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
  • lower abdominal wall
  • urinary bladder
  • bony pelvis
  • 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

More general terms

• congenital anomaly (birth defect)
• urogenital disease