Cloacal Exstrophy with Dr. Alberto Peña
With Dr. Alberto Peña · hosted by Dr. Todd Ponsky · StayCurrentMD
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Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
Cloacal exstrophy is a spectrum of congenital malformations affecting the gastrointestinal/colorectal area, urogenital tract, spine and cord, and sometimes lower extremity motion.
Babies with cloacal exstrophy are born with an omphalocele, bladder exstrophy (two separated hemibladders), open cecum between the hemibladders, and separated pubic bones.
The small bowel can become exstrophic through the ileocecal valve, creating an 'elephant trunk' appearance.
Male patients have two separated hemiphalluses with normal gonads; female patients have two hemivaginas below the exstrophic bladder leading to two hemiuteri.
Cloacal exstrophy represents a spectrum of colonic anatomy from normal colon to almost absent or completely absent colon, sometimes with two ceca or two appendices and bizarre blood supply.
The amount of colon present at birth has very important implications for the patient's management and outcomes.
A variant exists where babies are born with intact abdominal skin (no omphalocele, no bladder exstrophy externally) but have a completely open bladder inside with no bladder neck and a single large perineal orifice.
While surgical techniques, intensive care, parenteral nutrition, and metabolic management have improved, functional outcomes (bowel control, urinary control, sexual function, spinal abnormalities) remain severely limited and cannot be made normal.
Historical practice was to perform bilateral gonadectomy, remove hemiphalluses, create a vagina with bowel, and assign female gender to XY patients with cloacal exstrophy.
Patients raised as female despite XY chromosomes exhibited male attitudes and behavior, and many became upset upon learning their chromosomal sex and that gonads were removed without their consent.
Patients argued that sex is not the most important aspect of being male, that they wanted their gonads back for fertility (modern techniques allow fertilization and children), and that being male is much more than having a phallus.
Current consensus is that XY patients should be raised as male, with pediatric urologists and plastic surgeons working on phallus reconstruction techniques.
When a prominent pediatric urologist dominates management, patients receive good urologic attention but inadequate gastrointestinal care; the reverse occurs when pediatric surgeons dominate.
The pediatric surgeon's role in the initial operation is to close the omphalocele (if possible), separate urothelium from intestinal mucosa by placing stitches at the edges and making an incision, allowing the urologist to bring hemibladders together.
It is very common but very harmful for pediatric surgeons to simply create an ileostomy, leaving all colon distally attached to the urinary tract.
Leaving colon attached to the urinary tract creates a congenital bladder augmentation that causes hyperchloremic acidosis from urine absorption, interfering with growth and development.
Defunctionalized colonic tissue left distally will not grow; colon requires passage of fecal matter through its lumen to grow.
The pediatric surgeon must incorporate all gastrointestinal tissue into the fecal stream and create a true end colostomy to ensure fecal matter passes through all colonic tissue.
Patients who received ileostomy with defunctionalized colon present at 2-3 years with poor growth, hyperchloremic acidosis managed by nephrologists, and large ileostomy losses.
The rescue operation involves taking down the ileostomy, finding and incorporating colonic tissue into the GI tract, and creating an end colostomy; acidosis disappears the next day.
Surgeons must accept that cloacal exstrophy is a spectrum and be prepared to deal with complex, variable colonic anatomy rather than taking the easy way out with an ileostomy.
Some institutions routinely perform pelvic osteotomy at the initial operation to facilitate bladder and omphalocele reconstruction, while others wait 2-3 months to do it separately.
Even after osteotomy, it is very difficult to see a cloacal exstrophy patient with pubic bones completely together; they usually remain separated.
Even a technically correct end colostomy often has poor motility, and babies may not pass stool easily, sometimes developing bacterial overgrowth similar to Hirschsprung disease.
Families must be taught to irrigate the colostomy with small volumes of saline through a tube to evacuate fecal material when peristalsis is inadequate.
Decision-making for bowel control begins around age 3 when parents want to send the child to school clean and dry (no stool or urine in diaper).
Most cloacal exstrophy patients have an inadequate, tiny bladder requiring bladder augmentation with gastrointestinal tract, necessitating coordination between pediatric surgery and urology.
Patients born with no colon are candidates for permanent colostomy and should never have terminal ileum pulled through, even if sphincter evidence exists, because they will never have bowel control.
Pull-through is only considered for patients with capacity to form solid stool (adequate colon), as bowel management only works with solid stool.
It is extremely unusual for cloacal exstrophy patients to have spontaneous bowel control; the overwhelming majority need a bowel management program (enema administration to keep patient clean).
Pediatric surgeons should not underestimate the growth capacity of tiny colonic pieces during the newborn period; even small segments will grow over three years if fecal stream passes through them.
Annual contrast studies through the colostomy (retrograde injection) are performed to assess colonic growth.
Before committing to pull-through, a trial bowel management program is performed through the colostomy: enemas are given to empty the colonic pouch, and if the patient stays 24 hours without stool in the colostomy bag, pull-through is likely to succeed.
Bowel management trial through colostomy is typically started after age 3 when families consider avoiding the stoma for school.
If a patient has very little colon and cannot form solid stool, the urologist is free to use bowel for bladder augmentation; if the patient has borderline colon, the urologist must use stomach for augmentation to preserve bowel for fecal function.
The colon to be pulled through is the most posterior structure in the pelvis, with the bladder and augmentation anterior to it; therefore, bladder augmentation must not be done before deciding on pull-through, or accessing the colon will be extremely difficult.
Contrast enema through the colostomy can distinguish true liquid stool from paradoxical diarrhea (liquid stool around solid fecal impaction).
Pull-through and bladder augmentation are ideally performed together in a single operation lasting approximately 12 hours, with pediatric surgery going first (posterior dissection) followed by urology (anterior augmentation).
During pull-through, if the patient has one or two appendices, a Malone appendicostomy can be created for antegrade enema administration, as the appendix remains in the abdomen when colon is pulled down.
Urologists almost never use colon for bladder augmentation in cloacal exstrophy because colon is needed to form solid stool; they typically use small bowel or stomach.
Occasionally, when a patient has a giant colonic pouch with very poor motility, the poor motility makes it good for bowel management (irrigate once daily, stays clean between irrigations), and a piece can be shared with urology for augmentation.
Midline abdominal incision from xiphoid to pubis is used for pull-through to preserve the flanks and quadrants for potential future stomas.
The colostomy is circumferentially dissected and separated from the abdominal wall, then the blood supply is carefully studied because cloacal exstrophy patients have very bizarre, aberrant vascular anatomy.
Careful observation of the vascular anatomy allows the surgeon to decide which vessels can be ligated to mobilize the colon to the perineum without compromising blood supply; sometimes no vessels need to be ligated.
The space behind the bladder is easily created, and the bowel is placed posteriorly; patients do not need prone positioning because the exstrophy makes everything anterior, so a supine frog-leg position provides full perineal access.
Cloacal exstrophy patients are lifelong patients due to orthopedic problems (separated pubic bones causing abnormal gait) and spinal problems (tethered cord requiring neurosurgical follow-up and potential cord release).
Some teenagers are unhappy about separated pubic bones causing abnormal gait (feet pointing laterally); some dedicated orthopedic surgeons have been able to bring pubic bones closer together.
During pull-through, vaginal reconstruction is attempted by approximating the hemivaginas as much as possible; the degree of separation varies on the spectrum.
When hemivaginas are close with only a septum separating them superiorly, the septum is removed as high as possible; when vaginas run in completely different directions, one hemivagina may be removed, leaving the one with the better-looking cervix.
Patients with functional hemiuterus may become pregnant, but it is high-risk pregnancy requiring specialized pediatric gynecology follow-up; in general, pregnancy is not advised.
Hemiuterus has a great tendency to produce miscarriages and premature labor; delivery must be by cesarean section due to limited abdominal space.
Bladder augmentation requires a Mitrofanoff conduit (appendix or part of a long appendix, sometimes shared half for urology and half for Malone) for intermittent catheterization to empty the bladder.
Augmented bladders produce large amounts of mucus; if mucus is not removed, it forms stones, so families must be taught to irrigate the bladder (not just empty it) to remove mucus and prevent infections.
Bladder irrigation is sometimes performed with gentamicin to ensure all mucus is removed.
Lifelong urologic follow-up is needed to monitor for reflux and kidney damage; the Mitrofanoff may stop working or leak urine, requiring revision or valve tightening.
Patients transitioning to adult hospitals often feel uncomfortable because adult urologists and orthopedic surgeons lack experience with these congenital malformations; patients prefer to remain in pediatric environments even as adults.
Dr. Peña observes that cloacal exstrophy patients are particularly charming, intelligent, beautiful, and charismatic when they grow up, and some have energy to help others manage their own problems.
The Pull-Through Network is a national organization (similar to cystic fibrosis or breast cancer organizations) for parents and patients with anorectal malformations, Hirschsprung disease, or bowel/urinary control problems; it has over 1000 members, holds annual meetings, and invites doctors to give talks.
Colorectal and urogenital problems have been left behind in terms of scientific approach and research funding because they are not 'elegant' problems—they involve stool, urine, and sex—and institutions are not eager to receive these patients.
The initial operation includes omphalocele closure (sometimes requiring staged closure), bowel-bladder separation, end colostomy creation, bladder closure attempt (sometimes requiring multiple stages), and in some institutions, pelvic osteotomy (either at initial operation or 2-3 months later).