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bladder extrophy
Epidemiology:
- rare: 0.01-0.002% of live births
- male/female ratio of 2/1
Pathology:
- involves a spectrum of anomalies including the
a) lower abdominal wall
b) urinary bladder
c) bony pelvis
d) external genitalia
- results from failure of the abdominal wall to close during fetal development, thus fetal bladder communicates with amniotic fluid
Clinical manifestations:
- protrusion of the posterior bladder wall through the lower abdominal wall
- the bladder mucosa is exposed at birth & is quite Special sensitive laboratory:
- prenatal ultrasound may be helpful
Management:
- umbilical cord should be tied with a 2-0 silk suture rather than a clamp to prevent trauma to the bladder mucosa
- the bladder should be covered with a non-adherent film (Plastic Wrap) to prevent sticking of the bladder mucosa to diapers or clothing
- plastic wrap should be removed with each diaper change, the bladder irrigated with sterile saline, & a clean wrap placed
- transfer to a tertiary care pediatric unit
- surgical reconstruction of the bladder & genitalia, most often occurs in stages
General
congenital anomaly (birth defect)
urogenital disease
References
- Wikipedia: Bladder exstrophy
http://en.wikipedia.org/wiki/Bladder_exstrophy
- Medline Plus: Badder Extrophy repair
http://www.nlm.nih.gov/medlineplus/ency/article/002997.htm