•It is important to discuss the gender assignment in these patients.

–Many males with cloacal exstrophy have an unreconstructable phallus and undescended testes.

–The parents of these patients may elect to have their son undergo a gonadectomy and be raised as a female.

–This however may lead to possible psychosocial and behavioral outcomes of genotypic males being raised as females.

–Currently, it is possible to reconstruct a functional and cosmetically acceptable phallus and gender assignment should be consistent with the karyotype.

–On the other hand and as a result of advancement of surgical techniques, many are advocating for assigning gender that is consistent with karyotype, phallus reconstruction and orchidopexy.

13.5.8 Surgical Reconstruction

•Cloacal exstrophy reconstructive procedures can be done as a one-stage or two-stage.

•A one-stage closure is preferable to minimize the number of neonatal procedures and allow the bladder to be closed and protected.

•A central line should be inserted early for hydration, intravenous antibiotics and total parenteral nutrition.

•Reconstruction consists of the followings:

–Bilateral pelvic osteotomies are performed if reconstruction is done after the first 72 h of life.

–The omphalocele is excised.

–The bowel is separated from the hemi bladders and care should be taken to preserve the bowel length as much as possible to avoid a short bowel.

–The lateral vesicointestinal fissure is closed in continuity

–A short colostomy is created from the end of the distal colon segment and an ileostomy should be avoided as much as possible.

–The appendix should be preserved for possible later continent stoma construction if necessary.

–The hemi bladders are then re-approximated in the midline to create a single exstrophic bladder (classic bladder exstrophy).

–If a one-stage is selected, the entire bladder is closed completely.

–In patients with spinal dysraphism and myelocystocele, a closure by a neurosurgeon is undertaken as soon as the infant is medically stable.

•If reconstruction is to be done in one stage:

–Complete penile disassembly and division of the intersymphyseal band are crucial steps to allow for posterior positioning of the bladder and urethra.

•Then reconstruction of the bladder, penis, abdomen, and pelvis approximates the normal anatomy.

•Osteotomies are almost always necessary to assist in closure and posterior placement of the lower urinary tract. This allows a tensionfree approximation of the widely separated pubic bones and of the anterior abdominal wall. In cases of extreme pubic diastasis, combined anterior innominate and posterior osteotomy can be done or osteotomy is performed along with external fixator placement for 2–3 weeks.

•Postoperative drainage and immobilization are important via ureteral stents, a suprapubic catheter and possibly a urethral catheter along with traction and casting similar to that in classic bladder exstrophy.

•In males with cloacal exstrophy, the penis is usually represented by two widely separated small phallic structures. Reconstruction of these is difficult and challenging.

–Epispadias repair can be performed at the same time of initial closure when adequate corporal tissue is present.

–Repair of epispadias can also be delayed.

–In those with very small and insufficient phallic tissue, a female sex rearing should be considered and discussed with the

family. This however should be considered only in severe cases.

–In females, the hemi clitoris are brought together and duplicate vaginas are joined in the midline if possible.

–In the genotypic male patient raised as a female, new vagina construction is usually delayed till the time of puberty.

•Abdominal Wall and Bladder Closure

–Bilateral iliac osteotomies enable closure of the urinary bladder, abdominal wall and the widely separated pubic rami.

–Urinary bladder closure, omphalocele closure and intestinal diversion are performed with pelvic osteotomies within the first 48–72 h of life.

–A two-staged approach is indicated in those with other associated anomalies, widely separated pubic bones, a small bladder template or if the patient is not medically fit. The omphalocele is repaired together with intestinal diversion. At about 6 months later, the patient undergoes osteotomy and then, bladder closure and genital revision.

•Postclosure Management:

–The patients are kept immobilized postoperatively for 4–6 weeks to allow the osteotomies to heal.

–A modified Cantwell-Ransley repair of epispadias is planned at around 1 year of age.

–Following epispadias repair, the patient’s bladder capacity is measured annually with gravity cystogram.

–The majority of cloacal exstrophy patients require an augmentation cystoplasty and continent catheterizable stoma or a continent urinary diversion. This is done at 6–8 years of age.

–During this procedure the patient will either undergo a Young-Dees-Leadbetter bladder neck reconstruction or bladder neck transection along with ureteral reimplantation to prevent further vesicoureteral reflux.

–Male patients, who continue to be raised as males, may require a phalloplasty or neophallus if their original phallus is very small.

13.5.9Management of Urinary Incontinence

•Incontinence of urine is common in patients with cloacal exstrophy.

•This may be due in part to spinal defects, which can cause a neurologic deficit in bladder function, or to a small bladder capacity, which may require augmentation.

•Intermittent catheterization is important to enhance bladder outlet resistance.

•Urinary continence is possible in most children with cloacal exstrophy but usually will require a bladder augmentation and intermittent catheterization.

•Surgery to produce a continent reservoir should be delayed until the child is old enough to participate in self-care.

•The choice between a catheterizable urethra or an abdominal stoma depends on the adequacy of the urethra and bladder outlet, the intellect and dexterity of the child, and the child’s orthopedic status as regards to the spine, hip joints, braces and ambulation.

•Bladder augmentation should be delayed until bowel function is mature and nutrition and acidosis are no longer a problem.

•Bladder augmentation can be done using hindgut or ileum but to avoid further loss of bowel, part of the stomach can be used as a gastrocystoplasty.

•There is a small risk of long-term cancer with gastrocystoplasty.

13.5.10 Prognosis

•In bladder exstrophy and cloacal exstrophy, the single most important predictor of longterm bladder growth and continence is successful primary bladder closure.

•The postoperative continence in patients with bladder exstrophy and cloacal exstrophy ranges from 70 % to 80 %.