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Cloacal Exstrophy with Dr. Alberto Peña
With Dr. Alberto Peña · hosted by Dr. Todd Ponsky
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Cloacal Exstrophy 4 items
Chapter 1 of 9 · Fundamentals
Anatomy overview
Introduction and Anatomy of Cloacal Exstrophy
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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 consists of omphalocele, bladder exstrophy with separated hemibladders, exposed cecum (often with 'elephant trunk' prolapse through the ileocecal valve), variable colon length (spectrum from normal to absent), genital abnormalities (hemiphalluses in males, hemivaginas/hemiuteri in females), and associated spinal/orthopedic abnormalities.
The amount of colon a patient with cloacal exstrophy is born with has very important repercussions and implications for management and outcomes.
There is a variant of cloacal exstrophy where babies are born with intact abdominal skin (no omphalocele, no bladder exstrophy externally) but have all the internal malformations including completely open bladder inside and a single large perineal orifice.
While surgical techniques have improved (safer operations, better intensive care, better parenteral nutrition), functional outcomes have not improved significantly—patients still suffer lifelong limitations in bowel control, urinary control, sexual function, and spinal abnormalities.
Historical practice was to perform bilateral gonadectomy, remove hemiphalluses, create a neovagina, and raise chromosomal males as females, but these patients often had male attitudes and behavior despite female upbringing and hormones.
When patients raised as females learned they were chromosomically male, many became upset, stating that sex is not the most important thing and that they wanted their gonads back for potential fertility with modern techniques.
Current consensus is that patients with XY chromosomes should be raised as male, with ongoing efforts in phallic reconstruction, though functional outcomes remain uncertain.
When babies with cloacal exstrophy were born in institutions dominated by pediatric urologists, they received good urologic attention but poor gastrointestinal management; the reverse occurred in surgeon-dominated institutions.
At the initial operation, the pediatric surgeon must separate the urothelium (bladder) from intestinal mucosa by placing multiple stitches at the edges and making an incision to completely separate the gastrointestinal tract from the urinary tract.
It is very common for pediatric surgeons worldwide to simply create an ileostomy rather than incorporating all colonic tissue and creating a true end colostomy, which is a serious error.
Creating an ileostomy and leaving colon distally attached to the urinary tract causes hyperchloremic acidosis from urine absorption starting in the newborn period, which interferes with growth and development.
Colonic tissue left defunctionalized or attached to the urinary tract will not grow; colon requires the passage of fecal matter through its lumen to grow.
The 'rescue operation' for patients who received ileostomy involves taking down the ileostomy, finding and incorporating all colonic tissue into the GI tract, and creating a true end colostomy; hyperchloremic acidosis disappears the next day after this operation.
Surgeons often create ileostomies because the colonic anatomy is so complex with bizarre blood supply that they prefer not to deal with it, taking the easier way out.
The surgeon must be prepared for each case to be different, sometimes finding a large sac of colonic tissue with bizarre blood supply requiring careful study to determine which vessels can be ligated.
Some institutions routinely perform pelvic osteotomy at the initial operation to facilitate bladder and omphalocele reconstruction, though pubic bones often remain separated long-term despite osteotomy.
Even a technically correct end colostomy without stricture sometimes doesn't work well due to poor motility of the incorporated colon, requiring colostomy irrigation similar to management of Hirschsprung disease enterocolitis.
Pull-through is only considered for patients who can form solid stool; patients with liquid stool (inadequate colon) should never have pull-through even if they show evidence of sphincter, as they will never have bowel control.
Bowel management only works if the patient has solid stool, and solid stool only occurs if the patient has enough colon.
It is extremely unusual for patients with cloacal exstrophy to have voluntary bowel control; the overwhelming majority will need bowel management programs.
Small pieces of colon that look tiny at birth should not be discarded; they have the capacity to grow during the first three years of life.
Annual contrast studies through the colostomy are performed to assess colonic growth during the first three years.
Before pull-through, trial bowel management is performed through the colostomy to determine if enemas can keep the patient clean for 24 hours, predicting success of bowel management after pull-through.
Coordination with pediatric urology is essential before pull-through because if the urologist wants to use bowel for bladder augmentation and the patient has borderline colon, the surgeon must not allow the urologist to take more GI tract.
The colon to be pulled through is the most posterior structure in the pelvis, with the bladder augmentation anterior to it; the urologist should not perform augmentation before deciding about pull-through, as accessing the colon behind the reconstruction would be a nightmare.
Patients with very little or no colon will have a permanent colostomy, and at that point the urologist has free hands to use whatever bowel is needed for bladder augmentation.
The combined pull-through and bladder augmentation operation is ideally done together by urologist and pediatric surgeon, typically lasting about 12 hours, usually around age 3 when families want to send children to school clean.
The pediatric surgeon operates first because the colon goes posteriorly, and separated pubic bones make the dissection somewhat easier.
A Malone appendicostomy can be created for antegrade enema administration; patients may have one or two appendices, and if the appendix is very long, it can be split with half used for Malone and half for Mitrofanoff.
Urologists almost never use colon for bladder augmentation in these patients because the colon is needed to form solid stool; they typically use small bowel or stomach.
In colorectal pediatric surgery, midline abdominal incisions are always used to preserve the flanks, iliac fossae, and lateral quadrants for potential future stomas.
Occasionally when patients are born with a giant colonic pouch with very poor motility, the pouch is so large that a piece can be shared with the urologist for augmentation, but this is unusual.
The blood supply in cloacal exstrophy is very bizarre with aberrant abnormal vessels; careful study is required to determine which vessels can be ligated without compromising colonic viability.
Patients with cloacal exstrophy do not need to be placed prone for the perineal portion of pull-through; everything is anterior due to the exstrophy, so a supine frog-leg position provides access to the entire perineum.
Patients with cloacal exstrophy will be patients for life due to orthopedic problems, and some teenagers are unhappy about persistent pubic bone separation causing a wide-based gait with feet pointing laterally.
Patients with severe spinal problems require lifelong follow-up by pediatric orthopedics and neurosurgery for tethered cord, sometimes requiring cord release.
During pull-through, vaginal reconstruction attempts to approximate the hemivaginas as much as possible; when vaginas run in completely different directions and cannot be brought together due to blood supply, one vagina may be removed, leaving the one with the better-looking cervix.
Patients with functional hemiuteri may become pregnant but it is high-risk pregnancy requiring specialized pediatric gynecology follow-up; hemiuterus has great tendency to produce miscarriages and premature labor, and delivery is by cesarean section.
Most patients will require bladder augmentation and Mitrofanoff (using appendix or piece of bowel) for intermittent catheterization to empty the bladder.
Augmented bladders produce a lot of mucus; if mucus stays in the bladder it forms stones, so families must be taught to irrigate the bladder (sometimes with gentamicin) to remove mucus and prevent infections.
Urologic follow-up is needed to ensure kidneys are not suffering from reflux, and to manage Mitrofanoff complications such as leakage or valve failure requiring revision.
Patients with cloacal exstrophy struggle with transition to adult care because adult urologists and orthopedic surgeons often lack experience with these malformations, so patients feel more confident in pediatric environments even as adults.
Patients with cloacal exstrophy when they grow up are particularly charming, intelligent, and beautiful; some manage their own problems and still have energy to help others.
Colorectal and urogenital problems have been left behind in terms of scientific approach and research funding because they are not considered elegant problems, being related to stool, urine, and sex, and institutions are not enthusiastic about receiving these patients.
Prenatal diagnosis of cloacal exstrophy is easier in the most complex defects because they have visible spinal problems and absent bladder (bladder not visible in utero due to exstrophy), detectable as early as week 20 of pregnancy.
Simple anorectal malformations like rectoperineal fistula are difficult to detect prenatally because imaging lacks necessary definition and these patients don't have other associated defects.
Prenatal diagnosis allows families to make decisions about pregnancy continuation and, if continuing, to deliver at a center with a dedicated team for these malformations.