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URINARY BLADDER
EXSTROPHY
Raja Mohamed
URINARY BLADDER EXSTROPHY
• It is also termed as ‘Ectopic vesicae’
• Exstrophy means ‘turned inside out’.
• Bladder exstrophy is a congenital abnormality of
the bladder.
• Lower portion of the abdominal wall does not form
properly and the anterior wall of the bladder is
missing, so the bladder is open and exposed on
the outside of the abdomen.
• Associated with numbers of congenital anomalies,
related to Urogenital tract, Musculo- skeletal
system and sometimes of GI system.
• 1 in every 50,000 live births
• Males are affected 2-3 times more often than
In boys born with bladder exstrophy, the bladder is
on the outside of the body and the penis and urethra
are not fully closed
In girls born with bladder exstrophy, the bladder is
on the outside of the body and the vagina is not fully
formed
ETIOLOGY
• Idiopathic
• Due to any birth defect
• Genetic factors
• Environmental factor
CLINICAL MANIFESTATIONS
• Exposed bladder from the abdomen
• Flattened puborectal sling
• Pubis symphysis separated
• Ulcer in the bladder mucosa
• Dribbling of urine
• Skin excoriation
PATHOPHYSIOLOGY
Due to an unknown
etiology/birth defect
Cloacal
membrane
bursts
Abdominal
muscle not
formed fully
Urorectal
septum
separate the
bladder from
bowel and
intestine
Exstrophy of
bladder
DIAGNOSIS
• Fetal Ultrasound
• Cystoscopic
examination
• X-Ray
• USG IVP
• Urodynamic testing
• Clinically obvious at
birth
irregularity of the
inferior abdominal wall
abnormal external
genitalia
SURGICAL MANAGEMENT
MSRE – Modern Staged Repair of Exstrophy
Staged repair involves three operations.
•First procedure closes the bladder and the abdomen
- within 72 hours after birth
•Second procedure, repairs the urethra and sex organs.
- age 6 to 12 months
•Third procedure, when the child is old enough to participate in toilet
training, surgeons perform bladder neck reconstruction
- 4 to 5 years.
Stage I of
MSRE
SURGICAL MANAGEMENT
CPRE – Complete Primary Repair of Exstrophy
Complete repair surgery is performed in a single procedure that closes
the bladder and the abdomen and repairs the urethra and outer sex
organs.
This can be done soon after birth, or when the baby is around two to
three months old
THANK
YOU

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Urinary Bladder Exstrophy.pptx

  • 2. URINARY BLADDER EXSTROPHY • It is also termed as ‘Ectopic vesicae’ • Exstrophy means ‘turned inside out’. • Bladder exstrophy is a congenital abnormality of the bladder. • Lower portion of the abdominal wall does not form properly and the anterior wall of the bladder is missing, so the bladder is open and exposed on the outside of the abdomen. • Associated with numbers of congenital anomalies, related to Urogenital tract, Musculo- skeletal system and sometimes of GI system. • 1 in every 50,000 live births • Males are affected 2-3 times more often than
  • 3. In boys born with bladder exstrophy, the bladder is on the outside of the body and the penis and urethra are not fully closed
  • 4. In girls born with bladder exstrophy, the bladder is on the outside of the body and the vagina is not fully formed
  • 5. ETIOLOGY • Idiopathic • Due to any birth defect • Genetic factors • Environmental factor
  • 6. CLINICAL MANIFESTATIONS • Exposed bladder from the abdomen • Flattened puborectal sling • Pubis symphysis separated • Ulcer in the bladder mucosa • Dribbling of urine • Skin excoriation
  • 7. PATHOPHYSIOLOGY Due to an unknown etiology/birth defect Cloacal membrane bursts Abdominal muscle not formed fully Urorectal septum separate the bladder from bowel and intestine Exstrophy of bladder
  • 8. DIAGNOSIS • Fetal Ultrasound • Cystoscopic examination • X-Ray • USG IVP • Urodynamic testing • Clinically obvious at birth irregularity of the inferior abdominal wall abnormal external genitalia
  • 9. SURGICAL MANAGEMENT MSRE – Modern Staged Repair of Exstrophy Staged repair involves three operations. •First procedure closes the bladder and the abdomen - within 72 hours after birth •Second procedure, repairs the urethra and sex organs. - age 6 to 12 months •Third procedure, when the child is old enough to participate in toilet training, surgeons perform bladder neck reconstruction - 4 to 5 years.
  • 11. SURGICAL MANAGEMENT CPRE – Complete Primary Repair of Exstrophy Complete repair surgery is performed in a single procedure that closes the bladder and the abdomen and repairs the urethra and outer sex organs. This can be done soon after birth, or when the baby is around two to three months old