Anatomy: there are 2 bladder halves laterally; at the center, there is a cecal plate; the elephant trunk is intussuscepted ileum attached to the cecal plate. Omphalocele is on top.
Cloacal exstrophy surgical stages:
- Colostomy (made with the tubulirized cecum) and bladder plate closure
- Growth and nutrition
- Spinal surgery (closed defect repaired at around 3 months)
- Bladder closure with osteotomies, minimal urethral reconstruction at around 1-2 years of age.
- Staged osteotomies with external fixation starting 4 weeks prior to bladder closure to gradually close pelvis.
- Nutrition, psychosocial care, dryness (diversion, bladder neck closure).
Rectal pull through rarely done.
I personally would do an ileal conduit at the 4th stage instead of the bladder closure. Why put the child through all the surgeries required for dryness and then have him or her do CIC, irrigation, and deal with all the complications? The child will already have a permanent colostomy, so an ileal conduit would likely not decrease the quality of life. All the care associated with an augment, as well as all the complications and surgeries to correct the complications would likely decrease quality of life.