# Cloacal Exstrophy with Dr. Alberto Peña — GCMD Library

<p>Discussion between Dr. Alberto Pena and Dr. Todd Ponsky about cloaca exstrophy</p><p><a href="http://videolibrary.globalcastmd.com/cloacal-exstrophy-with-dr-alberto-pena"></a></p><h4><a>Podcast Discussion:</a></h4><h4><a>What is cloacal exstrophy?</a></h4><p></p><p></p><ul><li>A group of congenital diseases involving the lower gastrointestinal tract, urogenital tract, spine, and even the lower extremities. Patients may often be born with omphalocele</li><li>There may be bladder exstrophy. Female patients may have two hemi-vaginas and hemi-uteruses, and male patients may have two hemi-phalluses. Gonads are usually normal. There may be kidney problems, but they tend to be normal.</li><li>Exposed GI mucosa tends to involve the cecum. There may also be ileo-cecal intussusception.</li><li>Intra-abdominal findings include a shortened or absent colon. There may be duplication of the cecum or appendix. Most commonly found is a small pouch of colon with abnormal blood supply.</li><li>Spinal abnormalities can include tethered cord or myelomeningocele, which can affect the motion of the lower extremities.</li><li>Patients may be born without omphalocele, but can still have all of the above abnormalities, albeit intraabdominally.</li></ul><a>Gender Assignment</a><p></p><p></p><ul><li>Gender assignment remains controversial; however, it remains extremely difficult to create a function phallus in a patient born with hemi-phalluses.</li><li>Historically, male patients born with hemi-phalluses underwent bilateral orchiectomy, removal of the hemi-phalluses, and creation of a neo-vagina (typically from small bowel). They were also administered exogenous female hormones. These patients were reared as females. However, they tended to develop male attitudes and behaviors and many of these patients became resentful of their gender re-assignment as adults.</li><li>Now, patients who are born with XY chromosomes are raised as males. Creation of a better phallus for patients with hemi-phallus is an active area of research.</li></ul><p></p><p><a>Management of cloacal extrophy</a></p><ul><li>Multidisciplinary approach is paramount.</li><li>No significant improvements in the ultimate functional status of patients (serious limitations in bowel and urinary control, as well as sexual function and spinal abnormalities).</li></ul><h4><a>Role of the pediatric general surgeon in cloacal exstrophy</a></h4><ul><li> The three major elements of repair are:</li><ul><li>Closure of the omphalocele (although this is not always possible)</li><li>Differentiation, and subsequent separation, of urothelium from intestinal mucosa. This allows the urologist to approximate the edges of urothelium in an attempt to close the bladder.</li><li>End colostomy creation</li></ul></ul><a>Failure to create a real End Colostomy and Rescue Operation</a><p></p><ul><li>The pediatric general surgeon must be prepared to encounter very bizarre anatomy. However, he must nonetheless differentiate proximal from distal colon, with subsequent creation of an end colostomy. A common, yet very serious, error is creation of an end ileostomy. This leaves the colon in discontinuity and attached to the urinary tract. Consequences of this include large ileostomy losses and absorption of urine via the colon (which can cause acidosis and subsequent retardation of growth and development). The defunctionalized colon will also not grow.</li><li>For patients who have undergone end ileostomy and have been referred to the Cincinnati Children’s Hospital, a "rescue” operation has been created in order to reclaim the previously unused colonic gastrointestinal mucosa.</li><li>Rescue entails ileostomy takedown with re-anastomosis, location of colonic tissue with separation of colonic from urogenital tissue, and creation of end colostomy.</li></ul><p><a>Pubic Separation and Pelvic Osteotomies</a></p><p></p><ul><li>The separation of the pubic bones in bladder exstrophy is not as severe as in cloacal exstrophy. It is very difficult to properly correct the pubic bone separation.</li><li>It is not always necessary to perform Pelvic osteotomies during the initial operation.</li><li>Due to orthopedic problems, patients with cloacal exstrophy will be patients for life. These patients tend to have persistent separation of the pubic bones. This alters the gait, which may be a significant source of concern for some patients. Other patients may have severe spinal problems, including tethered cord.</li></ul><a>Colonic Dysmotility and Bowel Management</a><p></p><ul><li>The end colostomy may have poor motility, for unknown reasons. Under these circumstances, the children may develop bacterial overgrowth, similar to Hirschsprung’s enterocolitis. These patients require irrigation via the stoma.</li><li>Around 3 years of age, when these patients are getting ready for school, decisions must be made regarding stoma takedown and urinary continence. These patients have small bladders and will require bladder augmentation. This is typically performed using gastrointestinal tract.</li><li>To determine if the patient will be a good candidate for pull-through, a bowel management through stoma program should be used. This involves administration of enemas through the end colostomy.</li><li>If the patient has no colon whatsoever, they will have a stoma for life. The ileum should not be attempted to be pulled down to the perineum, as there will be lifelong incontinence. Only patients who can form solid stool are eligible for pull-through and only patients with adequate colon can form solid stools. During all pediatric surgical operations, all attempts should be made to include and preserve the maximum amount of colon.</li><li>The pediatric surgeon’s job is complete if the patient is to have a lifelong stoma.</li></ul><p><a>Surgical planning and technique</a></p><p></p><p></p><ul><li>Urologic and general surgery operations are typically performed together. The general surgery portion is first. The end colostomy is taken down and the colon pulled down to the pelvis. This may be performed with an appendicostomy (Malone procedure) in order to administer antegrade enemas. Typically, small bowel, and/or stomach is used for bladder augmentation. The colon is typically not used in order to aid in preservation of solid stools.</li><li>The patient is placed supine, usually in the frog leg position. There is no need for prone positioning. A midline abdominal incision is performed in order to preserve the flanks and lower quadrants for potential future stoma creation. Dense adhesions are to be anticipated. The stoma is taken down circumferentially from the abdominal wall. The space posterior to the bladder is created. Next, the colon is mobilized in order for the distal colon to be brought into the vessel. Some ligation of blood vessels is usually required, and again it should be noted that there is significant variation in vascular supply to the colon in patients with cloacal exstrophy.</li></ul><p><a>Vaginal and Urologic Issues in Cloacal Exstrophy</a></p><p></p><ul><li>In patients born with two hemi-vaginas, there is a wide spectrum of disease. In less severe cases, the intervaginal septum may be simply removed. Other patients may have two distinct vaginas and removal of one of the vaginas is elected. Typically, the vagina with the more normal-appearing cervix is preserved. This operation is performed at the time of colonic pull-through. These patients may become pregnant, but they have very high risk pregnancies. There is a high rate of miscarriage and premature labor and Caesarean section is recommended. Pregnancy is typically not advised.</li><li>A Mitroffanoff procedure may be required for intermittent bladder drainage.</li><li>Even as adults, these patients should follow up with pediatric surgeons.</li></ul><p><a>The Pull-Thru Network</a></p><p></p><p></p><ul><li>The Pull-Thru Network is a national organization of parents and children to support those born with anorectal malformations. Per their website, "Information, education, support and advocacy for families, children, teens and adults who are living with the challenges of congenital anorectal, colorectal or urogenital disorders.”</li></ul><p><a>Summary</a></p><p></p><ul><li>Initial operation (neonatal period)</li><ul><li>Close (or attempt to close) omphalocele</li><li>Separate bowel from urinary tract</li><li>Create end colostomy</li><li>Close (or attempt to close) bladder</li><li>Osteotomy and attempted approximation of pubis bones</li></ul><li>Subsequent operation (3 years of age)</li><ul><li>Colostomy closure</li><li>Colon pull-through</li><li>Bladder augmentation</li><li>Vaginal reconstruction</li><li>Possible Malone and Mitrofanoff procedures</li></ul></ul><p></p><a>Prenatal diagnosis</a><p></p><p></p><ul><li>Imaging technology is improving, aiding in prenatal detection. More complex anorectal disease are actually easier to diagnose prenatally, as they tend to have less subtle findings and more associated defects.</li></ul>

Type: podcast · 53 min · posted 2020-12-30
Canonical: https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309

## Chapters
- [0:00](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=0) Introduction and Anatomy of Cloacal Exstrophy
- [6:24](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=384) Gender Assignment and Phallus Reconstruction
- [11:39](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=699) Initial Surgical Management and the Ileostomy Problem
- [19:41](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1181) Colostomy Management and Pull-Through Candidacy
- [26:41](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1601) Coordination with Urology and Pull-Through Technique
- [37:10](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2230) Long-Term Outcomes and Multidisciplinary Follow-Up
- [46:55](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2815) Prenatal Diagnosis, Centers of Excellence, and Closing

## Statements
- "Cloacal exstrophy affects the gastrointestinal/colorectal area, urogenital tract, spine and cord, and sometimes motion of lower extremities" — Alberto Peña (clinical) [1:31](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=91)
- "Babies are born with omphalocele, bladder exstrophy with two hemibladders, open cecum between the hemibladders, and separated pubic bones" — Alberto Peña (clinical) [2:09](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=129)
- "The small bowel can become exstrophic through the ileocecal valve creating an 'elephant trunk' appearance" — Alberto Peña (clinical) [3:08](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=188)
- "Males have two separated hemiphalluses with normal gonads; females have two hemivaginas below the exstrophic bladder leading to two hemi-uteri" — Alberto Peña (clinical) [3:32](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=212)
- "Colonic anatomy exists on a spectrum from normal colon to almost absent colon or no colon at all, sometimes with two ceca or two appendices and bizarre blood supply" — Alberto Peña (clinical) [4:20](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=260)
- "The amount of colon present at birth has very important implications for the patient and surgeon" — Alberto Peña (clinical) [4:57](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=297)
- "A variant exists where babies have intact abdominal skin without omphalocele or bladder exstrophy externally, but have completely open bladder and all internal malformations" — Alberto Peña (clinical) [5:44](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=344)
- "We have progressed in safer surgical techniques, intensive care, and parenteral nutrition, but cannot claim much progress in functional sequelae" — Alberto Peña (opinion) [7:02](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=422)
- "Patients suffer lifelong serious limitations in bowel control, urinary control, sexual function, and spinal abnormalities that can be managed but not made normal" — Alberto Peña (clinical) [7:34](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=454)
- "Historical practice was bilateral gonadectomy, hemiphallus removal, vaginal creation with bowel, and female gender assignment for XY patients" — Alberto Peña (clinical) [8:31](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=511)
- "Patients raised as females despite XY chromosomes showed male attitudes and behavior, and became upset when learning of their chromosomal sex and surgical reassignment" — Alberto Peña (clinical) [9:16](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=556)
- "Patients argued that sex is not the most important thing, they wanted their gonads back, and with modern techniques can fertilize and have children" — Alberto Peña (clinical) [9:59](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=599)
- "Current consensus is that XY patients should be raised as male, with urologists and plastic surgeons working on phallic reconstruction" — Alberto Peña (guideline) [10:34](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=634)
- "When prominent pediatric urologists dominated surgical departments, patients received good urologic attention but poor gastrointestinal attention, and vice versa when pediatric surgeons led" — Alberto Peña (clinical) [12:13](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=733)
- "The pediatric surgeon must separate the urothelium of the bladder from intestinal mucosa by placing multiple stitches at the edges and making an incision" — Alberto Peña (clinical) [13:31](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=811)
- "It is very common and very bad for pediatric surgeons to simply create an ileostomy, leaving all colon distally attached to the urinary tract" — Alberto Peña (opinion) [14:55](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=895)
- "Leaving colon attached to the urinary tract creates a natural congenital bladder augmentation that urologists may appreciate" — Alberto Peña (clinical) [15:31](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=931)
- "Babies with ileostomy and colon left attached to bladder absorb urine from the newborn period causing hyperchloremic acidosis that interferes with growth and development" — Alberto Peña (clinical) [15:43](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=943)
- "Defunctionalized colon left distally will not grow and remain tiny; colon requires passing fecal matter through its lumen to grow" — Alberto Peña (clinical) [16:03](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=963)
- "The rescue operation involves taking down the ileostomy, finding colonic tissue, performing end-to-end anastomosis, and creating an end colostomy, which makes acidosis disappear the next day" — Alberto Peña (clinical) [17:03](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1023)
- "Surgeons must accept they are dealing with a spectrum and sometimes the colonic component is so complex they prefer to avoid it and simply open an ileostomy" — Alberto Peña (opinion) [19:13](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1153)
- "The surgeon must not leave gastrointestinal tract inside defunctionalized—this is the main principle" — Alberto Peña (guideline) [20:07](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1207)
- "Orthopedic surgeons in some institutions routinely perform pelvic osteotomy at initial operation, which facilitates bladder and omphalocele reconstruction" — Alberto Peña (clinical) [21:00](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1260)
- "Even technically correct colostomies sometimes don't work well due to poor motility of the incorporated colon" — Alberto Peña (clinical) [22:00](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1320)
- "Babies with poor colonic motility may have bacterial overgrowth similar to Hirschsprung disease and require colostomy irrigation" — Alberto Peña (clinical) [22:20](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1340)
- "Babies below 3 years of age are in diapers at home and don't care about them, but around age 3 when starting school is when the second phase of management begins" — Alberto Peña (clinical) [23:18](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1398)
- "Most patients require bladder augmentation using gastrointestinal tract, and the pediatric surgeon is the defender of the gastrointestinal tract" — Alberto Peña (opinion) [23:54](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1434)
- "Patients born with no colon are candidates for colostomy for life and should never have terminal ileum pulled through even if sphincter evidence exists, because they will never have bowel control" — Alberto Peña (guideline) [24:42](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1482)
- "Only patients with capacity to form solid stool should be considered for pull-through, as bowel management only works with solid stool" — Alberto Peña (guideline) [25:04](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1504)
- "Surgeons should not underestimate the capacity of tiny pieces of colon to grow and should incorporate everything rather than discarding it" — Alberto Peña (guideline) [25:47](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1547)
- "Trial of bowel management through the colostomy simulates the colostomy as the new anus; if enema keeps patient clean for 24 hours with no stool in bag, pull-through may work" — Alberto Peña (clinical) [26:41](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1601)
- "If patient has very little colon and cannot form solid stool, the urologist can freely use bowel for augmentation since pull-through is not an option" — Alberto Peña (clinical) [28:07](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1687)
- "The colon to be pulled through is the most posterior structure in the pelvis with bladder augmentation in front, so augmentation should not be done before deciding on pull-through or it becomes a nightmare to access" — Alberto Peña (clinical) [28:54](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1734)
- "Pull-through and bladder augmentation should ideally be done together in approximately a 12-hour operation" — Alberto Peña (clinical) [31:04](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1864)
- "Pediatric surgeon goes first because the colon goes in the back, and separated pubic bones make things easier" — Alberto Peña (clinical) [31:16](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1876)
- "Appendix stays up in abdomen when colon is pulled down and can be used for Malone procedure to administer enemas" — Alberto Peña (clinical) [31:28](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1888)
- "Urologists almost never use colon for augmentation because colon is needed to form solid stool" — Alberto Peña (clinical) [31:57](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1917)
- "Giant colonic pouches with poor motility are good for bowel management because lack of peristalsis means once-daily irrigation keeps patient clean" — Alberto Peña (clinical) [32:26](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1946)
- "Midline abdominal incisions are always used in colorectal pediatric surgery to keep flanks and quadrants available for potential stomas" — Alberto Peña (clinical) [33:13](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=1993)
- "The blood supply in cloacal exstrophy is very bizarre with aberrant abnormal vessels, requiring careful study to avoid ligating crucial vessels and losing the colon" — Alberto Peña (clinical) [34:20](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2060)
- "Patients don't need prone positioning because the exstrophy makes everything anterior; frog-leg supine position provides access to entire perineum" — Alberto Peña (clinical) [35:53](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2153)
- "Patients have orthopedic problems for life, and some teenagers are unhappy about persistent pubic bone separation" — Alberto Peña (clinical) [37:33](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2253)
- "Separated pubic bones cause walking with feet separated and pointing laterally, which looks ugly and patients complain about" — Alberto Peña (clinical) [37:55](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2275)
- "Patients with severe spinal problems need ongoing follow-up by pediatric orthopedics and neurosurgeon for tethered cord, sometimes requiring cord release" — Alberto Peña (clinical) [38:21](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2301)
- "During pull-through, vaginas are approximated as much as possible; degree of separation varies on the spectrum" — Alberto Peña (clinical) [38:50](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2330)
- "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 better-looking cervix" — Alberto Peña (clinical) [39:20](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2360)
- "Patients with functional hemi-uterus may become pregnant but it is high-risk and should be followed by specialized pediatric gynecologist" — Alberto Peña (clinical) [39:53](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2393)
- "General advice is not to become pregnant, but some patients want to and may deliver by cesarean section" — Alberto Peña (opinion) [40:10](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2410)
- "Hemi-uterus has great tendency to produce miscarriages and premature labors" — Alberto Peña (clinical) [40:30](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2430)
- "Augmented bladder produces a lot of mucus; if mucus stays it forms stones, requiring family teaching on bladder irrigation and mucus removal" — Alberto Peña (clinical) [41:07](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2467)
- "Mitrofanoff may stop working or leak urine and need revision or valve tightening, similar to Malone" — Alberto Peña (clinical) [42:04](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2524)
- "When patients transition to adult hospitals they don't feel well because adult specialists lack experience with these malformations" — Alberto Peña (clinical) [42:29](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2549)
- "Patients with cloacal exstrophy when they grow up are particularly charming, intelligent, charismatic, and beautiful" — Alberto Peña (opinion) [42:57](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2577)
- "Colorectal and urogenital problems have been left behind in terms of scientific approach and research funds because they are not elegant problems related to stool, urine, and sex" — Alberto Peña (opinion) [44:48](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2688)
- "Prenatal diagnosis of anorectal and urogenital malformations is easier in the most complex defects because they have visible spinal problems and absent bladder" — Alberto Peña (clinical) [47:58](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2878)
- "No bladder visible on ultrasound from week 20 of pregnancy is a bad sign" — Alberto Peña (clinical) [49:00](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2940)
- "Prenatal diagnosis allows families to make decisions about pregnancy interruption or to deliver in a place with a specialized team" — Alberto Peña (clinical) [49:38](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=2978)
- "Certain malformations require centers of excellence with dedicated focused teams, otherwise after 20 years there will be many damaged children and nobody properly trained" — Alberto Peña (opinion) [50:30](https://library.globalcastmd.com/watch/cloacal-exstrophy-with-dr-alberto-pe-a-309?t=3030)

## Transcript
Stay Current is a multimedia publication designed to keep healthcare professionals up to date with standards of care and new emerging ideas. Stay Current is created and edited by Todd Ponsky, Nicholas Bruns, and Ian Glenn in partnership with Globalcast MD and is recorded and produced at Akron Children's Hospital in Akron, Ohio. Welcome to Stay Current in Pediatric Surgery, and today we're going to be talking about a rare finding of pediatric surgery, and that is cloacal atrophy. And although it's rare, it is quite complicated, and many of us have a difficult time understanding exactly what we should be doing in certain situations and Uh, we have none other than the great Alberto Pena who is with us today and going to be guiding us through some of the current concepts and controversies of coal atrophy. Dr. Alberto Pena is a clinical professor of surgery at the University of Cincinnati and is the founding director of the Alberto Pena Colorectal Center at Cincinnati Children's Hospital. Dr. Pena, thank you so much for joining us today. My pleasure. Well, before we get going on some of the concepts and controversies, let me ask you, can you explain what cloacal atrophy is and what are the components of cloacal atrophy? Sure, the cloacal atrophy is a group of congenital malformations that affect the gastrointestinal colorectal area. It also affects the eurogenital tract. The, the, the spine and the cord sometimes affects the motion of the lower extremities and, and, and therefore has many implications in the quality of life of the unfortunate patients that suffer from this. Basically, it consists in the babies are born. And with an halocele, typically with an phallocele, they also have all the stigmata of the, of the atrophy of the bladder. In other words, they have a bladder atrophy. The pubic bones are separated, and the below the onfalocele is the bladder totally trophic. And it's actually one can identify two half bladders. In other words, one hemi bladder on one side, the other hemi bladder on the other side completely open, and in the middle in between both hemibladders, it is relatively easy to identify a gastrointestinal mucosa also protruding. It usually is the cecum that is also open, completely open. And through the ileocecal valve of that cecum, sometimes the small bowel becomes atrophic. In other words, it's like an like an insusception and the babies are born with a prominent piece of bowel. That is that is known as elephant trunk. So if the patient happens to be male, then they are born with two little hemiphalluses separated and different degrees of separation, and usually they have normal gonads. And if the patient is a female, what they have is two hemivaginas that the orifices are located below the trophic bladder, and if you, if we introduce an instrument or a cystoscope through that, we can see that both hemivaginas run. And laterally, in other words, away from the midline and the patients have two hemi uterus. Now inside the abdomen we find that these patients have a spectrum. Of gastrointestinal deformities, the most characteristic is a short sac, I'm sorry, short colon or almost absent colon or no colon at all, sometimes two secus, sometimes two appendices, and most commonly they have rather than having a normal colon, they have a pouch of colonic tissue with an extremely bizarre blood supply. There is a growing belief that these patients have no colon. Actually, as I mentioned, this is a spectrum that goes from a normal colon to almost absent colon, and that the amount of colon that the patient is born with has very important repercussions and implications for the For the patient and for the pediatric general pediatric surgeon and the urinary tract, they may have also problems in the kidneys, but, but most of the time the kidneys work well. In the spine they have Decord and different degrees of spinal abnormalities, sometimes myelomeningocele that affects the motion of the lower extremities. So, so basically that's the, that's a cloal atrophy, and there is one variant that I hope we can mention later that is babies that are born with no phallocele, with no bladder atrophy, but inside the abdomen they have all the malformations that I described. In other words, The only difference is that they have an intact skin, abdominal skin, but inside they have a completely open bladder. Open inside, in other words, they have no bladder neck, but it's not trophic. They have a single perineal orifice but very large. So you know this is a pretty complex and devastating. Collection of malformations that you just described and just to repeat it's the gastrointestinal tract, the urogenital tract, the spine, and sometimes the movement of the lower extremities and the abdominal wall. Have we progressed in the management of this condition and the sequelae? And you know, these, these patients must be treated by a group of subspecialties, and in each area there have been some progresses in the management of these patients, like the entire, as you know, the entire technology and science related with medicine is improving and therefore we have a better way to do operations to a safer way to do it, intensive care. Parental nutrition, knowledge of the metabolic concern of the patients, but it is true that we can repair these malformations in a safer way, but we, we cannot claim that we progress very much in terms of the functional sequela. Of these patients, in other words, a patient that is born with a cloal atrophy is a patient that would suffer for life, serious limitations, bowel control, urinary control, sexual function. And spinal abnormalities and we can manage those things, but we cannot make them normal. You've mentioned now twice, so I want to touch on this about the sexual function. You also talked about that in boys they can have a hemiphallus. Um, there's some controversy related to gender assignment, and I think the reason is that the male babies are born with the two little hemiphallus. And uh hemefala, I think, and it's it's extremely challenging to create a, a functional phallus. So, what is your opinion and what is the current accepted management of this problem? See, in the early times when a baby was born with a clocal extrophy and it happened to be male, the general idea was that it was better to do a bilateral gonadectomy, removal of those hemiphalluses, and create a vagina with a piece of bowel, assign the female gender to the patient. And raise the patient as a female, educate the like a like a female, and that when I was When I was in my training, that's what we, what everybody used to do, and several things happened through the years. First of all, those patients grew up and, and we, we got all scared when we saw that even when they were raised as females and they dressed like females and they received hormones to look like females. They had attitudes and behavior very much of a male, and worse than that is the fact that when many of them learn that they were actually chromosomically females and that somebody decided on behalf of them to raise them as females, they get very upset because they, and they are right, they said. And and the parents and the doctors explained that it was, it would be a very difficult life to be raised as a male with an inadequate phallus to function sexually, and the answer from these young individuals was, who told you that sex is the most important thing? I want, I wanted my go nuts back, and with modern techniques, those individuals can fertilize, they can have children. And they say being a male is much more than having a phallus, and they are right. So now the pendulum came back, and most institutions and doctors agree that if a patient is born with an XY chromosome should be raised as male, and many visionaries, ambitious pediatric neurologists and plastic surgeons are working. In the reconstructing of phallus to make it as functional as possible, which remains to be seen, but at least that's what's happening at the present time. So you think that they've made progress? I mean, I know we don't know yet, but as far as techniques have evolved recently, that they're becoming better at creating a functional phallus, possibly. Yes, I have seen very interesting creative ideas that shows the ingenuity of. Of the doctors, but we cannot claim that they have a normal functional thyrox. But they are, they are very interesting. I have seen reconstructed some of those, and it's better than nothing, I think. I hope that this conversation will be heard by pediatric surgeons and hopefully by pediatric urologists. This should be called a. I approach and most institutions are doing that and these babies should be delivered in places that have that that team of team approach to the problem. Dr. Pena, you mentioned a unified approach to collical atrophy and this multidisciplinary team, but what if there's a pediatric surgeon or a pediatric urologist that really feels comfortable doing this by themselves? Is there a problem with that? In my early experience in the management of clo electrophy is that when the baby was born in a place where there was a prominent pediatric urologist dominating the surgical department and those patients received a lot of attention from the urologic point of view and not very good attention from the gastrointestinal point of view. The same similar something similar happened when the babies were born in a place where the pediatric surgeons were the leaders in charge of the baby, and many times they kind of forgot about the urological concerns of these patients. OK, Dr. Pena, so let's say tomorrow I'm going in to do a clolaal atrophy repair. What are the elements of the case that I need to be preparing for? What am I going to be doing in that case tomorrow? The role of the pediatric surgeon would be to try to close the omphalocele. I say try because the onfalocele may be minimal or may be a giant, and as you know, not all onhaloces can be closed immediately. So to try to close the onphalocele, but the second more important part is to, if you look at the cloic trophy, he should be able to differentiate what is the urothelium, that is the bladder. From intestinal mucosa and separate those structures. The way we do it, we put multiple stitches in the edges that separate the urothelium of the bladder from the bowel and make an incision and separate completely the gastrointestinal tract from the urinary tract. By doing that, then the urologist can bring together both hemibladders, and the urologist can try to close that bladder. Sometimes they can, sometimes they cannot, but he can try to do that. OK, so just to summarize, the pediatric surgeon needs to close the phalocele. They need to separate the bowel from the bladder, and you talk about creating an end colostomy. Can you clarify exactly what you mean and what is the importance of creating an end colostomy? The pediatric surgeon, once she separated all the gastrointestinal tract, Then he has to figure out what what's there because he must be ready, prepared to see the most bizarre type of anatomy that may end up with two or three little pieces of colon, and he should figure out which one is proximal, which one is distal, looking at the mesentery and put everything together and create. an end colostomy, a real end colostomy. The reason why I repeat that is because it is very common for pediatric surgeons all over the world facing one of these cases simply look inside the abdomen, grab a piece of small bowel, and open an ileostomy. That is a very bad thing to do, and the patient will suffer several consequences because of that, because by doing that, He's leaving The all the colon, either small or large colon, and leaving all that distally attached, attached to the urinary tract. Then the urologist, the urologist may be very happy because of that, because he will close the bladder and now he has a natural congenital bladder augmentation. Isn't that beautiful from your point of view? But that has serious consequences because number one, The baby will be absorbing urine from the newborn period, which will give him hyperchloremic acidosis that may interfere with the growth and development of the development of the baby. In addition, those pieces of colon left distally deffunctionalized or attached to the urinary tract will not grow, will remain tiny. And for the colon to grow, for the coronary tissue to grow, it requires the passing of fecal matter through its lumen. So the obligation of the pediatric surgeon is to be sure that all gastrointestinal tissue is incorporated into the gastrointestinal tract and to be sure that the fecal stream will pass through that and open a real end colostomy. We, I say that because we frequently receive patients that receive a colostomy, and I'm sorry, an ileostomy in another place. They are not growing well. They are already 2 or 3 years old, and they have serious problems managed by a nephrologist because of the hyperchloremic acidosis and big losses to the ileostomy. And for that we designed an operation called rescue operation. We have to go there. And rescue the gastrointestinal tissue that belongs to the gastrointestinal tract, incorporate incorporate it in the gastrointestinal tract, and open an end colostomy. By doing that, the acidosis disappears the next day. So the pediatric surgeon is the is the advocate of the baby and is going is going to be there to be sure that all gastrointestinal tract is going to be used as gastrointestinal tract. When the patient received an ileostomy and left dysfunctionalized bowel, I already mentioned the consequences, the negative consequences of that. Then the operation that we offer to those patients consists in taking down the ileostomy, looking for the colonic tissue that is there, closing the ileostomy, and. End to end anastomosis, figure out the anatomy of that of the colon that is diesel and doing an end colostomy. That's a rescue operation separating all the colonic tissue from the neurologic, from the neurogenital tract and and and. And open a rear end colostomy, an ileostomy closure. That's the operation. So when the, when the colon was incorporated into the bladder closure, you, you go and take that down. Yes, you have to go there and And remove that from separate that. Sometimes it's very easy, maybe a little bit more complex, depends on how much manipulation the baby had at birth, but usually it's not, it's not very complicated. It's not a problem. Dr. Pena, before I want to interrupt you for one second. So you said that this is a problem that you've seen. What is the reason that that happens? What's the challenge that surgeons will mistakenly make an ileostomy and how can that be avoided? Are there any tricks for you to advise a surgeon how they may not lead to that problem? So the first problem is that that that most surgeons must accept the idea that they are dealing with the spectrum. And sometimes the, the, the colonic component of the malformation is so complex that they prefer not to, not to even look into that. It's much easier and simply, and a way to a way out is to open an ileostomy and forget about the rest. So, but the surgeon must be prepared to deal every case is going to be different from cases that have a, for instance, pediatric surgeon finds one of those pouches. It's a big sack of colonic tissue with a very bizarre blood supply, and he has to open a stomach and connect that to to try to figure out what's the most distant part of that and open a stomach, a real end colostomy. Do not leave gastrointestinal tract inside the functionalized. That's the main, main thing. OK, so I get the steps of the operation, but some talk about doing a pelvic osteotomy in the initial procedure for pubic approximation. Is this something that needs to be done or should be done at the initial operation or later on? In some institutions, they, the orthopedic surgeons are very active and they want to participate from day one. And they claim that they can try to approximate the pubic bones. That experience comes from from the bladder atrophy patients. Bladder atrophy patients have separated pubic bones, but the separation of the pubic bones and bladder atrophy is not as severe as the separation of the bones in bladder and cloical atrophies. So most of the times, even when the orthopedic surgeons go to the operating room, they want and they do a pelvic osteotomy. Is every time I see a patient years later, the pubic bones are separated, not perhaps not as much as when they were born, but it's very difficult to see a cloical atrophy with the pubic bones completely together. But in some institutions they routinely they do osteotomy, which is OK because it facilitates the reconstruction of the bladder and facilitates the reconstruction of the omphalocele. In your practice, Dr. Pena, what problems have you encountered with the creation of an end colostomy? When, when we open an end colostomy, the babies usually recover well and they grow and develop, and they don't have the big water and salt and sodium losses of an ileostomy. That's very good. But, but frequently that piece of colon that we incorporated. It doesn't have good motility. There is something, and that has not been described well. The fact is that even a beautiful, technically correct colostomy, not a stricture, sometimes doesn't work well. Like the motility of that colon that we incorporated is not good, and under those circumstances, the babies sometimes have behaved like a Like they are, they don't pass too easily and sometimes they have bacterial proliferation similar to what we what we see in disease and then the surgeon has to learn to teach the family how to irrigate the colostomy to try to Because the peristalsis is not good, so you have to irrigate that in the through the stomach like we do in enterocolitis and his spoon through the rectum, you know, we pass a tube and pass small volumes of saline solution and suck to try to get all the fecal material from that stomach, and that's a problem that I have seen in those patients. And then the pediatric surgeon has to wait. Until the time of bowel control, you know that babies below below 3 years of age, they are at home, they are on diapers. That's the only way they know that their life is like that, so they don't care about the diapers. But at some point the parents want to send them to school and usually in the United States that's around 3 years of age and at that time it starts the second part of the management, which is the decision making of how to achieve. Dryness and clean, cleaning to have a clean child in the end with no stool and no no stomach and no urine in the diaper, that's the time for decisions and and and that's what requires a lot of coordination with the urologist, the reason being that From the urologic point of view, these patients have an inadequate bladder, a tiny bladder, same as in bladder atrophy, so most patients will require something called bladder augmentation. And the urologist is going to use gastrointestinal tract to augment the bladder, and the defender of the patient is the of the gastrointestinal tract is the pediatric surgeon. If the patient has, if the patient was born with no colon whatsoever. Then the patient is a candidate for stomach for life. We should never try to pull the terminal ileum down, even if the patient shows some evidence of sphincter in the perineum, because the patients will never have bowel control. So we only consider for put through patients that have the capacity to form solid stool because patients with solid stool. They respond to the bowel management. Patients with clo atrophy. And it's extremely unusual for them to have bowel control, so the overwhelming majority of them will need of our bowel management program, which is, as you know, the administration of enemas to keep the patient clean, but that only works if the patient has solid stool, and the patient only has solid stool if he has enough colon. Can you explain what the importance is of preserving the colon length? So during the newborn period, these pediatric surgeons should not underestimate the capacity to grow of the little pieces of colon. Sometimes they look so tiny that it makes the pediatric surgeon feel like throwing that into the garbage. That should not be done. Should they incorporate everything, Little pieces of colon will grow during those three years, and And then the patient may need a contrast study through the stomach to see the size of the colon, like a retrograde injection of contrast material through the stomach to see how the bowel is growing. We do that every year. And then the question is, if we take that colostomy down as a pull through, as a neo anus, and can we keep the patient clean with bowel management? Rather than taking the risk, what we do is to do something called bowel management program through the stomach, enemas through the stomach, to simulate that the stomach is the new anus and teach the family to give an enema through the stomach. And find by trial and error the enema that is capable of keeping emptying that pouch completely, that colonic pouch, and then see if the patients can stay 24 hours without any stool in the stomach bag. In other words, if you give the enema through the stomach and for 24 hours there's no stool whatsoever, then we may assume. That if we pull that stomach down as a rectum, the bowel management is going to work. That's great. And to clarify, what age do you start trying to do that? Usually at the at the age when the patients and the family start thinking and considering the possibility of of avoiding the enemy. They want to send the. Child to school, so they want to send the child to school clean, hopefully without stoma, usually after 3 years of age, after 3, but, but before making the decision they have to to create a communication with the pediatric urologist because as I was telling you, if the pediatric urologist wants to use bowel for the augmentation. If our patient has very little colon, if the main problem is that it cannot form solid stool, then we don't let the pediatric surgeons to take more gastrointestinal tract. See, if the patient happens to have a lot of colon and a big pouch, we can even share part of that to the bladder augmentation, or If we don't want the pediatric urologist to touch the bowel because the baby's borderline forming solid stool, then he, the urologist, has the option of using stomach for the bladder augmentation, and the and, and this should be coordinated because remember the, the colonic that we are going to pull down was behind. Is the most posterior structure in the pelvis and then in front of that comes the bladder with the bladder augmentation. So you don't, don't let the pediatric urologist do the augmentation before we decide whether or not the patient will need a pull through, because if we decide that the patient needs a pull through after the bladder augmentation, it's going to be a nightmare to go behind all that reconstruction to do the pull through. So, I have a few questions for you. In a patient that comes to you with uh uh liquid stools, not solid stools, and, uh, uh, it, it, it, it's appearing to you that they probably have an ileostomy, how do you. Is that how you gain your suspicion, I guess through the contrast enema, through the stoma enema, how you determine whether or not to go looking to see if in fact, before giving up on them and saying they're going to have a stoma, that there might be a chance that they have colon somewhere in there. Yes, we do a contrast enema because sometimes, as you know, a patient may have what we call paradoxical diarrhea. In other words, with the contrast study, we may find. Fecal impaction, solid stool inside and liquid stool coming around the impaction. So if the patient has very little colon or no colon, the patient will stay with the stomach for life. And and at that point we give free hands to the urologist. He can do whatever he wants, right? OK, because you're, you're now, are you essentially done at that point if you have a patient that's just not a candidate for a pull through, the pediatric surgeon's job is done. That's right, yeah, just take care of the stomach, potential complications of the stomach. That's all. Let's say the patient does appear to be a candidate for a pull through, uh, and let's say they're about 3 years of age now. You're going to be doing a, planning a pull through. How do you, I mean this is such a, the bladder augmentation that may come into play. Do you do them at the same time? Who else is involved in the operation? How do you plan this pull through and who else might be doing simultaneous procedures? Yes, we, we work together, urologists and pediatric surgeons ideally should be done that operation at the same time. Usually it's like a 12 hour affair, you know. And 1st, 1st goes the pediatric surgeon because that goes in the back and the fact that the patient is separated pubic bones make things a little easier. But we take the stoma down and we put it behind the behind the bladder into the pelvis and once we finish that part, then the pediatric with, by the way, with or without the mone because the patient may have two appendixs, one or two appendices, and since we turn the colon down, the appendix stays up in the abdomen and we may do alo to administer the enemas through that. And then once we pull the colon, then the pediatric urologist works in the bladder augmentation and using usually small bowel and And or stomach, they almost never use colon because we need the colon to form solid stool. Occasionally when the patients are born with that giant pouch of colon, it's so large that and has very, very poor motility, but that the fact that his poor motility is a big pouch makes it makes that pouch good for bowel bowel management because, because it doesn't have peristasis, you just Irrigate that once a day and because it doesn't move the patient remains clean in between. Sometimes it's so giant that you can share a little piece with the pediatric neurologist, but that's unusual. Usually they use small bowel and or stomach. So can you talk to me for a second about the approach and the technique of doing the pull through in these patients? I know that you do that before they do the augmentation. How about any advice about exposure, about technique and doing this poulter since it's quite different than the usual? Sure, so we, you know, in colorectal pediatric surgery we have learned to use midline abdominal incisions always. The reason for the midline abdominal incision is because we want to keep the flanks and the iliac, the. the the lower quadrants and the upper quadrants lateral for the stomach for potential stomach, so we enter in the midline with a from the siphoid all the way down to the area of the pubis, open everything, and usually you have to deal with a lot of peritoneal adhesions and then we take the stomach. Down we place multiple silk stitches around the stomach and perform a dissection, circumferential dissection, until the stomach is completely separated from the abdominal wall. Then the next part is really ingenious because you have to, one has to look very carefully as to the type of blood supply that the patient has. Expect we should expect a very bizarre type of blood supply, all kinds of aberrant, abnormal vessels that these patients may have, and we should be very careful because not to ligate a crucial important vessel because we may lose that colon. And see the the the branches of the vessels to be able to manipulate the blood supply, divide some vessels without without damaging the blood supply as to be able to reach the perineum that I cannot describe more except that you know that when you deal with colon or small bowel that you want to mobilize that. You, you are allowed to like get some arcades provided you are looking at the other arcades that will give blood supply to the distant part of the bowel that, but, but that's a relatively easy in colon, normal colon and a small bowel. The problem here is that they sometimes they have a very bizarre type of blood supply, but if you, if you, if we are careful and observe and study the blood supply. We can make a decision as to which vessels we can ligate and which patients, which vessels we cannot ligate. Sometimes it reaches down without ligating any vessels because that's the anatomy of the patient, and then we just put it in the back. We have to create the space behind the bladder which is easy, it's not a problem, and put the bowel and then these patients don't have to go into prone position. Because they have the atrophy makes everything anterior, so you, we just work anteriorly with a kind of leg frog position and by doing that you have access to the entire perineum of the patient, so you don't have to be usually the sphincters in these patients are poor, so everything is done anteriorly, OK. Yeah, I was wondering about whether you did them prone when you did at the end, but you don't. You keep, no, there is no posterior side in these patients because everything is anterior. If you put the patient prone, you, you cannot see exactly you are limited because of the entire. Everything is anterior. Everything is the pubic bones are separated, so the, the coccyx is very much, so it's much more comfortable to work in a supine position with the knees a little separated like a leg frog. You have access to the entire cranium, OK. That's great. That was a great summary for me. All right, Dr. Pena, the mom says to you, Let's take two patients, one that you were able to do a pull through and one not. The mom says, Can you tell me what's the prognosis of my child? What's their quality of life going to be like, and how often do you want to see them and for how long do you want to follow them? Yes, the The patients will have problems. They will be patients for life because of orthopedic problems, and sometimes they, I have seen teenagers that are unhappy about the fact that the pubic bones remain separated. And some ambitious dedicated orthopedic surgeons have been able to bring the pubic bone much closer because when you have the, when a person has the pubic bone separated, then we walk like with the with the with the feet kind of separated pointing lateral. It looks kind of ugly and that's, that's they complain about that. All the patients that have severe spinal problems, they have to keep being followed by pediatric orthopedics and by a neurosurgeon because they have tested cord. Sometimes they need a cord release for the test cord, and they have to be followed by the neurosurgeon. Can you talk to me about the female reproduction in the patient with cloacal atrophy gynecologically speaking. And when we do the the pull through, we try to approximate the vaginas as much as possible. Remember that everything is a spectrum, so the degree of separation of the vaginas could be minimal. The vaginas are together with the vaginal septum, only separating a little bit one from the other in the upper part. In those cases, we remove the septum in between as high as possible. But some of the cases are the vaginas run in completely different directions, and you cannot bring them together because because of the blood supply prevents us from doing that. And sometimes we elect to remove one of the vaginas and leave another one, the one that has a better looking cervix, and, and that you make that, we make that decision at the time of the put through because You don't want to, we don't want to be there all the time because of the peritoneal adhesions. So these, these patients, if they have functional human uterus, may become pregnant, but it's a high risk pregnancy, might be followed by a specialized pediatric gynecologist. In general, we advise not to become pregnant, but Some, some of the patients want to be pregnant, and that's a problem because the abdomen is very limited in space and but but they may deliver by cesarean section. In addition, hemi uterus has a great tendency to produce miscarriages and premature labors. They should know that from the beginning and And then from the, from the urologic point of view, these patients will need a bladder augmentation and a mitrofenov, a piece of bowel could be the appendix, a second appendix, or part sometimes the patient has a very long appendix. We use half for the urologist, half for us, and with the appendix they make a conduit for catheterization to empty the bladder intermittently. But of course that's not free of complications because an augmented bladder, the bowel that we use produces a lot of mucus, and if the mucus stays there, it forms stones. So the family, we need a good team of nurses in charge of teaching the family how to keep the bladder clean, not only to empty the bladder, but to remove the mucus, irrigation to remove the mucus and avoid infections and teach the family how to do irrigations with sometimes, sometimes with gentamicin. To be sure that all the mucus that is produced comes out, should not be left there and needs a follow up from the urologic point of view to be sure that the patient, the kidneys are not suffering because the patient may have reflux and that should be followed carefully. Sometimes the metrofenil stops working or it leaks urine and needs to redo or. And tightening of the valve of the microfanov, the same as it happens in the Malone. So these are patients for life and one thing that we are Seeing now is the problem of moving toward adult life. When these patients go to adult hospitals, they don't feel well. Because the adult urologists, for instance, or adult orthopedic surgeons, not all of them have experience in these malformations, so the patients feel desolated, and we try to follow them as much as possible because, because they feel more confident in a pediatric environment even when they are adults. So what is interesting about this is a very personal observation. Patients with cloic atrophy when they grow up are particularly charming and intelligent. I don't know, it's just my feeling, but I'm very impressed by how charismatic, how intelligent, how beautiful are these patients when they grow up. And um and some of them are they they they. Manage their problem and they still have more energy to help others to manage their own problems. So now with the pull through network, the patients are getting together and talking about their problems and so forth. What is, can you clarify the pull through network? Yes, a Pull Through Network is a national organization that we were there at the beginning when it was created. And something similar to all the organizations that are done like cystic fibrosis, heart fund, and breast cancer, and so forth. So a group of parents of children born with anorectal malformations or history disease or patients that have problems with bowel and urinary control, they created, they got together to create this organization called Pull Through Network. And now it grew. It's great because they have over 1000 members now. They meet every year. They have their own meeting and they invite doctors to give, to give talks to the patients. They are very enthusiastic. And and that's very good because they, they become powerful. you see the colorectal problems I believe colorectal and eurogenital problems have been um. Left behind in terms of scientific approach and funds allocated for research and all that because they are not elegant problems. They are problems related with stool, with urine, with sex, and institutions are not crazy about receiving those patients. So we believe that those problems did not receive enough attention, you know, funds for research and And work and they need a lot of help and people don't talk much about that because they are considered kind of secret problems and therefore the society doesn't know much about that and actually there are many, many patients suffering from this. Well, the network's great and it's certainly something that I'm going to tell my patients about, so I'm glad you brought that up. I just, I want to clarify and make sure I understand this in summary. So the initial operation, the pediatric surgeon's going to close the hail, going to create a colostomy, and at the same time, the urologists are going to close the bladder, um. What else happens during the initial operation? Is there any other? Yes, the, you know, pharmacy is closed. The bladder, the, the, the bowel is separated from the urinary tract. An end colostom is created, and the urologist will try to close the bladder. Sometimes they cannot. They just approximate a little bit and then they have other stages to keep closing, trying to close the bladder. But the function of the pediatric surgeon is to, to, to try to close the umphalocele. Sometimes it requires another stage to completely close the umphphalocele. OK. And the orthopedic surgeons, and the orthopedic surgeons in some institutions, they go ahead and do an osteotomy. At the beginning of the procedure, other institutions, they prefer to wait 2 or 3 months and do the osteotomy alone without our participation, OK. And dealing with the, uh, so then, then the next operation's usually going to be about 3 years of age after once the bladder's closed, and then you're talking about doing the pull through, doing the bladder augmentation, the vaginal reconstruction if you're going to do that. Is that right? Yes, before, before that, before considering the put through stairs, there may be stomach problems, you know, functional stomach, the function of the stomach, the, the, as I said, the colon that we, the colostomy that we Open we open a colostomy in a very poor motility type of colon, so be prepared to do irrigation through that stomach to help with the poor peristalsis of the patient. That's, that's a great, yeah, element that's in the middle there between before pull through. Well, actually, one last question for you. What, where are we as far as prenatal diagnosis of this condition? Yes, it's that's, that's a very interesting, good question, because as you know, we are making big progresses in prenatal diagnosis with the imaging technology that is used and fortunately the the diagnosis of anorectal and neurogenital malformation. In utero is easier in the most complex defects. In other words, we cannot claim that we can diagnose a rectal perineal fistula, a simple type of anorectal malformation is difficult to detect. Number 1, because the imaging procedures also have the necessary definition. Number 2, because those patients with benign malformations don't have. Other associated defects, whereas when you get into the complex type of malformations, usually they have spinal problems and the spine, you can see it very early in utero. Usually they have no bladder. You don't see the bladder in utero because it's atrophic and therefore even from week 20 in the pregnancy, if you see no bladder, that's a bad sign. So yes, the answer is yes, we can detect. These malformations reasonable early but keep in mind that it's a spectrum, so we cannot claim that we can make a very accurate diagnosis in all of them, but yes, I would say that it's in this malformation that we can make the diagnosis earlier and that. will allow the family number one to make a decision about Interruption of the pregnancy beyond ethical or religious concerns that that's an alternative for the parents. And second is the fact that if they decide to continue with the pregnancy, they can take the, they can have the delivery. Done in a place where there is a team of people working with this malformation, with this type of malformation, you know, as we progress in science and technology and medicine and surgery, it's becoming evident that for certain type of malformations, it requires centers of excellence where people are dedicated. Focus into these kind of problems because otherwise if we try to train every pediatric surgeon and every pediatric neurologist in the management in the full management of this, after 20 years there will be a lot of children, damaged children, and nobody will be trained unless we concentrate the cases in a place where there are people that are willing to sacrifice other things. Because once you decided to get become an expert in something, And focus into something, then you are giving up other, other areas very interesting pediatric surgeons. If we become colorectal pedia surgeons, I will not be doing esophagus that I love to do, you know. Yeah, yeah, that's a great point actually. And uh and and speaking of that sacrifice, thankfully you made that sacrifice and you're able to. Uh, teach us, uh, what we need to know and, and probably the most important thing is when to refer when we've are over our heads and, and what's going on. Um, thank you, Dr. Pena. I, uh, before we finish up, I wanted to ask you if there was anything else that you thought we missed or we should be discussing about collical atrophy. No, I think, um, I think, um, the only thing that I miss is the, the imaging that we could, it would be beautiful to show some, some, um, but that would be another program, right? Another one of your where we could show really diagrams and illustrations and. But that in a little commercial, our book is finished and there's a chapter on cloakical atrophy that has great illustrations. Tell me about your book, Dr. The book is published by Springer, Springer from Germany, but it's already, it's already available. And right now in the electronic version and in a few days will be the real book, but the book is done, it's finished. I know you showed me a preview of this a couple of years ago, and I was blown away by some of the graphics that you showed me, so I cannot wait to see the book. Yeah, the book has 800 illustrations, so it's mainly. We hope you enjoyed this episode of Stay Current in Pediatric Surgery. You can listen, watch, or read all content by downloading the Stay Current and Surgery app. Please send questions or comments to us at staycurrent podcast@gmail.com. We'll see you next time.

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