Epispadias

Epispadias
Specialty	Urology
Symptoms	Males: Urethra opening on upper aspect of the penis, short penis[1] Females: Split clitoris, poorly formed mons pubis[1]
Complications	Urinary incontinence[1]
Usual onset	Present at birth[2]
Types	Males: Glandular, penile, penopubic[1] Females: Vestibular, sub-symphyseal, retro-symphyseal[1]
Causes	Unclear[1]
Differential diagnosis	Hypospadias, bladder exstrophy, common urogenital sinus[1]
Treatment	Surgery[1]
Frequency	Up to 1 in 10,000[1]

Epispadias is a malformation in which the top of the urethra fails to close properly.^[1] In males the opening of the urethra may occur on the upper aspect of the penis; which may be short and upward curved.^[1] In females there may be a split clitoris and poorly formed mons pubis.^[1] It often occurs in association with Bladder exstrophy.^[1] Complications may include urinary incontinence.^[1]

The cause is unclear.^[1] The more severe forms of disease occur more commonly when there is a lack of folate before pregnancy.^[1] Diagnosis is based on examination.^[1] An X-ray is recommended to find associated pubic diastasis.^[1]

Treatment may involved surgery.^[1] Rates of incontinence range from 50 to 90%.^[1] Epispadias may affect up to 1 in 10,000 people; though cases without bladder exstrophy are less than 1 in 100,000 males and 1 in 160,000 female births.^[1]

Signs and symptoms

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. Many parts of this article are incorrect.

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.

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

References

External links