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
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