Cloacal Exstrophy
Everything in the library about cloacal exstrophy — built automatically from the recorded discussions that name it
Educational content from recorded physician discussions — not medical advice. Always talk to your child's care team about your child's situation.
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Surgical Management
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Cloacal Exstrophy: A Modification of the Newborn Operation - Leaving the Cecal Plate Untouched
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Pediatric Colorectal & Pelvic Reconstruction | Children's National Hospital (childrensnational.org)
video6:48 · Oct 2024
In-Depth Reviews
2 items
Cloacal Exstrophy with Dr. Alberto Peña
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Discussion between Dr. Alberto Pena and Dr. Todd Ponsky about cloaca exstrophy
podcast53:06 · Jan 2019
Cloacal Exstrophy with Dr. Alberto Peña
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Discussion between Dr. Alberto Pena and Dr. Todd Ponsky about cloaca exstrophyPodcast Discussion:What is cloacal exstrophy?A group of congenital diseases involving the lower gastrointestinal tract, urogenital tract, spine, and even the lowe
podcast53:06 · Dec 2020
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Every expert statement below comes from the recorded discussions, with its speaker and moment.
Cloacal Exstrophy with Dr. Alberto Peña
Cloacal exstrophy is a spectrum of congenital malformations affecting the gastrointestinal/colorectal area, urogenital tract, spine and cord, and sometimes lower extremity motion.
clinicalAlberto Peña1:31 ↗
Babies with cloacal exstrophy are born with an omphalocele, bladder exstrophy (two separated hemibladders), open cecum between the hemibladders, and separated pubic bones.
clinicalAlberto Peña2:09 ↗
The small bowel can become exstrophic through the ileocecal valve, creating an 'elephant trunk' appearance.
clinicalAlberto Peña3:08 ↗
Male patients have two separated hemiphalluses with normal gonads; female patients have two hemivaginas below the exstrophic bladder leading to two hemiuteri.
clinicalAlberto Peña3:32 ↗
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.
clinicalAlberto Peña4:20 ↗
The amount of colon present at birth has very important implications for the patient's management and outcomes.
clinicalAlberto Peña4:57 ↗
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.
clinicalAlberto Peña5:44 ↗
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.
clinicalAlberto Peña7:02 ↗
Historical practice was to perform bilateral gonadectomy, remove hemiphalluses, create a vagina with bowel, and assign female gender to XY patients with cloacal exstrophy.
clinicalAlberto Peña8:31 ↗
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.
clinicalAlberto Peña9:16 ↗
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.
opinionAlberto Peña9:59 ↗
Current consensus is that XY patients should be raised as male, with pediatric urologists and plastic surgeons working on phallus reconstruction techniques.
guidelineAlberto Peña10:34 ↗
When a prominent pediatric urologist dominates management, patients receive good urologic attention but inadequate gastrointestinal care; the reverse occurs when pediatric surgeons dominate.
clinicalAlberto Peña12:13 ↗
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.
clinicalAlberto Peña13:03 ↗
It is very common but very harmful for pediatric surgeons to simply create an ileostomy, leaving all colon distally attached to the urinary tract.
clinicalAlberto Peña14:55 ↗
Leaving colon attached to the urinary tract creates a congenital bladder augmentation that causes hyperchloremic acidosis from urine absorption, interfering with growth and development.
clinicalAlberto Peña15:31 ↗
Defunctionalized colonic tissue left distally will not grow; colon requires passage of fecal matter through its lumen to grow.
clinicalAlberto Peña16:03 ↗
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.
clinicalAlberto Peña16:23 ↗
Patients who received ileostomy with defunctionalized colon present at 2-3 years with poor growth, hyperchloremic acidosis managed by nephrologists, and large ileostomy losses.
clinicalAlberto Peña16:41 ↗
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.
clinicalAlberto Peña17:03 ↗
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.
opinionAlberto Peña19:13 ↗
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.
clinicalAlberto Peña21:00 ↗
Even after osteotomy, it is very difficult to see a cloacal exstrophy patient with pubic bones completely together; they usually remain separated.
clinicalAlberto Peña21:09 ↗
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.
clinicalAlberto Peña22:06 ↗
Families must be taught to irrigate the colostomy with small volumes of saline through a tube to evacuate fecal material when peristalsis is inadequate.
clinicalAlberto Peña23:00 ↗
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).
clinicalAlberto Peña23:30 ↗
Most cloacal exstrophy patients have an inadequate, tiny bladder requiring bladder augmentation with gastrointestinal tract, necessitating coordination between pediatric surgery and urology.
clinicalAlberto Peña23:54 ↗
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.
clinicalAlberto Peña24:42 ↗
Pull-through is only considered for patients with capacity to form solid stool (adequate colon), as bowel management only works with solid stool.
clinicalAlberto Peña25:04 ↗
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).
clinicalAlberto Peña25:20 ↗
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