- 1 in 300,000 births.
- Low exomphalos, non-visible bladder and sacral spina bifida (in 50% of cases) with normal volume of the amniotic fluid.
- The anatomy of cloacal exstrophy is complex but essentially there is a low exomphalos at the superior margin of the defect, small or large bowel protruding through the middle of two widely separated hemi-bladders, hypoplasia of the colon and anal atresia, splaying of the pubic bones, bifid penis in males, hemiclitoris on either side of the bladder in females, and spina bifida.
- The incidence of chromosomal abnormalities is not increased.
- Other defects, mainly renal and vertebral, talipes, single umbilical artery, ambiguous genitalia (in males, the penis is divided and duplicated) are often found.
- Detailed ultrasound examination.
- Invasive testing to determine the genetic sex of the fetus.
- Follow-up should be standard.
- Place: hospital with neonatal intensive care and facilities for pediatric surgery.
- Time: 38 weeks.
- Method: induction of labor aiming for vaginal delivery.
- Survival: >90% after extensive reconstructive bladder, bowel and genital surgery.
- Both males and females with this condition are capable of normal lifestyle and both sexes are fertile after surgery. Some form of urinary tract diversion is required for all.
- No increased risk of recurrence.