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Epispadi
asDr. Zahoor Ahmad
Introduction
•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
Epidemioligy
around 1/ 120,000 male
1/500,000 female births
Etiology
•It is a partial form of a spectrum of failures of
abdominal and pelvic fusion in the first months of
embryogenesis known as the exstrophy - epispadias
complex.
•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.
Associated
anomalies•The causes of epispadias are not known. It may
occur because the pubic bone does not develop
properly.
•Epispadias can occur with bladder exstrophy. In
this rare birth defect, the bladder is inside out
and sticks through the abdomen wall. Epispadias
can also occur with other birth defects.
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.
Presentation………….
•Males usually have a short, wide penis with an
abnormal curve. The urethra usually opens on
the top or side of the penis instead of the tip.
However, the urethra may be open along the
whole length of the penis.
•Females have an abnormal clitoris and labia. The
opening is usually between the clitoris and the
labia, but it may be in the belly area. They may
have trouble controlling urination (urinary
incontinence).
Examination
•Abnormal opening from the bladder neck to the
area above the normal urethra opening
•Backward flow of urine into the kidney (reflux
nephropathy)
•Urinary incontinence
•Urinary tract infections
•Widened pubic bone
Tests (for assos.
anomaly)• Blood test
• 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 urinary system and genitals
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.
modified Cantwell-Ransley
approach
•The most popular and
successful technique is known
as the modified Cantwell-
Ransley approach.
complete penile
disassembly technique
•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
Complications
•Recurrence
•Sexual problems
•Incontinence
•UTI
•Infertility
•Psycho-social stress

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Epispadias 131104120306-phpapp01 (1)

  • 2. Introduction •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
  • 3. Epidemioligy around 1/ 120,000 male 1/500,000 female births
  • 4. Etiology •It is a partial form of a spectrum of failures of abdominal and pelvic fusion in the first months of embryogenesis known as the exstrophy - epispadias complex. •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.
  • 5. Associated anomalies•The causes of epispadias are not known. It may occur because the pubic bone does not develop properly. •Epispadias can occur with bladder exstrophy. In this rare birth defect, the bladder is inside out and sticks through the abdomen wall. Epispadias can also occur with other birth defects.
  • 6. 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.
  • 7. Presentation…………. •Males usually have a short, wide penis with an abnormal curve. The urethra usually opens on the top or side of the penis instead of the tip. However, the urethra may be open along the whole length of the penis. •Females have an abnormal clitoris and labia. The opening is usually between the clitoris and the labia, but it may be in the belly area. They may have trouble controlling urination (urinary incontinence).
  • 8. Examination •Abnormal opening from the bladder neck to the area above the normal urethra opening •Backward flow of urine into the kidney (reflux nephropathy) •Urinary incontinence •Urinary tract infections •Widened pubic bone
  • 9. Tests (for assos. anomaly)• Blood test • 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 urinary system and genitals
  • 10. 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.
  • 11. modified Cantwell-Ransley approach •The most popular and successful technique is known as the modified Cantwell- Ransley approach.
  • 12. complete penile disassembly technique •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