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Epispadias

 
Medical Encyclopedia: Hypospadias and Epispadias
More about Hypospadias and Epispadias:
Causes and symptoms
Diagnosis
Treatment
Prognosis
Resources

Definition

Hypospadias is a congenital defect, primarily of males, in which the urethra opens on the underside (ventrum) of the penis. The corresponding defect in females is an opening of the urethra into the vagina and is rare.

Epispadias (also called bladder exstrophy) is a congenital defect of males in which the urethra opens on the

upper surface (dorsum) of the penis. The corresponding defect in females is a fissure in the upper wall of the urethra and is quite rare.

Description

In a male, the external opening of the urinary tract (external meatus) is normally located at the tip of the penis. In a female, it is normally located between the clitoris and the vagina.

In males with hypospadias, the urethra opens on the inferior surface or underside of the penis. In females with hypospadias, the urethra opens into the cavity of the vagina.

In males with epispadias, the urethra opens on the superior surface or upper side of the penis. In females with epispadias, there is a crack or fissure in the wall of the urethra and out of the body through an opening in the skin above the clitoris.

During the embryological development of males, a groove of tissue folds inward and then fuses to form a tube that becomes the urethra. Hypospadias occurs when the tube does not form or does not fuse completely. Epispadias is due to a defect in the tissue that folds inward to form the urethra.

During the development of a female, similar processes occur to form the urethra. The problem is usually insufficient length of the tube that becomes the urethra. As a result, the urethra opens in an abnormal location, resulting in a hypospadias. Occasionally, fissures form in the bladder. These may extend to the surface of the abdomen and fuse with the adjacent skin. This is most often identified as a defect in the bladder although it is technically an epispadias.

Hypospadias in males generally occur alone. Female hypospadias may be associated with abnormalities of the genital tract, since the urinary and genital tracts are formed in the same embryonic process.

Because it represents incomplete development of the penis, some experts think that insufficient male hormone may be responsible for hypospadias.

In males, the incidence of hypospadias is approximately one per 250 to 300 live births. Epispadias is much less common, having an incidence of about one per 100,000 live male births.

In females, hypospadias is much less common than in males. It appears about once in every 500,000 live female births. Epispadias is even rarer. Reliable estimates of the prevalence of epispadias in females are not available. Epispadias in females is often diagnosed and recorded as a bladder anomaly.

— L. Fleming Fallon, Jr., MD, PhD, DrPH



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Sci-Tech Dictionary: epispadias
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(′ep·ə′spād·ē·əs)

(medicine) A congenital defect of the anterior urethra in which the canal terminates on the dorsum of the penis and posterior to its normal opening.


Veterinary Dictionary: epispadias
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A congenital malformation with absence of the upper wall of the urethra, occurring in both sexes, but more commonly in the male, the urethral opening being located anywhere on the dorsum of the penis.

Word Tutor: epispadias
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pronunciation

IN BRIEF: n. - A congenital abnormality in males in which the urethra is on the upper surface of the reproductive organ.

Wikipedia: Epispadias
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Epispadias
Classification and external resources
File:Epispadia.jpg
ICD-10 Q64.0
ICD-9 752.62
DiseasesDB 33378
eMedicine / 
MeSH [1]

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 occurs in around 1 in 120,000 male and 1 in 500,000 female births.

Contents

Causes

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.

Causes are still essentially unknown, with various theories put forward including chemical intake of the pregnant mother. They are not thought to involve androgens nor to be primarily genetic although there is some increased genetic frequency. The leading theory is that of a virus during pregnancy, manifesting as a moderate to serious common cold

Presentation

Most cases involve a small and bifid phallus 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.

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 disfunction

External links



 
 
Learn More
Hypospadias and Epispadias: Prognosis
Hypospadias and Epispadias: Causes and symptoms
Hypospadias and Epispadias: Treatment

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