Prevalence:

• 1 in 300,000 births.

Ultrasound diagnosis:

• 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.

Associated abnormalities:

• 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.

Investigations:

• Detailed ultrasound examination.
• Invasive testing to determine the genetic sex of the fetus.

Follow up:

• Follow-up should be standard.

Delivery:

• Place: hospital with neonatal intensive care and facilities for pediatric surgery.
• Time: 38 weeks.
• Method: induction of labor aiming for vaginal delivery.

Prognosis:

• 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.

Recurrence:

• No increased risk of recurrence.