Cloacal Exstrophy with Dr. Alberto Peña
With Dr. Alberto Peña · hosted by Dr. Todd Ponsky · StayCurrentMD
Part of
Cloacal Exstrophy 3 items
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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Cloacal Exstrophy with Dr. Alberto Peña
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What the experts said
Cloacal exstrophy is a spectrum of congenital malformations ranging from normal colon to almost absent colon, and the amount of colon present has very important implications for the patient and surgeon.
Cloacal exstrophy affects the gastrointestinal/colorectal area, urogenital tract, spine and cord, and sometimes the motion of lower extremities.
Babies with cloacal exstrophy are born with an omphalocele, bladder exstrophy with separated pubic bones, two hemibladders, and exposed gastrointestinal mucosa (usually cecum) between the hemibladders.
Male patients with cloacal exstrophy are born with two separated hemiphalluses and usually normal gonads; female patients have two hemivaginas with orifices below the exstrophic bladder and two hemi-uteri.
Inside the abdomen, cloacal exstrophy patients have a spectrum of gastrointestinal deformities including short or absent colon, sometimes two ceca, sometimes two appendices, and most commonly a pouch of colonic tissue with bizarre blood supply.
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 and operations can be done more safely with better intensive care and parenteral nutrition, functional outcomes (bowel control, urinary control, sexual function) have not progressed significantly—patients suffer lifelong serious limitations.
Historically, male babies with cloacal exstrophy underwent bilateral gonadectomy, removal of hemiphalluses, vaginal creation with bowel, and were assigned female gender and raised as females.
Patients who were assigned female gender despite XY chromosomes often displayed male attitudes and behavior, and many became upset when learning they were chromosomally male, stating that sex is not the most important thing and they wanted their gonads back.
Current consensus is that patients born with XY chromosomes should be raised as male, and pediatric urologists and plastic surgeons are working on phallic reconstruction techniques.
In Dr. Peña's early experience, when a prominent pediatric urologist dominated the surgical department, patients received good urologic attention but poor gastrointestinal attention; conversely, when pediatric surgeons led, urologic concerns were sometimes neglected.
The pediatric surgeon's role in initial cloacal exstrophy repair is to close the omphalocele (if possible), separate the urothelium from intestinal mucosa by placing stitches at the edges and making an incision, and create a true end colostomy incorporating all gastrointestinal tissue.
Creating an ileostomy instead of an end colostomy is a serious error that leaves dysfunctionalized colon attached to the urinary tract, causing hyperchloremic acidosis from urine absorption, poor growth, and preventing colonic growth (colon requires fecal stream to grow).
Patients who receive an ileostomy with dysfunctionalized colon attached to the bladder develop hyperchloremic acidosis, growth problems, and large ileostomy losses, often requiring nephrologist management.
The rescue operation for patients with ileostomy and dysfunctionalized colon consists of taking down the ileostomy, finding and incorporating the colonic tissue into the GI tract, performing end-to-end anastomosis, separating colonic tissue from the urogenital tract, and creating a true end colostomy.
After the rescue operation incorporating dysfunctionalized colon, hyperchloremic acidosis disappears the next day.
Surgeons create ileostomies instead of end colostomies because the colonic anatomy is so complex and bizarre that they prefer not to deal with it, finding it easier to simply open an ileostomy.
In some institutions, orthopedic surgeons perform pelvic osteotomy at the initial operation to approximate pubic bones, which facilitates bladder and omphalocele reconstruction, though long-term complete approximation is rare in cloacal exstrophy.
Even technically correct end colostomies in cloacal exstrophy patients often have poor motility, and babies may not pass stool easily, sometimes developing bacterial overgrowth similar to Hirschsprung disease, requiring colostomy irrigation.
It is extremely unusual for patients with cloacal exstrophy to have voluntary bowel control; the overwhelming majority will need bowel management programs.
Bowel management (enema administration to keep patients clean) only works if the patient has solid stool, which requires adequate colon length.
Pediatric surgeons should not discard tiny pieces of colon during the newborn operation—these pieces will grow over the first three years if fecal stream passes through them.
Before considering pull-through, surgeons should perform annual contrast studies through the colostomy to assess colon growth and trial bowel management through the colostomy to determine if the patient can stay clean for 24 hours after an enema.
Coordination between pediatric surgery and urology is critical before definitive reconstruction because if the urologist performs bladder augmentation first, subsequent pull-through becomes extremely difficult (the colon must pass behind the augmented bladder).
Patients with very little or no colon who cannot form solid stool are not candidates for pull-through and will require a permanent colostomy.
The combined pull-through and bladder augmentation operation is typically a 12-hour procedure with the pediatric surgeon operating first (pull-through) followed by the urologist (bladder augmentation).
If a patient has adequate colon for pull-through, the urologist should use small bowel or stomach (not colon) for bladder augmentation to preserve colonic tissue for solid stool formation.
During pull-through, surgeons use midline abdominal incisions to preserve the flanks and quadrants for potential future colostomy sites.
The blood supply in cloacal exstrophy is extremely bizarre with aberrant vessels, requiring careful study to determine which vessels can be ligated to mobilize the colon to the perineum without compromising blood supply.
Pull-through in cloacal exstrophy is performed with the patient supine in frog-leg position (not prone) because the exstrophy makes everything anterior and separated pubic bones provide access to the entire perineum.
Cloacal exstrophy patients are patients for life due to orthopedic problems, spinal abnormalities requiring neurosurgical follow-up for tethered cord, and ongoing urologic management.
During pull-through, surgeons attempt to approximate the hemivaginas as much as possible, sometimes removing the vaginal septum or, if vaginas run in completely different directions, removing one vagina and leaving the one with the better-looking cervix.
Female patients with cloacal exstrophy who have functional hemi-uteri may become pregnant but face high-risk pregnancies with increased miscarriage and premature labor rates, requiring specialized gynecologic follow-up and cesarean delivery.
Most cloacal exstrophy patients require bladder augmentation and a Mitrofanoff (appendiceal conduit) for intermittent catheterization to empty the bladder.
Augmented bladders produce mucus that can form stones if not removed, requiring family education on bladder irrigation and mucus removal, sometimes with gentamicin irrigation.
Transition to adult care is problematic for cloacal exstrophy patients because adult specialists often lack experience with these congenital malformations, and patients feel more confident in pediatric environments even as adults.
Dr. Peña observes that patients with cloacal exstrophy who grow up are particularly charming, intelligent, and beautiful, with some managing their own problems while helping others.
Prenatal diagnosis of cloacal exstrophy is easier than simpler anorectal malformations because complex defects have associated findings visible on ultrasound, such as absent bladder (exstrophic) and spinal abnormalities, detectable as early as 20 weeks gestation.
Prenatal diagnosis allows families to make decisions about pregnancy continuation and, if continuing, to deliver at a center with a multidisciplinary team experienced in cloacal exstrophy management.
Colorectal and urogenital problems have been left behind in terms of scientific approach and research funding because they are not considered elegant problems and relate to stool, urine, and sex—topics institutions are reluctant to prioritize.