:
Even with successful surgery, patients may have long-term problems with:
*incontinence, where serious usually treated with some form of continent urinary diversion such as the Mitrofanoff
*depression and psycho-social complications
*sexual dysfunction

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

Resources:
http://www.nlm.nih.gov/medlineplus/ency/article/001285.htm
http://en.wikipedia.org/wiki/Epispadias
http://health.stateuniversity.com/pages/794/Hypospadias-Epispadias.html
http://www.wikidoc.org/index.php/Epispadias
http://www.eclips.consult.com/eclips/article/Pediatrics/S0084-3954(07)70134-3

Enhanced by Zemanta

Leave a Reply

This site uses Akismet to reduce spam. Learn how your comment data is processed.

css.php