In the real life and we don't make a very accurate diagnosis of the of the intrinsic anatomy of the cloaca during the newborn period and in fact trying to be very precise in the anatomic diagnosis is kind of useless and may actually hurt the baby, but we are very interested in a newborn babies to know if the baby has hydronephrosis and mega ureters and to know if the patient has a hydrocorpus that may be compressing the ureters.
The surgeons should be careful not to stretch too much because you start the dissection and you forget you want to be comfortable. You put retractors and I walk into the operating room and see sometimes a big hole that what used to be the anus becomes a huge hole and that is damaging by definition the sphincter mechanism.
If you take a child that has been traumatized with rectal maneuvers and you try to do rectal irrigations, it's going to be a lot of problems, whereas if a patient has an ileostomy and the mother does rectal irrigations once in a while with a soft catheter, the baby will accept that.
Enemas consist in giving a certain amount of fluid with the, with the, with the ingredients that are irritant to the bowels that provoke a bowel contraction, but the, the disease consists in not having the normal peristalsis therefore are not indicated.
You are at that point it's not so crucial to know if the baby has a reflux or not. It's not, it's what you want is the patient to, you want to be able to decompress the gastrointestinal tract and to decompress the urogenital tract.
I don't pay attention to that and I don't perform myectomies and I don't recommend those myectomies and myotomies and buttock and those concepts because we don't know where where you inject. What area of the muscle, nobody knows
StatementCloacal exstrophy is a spectrum of congenital malformations affecting the gastrointestinal/colorectal area, urogenital tract, spine and cord, and sometimes lower extremity motion.Open
StatementCloacal exstrophy is a spectrum of congenital malformations affecting the gastrointestinal/colorectal area, urogenital tract, spine and cord, and sometimes lower extremity motion.Open
StatementBabies with cloacal exstrophy are born with an omphalocele, bladder exstrophy (two separated hemibladders), open cecum between the hemibladders, and separated pubic bones.Open
StatementBabies with cloacal exstrophy are born with an omphalocele, bladder exstrophy (two separated hemibladders), open cecum between the hemibladders, and separated pubic bones.Open
StatementThe small bowel can become exstrophic through the ileocecal valve, creating an 'elephant trunk' appearance.Open
StatementThe small bowel can become exstrophic through the ileocecal valve, creating an 'elephant trunk' appearance.Open
StatementMale patients have two separated hemiphalluses with normal gonads; female patients have two hemivaginas below the exstrophic bladder leading to two hemiuteri.Open
StatementMale patients have two separated hemiphalluses with normal gonads; female patients have two hemivaginas below the exstrophic bladder leading to two hemiuteri.Open
StatementCloacal 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.Open
StatementCloacal 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.Open
StatementThe amount of colon present at birth has very important implications for the patient's management and outcomes.Open
StatementThe amount of colon present at birth has very important implications for the patient's management and outcomes.Open
StatementA 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.Open
StatementA 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.Open
StatementWhile 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.Open
StatementWhile 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.Open
StatementHistorical practice was to perform bilateral gonadectomy, remove hemiphalluses, create a vagina with bowel, and assign female gender to XY patients with cloacal exstrophy.Open
StatementHistorical practice was to perform bilateral gonadectomy, remove hemiphalluses, create a vagina with bowel, and assign female gender to XY patients with cloacal exstrophy.Open
StatementPatients 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.Open
StatementPatients 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.Open
StatementPatients 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.Open
Quotationwho 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.Open
StatementPatients 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.Open
Quotationbeing a male is much more than having a phallusOpen
StatementCurrent consensus is that XY patients should be raised as male, with pediatric urologists and plastic surgeons working on phallus reconstruction techniques.Open
StatementCurrent consensus is that XY patients should be raised as male, with pediatric urologists and plastic surgeons working on phallus reconstruction techniques.Open
StatementWhen a prominent pediatric urologist dominates management, patients receive good urologic attention but inadequate gastrointestinal care; the reverse occurs when pediatric surgeons dominate.Open
StatementWhen a prominent pediatric urologist dominates management, patients receive good urologic attention but inadequate gastrointestinal care; the reverse occurs when pediatric surgeons dominate.Open
StatementThe 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.Open
StatementThe 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.Open
StatementIt is very common but very harmful for pediatric surgeons to simply create an ileostomy, leaving all colon distally attached to the urinary tract.Open
StatementIt is very common but very harmful for pediatric surgeons to simply create an ileostomy, leaving all colon distally attached to the urinary tract.Open
QuotationThat is a very bad thing to do, and the patient will suffer several consequences because of thatOpen
QuotationThat is a very bad thing to do, and the patient will suffer several consequences because of thatOpen
StatementLeaving colon attached to the urinary tract creates a congenital bladder augmentation that causes hyperchloremic acidosis from urine absorption, interfering with growth and development.Open
StatementLeaving colon attached to the urinary tract creates a congenital bladder augmentation that causes hyperchloremic acidosis from urine absorption, interfering with growth and development.Open
QuotationIsn't that beautiful from your point of view?Open
QuotationIsn't that beautiful from your point of view?Open
StatementDefunctionalized colonic tissue left distally will not grow; colon requires passage of fecal matter through its lumen to grow.Open
StatementDefunctionalized colonic tissue left distally will not grow; colon requires passage of fecal matter through its lumen to grow.Open
StatementThe 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.Open
StatementThe 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.Open
StatementPatients who received ileostomy with defunctionalized colon present at 2-3 years with poor growth, hyperchloremic acidosis managed by nephrologists, and large ileostomy losses.Open
StatementPatients who received ileostomy with defunctionalized colon present at 2-3 years with poor growth, hyperchloremic acidosis managed by nephrologists, and large ileostomy losses.Open
StatementThe 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.Open
StatementThe 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.Open
StatementSurgeons 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.Open
StatementSurgeons 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.Open
QuotationDo not leave gastrointestinal tract inside the functionalized. That's the main, main thing.Open
QuotationDo not leave gastrointestinal tract inside the functionalized. That's the main, main thing.Open
StatementSome 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.Open
StatementSome 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.Open
StatementEven after osteotomy, it is very difficult to see a cloacal exstrophy patient with pubic bones completely together; they usually remain separated.Open
StatementEven after osteotomy, it is very difficult to see a cloacal exstrophy patient with pubic bones completely together; they usually remain separated.Open
StatementEven a technically correct end colostomy often has poor motility, and babies may not pass stool easily, sometimes developing bacterial overgrowth similar to Hirschsprung disease.Open
StatementEven a technically correct end colostomy often has poor motility, and babies may not pass stool easily, sometimes developing bacterial overgrowth similar to Hirschsprung disease.Open
StatementFamilies must be taught to irrigate the colostomy with small volumes of saline through a tube to evacuate fecal material when peristalsis is inadequate.Open
StatementFamilies must be taught to irrigate the colostomy with small volumes of saline through a tube to evacuate fecal material when peristalsis is inadequate.Open
StatementDecision-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).Open
StatementDecision-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).Open
StatementMost cloacal exstrophy patients have an inadequate, tiny bladder requiring bladder augmentation with gastrointestinal tract, necessitating coordination between pediatric surgery and urology.Open
StatementMost cloacal exstrophy patients have an inadequate, tiny bladder requiring bladder augmentation with gastrointestinal tract, necessitating coordination between pediatric surgery and urology.Open
StatementPatients 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.Open
StatementPatients 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.Open
StatementPull-through is only considered for patients with capacity to form solid stool (adequate colon), as bowel management only works with solid stool.Open
StatementPull-through is only considered for patients with capacity to form solid stool (adequate colon), as bowel management only works with solid stool.Open
StatementIt 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).Open
StatementIt 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).Open
StatementPediatric surgeons should not underestimate the growth capacity of tiny colonic pieces during the newborn period; even small segments will grow over three years if fecal stream passes through them.Open
StatementPediatric surgeons should not underestimate the growth capacity of tiny colonic pieces during the newborn period; even small segments will grow over three years if fecal stream passes through them.Open
StatementAnnual contrast studies through the colostomy (retrograde injection) are performed to assess colonic growth.Open
StatementAnnual contrast studies through the colostomy (retrograde injection) are performed to assess colonic growth.Open
StatementBefore committing to pull-through, a trial bowel management program is performed through the colostomy: enemas are given to empty the colonic pouch, and if the patient stays 24 hours without stool in the colostomy bag, pull-through is likely to succeed.Open
StatementBefore committing to pull-through, a trial bowel management program is performed through the colostomy: enemas are given to empty the colonic pouch, and if the patient stays 24 hours without stool in the colostomy bag, pull-through is likely to succeed.Open
StatementBowel management trial through colostomy is typically started after age 3 when families consider avoiding the stoma for school.Open
StatementBowel management trial through colostomy is typically started after age 3 when families consider avoiding the stoma for school.Open
StatementIf a patient has very little colon and cannot form solid stool, the urologist is free to use bowel for bladder augmentation; if the patient has borderline colon, the urologist must use stomach for augmentation to preserve bowel for fecal function.Open
StatementIf a patient has very little colon and cannot form solid stool, the urologist is free to use bowel for bladder augmentation; if the patient has borderline colon, the urologist must use stomach for augmentation to preserve bowel for fecal function.Open
StatementThe colon to be pulled through is the most posterior structure in the pelvis, with the bladder and augmentation anterior to it; therefore, bladder augmentation must not be done before deciding on pull-through, or accessing the colon will be extremely difficult.Open
StatementThe colon to be pulled through is the most posterior structure in the pelvis, with the bladder and augmentation anterior to it; therefore, bladder augmentation must not be done before deciding on pull-through, or accessing the colon will be extremely difficult.Open
StatementContrast enema through the colostomy can distinguish true liquid stool from paradoxical diarrhea (liquid stool around solid fecal impaction).Open
StatementContrast enema through the colostomy can distinguish true liquid stool from paradoxical diarrhea (liquid stool around solid fecal impaction).Open
StatementPull-through and bladder augmentation are ideally performed together in a single operation lasting approximately 12 hours, with pediatric surgery going first (posterior dissection) followed by urology (anterior augmentation).Open
StatementPull-through and bladder augmentation are ideally performed together in a single operation lasting approximately 12 hours, with pediatric surgery going first (posterior dissection) followed by urology (anterior augmentation).Open
StatementDuring pull-through, if the patient has one or two appendices, a Malone appendicostomy can be created for antegrade enema administration, as the appendix remains in the abdomen when colon is pulled down.Open
StatementDuring pull-through, if the patient has one or two appendices, a Malone appendicostomy can be created for antegrade enema administration, as the appendix remains in the abdomen when colon is pulled down.Open
StatementUrologists almost never use colon for bladder augmentation in cloacal exstrophy because colon is needed to form solid stool; they typically use small bowel or stomach.Open
StatementUrologists almost never use colon for bladder augmentation in cloacal exstrophy because colon is needed to form solid stool; they typically use small bowel or stomach.Open
StatementOccasionally, when a patient has a giant colonic pouch with very poor motility, the poor motility makes it good for bowel management (irrigate once daily, stays clean between irrigations), and a piece can be shared with urology for augmentation.Open
StatementOccasionally, when a patient has a giant colonic pouch with very poor motility, the poor motility makes it good for bowel management (irrigate once daily, stays clean between irrigations), and a piece can be shared with urology for augmentation.Open
StatementMidline abdominal incision from xiphoid to pubis is used for pull-through to preserve the flanks and quadrants for potential future stomas.Open
StatementMidline abdominal incision from xiphoid to pubis is used for pull-through to preserve the flanks and quadrants for potential future stomas.Open
StatementThe colostomy is circumferentially dissected and separated from the abdominal wall, then the blood supply is carefully studied because cloacal exstrophy patients have very bizarre, aberrant vascular anatomy.Open
StatementThe colostomy is circumferentially dissected and separated from the abdominal wall, then the blood supply is carefully studied because cloacal exstrophy patients have very bizarre, aberrant vascular anatomy.Open
StatementCareful observation of the vascular anatomy allows the surgeon to decide which vessels can be ligated to mobilize the colon to the perineum without compromising blood supply; sometimes no vessels need to be ligated.Open
StatementCareful observation of the vascular anatomy allows the surgeon to decide which vessels can be ligated to mobilize the colon to the perineum without compromising blood supply; sometimes no vessels need to be ligated.Open
StatementThe space behind the bladder is easily created, and the bowel is placed posteriorly; patients do not need prone positioning because the exstrophy makes everything anterior, so a supine frog-leg position provides full perineal access.Open
StatementThe space behind the bladder is easily created, and the bowel is placed posteriorly; patients do not need prone positioning because the exstrophy makes everything anterior, so a supine frog-leg position provides full perineal access.Open
StatementCloacal exstrophy patients are lifelong patients due to orthopedic problems (separated pubic bones causing abnormal gait) and spinal problems (tethered cord requiring neurosurgical follow-up and potential cord release).Open
StatementCloacal exstrophy patients are lifelong patients due to orthopedic problems (separated pubic bones causing abnormal gait) and spinal problems (tethered cord requiring neurosurgical follow-up and potential cord release).Open
StatementSome teenagers are unhappy about separated pubic bones causing abnormal gait (feet pointing laterally); some dedicated orthopedic surgeons have been able to bring pubic bones closer together.Open
StatementSome teenagers are unhappy about separated pubic bones causing abnormal gait (feet pointing laterally); some dedicated orthopedic surgeons have been able to bring pubic bones closer together.Open
StatementDuring pull-through, vaginal reconstruction is attempted by approximating the hemivaginas as much as possible; the degree of separation varies on the spectrum.Open
StatementDuring pull-through, vaginal reconstruction is attempted by approximating the hemivaginas as much as possible; the degree of separation varies on the spectrum.Open
StatementWhen hemivaginas are close with only a septum separating them superiorly, the septum is removed as high as possible; when vaginas run in completely different directions, one hemivagina may be removed, leaving the one with the better-looking cervix.Open
StatementWhen hemivaginas are close with only a septum separating them superiorly, the septum is removed as high as possible; when vaginas run in completely different directions, one hemivagina may be removed, leaving the one with the better-looking cervix.Open
StatementPatients with functional hemiuterus may become pregnant, but it is high-risk pregnancy requiring specialized pediatric gynecology follow-up; in general, pregnancy is not advised.Open
StatementPatients with functional hemiuterus may become pregnant, but it is high-risk pregnancy requiring specialized pediatric gynecology follow-up; in general, pregnancy is not advised.Open
StatementHemiuterus has a great tendency to produce miscarriages and premature labor; delivery must be by cesarean section due to limited abdominal space.Open
StatementHemiuterus has a great tendency to produce miscarriages and premature labor; delivery must be by cesarean section due to limited abdominal space.Open
StatementBladder augmentation requires a Mitrofanoff conduit (appendix or part of a long appendix, sometimes shared half for urology and half for Malone) for intermittent catheterization to empty the bladder.Open
StatementBladder augmentation requires a Mitrofanoff conduit (appendix or part of a long appendix, sometimes shared half for urology and half for Malone) for intermittent catheterization to empty the bladder.Open
StatementAugmented bladders produce large amounts of mucus; if mucus is not removed, it forms stones, so families must be taught to irrigate the bladder (not just empty it) to remove mucus and prevent infections.Open
StatementAugmented bladders produce large amounts of mucus; if mucus is not removed, it forms stones, so families must be taught to irrigate the bladder (not just empty it) to remove mucus and prevent infections.Open
StatementBladder irrigation is sometimes performed with gentamicin to ensure all mucus is removed.Open
StatementBladder irrigation is sometimes performed with gentamicin to ensure all mucus is removed.Open
StatementLifelong urologic follow-up is needed to monitor for reflux and kidney damage; the Mitrofanoff may stop working or leak urine, requiring revision or valve tightening.Open
StatementLifelong urologic follow-up is needed to monitor for reflux and kidney damage; the Mitrofanoff may stop working or leak urine, requiring revision or valve tightening.Open
StatementPatients transitioning to adult hospitals often feel uncomfortable because adult urologists and orthopedic surgeons lack experience with these congenital malformations; patients prefer to remain in pediatric environments even as adults.Open
StatementPatients transitioning to adult hospitals often feel uncomfortable because adult urologists and orthopedic surgeons lack experience with these congenital malformations; patients prefer to remain in pediatric environments even as adults.Open
StatementDr. Peña observes that cloacal exstrophy patients are particularly charming, intelligent, beautiful, and charismatic when they grow up, and some have energy to help others manage their own problems.Open
StatementDr. Peña observes that cloacal exstrophy patients are particularly charming, intelligent, beautiful, and charismatic when they grow up, and some have energy to help others manage their own problems.Open
StatementThe Pull-Through Network is a national organization (similar to cystic fibrosis or breast cancer organizations) for parents and patients with anorectal malformations, Hirschsprung disease, or bowel/urinary control problems; it has over 1000 members, holds annual meetings, and invites doctors to give talks.Open
StatementThe Pull-Through Network is a national organization (similar to cystic fibrosis or breast cancer organizations) for parents and patients with anorectal malformations, Hirschsprung disease, or bowel/urinary control problems; it has over 1000 members, holds annual meetings, and invites doctors to give talks.Open
StatementColorectal and urogenital problems have been left behind in terms of scientific approach and research funding because they are not 'elegant' problems—they involve stool, urine, and sex—and institutions are not eager to receive these patients.Open
StatementColorectal and urogenital problems have been left behind in terms of scientific approach and research funding because they are not 'elegant' problems—they involve stool, urine, and sex—and institutions are not eager to receive these patients.Open
StatementThe initial operation includes omphalocele closure (sometimes requiring staged closure), bowel-bladder separation, end colostomy creation, bladder closure attempt (sometimes requiring multiple stages), and in some institutions, pelvic osteotomy (either at initial operation or 2-3 months later).Open
StatementThe initial operation includes omphalocele closure (sometimes requiring staged closure), bowel-bladder separation, end colostomy creation, bladder closure attempt (sometimes requiring multiple stages), and in some institutions, pelvic osteotomy (either at initial operation or 2-3 months later).Open
StatementBetween the initial operation and pull-through, colostomy irrigation is often needed to manage poor colonic motility.Open
StatementBetween the initial operation and pull-through, colostomy irrigation is often needed to manage poor colonic motility.Open
StatementPrenatal diagnosis of anorectal and urogenital malformations is easier for complex defects (like cloacal exstrophy) than simple defects because complex cases have associated findings (spinal problems, absent bladder) visible on imaging.Open
StatementPrenatal diagnosis of anorectal and urogenital malformations is easier for complex defects (like cloacal exstrophy) than simple defects because complex cases have associated findings (spinal problems, absent bladder) visible on imaging.Open
StatementAbsent bladder on prenatal ultrasound (due to exstrophy) is a bad sign and can be detected as early as week 20 of pregnancy.Open
StatementAbsent bladder on prenatal ultrasound (due to exstrophy) is a bad sign and can be detected as early as week 20 of pregnancy.Open
StatementPrenatal diagnosis allows families to decide about pregnancy continuation and, if continuing, to deliver at a center with a multidisciplinary team experienced in these malformations.Open
StatementPrenatal diagnosis allows families to decide about pregnancy continuation and, if continuing, to deliver at a center with a multidisciplinary team experienced in these malformations.Open
StatementCertain malformations require centers of excellence where surgeons focus and sacrifice other areas of practice; attempting to train all surgeons superficially in complex conditions results in damaged children and no one becoming truly trained.Open
StatementCertain malformations require centers of excellence where surgeons focus and sacrifice other areas of practice; attempting to train all surgeons superficially in complex conditions results in damaged children and no one becoming truly trained.Open
Alberto's statements about Anorectal Malformation4 statements
QuotationNot everybody gets the privilege to get a specialized radiologist is dedicated to the prenatal diagnosis of this malformation that we have.Open
StatementDr. Peña reviewed mothers of babies born with cloacas who did not have prenatal diagnosis; when he obtained their ultrasounds, many had abnormalities but radiologists misdiagnosed them as urethrocele, double bladder, ovarian cysts, or bladder diverticulum instead of recognizing hydrocolposOpen
QuotationThe images were there. What failed was the index of suspicion in the those radiologist.Open
StatementIf a female fetus has a prenatal diagnosis of ovarian cyst, double bladder, or urethrocele, suspect the patient may have a cloaca; if the patient also has abnormal vertebrae, hydronephrosis, and dilated bowel, that confirms the diagnosisOpen
Alberto's statements about Anorectal Malformations & Cloacal Reconstruction8 statements
Tell Me a Story: Pediatric Surgeons Putting Colorectal Center on the Map
QuotationRevealing episodes in your life, you read a book, you meet somebody, you, you hear some music, you see some painting, you see an operation that's, and then you say, I want this, and that changes your life.Open
StatementDr. Alberto Peña is the director of the Colorectal Center at Cincinnati Children's Hospital.Open
QuotationI'm the director of the Colorectal Center.Open
StatementDr. Peña advises young surgeons asking about the key to success to focus on doing the best version of whatever operation they are performing, even routine procedures like hernias.Open
QuotationMany surgeons come and many young surgeons say, what should I do? What's the key for success? And I said, What are you doing tomorrow? I said, they said, I'm doing a hernia, which is a kind of a simple operation.Open
QuotationAnd my answer is, why don't you? To think overnight and say tomorrow I'm going to do the best hernia in the world.Open
QuotationAnd they look at me like saying this man didn't understand the question and he's crazy, and I look at them and I said they didn't understand my answer.Open
QuotationYou just concentrate on what you do and let the rest of the gods. That's all that I can say.Open
StatementThe measurements from 3D cloacography are accurate to within millimeters; when the radiologist reports 3.5 cm, the intraoperative measurement is typically 3.5 cm.Open
QuotationI was very skeptical about their measurements, but then little by little they become more and more accurate, and now they are, they are just there, but the differences in millimeters very subjective, so they are. I don't know what they did, but right now when they say 3.5 3.5 centimeters, usually it's 3.5 centimeters, so we really, we gain a lot, you gain a lot of experience for the benefit of these children.Open
StatementIn neonatal cloacas, trying to make a very accurate anatomic diagnosis during the newborn period is useless and may actually hurt the baby.Open
QuotationIn the real life and we don't make a very accurate diagnosis of the of the intrinsic anatomy of the cloaca during the newborn period and in fact trying to be very precise in the anatomic diagnosis is kind of useless and may actually hurt the baby, but we are very interested in a newborn babies to know if the baby has hydronephrosis and mega ureters and to know if the patient has a hydrocorpus that may be compressing the ureters.Open
StatementIn the neonatal period, the crucial information is whether the baby has hydronephrosis, megaureters, or hydrocolpos compressing the ureters, which can be determined by ultrasound without endoscopy.Open
StatementKnowing whether a neonate with cloaca has reflux is not crucial; the priority is decompressing the gastrointestinal and urogenital tracts.Open
QuotationYou are at that point it's not so crucial to know if the baby has a reflux or not. It's not, it's what you want is the patient to, you want to be able to decompress the gastrointestinal tract and to decompress the urogenital tract.Open
QuotationNot everybody has the capacity to do it and don't force the endoscope in a tiny structure because you may end up hurting.Open
StatementForcing an endoscope into a tiny neonatal cloacal structure can cause harm.Open
QuotationMany urologists don't have experience in cloacas and will end up doing nephrostom is not indicated, ureterostom is not indicated, and vesicostom is not indicated.Open
StatementNephrostomy, ureterostomy, and vesicostomy are not indicated in most neonatal cloacas.Open
StatementThe recommended neonatal management for cloaca is to drain hydrocolpos, open a colostomy, and re-evaluate from the urologic point of view 48 hours later before making further decisions.Open
QuotationOur specific recommendation is to drain the hydrocorpus, to open a colostomy, and re-evaluate the patient from the urologic point of view 48 hours later and then make a decision.Open
StatementSome cloacal patients may need vesicostomy if there is obstruction in the common channel, but many do not; decompressing the hydrocolpos often completely changes the clinical picture.Open
QuotationBy by simply decompressing the hydrocorpus, the, the picture completely changes.Open
Cloaca - Prental Imaging & Diagnosis - Counseling
QuotationNot everybody gets the privilege to get a specialized radiologist is dedicated to the prenatal diagnosis of this malformation that we have.Open
StatementDr. Peña reviewed mothers of babies born with cloacas who did not have prenatal diagnosis; when he obtained their ultrasounds, many had abnormalities but radiologists misdiagnosed them as urethrocele, double bladder, ovarian cysts, or bladder diverticulum instead of recognizing hydrocolposOpen
QuotationThe images were there. What failed was the index of suspicion in the those radiologist.Open
StatementIf a female fetus has a prenatal diagnosis of ovarian cyst, double bladder, or urethrocele, suspect the patient may have a cloaca; if the patient also has abnormal vertebrae, hydronephrosis, and dilated bowel, that confirms the diagnosisOpen
Alberto's statements about Cloacal Exstrophy115 statements
StatementCloacal 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.Open
StatementThe amount of colon a patient with cloacal exstrophy is born with has very important repercussions and implications for management and outcomes.Open
StatementThere 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.Open
StatementWhile 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.Open
StatementHistorical 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.Open
StatementWhen 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.Open
StatementCurrent consensus is that patients with XY chromosomes should be raised as male, with ongoing efforts in phallic reconstruction, though functional outcomes remain uncertain.Open
StatementWhen 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.Open
StatementAt 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.Open
StatementIt 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.Open
StatementCreating 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.Open
StatementColonic tissue left defunctionalized or attached to the urinary tract will not grow; colon requires the passage of fecal matter through its lumen to grow.Open
StatementThe '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.Open
StatementSurgeons 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.Open
StatementThe 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.Open
StatementSome 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.Open
StatementEven 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.Open
StatementPull-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.Open
StatementIt is extremely unusual for patients with cloacal exstrophy to have voluntary bowel control; the overwhelming majority will need bowel management programs.Open
StatementBowel management only works if the patient has solid stool, and solid stool only occurs if the patient has enough colon.Open
StatementSmall 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.Open
StatementAnnual contrast studies through the colostomy are performed to assess colonic growth during the first three years.Open
StatementBefore 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.Open
StatementCoordination 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.Open
StatementThe 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.Open
StatementPatients 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.Open
StatementThe 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.Open
StatementThe pediatric surgeon operates first because the colon goes posteriorly, and separated pubic bones make the dissection somewhat easier.Open
StatementA 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.Open
StatementUrologists 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.Open
StatementIn colorectal pediatric surgery, midline abdominal incisions are always used to preserve the flanks, iliac fossae, and lateral quadrants for potential future stomas.Open
StatementOccasionally 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.Open
StatementThe 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.Open
StatementPatients 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.Open
StatementPatients 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.Open
StatementPatients with severe spinal problems require lifelong follow-up by pediatric orthopedics and neurosurgery for tethered cord, sometimes requiring cord release.Open
StatementDuring 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.Open
StatementPatients 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.Open
StatementMost patients will require bladder augmentation and Mitrofanoff (using appendix or piece of bowel) for intermittent catheterization to empty the bladder.Open
StatementAugmented 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.Open
StatementUrologic 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.Open
StatementPatients 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.Open
StatementPatients 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.Open
StatementColorectal 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.Open
StatementPrenatal 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.Open
StatementSimple anorectal malformations like rectoperineal fistula are difficult to detect prenatally because imaging lacks necessary definition and these patients don't have other associated defects.Open
StatementPrenatal diagnosis allows families to make decisions about pregnancy continuation and, if continuing, to deliver at a center with a dedicated team for these malformations.Open
Cloacal Exstrophy with Dr. Alberto Peña
StatementCloacal exstrophy is a spectrum of congenital malformations affecting the gastrointestinal/colorectal area, urogenital tract, spine and cord, and sometimes lower extremity motion.Open
StatementBabies with cloacal exstrophy are born with an omphalocele, bladder exstrophy (two separated hemibladders), open cecum between the hemibladders, and separated pubic bones.Open
StatementThe small bowel can become exstrophic through the ileocecal valve, creating an 'elephant trunk' appearance.Open
StatementMale patients have two separated hemiphalluses with normal gonads; female patients have two hemivaginas below the exstrophic bladder leading to two hemiuteri.Open
StatementCloacal 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.Open
StatementThe amount of colon present at birth has very important implications for the patient's management and outcomes.Open
StatementA 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.Open
StatementWhile 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.Open
StatementHistorical practice was to perform bilateral gonadectomy, remove hemiphalluses, create a vagina with bowel, and assign female gender to XY patients with cloacal exstrophy.Open
StatementPatients 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.Open
StatementPatients 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.Open
StatementCurrent consensus is that XY patients should be raised as male, with pediatric urologists and plastic surgeons working on phallus reconstruction techniques.Open
StatementWhen a prominent pediatric urologist dominates management, patients receive good urologic attention but inadequate gastrointestinal care; the reverse occurs when pediatric surgeons dominate.Open
StatementThe 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.Open
StatementIt is very common but very harmful for pediatric surgeons to simply create an ileostomy, leaving all colon distally attached to the urinary tract.Open
QuotationThat is a very bad thing to do, and the patient will suffer several consequences because of thatOpen
StatementLeaving colon attached to the urinary tract creates a congenital bladder augmentation that causes hyperchloremic acidosis from urine absorption, interfering with growth and development.Open
QuotationIsn't that beautiful from your point of view?Open
StatementDefunctionalized colonic tissue left distally will not grow; colon requires passage of fecal matter through its lumen to grow.Open
StatementThe 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.Open
StatementPatients who received ileostomy with defunctionalized colon present at 2-3 years with poor growth, hyperchloremic acidosis managed by nephrologists, and large ileostomy losses.Open
StatementThe 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.Open
StatementSurgeons 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.Open
QuotationDo not leave gastrointestinal tract inside the functionalized. That's the main, main thing.Open
StatementSome 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.Open
StatementEven after osteotomy, it is very difficult to see a cloacal exstrophy patient with pubic bones completely together; they usually remain separated.Open
StatementEven a technically correct end colostomy often has poor motility, and babies may not pass stool easily, sometimes developing bacterial overgrowth similar to Hirschsprung disease.Open
StatementFamilies must be taught to irrigate the colostomy with small volumes of saline through a tube to evacuate fecal material when peristalsis is inadequate.Open
StatementDecision-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).Open
StatementMost cloacal exstrophy patients have an inadequate, tiny bladder requiring bladder augmentation with gastrointestinal tract, necessitating coordination between pediatric surgery and urology.Open
StatementPatients 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.Open
StatementPull-through is only considered for patients with capacity to form solid stool (adequate colon), as bowel management only works with solid stool.Open
StatementIt 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).Open
StatementPediatric surgeons should not underestimate the growth capacity of tiny colonic pieces during the newborn period; even small segments will grow over three years if fecal stream passes through them.Open
StatementAnnual contrast studies through the colostomy (retrograde injection) are performed to assess colonic growth.Open
StatementBefore committing to pull-through, a trial bowel management program is performed through the colostomy: enemas are given to empty the colonic pouch, and if the patient stays 24 hours without stool in the colostomy bag, pull-through is likely to succeed.Open
StatementBowel management trial through colostomy is typically started after age 3 when families consider avoiding the stoma for school.Open
StatementIf a patient has very little colon and cannot form solid stool, the urologist is free to use bowel for bladder augmentation; if the patient has borderline colon, the urologist must use stomach for augmentation to preserve bowel for fecal function.Open
StatementThe colon to be pulled through is the most posterior structure in the pelvis, with the bladder and augmentation anterior to it; therefore, bladder augmentation must not be done before deciding on pull-through, or accessing the colon will be extremely difficult.Open
StatementContrast enema through the colostomy can distinguish true liquid stool from paradoxical diarrhea (liquid stool around solid fecal impaction).Open
StatementPull-through and bladder augmentation are ideally performed together in a single operation lasting approximately 12 hours, with pediatric surgery going first (posterior dissection) followed by urology (anterior augmentation).Open
StatementDuring pull-through, if the patient has one or two appendices, a Malone appendicostomy can be created for antegrade enema administration, as the appendix remains in the abdomen when colon is pulled down.Open
StatementUrologists almost never use colon for bladder augmentation in cloacal exstrophy because colon is needed to form solid stool; they typically use small bowel or stomach.Open
StatementOccasionally, when a patient has a giant colonic pouch with very poor motility, the poor motility makes it good for bowel management (irrigate once daily, stays clean between irrigations), and a piece can be shared with urology for augmentation.Open
StatementMidline abdominal incision from xiphoid to pubis is used for pull-through to preserve the flanks and quadrants for potential future stomas.Open
StatementThe colostomy is circumferentially dissected and separated from the abdominal wall, then the blood supply is carefully studied because cloacal exstrophy patients have very bizarre, aberrant vascular anatomy.Open
StatementCareful observation of the vascular anatomy allows the surgeon to decide which vessels can be ligated to mobilize the colon to the perineum without compromising blood supply; sometimes no vessels need to be ligated.Open
StatementThe space behind the bladder is easily created, and the bowel is placed posteriorly; patients do not need prone positioning because the exstrophy makes everything anterior, so a supine frog-leg position provides full perineal access.Open
StatementCloacal exstrophy patients are lifelong patients due to orthopedic problems (separated pubic bones causing abnormal gait) and spinal problems (tethered cord requiring neurosurgical follow-up and potential cord release).Open
StatementSome teenagers are unhappy about separated pubic bones causing abnormal gait (feet pointing laterally); some dedicated orthopedic surgeons have been able to bring pubic bones closer together.Open
StatementDuring pull-through, vaginal reconstruction is attempted by approximating the hemivaginas as much as possible; the degree of separation varies on the spectrum.Open
StatementWhen hemivaginas are close with only a septum separating them superiorly, the septum is removed as high as possible; when vaginas run in completely different directions, one hemivagina may be removed, leaving the one with the better-looking cervix.Open
StatementPatients with functional hemiuterus may become pregnant, but it is high-risk pregnancy requiring specialized pediatric gynecology follow-up; in general, pregnancy is not advised.Open
StatementHemiuterus has a great tendency to produce miscarriages and premature labor; delivery must be by cesarean section due to limited abdominal space.Open
StatementBladder augmentation requires a Mitrofanoff conduit (appendix or part of a long appendix, sometimes shared half for urology and half for Malone) for intermittent catheterization to empty the bladder.Open
StatementAugmented bladders produce large amounts of mucus; if mucus is not removed, it forms stones, so families must be taught to irrigate the bladder (not just empty it) to remove mucus and prevent infections.Open
StatementBladder irrigation is sometimes performed with gentamicin to ensure all mucus is removed.Open
StatementLifelong urologic follow-up is needed to monitor for reflux and kidney damage; the Mitrofanoff may stop working or leak urine, requiring revision or valve tightening.Open
StatementPatients transitioning to adult hospitals often feel uncomfortable because adult urologists and orthopedic surgeons lack experience with these congenital malformations; patients prefer to remain in pediatric environments even as adults.Open
StatementDr. Peña observes that cloacal exstrophy patients are particularly charming, intelligent, beautiful, and charismatic when they grow up, and some have energy to help others manage their own problems.Open
StatementThe Pull-Through Network is a national organization (similar to cystic fibrosis or breast cancer organizations) for parents and patients with anorectal malformations, Hirschsprung disease, or bowel/urinary control problems; it has over 1000 members, holds annual meetings, and invites doctors to give talks.Open
StatementColorectal and urogenital problems have been left behind in terms of scientific approach and research funding because they are not 'elegant' problems—they involve stool, urine, and sex—and institutions are not eager to receive these patients.Open
StatementThe initial operation includes omphalocele closure (sometimes requiring staged closure), bowel-bladder separation, end colostomy creation, bladder closure attempt (sometimes requiring multiple stages), and in some institutions, pelvic osteotomy (either at initial operation or 2-3 months later).Open
StatementBetween the initial operation and pull-through, colostomy irrigation is often needed to manage poor colonic motility.Open
StatementPrenatal diagnosis of anorectal and urogenital malformations is easier for complex defects (like cloacal exstrophy) than simple defects because complex cases have associated findings (spinal problems, absent bladder) visible on imaging.Open
StatementAbsent bladder on prenatal ultrasound (due to exstrophy) is a bad sign and can be detected as early as week 20 of pregnancy.Open
StatementPrenatal diagnosis allows families to decide about pregnancy continuation and, if continuing, to deliver at a center with a multidisciplinary team experienced in these malformations.Open
StatementCertain malformations require centers of excellence where surgeons focus and sacrifice other areas of practice; attempting to train all surgeons superficially in complex conditions results in damaged children and no one becoming truly trained.Open
Alberto's statements about Colorectal / ARM & Hirschsprung572 statements
StatementDr. Pena would perform rectal biopsy in a case of apparent small left colon because he cannot distinguish it from Hirschsprung disease.Open
StatementDr. Pena would perform rectal biopsy in a case of apparent small left colon because he cannot distinguish it from Hirschsprung disease.Open
StatementDr. Pena questions whether patients who develop the characteristic dilated Duhamel pouch actually had true Hirschsprung disease, since by definition aganglionic bowel should not distend even after 10-15 years.Open
QuotationI have been wondering why patients with Duhamel get this image. If you think about it, we have seen patients with his disease that are 1012, 1415 years old. We have never seen a dilated rectum in a patient with Hirp disease. By definition, Hirp disease is a condition in which the egg ganglionic segment doesn't get distended.Open
StatementDr. Pena questions whether patients who develop the characteristic dilated Duhamel pouch actually had true Hirschsprung disease, since by definition aganglionic bowel should not distend even after 10-15 years.Open
QuotationI have been wondering why patients with Duhamel get this image. If you think about it, we have seen patients with his disease that are 1012, 1415 years old. We have never seen a dilated rectum in a patient with Hirp disease. By definition, Hirp disease is a condition in which the egg ganglionic segment doesn't get distended.Open
QuotationI wonder if all the patients that developed this traditional characteristic pouch of the duja male are actually patients that never had Hirk diseaseOpen
QuotationI wonder if all the patients that developed this traditional characteristic pouch of the duja male are actually patients that never had Hirk diseaseOpen
StatementDr. Pena recommends that when resecting a Duhamel pouch, the specimen should be oriented and the pathologist alerted to determine whether ganglion cells are present in the rectal portion, to test the hypothesis that these were misdiagnosed cases.Open
StatementDr. Pena recommends that when resecting a Duhamel pouch, the specimen should be oriented and the pathologist alerted to determine whether ganglion cells are present in the rectal portion, to test the hypothesis that these were misdiagnosed cases.Open
StatementDr. Pena states that if he sees a patient with megacolon and constipation on enema, he will not perform a rectal biopsy because it is a waste of time from his point of view.Open
StatementDr. Pena states that if he sees a patient with megacolon and constipation on enema, he will not perform a rectal biopsy because it is a waste of time from his point of view.Open
Quotationif I see a patient with this contrast enema, first of all, I will not take a rectal biopsy. I will not take your time because it's a waste of time from my point of view.Open
Quotationif I see a patient with this contrast enema, first of all, I will not take a rectal biopsy. I will not take your time because it's a waste of time from my point of view.Open
StatementDr. Pena argues that taking a biopsy in a patient with idiopathic constipation risks getting an aganglionic result that does not mean anything, because the patient does not have Hirschsprung disease.Open
StatementDr. Pena argues that taking a biopsy in a patient with idiopathic constipation risks getting an aganglionic result that does not mean anything, because the patient does not have Hirschsprung disease.Open
StatementThere is a normal physiologic aganglionic segment in the distal rectum, but its length at different ages (preterm, term, 6 months) has never been accurately determined in humans.Open
StatementThere is a normal physiologic aganglionic segment in the distal rectum, but its length at different ages (preterm, term, 6 months) has never been accurately determined in humans.Open
Quotationthere is one area. And the length of that area with no ganglion cells has never been accurately determined at different ages in the human being.Open
Quotationthere is one area. And the length of that area with no ganglion cells has never been accurately determined at different ages in the human being.Open
StatementSomeone could take a biopsy in the normal physiologic aganglionic area and get a result of no ganglion cells, which does not indicate Hirschsprung disease.Open
StatementSomeone could take a biopsy in the normal physiologic aganglionic area and get a result of no ganglion cells, which does not indicate Hirschsprung disease.Open
StatementThe internal sphincter has been defined as a thickening of the circular layer of normal smooth muscle bowel, but Dr. Pena states he has never personally observed this thickening when opening normal rectums at different ages.Open
Quotationthe internal sphincter has been defined as a thickening, as you can see in this diagram. A thickening of the circular layer of the normal smooth muscle bowel. In other words, and I have never seen that thickening personallyOpen
StatementThe internal sphincter has been defined as a thickening of the circular layer of normal smooth muscle bowel, but Dr. Pena states he has never personally observed this thickening when opening normal rectums at different ages.Open
Quotationthe internal sphincter has been defined as a thickening, as you can see in this diagram. A thickening of the circular layer of the normal smooth muscle bowel. In other words, and I have never seen that thickening personallyOpen
StatementIf the internal sphincter thickening exists, nobody has determined its exact boundaries at different ages.Open
StatementIf the internal sphincter thickening exists, nobody has determined its exact boundaries at different ages.Open
Quotationalacia of alacia of the internal sphincter is a manometric concept, not an anatomic conceptOpen
Quotationalacia of alacia of the internal sphincter is a manometric concept, not an anatomic conceptOpen
StatementInternal sphincter achalasia is a manometric concept, not an anatomic concept, similar to the lower esophageal sphincter.Open
StatementDr. Pena does not perform or recommend myectomies, myotomies, or botulinum toxin injections for internal sphincter achalasia because the target muscle area is undefined and these procedures paralyze muscle to facilitate stool passage rather than curing a condition of unknown origin.Open
StatementDr. Pena does not perform or recommend myectomies, myotomies, or botulinum toxin injections for internal sphincter achalasia because the target muscle area is undefined and these procedures paralyze muscle to facilitate stool passage rather than curing a condition of unknown origin.Open
StatementInternal sphincter achalasia is a manometric concept, not an anatomic concept, similar to the lower esophageal sphincter.Open
Cloaca - Workup & Evaluation
StatementThe measurements from 3D cloacography are accurate to within millimeters; when the radiologist reports 3.5 cm, the intraoperative measurement is typically 3.5 cm.Open
StatementThe measurements from 3D cloacography are accurate to within millimeters; when the radiologist reports 3.5 cm, the intraoperative measurement is typically 3.5 cm.Open
QuotationI was very skeptical about their measurements, but then little by little they become more and more accurate, and now they are, they are just there, but the differences in millimeters very subjective, so they are. I don't know what they did, but right now when they say 3.5 3.5 centimeters, usually it's 3.5 centimeters, so we really, we gain a lot, you gain a lot of experience for the benefit of these children.Open
QuotationI was very skeptical about their measurements, but then little by little they become more and more accurate, and now they are, they are just there, but the differences in millimeters very subjective, so they are. I don't know what they did, but right now when they say 3.5 3.5 centimeters, usually it's 3.5 centimeters, so we really, we gain a lot, you gain a lot of experience for the benefit of these children.Open
StatementIn neonatal cloacas, trying to make a very accurate anatomic diagnosis during the newborn period is useless and may actually hurt the baby.Open
StatementIn neonatal cloacas, trying to make a very accurate anatomic diagnosis during the newborn period is useless and may actually hurt the baby.Open
StatementIn the neonatal period, the crucial information is whether the baby has hydronephrosis, megaureters, or hydrocolpos compressing the ureters, which can be determined by ultrasound without endoscopy.Open
QuotationIn the real life and we don't make a very accurate diagnosis of the of the intrinsic anatomy of the cloaca during the newborn period and in fact trying to be very precise in the anatomic diagnosis is kind of useless and may actually hurt the baby, but we are very interested in a newborn babies to know if the baby has hydronephrosis and mega ureters and to know if the patient has a hydrocorpus that may be compressing the ureters.Open
QuotationIn the real life and we don't make a very accurate diagnosis of the of the intrinsic anatomy of the cloaca during the newborn period and in fact trying to be very precise in the anatomic diagnosis is kind of useless and may actually hurt the baby, but we are very interested in a newborn babies to know if the baby has hydronephrosis and mega ureters and to know if the patient has a hydrocorpus that may be compressing the ureters.Open
StatementIn the neonatal period, the crucial information is whether the baby has hydronephrosis, megaureters, or hydrocolpos compressing the ureters, which can be determined by ultrasound without endoscopy.Open
StatementKnowing whether a neonate with cloaca has reflux is not crucial; the priority is decompressing the gastrointestinal and urogenital tracts.Open
QuotationYou are at that point it's not so crucial to know if the baby has a reflux or not. It's not, it's what you want is the patient to, you want to be able to decompress the gastrointestinal tract and to decompress the urogenital tract.Open
StatementKnowing whether a neonate with cloaca has reflux is not crucial; the priority is decompressing the gastrointestinal and urogenital tracts.Open
QuotationYou are at that point it's not so crucial to know if the baby has a reflux or not. It's not, it's what you want is the patient to, you want to be able to decompress the gastrointestinal tract and to decompress the urogenital tract.Open
QuotationNot everybody has the capacity to do it and don't force the endoscope in a tiny structure because you may end up hurting.Open
QuotationNot everybody has the capacity to do it and don't force the endoscope in a tiny structure because you may end up hurting.Open
StatementForcing an endoscope into a tiny neonatal cloacal structure can cause harm.Open
StatementForcing an endoscope into a tiny neonatal cloacal structure can cause harm.Open
QuotationMany urologists don't have experience in cloacas and will end up doing nephrostom is not indicated, ureterostom is not indicated, and vesicostom is not indicated.Open
QuotationMany urologists don't have experience in cloacas and will end up doing nephrostom is not indicated, ureterostom is not indicated, and vesicostom is not indicated.Open
StatementNephrostomy, ureterostomy, and vesicostomy are not indicated in most neonatal cloacas.Open
StatementNephrostomy, ureterostomy, and vesicostomy are not indicated in most neonatal cloacas.Open
QuotationOur specific recommendation is to drain the hydrocorpus, to open a colostomy, and re-evaluate the patient from the urologic point of view 48 hours later and then make a decision.Open
StatementThe recommended neonatal management for cloaca is to drain hydrocolpos, open a colostomy, and re-evaluate from the urologic point of view 48 hours later before making further decisions.Open
StatementThe recommended neonatal management for cloaca is to drain hydrocolpos, open a colostomy, and re-evaluate from the urologic point of view 48 hours later before making further decisions.Open
QuotationOur specific recommendation is to drain the hydrocorpus, to open a colostomy, and re-evaluate the patient from the urologic point of view 48 hours later and then make a decision.Open
StatementSome cloacal patients may need vesicostomy if there is obstruction in the common channel, but many do not; decompressing the hydrocolpos often completely changes the clinical picture.Open
StatementSome cloacal patients may need vesicostomy if there is obstruction in the common channel, but many do not; decompressing the hydrocolpos often completely changes the clinical picture.Open
QuotationBy by simply decompressing the hydrocorpus, the, the picture completely changes.Open
QuotationBy by simply decompressing the hydrocorpus, the, the picture completely changes.Open
Cloaca - Prental Imaging & Diagnosis - Counseling
QuotationNot everybody gets the privilege to get a specialized radiologist is dedicated to the prenatal diagnosis of this malformation that we have.Open
StatementDr. Peña reviewed mothers of babies born with cloacas who did not have prenatal diagnosis; when he obtained their ultrasounds, many had abnormalities but radiologists misdiagnosed them as urethrocele, double bladder, ovarian cysts, or bladder diverticulum instead of recognizing hydrocolposOpen
QuotationThe images were there. What failed was the index of suspicion in the those radiologist.Open
StatementIf a female fetus has a prenatal diagnosis of ovarian cyst, double bladder, or urethrocele, suspect the patient may have a cloaca; if the patient also has abnormal vertebrae, hydronephrosis, and dilated bowel, that confirms the diagnosisOpen
Radiology and Image Diagnosis of Hirschsprung Disease
StatementDr. Peña would not do biopsy if sure it is meconium ileus, but would do biopsy for a picture of small left colon because he does not know how to distinguish it from Hirschsprung.Open
StatementDr. Peña would not do biopsy if sure it is meconium ileus, but would do biopsy for a picture of small left colon because he does not know how to distinguish it from Hirschsprung.Open
QuotationI have been wondering why patients with Duhamel get this image. If you think about it, we have seen patients with his disease that are 1012, 1415 years old. We have never seen a dilated rectum in a patient with Hirp disease. By definition, Hirp disease is a condition in which the egg ganglionic segment doesn't get distended. Even if you go 10 years, 15 years, and yet in these patients we see the rectum, the rectum was opposed to this angryonic, very stretched with fecally impacted.Open
QuotationI have been wondering why patients with Duhamel get this image. If you think about it, we have seen patients with his disease that are 1012, 1415 years old. We have never seen a dilated rectum in a patient with Hirp disease. By definition, Hirp disease is a condition in which the egg ganglionic segment doesn't get distended. Even if you go 10 years, 15 years, and yet in these patients we see the rectum, the rectum was opposed to this angryonic, very stretched with fecally impacted.Open
StatementDr. Peña wonders why Duhamel patients get dilated rectum; by definition Hirschsprung disease is a condition where the aganglionic segment does not get distended even after 10-15 years, yet in these patients the rectum is very stretched and fecally impacted.Open
StatementDr. Peña wonders why Duhamel patients get dilated rectum; by definition Hirschsprung disease is a condition where the aganglionic segment does not get distended even after 10-15 years, yet in these patients the rectum is very stretched and fecally impacted.Open
StatementDr. Peña suspects that all patients who developed the traditional Duhamel pouch may never have had Hirschsprung disease; he invites surgeons to orient the resected pouch and have pathologists study whether there are ganglion cells in that rectum.Open
StatementDr. Peña suspects that all patients who developed the traditional Duhamel pouch may never have had Hirschsprung disease; he invites surgeons to orient the resected pouch and have pathologists study whether there are ganglion cells in that rectum.Open
QuotationI suspect that this patient never had Hodgkin disease.Open
QuotationI suspect that this patient never had Hodgkin disease.Open
StatementDr. Peña states that if he sees a patient with a contrast enema showing redundant stool-filled colon with normal rectum, he will not take a rectal biopsy because it is a waste of time and the patient has no Hirschsprung disease.Open
StatementDr. Peña states that if he sees a patient with a contrast enema showing redundant stool-filled colon with normal rectum, he will not take a rectal biopsy because it is a waste of time and the patient has no Hirschsprung disease.Open
Quotationif I see a patient with this contrast enema, first of all, I will not take a rectal biopsy. I will not take your time because it's a waste of time from my point of view.Open
Quotationif I see a patient with this contrast enema, first of all, I will not take a rectal biopsy. I will not take your time because it's a waste of time from my point of view.Open
Quotationthere is no way to differentiate the so-called ultra short with idiopathic constipationOpen
Quotationthere is no way to differentiate the so-called ultra short with idiopathic constipationOpen
StatementDr. Peña states there is no way to differentiate so-called ultra-short segment Hirschsprung from idiopathic constipation.Open
StatementDr. Peña states there is no way to differentiate so-called ultra-short segment Hirschsprung from idiopathic constipation.Open
Quotationthere is one area. And the length of that area with no ganglion cells has never been accurately determined at different ages in the human being.Open
Quotationthere is one area. And the length of that area with no ganglion cells has never been accurately determined at different ages in the human being.Open
StatementThe rectum has ganglion cells in normal ganglionic bowel, then there is one area with no ganglion cells, but the length of that normal aganglionic segment has never been accurately determined at different ages in humans.Open
StatementThe rectum has ganglion cells in normal ganglionic bowel, then there is one area with no ganglion cells, but the length of that normal aganglionic segment has never been accurately determined at different ages in humans.Open
StatementThere is no accurate study of the normal aganglionic segment length in a preemie, full-term baby, or 6-month-old baby; this is a challenge for young pediatric surgeons to contribute.Open
StatementThere is no accurate study of the normal aganglionic segment length in a preemie, full-term baby, or 6-month-old baby; this is a challenge for young pediatric surgeons to contribute.Open
QuotationThat's another challenge for young pediatric surgeons. If you want to make a contribution. studies, this is a real challenge.Open
QuotationThat's another challenge for young pediatric surgeons. If you want to make a contribution. studies, this is a real challenge.Open
StatementSomebody could take a biopsy in the normal aganglionic area and get a result of no ganglion cells, but it does not mean anything clinically.Open
Quotationconceivably somebody could take a biopsy in that area and come up with the result of no ganglion cells. It doesn't mean anything for us.Open
StatementSomebody could take a biopsy in the normal aganglionic area and get a result of no ganglion cells, but it does not mean anything clinically.Open
Quotationconceivably somebody could take a biopsy in that area and come up with the result of no ganglion cells. It doesn't mean anything for us.Open
Quotationthe internal sphincter has been defined as a thickening, as you can see in this diagram. A thickening of the circular layer of the normal smooth muscle bowel. In other words, and I have never seen that thickening personally, and I have opened these normal rectums in different, different ages. I have been looking at that. I don't have a microscope, but I have never seen that thickening.Open
StatementThe internal sphincter has been defined as a thickening of the circular layer of normal smooth muscle bowel, but Dr. Peña has never personally seen that thickening despite opening normal rectums at different ages.Open
Quotationthe internal sphincter has been defined as a thickening, as you can see in this diagram. A thickening of the circular layer of the normal smooth muscle bowel. In other words, and I have never seen that thickening personally, and I have opened these normal rectums in different, different ages. I have been looking at that. I don't have a microscope, but I have never seen that thickening.Open
StatementThe internal sphincter has been defined as a thickening of the circular layer of normal smooth muscle bowel, but Dr. Peña has never personally seen that thickening despite opening normal rectums at different ages.Open
StatementIf the internal sphincter thickening exists, nobody has determined the exact limit of that thickening at different ages, so it becomes a kind of magic or witchery type of diagnosis.Open
StatementIf the internal sphincter thickening exists, nobody has determined the exact limit of that thickening at different ages, so it becomes a kind of magic or witchery type of diagnosis.Open
Quotationalacia of alacia of the internal sphincter is a manometric concept, not an anatomic conceptOpen
StatementInternal anal sphincter achalasia is a manometric concept, not an anatomic concept, similar to the lower esophageal sphincter and other sphincters that have been defined but never anatomically seen.Open
Quotationalacia of alacia of the internal sphincter is a manometric concept, not an anatomic conceptOpen
StatementInternal anal sphincter achalasia is a manometric concept, not an anatomic concept, similar to the lower esophageal sphincter and other sphincters that have been defined but never anatomically seen.Open
StatementDr. Peña does not perform myectomies or myotomies and does not recommend those procedures because we do not know where to inject or what area of muscle to target.Open
StatementDr. Peña does not perform myectomies or myotomies and does not recommend those procedures because we do not know where to inject or what area of muscle to target.Open
QuotationI don't pay attention to that and I don't perform myectomies and I don't recommend those myectomies and myotomies and buttock and those concepts because we don't know where where you inject. What area of the muscle, nobody knowsOpen
QuotationI don't pay attention to that and I don't perform myectomies and I don't recommend those myectomies and myotomies and buttock and those concepts because we don't know where where you inject. What area of the muscle, nobody knowsOpen
StatementBotox and similar injections paralyze whatever muscle is there and facilitate passing of stool, but they are not curing a condition of unknown origin.Open
StatementBotox and similar injections paralyze whatever muscle is there and facilitate passing of stool, but they are not curing a condition of unknown origin.Open
Outcomes and Complications in Hirschsprung Disease
StatementDr. Peña has more re-operation cases than primary procedures because most pediatric surgeons believe they can operate on Hirschsprung disease, so he receives referrals for complex cases but usually not for primary Hirschsprung.Open
StatementDr. Peña has more re-operation cases than primary procedures because most pediatric surgeons believe they can operate on Hirschsprung disease, so he receives referrals for complex cases but usually not for primary Hirschsprung.Open
QuotationI have much more experience in re-operation than primaries or simply in patients that come because they suffer from complications.Open
QuotationI have much more experience in re-operation than primaries or simply in patients that come because they suffer from complications.Open
StatementComplications and sequelae are classified into three categories: non-preventable (enterocolitis), partially preventable (constipation), and preventable (dehiscence, strictures, perianal/urogenital fistulas).Open
StatementComplications and sequelae are classified into three categories: non-preventable (enterocolitis), partially preventable (constipation), and preventable (dehiscence, strictures, perianal/urogenital fistulas).Open
QuotationWe look forward for the, as I said before, our, the new generation of pediatric surgeons to go into serious scientific research to find out what's the problem with that, to explain this and to treat this adequately.Open
QuotationWe look forward for the, as I said before, our, the new generation of pediatric surgeons to go into serious scientific research to find out what's the problem with that, to explain this and to treat this adequately.Open
StatementConstipation is partially preventable; if a very dilated piece of bowel is left, even if ganglionic, patients will likely suffer from constipation because very dilated colon suffers from hypomotility.Open
QuotationThe very dilated colon suffers for hypomotility, poor motility, and therefore the patients suffer from constipation.Open
StatementThe rule is to resect not only the aganglionic segment but as much as possible the dilated portion of the colon.Open
StatementThe rule is to resect not only the aganglionic segment but as much as possible the dilated portion of the colon.Open
QuotationIt's very sad because those are, I believe, 100% preventable, and yet we see them all the time.Open
QuotationIt's very sad because those are, I believe, 100% preventable, and yet we see them all the time.Open
QuotationI don't remember a patient that I operated, came back with symptoms. I re-biopsied and found no ganglion cells after one of my put-throughs.Open
StatementDr. Peña has never documented a case where a patient he operated on returned with symptoms, was re-biopsied, and was found to have aganglionosis in the pull-through area that previously had normal ganglion cells.Open
StatementDr. Peña has never documented a case where a patient he operated on returned with symptoms, was re-biopsied, and was found to have aganglionosis in the pull-through area that previously had normal ganglion cells.Open
QuotationI don't remember a patient that I operated, came back with symptoms. I re-biopsied and found no ganglion cells after one of my put-throughs.Open
StatementDr. Peña resects 5 cm proximal to the last positive biopsy site when the bowel is not dilated.Open
StatementDr. Peña resects 5 cm proximal to the last positive biopsy site when the bowel is not dilated.Open
StatementWith no anal canal, there will be no bowel control, and liquid stool with no bowel control is tragic; the quality of life is seriously affected.Open
StatementWith no anal canal, there will be no bowel control, and liquid stool with no bowel control is tragic; the quality of life is seriously affected.Open
QuotationWith no anal canal, there will be no bowel control, and liquid stool with no bowel control is really tragic.Open
QuotationWith no anal canal, there will be no bowel control, and liquid stool with no bowel control is really tragic.Open
QuotationWe pediatric surgeons don't talk much about the diaper problems, you know, the nurses, the mothers are the ones who struggle with this, and we don't discuss much of this, and we should do it because the quality of life of these patients is seriously affected.Open
QuotationWe pediatric surgeons don't talk much about the diaper problems, you know, the nurses, the mothers are the ones who struggle with this, and we don't discuss much of this, and we should do it because the quality of life of these patients is seriously affected.Open
StatementThe key part of evaluation for fecal incontinence after Hirschsprung surgery is examination under anesthesia to determine if the anal canal has been damaged.Open
StatementThe key part of evaluation for fecal incontinence after Hirschsprung surgery is examination under anesthesia to determine if the anal canal has been damaged.Open
StatementIf the anal canal is preserved, there is more chance of bowel control, though no guarantee; if damaged, the patient will most likely need enemas for life (bowel management for fecal incontinence).Open
StatementIf the anal canal is preserved, there is more chance of bowel control, though no guarantee; if damaged, the patient will most likely need enemas for life (bowel management for fecal incontinence).Open
QuotationIf the patient doesn't poop only every 3 days and has a dilated colon, that's hypermotility with and without colonic manometry.Open
QuotationIf the patient doesn't poop only every 3 days and has a dilated colon, that's hypermotility with and without colonic manometry.Open
QuotationI don't know how to call it that in other words, for that patient, we give laxatives and if the incontinence persists, then we go for bowel management.Open
QuotationI don't know how to call it that in other words, for that patient, we give laxatives and if the incontinence persists, then we go for bowel management.Open
StatementIn Hirschsprung disease, a patient may have a stricture and yet suffer from terrible diarrhea; stricture and stasis of stool in the colon produces secretory diarrhea even with normal ganglionic bowel.Open
QuotationA patient may have stricture and yet suffer from terrible diarrhea. And yet when you we go for the to see the films that you took that you take that you took um that you took after you put through shows um shows solid stored in the column.Open
QuotationA patient may have stricture and yet suffer from terrible diarrhea. And yet when you we go for the to see the films that you took that you take that you took um that you took after you put through shows um shows solid stored in the column.Open
StatementIn Hirschsprung disease, a patient may have a stricture and yet suffer from terrible diarrhea; stricture and stasis of stool in the colon produces secretory diarrhea even with normal ganglionic bowel.Open
QuotationStricture and stasis of stool in the colon in Kishman disease, even if you have a normal galionic bowel, produces secretory diarrhea.Open
QuotationStricture and stasis of stool in the colon in Kishman disease, even if you have a normal galionic bowel, produces secretory diarrhea.Open
QuotationMost of the mistakes that we saw in the old time were consecutive to lack of exposure.Open
QuotationMost of the mistakes that we saw in the old time were consecutive to lack of exposure.Open
QuotationThe priority is to do an operation that you finish happy. The happiness of the surgeon at the end of the procedure is very important, and you look at the patients and you say, you know something, I'm happy and the family feels happy and you sleep well.Open
StatementThe priority in surgery is to finish the operation happy; the happiness of the surgeon at the end of the procedure is very important, the family feels it, and you sleep well.Open
StatementThe priority in surgery is to finish the operation happy; the happiness of the surgeon at the end of the procedure is very important, the family feels it, and you sleep well.Open
QuotationThe priority is to do an operation that you finish happy. The happiness of the surgeon at the end of the procedure is very important, and you look at the patients and you say, you know something, I'm happy and the family feels happy and you sleep well.Open
QuotationEnemas are contraindicated in Hik disease irrigation. We do it on prophylactic.Open
QuotationEnemas are contraindicated in Hik disease irrigation. We do it on prophylactic.Open
StatementProphylactic postoperative irrigations (3 times per day for the first month with oral Flagyl, tapering over 3 months) can prevent enterocolitis after pull-through; a Salt Lake City study demonstrated this approach.Open
QuotationThe enterocolitis is such a bad complication, such a bad problem. The baby sometimes died from home to the hospital and that's why we don't want to run into that.Open
StatementEnterocolitis is such a bad complication that babies sometimes die between home and hospital; prophylactic management is preferred over having patients return with distention and enterocolitis two weeks post-operatively.Open
QuotationThe enterocolitis is such a bad complication, such a bad problem. The baby sometimes died from home to the hospital and that's why we don't want to run into that.Open
StatementEnterocolitis is such a bad complication that babies sometimes die between home and hospital; prophylactic management is preferred over having patients return with distention and enterocolitis two weeks post-operatively.Open
StatementEnemas consist of giving fluid with ingredients that irritate the bowel to provoke contraction; they are contraindicated in Hirschsprung disease because the disease involves absent normal peristalsis.Open
StatementEnemas consist of giving fluid with ingredients that irritate the bowel to provoke contraction; they are contraindicated in Hirschsprung disease because the disease involves absent normal peristalsis.Open
QuotationEnemas consist in giving a certain amount of fluid with the, with the, with the ingredients that are irritant to the bowels that provoke a bowel contraction, but the, the disease consists in not having the normal peristalsis therefore are not indicated.Open
QuotationEnemas consist in giving a certain amount of fluid with the, with the, with the ingredients that are irritant to the bowels that provoke a bowel contraction, but the, the disease consists in not having the normal peristalsis therefore are not indicated.Open
StatementIrrigation means passing a tube through the rectum; liquid stool comes out through the tube lumen, and only a small amount of saline is passed to clear the tube.Open
QuotationIrrigation means to take a tube like you saw in the video, pass it through the rectum, and through the lumen of the tube, liquid stool will come out. We just pass a small amount of saline to clear the lumen of the tube.Open
StatementIrrigation means passing a tube through the rectum; liquid stool comes out through the tube lumen, and only a small amount of saline is passed to clear the tube.Open
QuotationIrrigation means to take a tube like you saw in the video, pass it through the rectum, and through the lumen of the tube, liquid stool will come out. We just pass a small amount of saline to clear the lumen of the tube.Open
QuotationIf you don't do, if you decide not to go for irrigations, my suggestion is to be very careful. And follow the patient. First of all, it's very important how reliable is the mother for you.Open
QuotationIf you don't do, if you decide not to go for irrigations, my suggestion is to be very careful. And follow the patient. First of all, it's very important how reliable is the mother for you.Open
QuotationThere are mothers that are very reliable. They will be calling you every 3 days, and there are mothers that just disappear, and then you hear that they went to the emergency room in a general hospital where they don't know what enterocolitis is all about.Open
QuotationThere are mothers that are very reliable. They will be calling you every 3 days, and there are mothers that just disappear, and then you hear that they went to the emergency room in a general hospital where they don't know what enterocolitis is all about.Open
StatementDilations are performed twice daily with diaper changes; the dilation also provokes bowel movements in babies.Open
StatementDilations are performed twice daily with diaper changes; the dilation also provokes bowel movements in babies.Open
QuotationThe association between Hirschsprung disease and anorectal malformations is extremely uncommon; personally, I have seen in my life two cases, demonstrated cases of Hishman with an rectum malformation, which is a very bad association.Open
StatementThe association between Hirschsprung disease and anorectal malformations is extremely uncommon; Dr. Peña has seen only two demonstrated cases in his career.Open
QuotationThe association between Hirschsprung disease and anorectal malformations is extremely uncommon; personally, I have seen in my life two cases, demonstrated cases of Hishman with an rectum malformation, which is a very bad association.Open
StatementThe association between Hirschsprung disease and anorectal malformations is extremely uncommon; Dr. Peña has seen only two demonstrated cases in his career.Open
StatementMany patients with anorectal malformations suffer from constipation; surgeons accustomed to suspecting Hirschsprung in constipation cases have taken biopsies showing no ganglion cells (which can occur in normal children for various reasons) and performed pull-throughs, leaving patients incontinent.Open
StatementMany patients with anorectal malformations suffer from constipation; surgeons accustomed to suspecting Hirschsprung in constipation cases have taken biopsies showing no ganglion cells (which can occur in normal children for various reasons) and performed pull-throughs, leaving patients incontinent.Open
StatementBefore making a Hirschsprung diagnosis and embarking on a pull-through in a patient with anorectal malformation, think carefully—most likely it is not Hirschsprung; many patients suffer from constipation but very few have Hirschsprung.Open
QuotationBefore you make that uh diagnosis and before you embark yourself in a push-through for history, a patient with anorectal malformation, think about it. Most likely it's not Hines.Open
QuotationBefore you make that uh diagnosis and before you embark yourself in a push-through for history, a patient with anorectal malformation, think about it. Most likely it's not Hines.Open
StatementBefore making a Hirschsprung diagnosis and embarking on a pull-through in a patient with anorectal malformation, think carefully—most likely it is not Hirschsprung; many patients suffer from constipation but very few have Hirschsprung.Open
History of Hirschsprung Disease
QuotationWhat would happen if Harold Hib could be here today? Contemplating these fantastic advances in technology and hearing, repeating and repeating his name are related with a condition that he described so many years ago.Open
QuotationWhat would happen if Harold Hib could be here today? Contemplating these fantastic advances in technology and hearing, repeating and repeating his name are related with a condition that he described so many years ago.Open
QuotationWe are in depth with all those through history that contribute to the progress in the management of these serious condition that affects so many children.Open
QuotationWe are in depth with all those through history that contribute to the progress in the management of these serious condition that affects so many children.Open
QuotationWe are particularly grateful with Dr. Ova Swenson for his seminal contribution, and, and we still followed his principles in the management of this condition.Open
QuotationWe are particularly grateful with Dr. Ova Swenson for his seminal contribution, and, and we still followed his principles in the management of this condition.Open
StatementThe most serious challenge in Hirschsprung disease is the basic science approach to solve the problem of enterocolitis and many other problems affecting children with the disease.Open
StatementThe most serious challenge in Hirschsprung disease is the basic science approach to solve the problem of enterocolitis and many other problems affecting children with the disease.Open
StatementHirschsprung disease is not only about ganglion cells or no ganglion cells; it is a much more complex condition.Open
StatementHirschsprung disease is not only about ganglion cells or no ganglion cells; it is a much more complex condition.Open
StatementElectric enemas were described around 1908, involving passing a tube through the rectum with an electrode inside and another electrode on the abdomen, delivering about 40 milliamps of interrupted cycling current to facilitate expulsion of saline solution from the colon.Open
StatementElectric enemas were described around 1908, involving passing a tube through the rectum with an electrode inside and another electrode on the abdomen, delivering about 40 milliamps of interrupted cycling current to facilitate expulsion of saline solution from the colon.Open
Surgical Procedures for Hirschsprung Disease
StatementBetween 75 and 80% of Hirschsprung cases can be completed transanally, reaching normal ganglionic bowel from below.Open
StatementBetween 75 and 80% of Hirschsprung cases can be completed transanally, reaching normal ganglionic bowel from below.Open
StatementIf you start transanally and cannot reach ganglionic bowel, you simply open the abdomen and continue the resection—this is not a complication.Open
StatementIf you start transanally and cannot reach ganglionic bowel, you simply open the abdomen and continue the resection—this is not a complication.Open
QuotationThe main problems that we have seen in Hirschsprung disease are not related with the approach that you use—are related with the inexperience and technical incapacity of the surgeon that damaged the baby on a permanent basis both ways, laparoscopically or transanally. Those are the real problems that we see.Open
StatementThe main problems in Hirschsprung surgery are related to surgeon inexperience and technical incapacity, not the approach (laparoscopic vs. transanal vs. open).Open
StatementThe main problems in Hirschsprung surgery are related to surgeon inexperience and technical incapacity, not the approach (laparoscopic vs. transanal vs. open).Open
QuotationThe main problems that we have seen in Hirschsprung disease are not related with the approach that you use—are related with the inexperience and technical incapacity of the surgeon that damaged the baby on a permanent basis both ways, laparoscopically or transanally. Those are the real problems that we see.Open
StatementA bad surgeon will damage the patient both ways—laparoscopically or transanally.Open
StatementA bad surgeon will damage the patient both ways—laparoscopically or transanally.Open
StatementThe basic goal of Hirschsprung surgery is not to damage the sphincter mechanism, which has been damaged by both laparoscopic and non-laparoscopic techniques.Open
StatementThe basic goal of Hirschsprung surgery is not to damage the sphincter mechanism, which has been damaged by both laparoscopic and non-laparoscopic techniques.Open
QuotationI never heard people complaining about the scar or not the scar. I see patients complaining about fecal incontinence remaining—that's the real problem, not the size of the scar.Open
QuotationI never heard people complaining about the scar or not the scar. I see patients complaining about fecal incontinence remaining—that's the real problem, not the size of the scar.Open
StatementPatients complain about fecal incontinence, not the size of the scar.Open
StatementPatients complain about fecal incontinence, not the size of the scar.Open
StatementWhen doing transanal dissection, the Lone Star retractor hooks should be placed at the pectinate line to protect the entire anal canal.Open
StatementWhen doing transanal dissection, the Lone Star retractor hooks should be placed at the pectinate line to protect the entire anal canal.Open
StatementSurgeons must be careful not to stretch the anus too much during transanal dissection, as excessive stretch damages the sphincter mechanism and causes fecal incontinence.Open
StatementSurgeons must be careful not to stretch the anus too much during transanal dissection, as excessive stretch damages the sphincter mechanism and causes fecal incontinence.Open
StatementThe dissection should start 2 centimeters deep inside the rectum from the pectinate line, using multiple silk stitches to distribute tension and avoid tissue damage.Open
StatementThe dissection should start 2 centimeters deep inside the rectum from the pectinate line, using multiple silk stitches to distribute tension and avoid tissue damage.Open
StatementDr. de la Torre originally started the transanal operation submucosally and rectally; Dr. Peña prefers full-thickness dissection like Dr. Swenson used to do.Open
StatementDr. de la Torre originally started the transanal operation submucosally and rectally; Dr. Peña prefers full-thickness dissection like Dr. Swenson used to do.Open
StatementBiopsies should be taken every 5 centimeters during transanal dissection until normal ganglionic bowel is found, then go 5 centimeters higher.Open
StatementBiopsies should be taken every 5 centimeters during transanal dissection until normal ganglionic bowel is found, then go 5 centimeters higher.Open
StatementA two-layer anastomosis is performed: the first layer takes seromuscular of the bowel and tissue above the divided rectum; the second layer is mucosa-to-mucosa.Open
StatementA two-layer anastomosis is performed: the first layer takes seromuscular of the bowel and tissue above the divided rectum; the second layer is mucosa-to-mucosa.Open
StatementKeys to successful transanal surgery: respect the pectinate line and anal canal, don't stretch the anus too much, mobilize rectum to ensure ganglionic bowel with good blood supply, and perform anastomosis with no tension.Open
StatementKeys to successful transanal surgery: respect the pectinate line and anal canal, don't stretch the anus too much, mobilize rectum to ensure ganglionic bowel with good blood supply, and perform anastomosis with no tension.Open
StatementProne position is preferred over lithotomy for transanal surgery because the surgeon is not the only one who can see, the field is not vertical, and instruments are not lost.Open
StatementProne position is preferred over lithotomy for transanal surgery because the surgeon is not the only one who can see, the field is not vertical, and instruments are not lost.Open
StatementLeaving 1-2 centimeters of aganglionic bowel does not explain why patients don't behave well postoperatively.Open
StatementLeaving 1-2 centimeters of aganglionic bowel does not explain why patients don't behave well postoperatively.Open
StatementSome patients operated with exactly the same technique do beautifully like normal individuals, while others have symptoms of enterocolitis, and we don't know why.Open
StatementSome patients operated with exactly the same technique do beautifully like normal individuals, while others have symptoms of enterocolitis, and we don't know why.Open
StatementThe majority of patients who come with symptoms of retention (enterocolitis or constipation) after pull-through do NOT have a portion of aganglionic bowel left—they simply behave that way.Open
StatementThe majority of patients who come with symptoms of retention (enterocolitis or constipation) after pull-through do NOT have a portion of aganglionic bowel left—they simply behave that way.Open
StatementA few patients do have an obvious piece of aganglionic bowel left, but usually it's much more than 2 centimeters.Open
StatementA few patients do have an obvious piece of aganglionic bowel left, but usually it's much more than 2 centimeters.Open
StatementWhen you finish the operation, the 2 centimeters of bowel you left above the pectinate line are already damaged, so you are very near the pectinate line.Open
StatementWhen you finish the operation, the 2 centimeters of bowel you left above the pectinate line are already damaged, so you are very near the pectinate line.Open
StatementThe real concern about transanal surgery is fecal incontinence, which happens when the anal canal is damaged.Open
StatementThe real concern about transanal surgery is fecal incontinence, which happens when the anal canal is damaged.Open
QuotationWhen we remove the rectum of a human being, we are already affecting seriously the mechanisms of bowel control because we are removing the natural reservoir of the patient.Open
QuotationWhen we remove the rectum of a human being, we are already affecting seriously the mechanisms of bowel control because we are removing the natural reservoir of the patient.Open
StatementWhen we remove the rectum of a human being, we are already seriously affecting the mechanisms of bowel control because we are removing the natural reservoir.Open
StatementWhen we remove the rectum of a human being, we are already seriously affecting the mechanisms of bowel control because we are removing the natural reservoir.Open
StatementEven adult ulcerative colitis patients with perfect operations and intact anal canals have problems with bowel control—they have accidents at night.Open
StatementEven adult ulcerative colitis patients with perfect operations and intact anal canals have problems with bowel control—they have accidents at night.Open
StatementAfter removing the rectum, we connect a piece of colon that is constantly moving with peristalsis, whereas the rectum normally rests and only moves when it wants to empty.Open
StatementAfter removing the rectum, we connect a piece of colon that is constantly moving with peristalsis, whereas the rectum normally rests and only moves when it wants to empty.Open
StatementRemoving the rectum in a child results in passing stool constantly, requiring an intact anal canal, sensation, intact sphincter, and cooperation for bowel control.Open
StatementRemoving the rectum in a child results in passing stool constantly, requiring an intact anal canal, sensation, intact sphincter, and cooperation for bowel control.Open
StatementEven in patients with a very well-preserved anal canal, some children have different degrees of fecal incontinence after Hirschsprung surgery.Open
StatementEven in patients with a very well-preserved anal canal, some children have different degrees of fecal incontinence after Hirschsprung surgery.Open
QuotationWe have not been discussing enough in our pediatric surgical meetings the problem of fecal incontinence that is much more common than we believe.Open
StatementFecal incontinence after Hirschsprung surgery is much more common than we believe, and we have not been discussing it enough in pediatric surgical meetings.Open
QuotationWe have not been discussing enough in our pediatric surgical meetings the problem of fecal incontinence that is much more common than we believe.Open
StatementFecal incontinence after Hirschsprung surgery is much more common than we believe, and we have not been discussing it enough in pediatric surgical meetings.Open
StatementMost patients with symptoms of enterocolitis and constipation after pull-through do not have residual aganglionic bowel.Open
StatementMost patients with symptoms of enterocolitis and constipation after pull-through do not have residual aganglionic bowel.Open
StatementPatients are born with bowel control; we provoke fecal incontinence through surgical technique.Open
StatementPatients are born with bowel control; we provoke fecal incontinence through surgical technique.Open
QuotationPatients are born with bowel control. We provoke the fecal incontinence.Open
QuotationPatients are born with bowel control. We provoke the fecal incontinence.Open
StatementUntil the time of the video, Dr. Peña's group had performed 125 transanal operations: 56 primary Hirschsprung, 42 redo Hirschsprung, 21 for idiopathic constipation.Open
StatementUntil the time of the video, Dr. Peña's group had performed 125 transanal operations: 56 primary Hirschsprung, 42 redo Hirschsprung, 21 for idiopathic constipation.Open
StatementTransanal pull-through for idiopathic constipation is not a good operation.Open
StatementTransanal pull-through for idiopathic constipation is not a good operation.Open
StatementWhen dissecting the anterior rectal wall transanally, the dissection must be conducted very meticulously because the rectum has a common wall with the vagina and prostatic urethra.Open
StatementWhen dissecting the anterior rectal wall transanally, the dissection must be conducted very meticulously because the rectum has a common wall with the vagina and prostatic urethra.Open
StatementCases with fistulas to the vagina or urinary tract after Hirschsprung surgery are unacceptable complications.Open
StatementCases with fistulas to the vagina or urinary tract after Hirschsprung surgery are unacceptable complications.Open
StatementDuring full-thickness transanal dissection, if you see fat around the rectum, you can get closer to the rectum because that means you are not in the real rectal wall.Open
StatementDuring full-thickness transanal dissection, if you see fat around the rectum, you can get closer to the rectum because that means you are not in the real rectal wall.Open
StatementIf you stay right on the bowel wall during dissection, you will not provoke denervation of the urinary tract, as Dr. Swenson emphasized.Open
StatementThe outer layer of sutures fixes the rectum in the right position and releases tension from the inner layer.Open
StatementThe outer layer of sutures fixes the rectum in the right position and releases tension from the inner layer.Open
StatementAvoid using big retractors pulling in different directions during transanal surgery because that stretches the sphincter too much.Open
StatementAvoid using big retractors pulling in different directions during transanal surgery because that stretches the sphincter too much.Open
StatementPediatric surgeons compete with each other trying to close stomas as early as possible, but this may not be best for the patient.Open
StatementPediatric surgeons compete with each other trying to close stomas as early as possible, but this may not be best for the patient.Open
StatementIn Hirschsprung disease with or without total colonic aganglionosis, we resect the natural reservoir and connect high-motility bowel to the anal canal.Open
StatementIn Hirschsprung disease with or without total colonic aganglionosis, we resect the natural reservoir and connect high-motility bowel to the anal canal.Open
StatementAdults who receive ileoanal anastomosis for ulcerative colitis live the rest of their lives trying to avoid accidents, with terrible diarrhea.Open
StatementAdults who receive ileoanal anastomosis for ulcerative colitis live the rest of their lives trying to avoid accidents, with terrible diarrhea.Open
StatementCreating intentional stasis in the bowel leads to bacterial proliferation, colitis, inflammatory changes, secretory diarrhea, and worsening symptoms.Open
StatementCreating intentional stasis in the bowel leads to bacterial proliferation, colitis, inflammatory changes, secretory diarrhea, and worsening symptoms.Open
QuotationThe baby is happy with the ileostomy. The only unhappy people are others, but not the baby.Open
StatementIf you wait until the patient is toilet-trained for urine (usually around 3 years), then close the ileostomy, the baby becomes totally trained for stool soon afterward, provided you did a correct operation and preserved the anal canal.Open
QuotationThe baby is happy with the ileostomy. The only unhappy people are others, but not the baby.Open
StatementIf you wait until the patient is toilet-trained for urine (usually around 3 years), then close the ileostomy, the baby becomes totally trained for stool soon afterward, provided you did a correct operation and preserved the anal canal.Open
StatementPatients with total colonic aganglionosis have a high incidence of enterocolitis and will most likely need rectal irrigation.Open
StatementPatients with total colonic aganglionosis have a high incidence of enterocolitis and will most likely need rectal irrigation.Open
StatementIt's not easy to do rectal irrigations in a 3-year-old with severe diaper rash who doesn't want anything near the anus.Open
StatementIt's not easy to do rectal irrigations in a 3-year-old with severe diaper rash who doesn't want anything near the anus.Open
StatementFor contrast enema in Hirschsprung, do not overfill the colon—some technicians open the contrast and fill the entire colon, even the small bowel, and patients vomit.Open
StatementFor contrast enema in Hirschsprung, do not overfill the colon—some technicians open the contrast and fill the entire colon, even the small bowel, and patients vomit.Open
StatementA good contrast study for Hirschsprung requires multiple films, including lateral views, to see if dilation goes all the way down to the pubococcygeal line.Open
StatementA good contrast study for Hirschsprung requires multiple films, including lateral views, to see if dilation goes all the way down to the pubococcygeal line.Open
StatementIf dilation on contrast enema goes all the way down to the pubococcygeal line, that is not Hirschsprung, even if the biopsy says something different.Open
StatementIf dilation on contrast enema goes all the way down to the pubococcygeal line, that is not Hirschsprung, even if the biopsy says something different.Open
StatementFor newborn babies with obvious Hirschsprung on contrast enema, keep NPO, place nasogastric tube, place central line, give parenteral nutrition, and do irrigations until abdomen is completely flat and you obtain bile through irrigations—then the baby is ready for surgery.Open
StatementFor newborn babies with obvious Hirschsprung on contrast enema, keep NPO, place nasogastric tube, place central line, give parenteral nutrition, and do irrigations until abdomen is completely flat and you obtain bile through irrigations—then the baby is ready for surgery.Open
StatementThere are two types of Hirschsprung disease: a benign type where patients never have enterocolitis and can go for years with huge megacolon, and a bad group with manifestations during the newborn period who have great tendency to suffer enterocolitis even with good operation.Open
StatementThere are two types of Hirschsprung disease: a benign type where patients never have enterocolitis and can go for years with huge megacolon, and a bad group with manifestations during the newborn period who have great tendency to suffer enterocolitis even with good operation.Open
StatementCongenital central hypoventilation syndrome is associated with Ondine's curse, where patients stop breathing when they fall asleep.Open
CinciHirsch - Pathology of Hirschprung Disease
StatementAfter 6 months of age, the clinical picture of Hirschsprung disease is often so obvious that suction biopsy becomes less relevant.Open
StatementAfter 6 months of age, the clinical picture of Hirschsprung disease is often so obvious that suction biopsy becomes less relevant.Open
QuotationI don't believe that the suction rectal biopsy is the main element for the diagnosis, and it's it's one extra piece of information.Open
QuotationI don't believe that the suction rectal biopsy is the main element for the diagnosis, and it's it's one extra piece of information.Open
StatementThe suction rectal biopsy is not the main element for diagnosis but one extra piece of information, particularly useful in newborn babies.Open
StatementThe suction rectal biopsy is not the main element for diagnosis but one extra piece of information, particularly useful in newborn babies.Open
StatementThere is not a single topographic study of intestinal neuronal dysplasia describing the anatomical extent of the histological abnormality.Open
QuotationThere is not a single topographic study of so-called neuronal intestinal dysplasia, and yet people are doing resections, treating with laxatives, treating with enemas, and but they don't, they don't describe that they resected the the histologically abnormal portion of the colon.Open
QuotationThere is not a single topographic study of so-called neuronal intestinal dysplasia, and yet people are doing resections, treating with laxatives, treating with enemas, and but they don't, they don't describe that they resected the the histologically abnormal portion of the colon.Open
StatementThere is not a single topographic study of intestinal neuronal dysplasia describing the anatomical extent of the histological abnormality.Open
StatementWithout topographic studies, there is no basis for resecting histologically abnormal portions of colon in IND, unlike in Hirschsprung disease where pathology determines resection extent.Open
StatementWithout topographic studies, there is no basis for resecting histologically abnormal portions of colon in IND, unlike in Hirschsprung disease where pathology determines resection extent.Open
StatementThe concept of IND is currently not clinically relevant, though there may be secrets in histological characterization of motility disorders to be studied in the future.Open
StatementThe concept of IND is currently not clinically relevant, though there may be secrets in histological characterization of motility disorders to be studied in the future.Open
QuotationThe concept is still irrelevant. I'm sure that there are many secrets in the in histological characterization of the many motility disorders that have to be studied in the future.Open
QuotationThe concept is still irrelevant. I'm sure that there are many secrets in the in histological characterization of the many motility disorders that have to be studied in the future.Open
Hirschsprung Disease: Surgical Procedures
StatementBetween 75 and 80% of the time, transanal dissection with sequential biopsies reaches normal ganglionic bowel; in the other 20%, conversion to abdominal approach is needed.Open
StatementIf you start transanally and cannot mobilize more bowel, you simply go into the abdomen and continue the resection—this is not considered a complication.Open
QuotationI don't consider that a complication. I don't consider that a problem. You see they go and do what, what you was, what you were going to do in the first place.Open
StatementThe main problems in Hirschsprung disease are not related to the surgical approach (laparoscopic vs. transanal) but to inexperience and technical incapacity of the surgeon that damages the patient permanently.Open
QuotationThe main problems that we have seen in history from disease are not related with the approach that you got are related with the inexperience and technical incapacity of the surgeon that damaged the baby on a permanent basis both ways laparoscopically or transenally, a bad surgeon will damage the patient.Open
QuotationI never heard people complaining about the scar or not the scar. I see patients complaining about fecal incontinence remaining that's the real problem, not the size of the scar.Open
StatementPatients complain about fecal incontinence, not about the size of the scar—that is the real problem.Open
StatementThe Lone Star retractor hooks should be placed at the pectinate line itself, not in the anal canal, to protect the anal canal by definition.Open
QuotationThe surgeons should be careful not to stretch too much because you start the dissection and you forget you want to be comfortable. You put retractors and I walk into the operating room and see sometimes a big hole that what used to be the anus becomes a huge hole and that is damaging by definition the sphincter mechanism.Open
StatementSurgeons should be careful not to stretch too much during transanal dissection because excessive stretching damages the sphincter mechanism by definition, potentially causing fecal incontinence.Open
StatementThe keys to avoiding strictures and retractions are: respecting the pectinate line and anal canal, not stretching the anus too much, mobilizing the rectum to ensure normal ganglionic bowel with good blood supply, and performing anastomosis with no tension.Open
StatementProne position is preferred over lithotomy position for transanal procedures because in lithotomy the surgeon is the only one who can see, the field is vertical causing instrument loss, and the scrub nurse has difficulty seeing.Open
StatementLeaving 1-2 centimeters of aganglionosis does not explain why patients don't behave well—some patients operated with exactly the same technique do beautifully while others have enterocolitis symptoms, and we don't know why.Open
QuotationI don't blame on the 1 centimeter or 1.5 centimeters or 2 centimeters of ganglionnosis to explain why a patient doesn't behave well.Open
QuotationWe have been obsessed with the idea of leaving 1 centimeter or two a ganglionnosis, and that explains symptoms. That simply is not true from my point of view.Open
StatementThe majority of patients who come with symptoms of retention (enterocolitis or constipation) after pull-through do not have a portion of ganglionic bowel left—they simply behave like that and we do not know why.Open
QuotationWhen we remove the rectum of a human being, we are already affecting seriously the mechanisms of bowel control because we are removing the natural reservoir of the patient.Open
StatementWhen we remove the rectum of a human being, we are already seriously affecting the mechanisms of bowel control because we are removing the natural reservoir of the patient.Open
StatementEven adult ulcerative colitis patients with perfect operations have problems with bowel control—they have accidents at night—because we are connecting a piece of colon that is constantly moving to where the rectum normally rests.Open
StatementEven in patients with a very well preserved anal canal, some children have different degrees of fecal incontinence after Hirschsprung surgery.Open
QuotationWe have not been discussing enough in our pediatric surgical meetings the problem of fecal incontinence that is much more common than we believe.Open
StatementFecal incontinence in Hirschsprung disease is much more common than we believe, and we have not been discussing it enough in pediatric surgical meetings.Open
StatementIf you preserve the pectinate line, by definition you are preserving this crucial part of the bowel.Open
StatementPatients are born with bowel control—we provoke the fecal incontinence through surgical technique.Open
QuotationThe patients are born with bowel control. We provoke the fecal incontinence.Open
StatementThe anterior dissection of the rectal wall must be conducted in a very meticulous way because the rectum has a common wall with the vagina and the prostatic urethra—fistulas to the vagina or urinary tract are unacceptable complications.Open
StatementIf you see fat around the rectum during full-thickness dissection, you can get closer to the rectum because that means you are not in the real rectal wall.Open
QuotationIf you stay right in the bowel wall, you will not provoke the innervation of the urinary tract.Open
StatementIf you stay right on the bowel wall during dissection, you will not provoke denervation of the urinary tract.Open
StatementWaiting until the patient is toilet trained for urine (usually around 3 years of age) before closing the ileostomy results in the baby becoming totally trained for stool soon after closure, provided you did a correct operation and preserved the anal canal.Open
QuotationThe baby is happy with the ileostomy. The only unhappy people are others, but not the baby.Open
StatementIf you take a child that has been traumatized with rectal maneuvers and try to do rectal irrigations, there will be problems, whereas if a patient has an ileostomy and the mother does rectal irrigations once in a while with a soft catheter, the baby will accept that.Open
QuotationIf you take a child that has been traumatized with rectal maneuvers and you try to do rectal irrigations, it's going to be a lot of problems, whereas if a patient has an ileostomy and the mother does rectal irrigations once in a while with a soft catheter, the baby will accept that.Open
StatementPatients with total colonic aganglionosis have a high incidence of enterocolitis and will most likely need rectal irrigation, which is difficult to do in a 3-year-old with severe diaper rash who doesn't want anything near the anus.Open
StatementThere are two big types of Hirschsprung disease: a benign type where patients never have enterocolitis and can go for years with huge megacolon, and a bad group with manifestations during the newborn period who have great tendency to suffer enterocolitis even with a good operation.Open
Panel Discussion and Case Presentation Part II: Pediatric Bowel Management 2013
StatementIn Spain, de la Torre technique is used; for total colonic aganglionosis, Lester Martin procedure is used.Open
StatementIn Spain, de la Torre technique is used; for total colonic aganglionosis, Lester Martin procedure is used.Open
StatementWhen the anal canal is destroyed, the patient will not have bowel control; with total colonic aganglionosis producing liquid stool, there is no bowel management possible.Open
StatementWhen the anal canal is destroyed, the patient will not have bowel control; with total colonic aganglionosis producing liquid stool, there is no bowel management possible.Open
StatementA permanent stoma is indicated when the anal canal is destroyed and the patient has total colonic aganglionosis.Open
StatementA permanent stoma is indicated when the anal canal is destroyed and the patient has total colonic aganglionosis.Open
StatementSometimes constipating diet and fiber are tried to convince parents that there is no other option except permanent stoma; this is one of the few indications for permanent stoma.Open
StatementSometimes constipating diet and fiber are tried to convince parents that there is no other option except permanent stoma; this is one of the few indications for permanent stoma.Open
QuotationMost pediatricians all over the world don't know the difference between enterocolitis and gastroenteritis.Open
StatementMost pediatricians worldwide do not know the difference between enterocolitis and gastroenteritis; they do not understand the entity called post-Hirschsprung enterocolitis.Open
QuotationMost pediatricians all over the world don't know the difference between enterocolitis and gastroenteritis.Open
StatementMost pediatricians worldwide do not know the difference between enterocolitis and gastroenteritis; they do not understand the entity called post-Hirschsprung enterocolitis.Open
StatementPediatricians do not understand why irrigations are necessary; they see dilated bowel on X-ray and think it is intestinal obstruction, not recognizing the entity itself.Open
StatementPediatricians do not understand why irrigations are necessary; they see dilated bowel on X-ray and think it is intestinal obstruction, not recognizing the entity itself.Open
StatementWhen starting dissection 2 cm above the dentate line and pulling bowel through, the upper mucosa is often damaged, and the anastomosis ends up about 1 cm above the dentate line.Open
StatementWhen starting dissection 2 cm above the dentate line and pulling bowel through, the upper mucosa is often damaged, and the anastomosis ends up about 1 cm above the dentate line.Open
StatementDr. Pena does not believe that leaving 1-2 cm of rectal mucosa is the simple cause of enterocolitis.Open
StatementDr. Pena does not believe that leaving 1-2 cm of rectal mucosa is the simple cause of enterocolitis.Open
StatementA group in New York doing neonatal Soave primary procedures reported zero enterocolitis; when Dr. Pena followed some of those patients, many had fecal incontinence.Open
StatementA group in New York doing neonatal Soave primary procedures reported zero enterocolitis; when Dr. Pena followed some of those patients, many had fecal incontinence.Open
QuotationIf you produce fecal incontinence in the patient, the enterocolitis is zero.Open
StatementIf you produce fecal incontinence in a patient, enterocolitis is zero; a patient with destroyed anal canal is equivalent to a stoma, and patients with stomas rarely have enterocolitis.Open
StatementIf you produce fecal incontinence in a patient, enterocolitis is zero; a patient with destroyed anal canal is equivalent to a stoma, and patients with stomas rarely have enterocolitis.Open
QuotationIf you produce fecal incontinence in the patient, the enterocolitis is zero.Open
StatementA good operation preserving the sphincter and anal canal creates sphincter closure, which creates stasis, and stasis produces enterocolitis.Open
StatementA good operation preserving the sphincter and anal canal creates sphincter closure, which creates stasis, and stasis produces enterocolitis.Open
QuotationWe don't know why the patients have enterocolitis, but if you produce, if you have very little enterocolitis, chances are that you are damaging the anal canal.Open
QuotationWe don't know why the patients have enterocolitis, but if you produce, if you have very little enterocolitis, chances are that you are damaging the anal canal.Open
QuotationI prefer to deal with enterocolitis than with fecal incontinence. Fecal incontinence is for life.Open
StatementDr. Pena prefers to deal with enterocolitis rather than fecal incontinence; fecal incontinence is for life.Open
QuotationI prefer to deal with enterocolitis than with fecal incontinence. Fecal incontinence is for life.Open
StatementDr. Pena prefers to deal with enterocolitis rather than fecal incontinence; fecal incontinence is for life.Open
StatementDr. Pena's incidence of enterocolitis in pull-through patients is about 30%.Open
StatementDr. Pena's incidence of enterocolitis in pull-through patients is about 30%.Open
StatementResecting the rectosigmoid introduces a major pathophysiological change; children with perfect pull-throughs preserving the anal canal sometimes have toilet-training problems without explanation.Open
StatementResecting the rectosigmoid introduces a major pathophysiological change; children with perfect pull-throughs preserving the anal canal sometimes have toilet-training problems without explanation.Open
StatementHyperactive children with attention deficit disorder have more toilet-training problems because they have a piece of colon that does not act like a reservoir, connected to the rectum and moving constantly; significant cooperation from the child is required.Open
StatementHyperactive children with attention deficit disorder have more toilet-training problems because they have a piece of colon that does not act like a reservoir, connected to the rectum and moving constantly; significant cooperation from the child is required.Open
StatementEven with a perfect operation, patients may have certain toilet-training problems; if the anal canal is destroyed, they will be totally incontinent; partial anal canal destruction causes more problems.Open
StatementEven with a perfect operation, patients may have certain toilet-training problems; if the anal canal is destroyed, they will be totally incontinent; partial anal canal destruction causes more problems.Open
StatementDr. Pena does not find rectal manometry useful in Hirschsprung disease, constipation, or anorectal malformations after many years of experience.Open
StatementDr. Pena does not find rectal manometry useful in Hirschsprung disease, constipation, or anorectal malformations after many years of experience.Open
StatementPatients are discharged with three irrigations per day and metronidazole (Flagyl); every month the number of irrigations is decreased.Open
StatementPatients are discharged with three irrigations per day and metronidazole (Flagyl); every month the number of irrigations is decreased.Open
StatementIf decreasing irrigations causes recurrent enterocolitis and the patient does not tolerate lack of irrigation, and by 6 months post-op the patient is still on irrigations, parents become very nervous; at that point, other options are discussed.Open
StatementIf decreasing irrigations causes recurrent enterocolitis and the patient does not tolerate lack of irrigation, and by 6 months post-op the patient is still on irrigations, parents become very nervous; at that point, other options are discussed.Open
StatementFor refractory enterocolitis, another option is further resection of normal ganglionic colon to remove more.Open
StatementFor refractory enterocolitis, another option is further resection of normal ganglionic colon to remove more.Open
StatementHirschsprung disease is much more than ganglion cells vs. no ganglion cells; we do not know why some patients never have enterocolitis and toilet-train early, behaving like normal children, while others have severe enterocolitis from day one.Open
StatementHirschsprung disease is much more than ganglion cells vs. no ganglion cells; we do not know why some patients never have enterocolitis and toilet-train early, behaving like normal children, while others have severe enterocolitis from day one.Open
Statement'Benign Hirschsprung disease' patients present at 8-10 years old with classic imaging and abdominal distention but never had enterocolitis, grew and developed normally, and do very well after surgery.Open
Statement'Benign Hirschsprung disease' patients present at 8-10 years old with classic imaging and abdominal distention but never had enterocolitis, grew and developed normally, and do very well after surgery.Open
StatementIn the United States, earlier diagnosis is being made of patients with 'bad Hirschsprung'—enterocolitis from day one, very sick, and high incidence of enterocolitis after surgery.Open
StatementIn the United States, earlier diagnosis is being made of patients with 'bad Hirschsprung'—enterocolitis from day one, very sick, and high incidence of enterocolitis after surgery.Open
QuotationThere is a lot of things that we don't know about Higp disease. Some bowel has no the story is much more than absent ganglion cells.Open
QuotationThere is a lot of things that we don't know about Higp disease. Some bowel has no the story is much more than absent ganglion cells.Open
StatementThere is much we do not know about Hirschsprung disease; the story is much more than absent ganglion cells, and taking bowel with normal ganglion cells down does not mean that bowel is 100% normal.Open
StatementThere is much we do not know about Hirschsprung disease; the story is much more than absent ganglion cells, and taking bowel with normal ganglion cells down does not mean that bowel is 100% normal.Open
StatementSome believe that ganglionic bowel may have neuronal intestinal dysplasia, but this is a very controversial histopathological diagnosis; we do not know what is wrong and must learn much more.Open
StatementSome believe that ganglionic bowel may have neuronal intestinal dysplasia, but this is a very controversial histopathological diagnosis; we do not know what is wrong and must learn much more.Open
StatementDr. Pena is skeptical that the cuff produces obstruction; to believe it, he would need to see the cuff producing real obstruction manifested by very dilated colon above the cuff, which is very unusual.Open
StatementDr. Pena is skeptical that the cuff produces obstruction; to believe it, he would need to see the cuff producing real obstruction manifested by very dilated colon above the cuff, which is very unusual.Open
StatementDr. Mark Levitt has experience dealing with obstructive cuffs transanally; laparoscopic approach is not a bad idea but Dr. Pena has never heard of it being done laparoscopically.Open
StatementDr. Mark Levitt has experience dealing with obstructive cuffs transanally; laparoscopic approach is not a bad idea but Dr. Pena has never heard of it being done laparoscopically.Open
StatementPatients with total fecal incontinence have no enterocolitis; all operations moving toward fecal incontinence (myectomies, myotomies, Botox, massive dilatation, putting 3 fingers in the rectum) are temporary or permanent moves toward incontinence.Open
StatementPatients with total fecal incontinence have no enterocolitis; all operations moving toward fecal incontinence (myectomies, myotomies, Botox, massive dilatation, putting 3 fingers in the rectum) are temporary or permanent moves toward incontinence.Open
StatementPatients subjected to myotomies, myectomies, or repeated Botox injections eventually develop more severe fecal incontinence.Open
StatementPatients subjected to myotomies, myectomies, or repeated Botox injections eventually develop more severe fecal incontinence.Open
StatementDr. Pena does not believe in myectomy/myotomy/Botox procedures and does not use Botox.Open
StatementDr. Pena does not believe in myectomy/myotomy/Botox procedures and does not use Botox.Open
Cloacal Exstrophy with Dr. Alberto Peña
StatementCloacal exstrophy is a spectrum of congenital malformations affecting the gastrointestinal/colorectal area, urogenital tract, spine and cord, and sometimes lower extremity motion.Open
StatementCloacal exstrophy is a spectrum of congenital malformations affecting the gastrointestinal/colorectal area, urogenital tract, spine and cord, and sometimes lower extremity motion.Open
StatementBabies with cloacal exstrophy are born with an omphalocele, bladder exstrophy (two separated hemibladders), open cecum between the hemibladders, and separated pubic bones.Open
StatementBabies with cloacal exstrophy are born with an omphalocele, bladder exstrophy (two separated hemibladders), open cecum between the hemibladders, and separated pubic bones.Open
StatementThe small bowel can become exstrophic through the ileocecal valve, creating an 'elephant trunk' appearance.Open
StatementThe small bowel can become exstrophic through the ileocecal valve, creating an 'elephant trunk' appearance.Open
StatementMale patients have two separated hemiphalluses with normal gonads; female patients have two hemivaginas below the exstrophic bladder leading to two hemiuteri.Open
StatementMale patients have two separated hemiphalluses with normal gonads; female patients have two hemivaginas below the exstrophic bladder leading to two hemiuteri.Open
StatementCloacal 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.Open
StatementCloacal 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.Open
StatementThe amount of colon present at birth has very important implications for the patient's management and outcomes.Open
StatementThe amount of colon present at birth has very important implications for the patient's management and outcomes.Open
StatementA 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.Open
StatementA 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.Open
StatementWhile 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.Open
StatementWhile 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.Open
StatementHistorical practice was to perform bilateral gonadectomy, remove hemiphalluses, create a vagina with bowel, and assign female gender to XY patients with cloacal exstrophy.Open
StatementHistorical practice was to perform bilateral gonadectomy, remove hemiphalluses, create a vagina with bowel, and assign female gender to XY patients with cloacal exstrophy.Open
StatementPatients 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.Open
StatementPatients 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.Open
StatementPatients 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.Open
StatementPatients 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.Open
Quotationwho 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.Open
Quotationbeing a male is much more than having a phallusOpen
StatementCurrent consensus is that XY patients should be raised as male, with pediatric urologists and plastic surgeons working on phallus reconstruction techniques.Open
StatementCurrent consensus is that XY patients should be raised as male, with pediatric urologists and plastic surgeons working on phallus reconstruction techniques.Open
StatementWhen a prominent pediatric urologist dominates management, patients receive good urologic attention but inadequate gastrointestinal care; the reverse occurs when pediatric surgeons dominate.Open
StatementWhen a prominent pediatric urologist dominates management, patients receive good urologic attention but inadequate gastrointestinal care; the reverse occurs when pediatric surgeons dominate.Open
StatementThe 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.Open
StatementThe 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.Open
StatementIt is very common but very harmful for pediatric surgeons to simply create an ileostomy, leaving all colon distally attached to the urinary tract.Open
StatementIt is very common but very harmful for pediatric surgeons to simply create an ileostomy, leaving all colon distally attached to the urinary tract.Open
QuotationThat is a very bad thing to do, and the patient will suffer several consequences because of thatOpen
QuotationThat is a very bad thing to do, and the patient will suffer several consequences because of thatOpen
StatementLeaving colon attached to the urinary tract creates a congenital bladder augmentation that causes hyperchloremic acidosis from urine absorption, interfering with growth and development.Open
StatementLeaving colon attached to the urinary tract creates a congenital bladder augmentation that causes hyperchloremic acidosis from urine absorption, interfering with growth and development.Open
QuotationIsn't that beautiful from your point of view?Open
QuotationIsn't that beautiful from your point of view?Open
StatementDefunctionalized colonic tissue left distally will not grow; colon requires passage of fecal matter through its lumen to grow.Open
StatementDefunctionalized colonic tissue left distally will not grow; colon requires passage of fecal matter through its lumen to grow.Open
StatementThe 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.Open
StatementThe 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.Open
StatementPatients who received ileostomy with defunctionalized colon present at 2-3 years with poor growth, hyperchloremic acidosis managed by nephrologists, and large ileostomy losses.Open
StatementPatients who received ileostomy with defunctionalized colon present at 2-3 years with poor growth, hyperchloremic acidosis managed by nephrologists, and large ileostomy losses.Open
StatementThe 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.Open
StatementThe 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.Open
StatementSurgeons 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.Open
StatementSurgeons 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.Open
QuotationDo not leave gastrointestinal tract inside the functionalized. That's the main, main thing.Open
QuotationDo not leave gastrointestinal tract inside the functionalized. That's the main, main thing.Open
StatementSome 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.Open
StatementSome 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.Open
StatementEven after osteotomy, it is very difficult to see a cloacal exstrophy patient with pubic bones completely together; they usually remain separated.Open
StatementEven after osteotomy, it is very difficult to see a cloacal exstrophy patient with pubic bones completely together; they usually remain separated.Open
StatementEven a technically correct end colostomy often has poor motility, and babies may not pass stool easily, sometimes developing bacterial overgrowth similar to Hirschsprung disease.Open
StatementEven a technically correct end colostomy often has poor motility, and babies may not pass stool easily, sometimes developing bacterial overgrowth similar to Hirschsprung disease.Open
StatementFamilies must be taught to irrigate the colostomy with small volumes of saline through a tube to evacuate fecal material when peristalsis is inadequate.Open
StatementFamilies must be taught to irrigate the colostomy with small volumes of saline through a tube to evacuate fecal material when peristalsis is inadequate.Open
StatementDecision-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).Open
StatementDecision-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).Open
StatementMost cloacal exstrophy patients have an inadequate, tiny bladder requiring bladder augmentation with gastrointestinal tract, necessitating coordination between pediatric surgery and urology.Open
StatementMost cloacal exstrophy patients have an inadequate, tiny bladder requiring bladder augmentation with gastrointestinal tract, necessitating coordination between pediatric surgery and urology.Open
StatementPatients 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.Open
StatementPatients 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.Open
StatementPull-through is only considered for patients with capacity to form solid stool (adequate colon), as bowel management only works with solid stool.Open
StatementPull-through is only considered for patients with capacity to form solid stool (adequate colon), as bowel management only works with solid stool.Open
StatementIt 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).Open
StatementIt 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).Open
StatementPediatric surgeons should not underestimate the growth capacity of tiny colonic pieces during the newborn period; even small segments will grow over three years if fecal stream passes through them.Open
StatementPediatric surgeons should not underestimate the growth capacity of tiny colonic pieces during the newborn period; even small segments will grow over three years if fecal stream passes through them.Open
StatementAnnual contrast studies through the colostomy (retrograde injection) are performed to assess colonic growth.Open
StatementAnnual contrast studies through the colostomy (retrograde injection) are performed to assess colonic growth.Open
StatementBefore committing to pull-through, a trial bowel management program is performed through the colostomy: enemas are given to empty the colonic pouch, and if the patient stays 24 hours without stool in the colostomy bag, pull-through is likely to succeed.Open
StatementBefore committing to pull-through, a trial bowel management program is performed through the colostomy: enemas are given to empty the colonic pouch, and if the patient stays 24 hours without stool in the colostomy bag, pull-through is likely to succeed.Open
StatementBowel management trial through colostomy is typically started after age 3 when families consider avoiding the stoma for school.Open
StatementBowel management trial through colostomy is typically started after age 3 when families consider avoiding the stoma for school.Open
StatementIf a patient has very little colon and cannot form solid stool, the urologist is free to use bowel for bladder augmentation; if the patient has borderline colon, the urologist must use stomach for augmentation to preserve bowel for fecal function.Open
StatementIf a patient has very little colon and cannot form solid stool, the urologist is free to use bowel for bladder augmentation; if the patient has borderline colon, the urologist must use stomach for augmentation to preserve bowel for fecal function.Open
StatementThe colon to be pulled through is the most posterior structure in the pelvis, with the bladder and augmentation anterior to it; therefore, bladder augmentation must not be done before deciding on pull-through, or accessing the colon will be extremely difficult.Open
StatementThe colon to be pulled through is the most posterior structure in the pelvis, with the bladder and augmentation anterior to it; therefore, bladder augmentation must not be done before deciding on pull-through, or accessing the colon will be extremely difficult.Open
StatementContrast enema through the colostomy can distinguish true liquid stool from paradoxical diarrhea (liquid stool around solid fecal impaction).Open
StatementContrast enema through the colostomy can distinguish true liquid stool from paradoxical diarrhea (liquid stool around solid fecal impaction).Open
StatementPull-through and bladder augmentation are ideally performed together in a single operation lasting approximately 12 hours, with pediatric surgery going first (posterior dissection) followed by urology (anterior augmentation).Open
StatementPull-through and bladder augmentation are ideally performed together in a single operation lasting approximately 12 hours, with pediatric surgery going first (posterior dissection) followed by urology (anterior augmentation).Open
StatementDuring pull-through, if the patient has one or two appendices, a Malone appendicostomy can be created for antegrade enema administration, as the appendix remains in the abdomen when colon is pulled down.Open
StatementDuring pull-through, if the patient has one or two appendices, a Malone appendicostomy can be created for antegrade enema administration, as the appendix remains in the abdomen when colon is pulled down.Open
StatementUrologists almost never use colon for bladder augmentation in cloacal exstrophy because colon is needed to form solid stool; they typically use small bowel or stomach.Open
StatementUrologists almost never use colon for bladder augmentation in cloacal exstrophy because colon is needed to form solid stool; they typically use small bowel or stomach.Open
StatementOccasionally, when a patient has a giant colonic pouch with very poor motility, the poor motility makes it good for bowel management (irrigate once daily, stays clean between irrigations), and a piece can be shared with urology for augmentation.Open
StatementOccasionally, when a patient has a giant colonic pouch with very poor motility, the poor motility makes it good for bowel management (irrigate once daily, stays clean between irrigations), and a piece can be shared with urology for augmentation.Open
StatementMidline abdominal incision from xiphoid to pubis is used for pull-through to preserve the flanks and quadrants for potential future stomas.Open
StatementMidline abdominal incision from xiphoid to pubis is used for pull-through to preserve the flanks and quadrants for potential future stomas.Open
StatementThe colostomy is circumferentially dissected and separated from the abdominal wall, then the blood supply is carefully studied because cloacal exstrophy patients have very bizarre, aberrant vascular anatomy.Open
StatementThe colostomy is circumferentially dissected and separated from the abdominal wall, then the blood supply is carefully studied because cloacal exstrophy patients have very bizarre, aberrant vascular anatomy.Open
StatementCareful observation of the vascular anatomy allows the surgeon to decide which vessels can be ligated to mobilize the colon to the perineum without compromising blood supply; sometimes no vessels need to be ligated.Open
StatementCareful observation of the vascular anatomy allows the surgeon to decide which vessels can be ligated to mobilize the colon to the perineum without compromising blood supply; sometimes no vessels need to be ligated.Open
StatementThe space behind the bladder is easily created, and the bowel is placed posteriorly; patients do not need prone positioning because the exstrophy makes everything anterior, so a supine frog-leg position provides full perineal access.Open
StatementThe space behind the bladder is easily created, and the bowel is placed posteriorly; patients do not need prone positioning because the exstrophy makes everything anterior, so a supine frog-leg position provides full perineal access.Open
StatementCloacal exstrophy patients are lifelong patients due to orthopedic problems (separated pubic bones causing abnormal gait) and spinal problems (tethered cord requiring neurosurgical follow-up and potential cord release).Open
StatementCloacal exstrophy patients are lifelong patients due to orthopedic problems (separated pubic bones causing abnormal gait) and spinal problems (tethered cord requiring neurosurgical follow-up and potential cord release).Open
StatementSome teenagers are unhappy about separated pubic bones causing abnormal gait (feet pointing laterally); some dedicated orthopedic surgeons have been able to bring pubic bones closer together.Open
StatementSome teenagers are unhappy about separated pubic bones causing abnormal gait (feet pointing laterally); some dedicated orthopedic surgeons have been able to bring pubic bones closer together.Open
StatementDuring pull-through, vaginal reconstruction is attempted by approximating the hemivaginas as much as possible; the degree of separation varies on the spectrum.Open
StatementDuring pull-through, vaginal reconstruction is attempted by approximating the hemivaginas as much as possible; the degree of separation varies on the spectrum.Open
StatementWhen hemivaginas are close with only a septum separating them superiorly, the septum is removed as high as possible; when vaginas run in completely different directions, one hemivagina may be removed, leaving the one with the better-looking cervix.Open
StatementWhen hemivaginas are close with only a septum separating them superiorly, the septum is removed as high as possible; when vaginas run in completely different directions, one hemivagina may be removed, leaving the one with the better-looking cervix.Open
StatementPatients with functional hemiuterus may become pregnant, but it is high-risk pregnancy requiring specialized pediatric gynecology follow-up; in general, pregnancy is not advised.Open
StatementPatients with functional hemiuterus may become pregnant, but it is high-risk pregnancy requiring specialized pediatric gynecology follow-up; in general, pregnancy is not advised.Open
StatementHemiuterus has a great tendency to produce miscarriages and premature labor; delivery must be by cesarean section due to limited abdominal space.Open
StatementHemiuterus has a great tendency to produce miscarriages and premature labor; delivery must be by cesarean section due to limited abdominal space.Open
StatementBladder augmentation requires a Mitrofanoff conduit (appendix or part of a long appendix, sometimes shared half for urology and half for Malone) for intermittent catheterization to empty the bladder.Open
StatementBladder augmentation requires a Mitrofanoff conduit (appendix or part of a long appendix, sometimes shared half for urology and half for Malone) for intermittent catheterization to empty the bladder.Open
StatementAugmented bladders produce large amounts of mucus; if mucus is not removed, it forms stones, so families must be taught to irrigate the bladder (not just empty it) to remove mucus and prevent infections.Open
StatementAugmented bladders produce large amounts of mucus; if mucus is not removed, it forms stones, so families must be taught to irrigate the bladder (not just empty it) to remove mucus and prevent infections.Open
StatementBladder irrigation is sometimes performed with gentamicin to ensure all mucus is removed.Open
StatementBladder irrigation is sometimes performed with gentamicin to ensure all mucus is removed.Open
StatementLifelong urologic follow-up is needed to monitor for reflux and kidney damage; the Mitrofanoff may stop working or leak urine, requiring revision or valve tightening.Open
StatementLifelong urologic follow-up is needed to monitor for reflux and kidney damage; the Mitrofanoff may stop working or leak urine, requiring revision or valve tightening.Open
StatementPatients transitioning to adult hospitals often feel uncomfortable because adult urologists and orthopedic surgeons lack experience with these congenital malformations; patients prefer to remain in pediatric environments even as adults.Open
StatementPatients transitioning to adult hospitals often feel uncomfortable because adult urologists and orthopedic surgeons lack experience with these congenital malformations; patients prefer to remain in pediatric environments even as adults.Open
StatementDr. Peña observes that cloacal exstrophy patients are particularly charming, intelligent, beautiful, and charismatic when they grow up, and some have energy to help others manage their own problems.Open
StatementDr. Peña observes that cloacal exstrophy patients are particularly charming, intelligent, beautiful, and charismatic when they grow up, and some have energy to help others manage their own problems.Open
StatementThe Pull-Through Network is a national organization (similar to cystic fibrosis or breast cancer organizations) for parents and patients with anorectal malformations, Hirschsprung disease, or bowel/urinary control problems; it has over 1000 members, holds annual meetings, and invites doctors to give talks.Open
StatementThe Pull-Through Network is a national organization (similar to cystic fibrosis or breast cancer organizations) for parents and patients with anorectal malformations, Hirschsprung disease, or bowel/urinary control problems; it has over 1000 members, holds annual meetings, and invites doctors to give talks.Open
StatementColorectal and urogenital problems have been left behind in terms of scientific approach and research funding because they are not 'elegant' problems—they involve stool, urine, and sex—and institutions are not eager to receive these patients.Open
StatementColorectal and urogenital problems have been left behind in terms of scientific approach and research funding because they are not 'elegant' problems—they involve stool, urine, and sex—and institutions are not eager to receive these patients.Open
StatementThe initial operation includes omphalocele closure (sometimes requiring staged closure), bowel-bladder separation, end colostomy creation, bladder closure attempt (sometimes requiring multiple stages), and in some institutions, pelvic osteotomy (either at initial operation or 2-3 months later).Open
StatementThe initial operation includes omphalocele closure (sometimes requiring staged closure), bowel-bladder separation, end colostomy creation, bladder closure attempt (sometimes requiring multiple stages), and in some institutions, pelvic osteotomy (either at initial operation or 2-3 months later).Open
StatementBetween the initial operation and pull-through, colostomy irrigation is often needed to manage poor colonic motility.Open
StatementBetween the initial operation and pull-through, colostomy irrigation is often needed to manage poor colonic motility.Open
StatementPrenatal diagnosis of anorectal and urogenital malformations is easier for complex defects (like cloacal exstrophy) than simple defects because complex cases have associated findings (spinal problems, absent bladder) visible on imaging.Open
StatementPrenatal diagnosis of anorectal and urogenital malformations is easier for complex defects (like cloacal exstrophy) than simple defects because complex cases have associated findings (spinal problems, absent bladder) visible on imaging.Open
StatementAbsent bladder on prenatal ultrasound (due to exstrophy) is a bad sign and can be detected as early as week 20 of pregnancy.Open
StatementAbsent bladder on prenatal ultrasound (due to exstrophy) is a bad sign and can be detected as early as week 20 of pregnancy.Open
StatementPrenatal diagnosis allows families to decide about pregnancy continuation and, if continuing, to deliver at a center with a multidisciplinary team experienced in these malformations.Open
StatementPrenatal diagnosis allows families to decide about pregnancy continuation and, if continuing, to deliver at a center with a multidisciplinary team experienced in these malformations.Open
StatementCertain malformations require centers of excellence where surgeons focus and sacrifice other areas of practice; attempting to train all surgeons superficially in complex conditions results in damaged children and no one becoming truly trained.Open
StatementCertain malformations require centers of excellence where surgeons focus and sacrifice other areas of practice; attempting to train all surgeons superficially in complex conditions results in damaged children and no one becoming truly trained.Open
Alberto's statements about Enterocolitis78 statements
StatementDr. Pena would perform rectal biopsy in a case of apparent small left colon because he cannot distinguish it from Hirschsprung disease.Open
StatementDr. Pena questions whether patients who develop the characteristic dilated Duhamel pouch actually had true Hirschsprung disease, since by definition aganglionic bowel should not distend even after 10-15 years.Open
QuotationI have been wondering why patients with Duhamel get this image. If you think about it, we have seen patients with his disease that are 1012, 1415 years old. We have never seen a dilated rectum in a patient with Hirp disease. By definition, Hirp disease is a condition in which the egg ganglionic segment doesn't get distended.Open
QuotationI wonder if all the patients that developed this traditional characteristic pouch of the duja male are actually patients that never had Hirk diseaseOpen
StatementDr. Pena recommends that when resecting a Duhamel pouch, the specimen should be oriented and the pathologist alerted to determine whether ganglion cells are present in the rectal portion, to test the hypothesis that these were misdiagnosed cases.Open
StatementDr. Pena states that if he sees a patient with megacolon and constipation on enema, he will not perform a rectal biopsy because it is a waste of time from his point of view.Open
Quotationif I see a patient with this contrast enema, first of all, I will not take a rectal biopsy. I will not take your time because it's a waste of time from my point of view.Open
StatementDr. Pena argues that taking a biopsy in a patient with idiopathic constipation risks getting an aganglionic result that does not mean anything, because the patient does not have Hirschsprung disease.Open
Quotationthere is one area. And the length of that area with no ganglion cells has never been accurately determined at different ages in the human being.Open
StatementThere is a normal physiologic aganglionic segment in the distal rectum, but its length at different ages (preterm, term, 6 months) has never been accurately determined in humans.Open
StatementSomeone could take a biopsy in the normal physiologic aganglionic area and get a result of no ganglion cells, which does not indicate Hirschsprung disease.Open
Quotationthe internal sphincter has been defined as a thickening, as you can see in this diagram. A thickening of the circular layer of the normal smooth muscle bowel. In other words, and I have never seen that thickening personallyOpen
StatementThe internal sphincter has been defined as a thickening of the circular layer of normal smooth muscle bowel, but Dr. Pena states he has never personally observed this thickening when opening normal rectums at different ages.Open
StatementIf the internal sphincter thickening exists, nobody has determined its exact boundaries at different ages.Open
StatementDr. Pena does not perform or recommend myectomies, myotomies, or botulinum toxin injections for internal sphincter achalasia because the target muscle area is undefined and these procedures paralyze muscle to facilitate stool passage rather than curing a condition of unknown origin.Open
Quotationalacia of alacia of the internal sphincter is a manometric concept, not an anatomic conceptOpen
StatementInternal sphincter achalasia is a manometric concept, not an anatomic concept, similar to the lower esophageal sphincter.Open
Radiology and Image Diagnosis of Hirschsprung Disease
StatementDr. Peña would not do biopsy if sure it is meconium ileus, but would do biopsy for a picture of small left colon because he does not know how to distinguish it from Hirschsprung.Open
QuotationI have been wondering why patients with Duhamel get this image. If you think about it, we have seen patients with his disease that are 1012, 1415 years old. We have never seen a dilated rectum in a patient with Hirp disease. By definition, Hirp disease is a condition in which the egg ganglionic segment doesn't get distended. Even if you go 10 years, 15 years, and yet in these patients we see the rectum, the rectum was opposed to this angryonic, very stretched with fecally impacted.Open
StatementDr. Peña wonders why Duhamel patients get dilated rectum; by definition Hirschsprung disease is a condition where the aganglionic segment does not get distended even after 10-15 years, yet in these patients the rectum is very stretched and fecally impacted.Open
StatementDr. Peña suspects that all patients who developed the traditional Duhamel pouch may never have had Hirschsprung disease; he invites surgeons to orient the resected pouch and have pathologists study whether there are ganglion cells in that rectum.Open
QuotationI suspect that this patient never had Hodgkin disease.Open
StatementDr. Peña states that if he sees a patient with a contrast enema showing redundant stool-filled colon with normal rectum, he will not take a rectal biopsy because it is a waste of time and the patient has no Hirschsprung disease.Open
Quotationif I see a patient with this contrast enema, first of all, I will not take a rectal biopsy. I will not take your time because it's a waste of time from my point of view.Open
Quotationthere is no way to differentiate the so-called ultra short with idiopathic constipationOpen
StatementDr. Peña states there is no way to differentiate so-called ultra-short segment Hirschsprung from idiopathic constipation.Open
StatementThe rectum has ganglion cells in normal ganglionic bowel, then there is one area with no ganglion cells, but the length of that normal aganglionic segment has never been accurately determined at different ages in humans.Open
Quotationthere is one area. And the length of that area with no ganglion cells has never been accurately determined at different ages in the human being.Open
StatementThere is no accurate study of the normal aganglionic segment length in a preemie, full-term baby, or 6-month-old baby; this is a challenge for young pediatric surgeons to contribute.Open
QuotationThat's another challenge for young pediatric surgeons. If you want to make a contribution. studies, this is a real challenge.Open
Quotationconceivably somebody could take a biopsy in that area and come up with the result of no ganglion cells. It doesn't mean anything for us.Open
StatementSomebody could take a biopsy in the normal aganglionic area and get a result of no ganglion cells, but it does not mean anything clinically.Open
Quotationthe internal sphincter has been defined as a thickening, as you can see in this diagram. A thickening of the circular layer of the normal smooth muscle bowel. In other words, and I have never seen that thickening personally, and I have opened these normal rectums in different, different ages. I have been looking at that. I don't have a microscope, but I have never seen that thickening.Open
StatementThe internal sphincter has been defined as a thickening of the circular layer of normal smooth muscle bowel, but Dr. Peña has never personally seen that thickening despite opening normal rectums at different ages.Open
StatementIf the internal sphincter thickening exists, nobody has determined the exact limit of that thickening at different ages, so it becomes a kind of magic or witchery type of diagnosis.Open
StatementInternal anal sphincter achalasia is a manometric concept, not an anatomic concept, similar to the lower esophageal sphincter and other sphincters that have been defined but never anatomically seen.Open
Quotationalacia of alacia of the internal sphincter is a manometric concept, not an anatomic conceptOpen
StatementDr. Peña does not perform myectomies or myotomies and does not recommend those procedures because we do not know where to inject or what area of muscle to target.Open
QuotationI don't pay attention to that and I don't perform myectomies and I don't recommend those myectomies and myotomies and buttock and those concepts because we don't know where where you inject. What area of the muscle, nobody knowsOpen
StatementBotox and similar injections paralyze whatever muscle is there and facilitate passing of stool, but they are not curing a condition of unknown origin.Open
Outcomes and Complications in Hirschsprung Disease
StatementDr. Peña has more re-operation cases than primary procedures because most pediatric surgeons believe they can operate on Hirschsprung disease, so he receives referrals for complex cases but usually not for primary Hirschsprung.Open
QuotationI have much more experience in re-operation than primaries or simply in patients that come because they suffer from complications.Open
StatementComplications and sequelae are classified into three categories: non-preventable (enterocolitis), partially preventable (constipation), and preventable (dehiscence, strictures, perianal/urogenital fistulas).Open
QuotationWe look forward for the, as I said before, our, the new generation of pediatric surgeons to go into serious scientific research to find out what's the problem with that, to explain this and to treat this adequately.Open
StatementThe rule is to resect not only the aganglionic segment but as much as possible the dilated portion of the colon.Open
QuotationIt's very sad because those are, I believe, 100% preventable, and yet we see them all the time.Open
QuotationI don't remember a patient that I operated, came back with symptoms. I re-biopsied and found no ganglion cells after one of my put-throughs.Open
StatementDr. Peña has never documented a case where a patient he operated on returned with symptoms, was re-biopsied, and was found to have aganglionosis in the pull-through area that previously had normal ganglion cells.Open
StatementDr. Peña resects 5 cm proximal to the last positive biopsy site when the bowel is not dilated.Open
QuotationWith no anal canal, there will be no bowel control, and liquid stool with no bowel control is really tragic.Open
StatementWith no anal canal, there will be no bowel control, and liquid stool with no bowel control is tragic; the quality of life is seriously affected.Open
QuotationWe pediatric surgeons don't talk much about the diaper problems, you know, the nurses, the mothers are the ones who struggle with this, and we don't discuss much of this, and we should do it because the quality of life of these patients is seriously affected.Open
StatementThe key part of evaluation for fecal incontinence after Hirschsprung surgery is examination under anesthesia to determine if the anal canal has been damaged.Open
StatementIf the anal canal is preserved, there is more chance of bowel control, though no guarantee; if damaged, the patient will most likely need enemas for life (bowel management for fecal incontinence).Open
QuotationIf the patient doesn't poop only every 3 days and has a dilated colon, that's hypermotility with and without colonic manometry.Open
QuotationI don't know how to call it that in other words, for that patient, we give laxatives and if the incontinence persists, then we go for bowel management.Open
QuotationA patient may have stricture and yet suffer from terrible diarrhea. And yet when you we go for the to see the films that you took that you take that you took um that you took after you put through shows um shows solid stored in the column.Open
StatementIn Hirschsprung disease, a patient may have a stricture and yet suffer from terrible diarrhea; stricture and stasis of stool in the colon produces secretory diarrhea even with normal ganglionic bowel.Open
QuotationStricture and stasis of stool in the colon in Kishman disease, even if you have a normal galionic bowel, produces secretory diarrhea.Open
QuotationMost of the mistakes that we saw in the old time were consecutive to lack of exposure.Open
QuotationThe priority is to do an operation that you finish happy. The happiness of the surgeon at the end of the procedure is very important, and you look at the patients and you say, you know something, I'm happy and the family feels happy and you sleep well.Open
StatementThe priority in surgery is to finish the operation happy; the happiness of the surgeon at the end of the procedure is very important, the family feels it, and you sleep well.Open
QuotationEnemas are contraindicated in Hik disease irrigation. We do it on prophylactic.Open
QuotationThe enterocolitis is such a bad complication, such a bad problem. The baby sometimes died from home to the hospital and that's why we don't want to run into that.Open
StatementEnterocolitis is such a bad complication that babies sometimes die between home and hospital; prophylactic management is preferred over having patients return with distention and enterocolitis two weeks post-operatively.Open
QuotationEnemas consist in giving a certain amount of fluid with the, with the, with the ingredients that are irritant to the bowels that provoke a bowel contraction, but the, the disease consists in not having the normal peristalsis therefore are not indicated.Open
StatementEnemas consist of giving fluid with ingredients that irritate the bowel to provoke contraction; they are contraindicated in Hirschsprung disease because the disease involves absent normal peristalsis.Open
StatementIrrigation means passing a tube through the rectum; liquid stool comes out through the tube lumen, and only a small amount of saline is passed to clear the tube.Open
QuotationIrrigation means to take a tube like you saw in the video, pass it through the rectum, and through the lumen of the tube, liquid stool will come out. We just pass a small amount of saline to clear the lumen of the tube.Open
QuotationIf you don't do, if you decide not to go for irrigations, my suggestion is to be very careful. And follow the patient. First of all, it's very important how reliable is the mother for you.Open
QuotationThere are mothers that are very reliable. They will be calling you every 3 days, and there are mothers that just disappear, and then you hear that they went to the emergency room in a general hospital where they don't know what enterocolitis is all about.Open
StatementDilations are performed twice daily with diaper changes; the dilation also provokes bowel movements in babies.Open
StatementThe association between Hirschsprung disease and anorectal malformations is extremely uncommon; Dr. Peña has seen only two demonstrated cases in his career.Open
QuotationThe association between Hirschsprung disease and anorectal malformations is extremely uncommon; personally, I have seen in my life two cases, demonstrated cases of Hishman with an rectum malformation, which is a very bad association.Open
StatementMany patients with anorectal malformations suffer from constipation; surgeons accustomed to suspecting Hirschsprung in constipation cases have taken biopsies showing no ganglion cells (which can occur in normal children for various reasons) and performed pull-throughs, leaving patients incontinent.Open
QuotationBefore you make that uh diagnosis and before you embark yourself in a push-through for history, a patient with anorectal malformation, think about it. Most likely it's not Hines.Open
StatementBefore making a Hirschsprung diagnosis and embarking on a pull-through in a patient with anorectal malformation, think carefully—most likely it is not Hirschsprung; many patients suffer from constipation but very few have Hirschsprung.Open
Alberto's statements about Functional Colorectal Disorders & Bowel Management8 statements
Tell Me a Story: Pediatric Surgeons Putting Colorectal Center on the Map
QuotationRevealing episodes in your life, you read a book, you meet somebody, you, you hear some music, you see some painting, you see an operation that's, and then you say, I want this, and that changes your life.Open
StatementDr. Alberto Peña is the director of the Colorectal Center at Cincinnati Children's Hospital.Open
QuotationI'm the director of the Colorectal Center.Open
StatementDr. Peña advises young surgeons asking about the key to success to focus on doing the best version of whatever operation they are performing, even routine procedures like hernias.Open
QuotationMany surgeons come and many young surgeons say, what should I do? What's the key for success? And I said, What are you doing tomorrow? I said, they said, I'm doing a hernia, which is a kind of a simple operation.Open
QuotationAnd my answer is, why don't you? To think overnight and say tomorrow I'm going to do the best hernia in the world.Open
QuotationAnd they look at me like saying this man didn't understand the question and he's crazy, and I look at them and I said they didn't understand my answer.Open
QuotationYou just concentrate on what you do and let the rest of the gods. That's all that I can say.Open
Alberto's statements about Hirschsprung disease334 statements
StatementDr. Pena would perform rectal biopsy in a case of apparent small left colon because he cannot distinguish it from Hirschsprung disease.Open
StatementDr. Pena would perform rectal biopsy in a case of apparent small left colon because he cannot distinguish it from Hirschsprung disease.Open
StatementDr. Pena questions whether patients who develop the characteristic dilated Duhamel pouch actually had true Hirschsprung disease, since by definition aganglionic bowel should not distend even after 10-15 years.Open
StatementDr. Pena questions whether patients who develop the characteristic dilated Duhamel pouch actually had true Hirschsprung disease, since by definition aganglionic bowel should not distend even after 10-15 years.Open
QuotationI have been wondering why patients with Duhamel get this image. If you think about it, we have seen patients with his disease that are 1012, 1415 years old. We have never seen a dilated rectum in a patient with Hirp disease. By definition, Hirp disease is a condition in which the egg ganglionic segment doesn't get distended.Open
QuotationI have been wondering why patients with Duhamel get this image. If you think about it, we have seen patients with his disease that are 1012, 1415 years old. We have never seen a dilated rectum in a patient with Hirp disease. By definition, Hirp disease is a condition in which the egg ganglionic segment doesn't get distended.Open
QuotationI wonder if all the patients that developed this traditional characteristic pouch of the duja male are actually patients that never had Hirk diseaseOpen
QuotationI wonder if all the patients that developed this traditional characteristic pouch of the duja male are actually patients that never had Hirk diseaseOpen
StatementDr. Pena recommends that when resecting a Duhamel pouch, the specimen should be oriented and the pathologist alerted to determine whether ganglion cells are present in the rectal portion, to test the hypothesis that these were misdiagnosed cases.Open
StatementDr. Pena recommends that when resecting a Duhamel pouch, the specimen should be oriented and the pathologist alerted to determine whether ganglion cells are present in the rectal portion, to test the hypothesis that these were misdiagnosed cases.Open
Quotationif I see a patient with this contrast enema, first of all, I will not take a rectal biopsy. I will not take your time because it's a waste of time from my point of view.Open
StatementDr. Pena states that if he sees a patient with megacolon and constipation on enema, he will not perform a rectal biopsy because it is a waste of time from his point of view.Open
Quotationif I see a patient with this contrast enema, first of all, I will not take a rectal biopsy. I will not take your time because it's a waste of time from my point of view.Open
StatementDr. Pena states that if he sees a patient with megacolon and constipation on enema, he will not perform a rectal biopsy because it is a waste of time from his point of view.Open
StatementDr. Pena argues that taking a biopsy in a patient with idiopathic constipation risks getting an aganglionic result that does not mean anything, because the patient does not have Hirschsprung disease.Open
StatementDr. Pena argues that taking a biopsy in a patient with idiopathic constipation risks getting an aganglionic result that does not mean anything, because the patient does not have Hirschsprung disease.Open
Quotationthere is one area. And the length of that area with no ganglion cells has never been accurately determined at different ages in the human being.Open
StatementThere is a normal physiologic aganglionic segment in the distal rectum, but its length at different ages (preterm, term, 6 months) has never been accurately determined in humans.Open
StatementThere is a normal physiologic aganglionic segment in the distal rectum, but its length at different ages (preterm, term, 6 months) has never been accurately determined in humans.Open
Quotationthere is one area. And the length of that area with no ganglion cells has never been accurately determined at different ages in the human being.Open
StatementSomeone could take a biopsy in the normal physiologic aganglionic area and get a result of no ganglion cells, which does not indicate Hirschsprung disease.Open
StatementSomeone could take a biopsy in the normal physiologic aganglionic area and get a result of no ganglion cells, which does not indicate Hirschsprung disease.Open
StatementThe internal sphincter has been defined as a thickening of the circular layer of normal smooth muscle bowel, but Dr. Pena states he has never personally observed this thickening when opening normal rectums at different ages.Open
StatementThe internal sphincter has been defined as a thickening of the circular layer of normal smooth muscle bowel, but Dr. Pena states he has never personally observed this thickening when opening normal rectums at different ages.Open
Quotationthe internal sphincter has been defined as a thickening, as you can see in this diagram. A thickening of the circular layer of the normal smooth muscle bowel. In other words, and I have never seen that thickening personallyOpen
Quotationthe internal sphincter has been defined as a thickening, as you can see in this diagram. A thickening of the circular layer of the normal smooth muscle bowel. In other words, and I have never seen that thickening personallyOpen
StatementIf the internal sphincter thickening exists, nobody has determined its exact boundaries at different ages.Open
StatementIf the internal sphincter thickening exists, nobody has determined its exact boundaries at different ages.Open
Quotationalacia of alacia of the internal sphincter is a manometric concept, not an anatomic conceptOpen
StatementInternal sphincter achalasia is a manometric concept, not an anatomic concept, similar to the lower esophageal sphincter.Open
StatementDr. Pena does not perform or recommend myectomies, myotomies, or botulinum toxin injections for internal sphincter achalasia because the target muscle area is undefined and these procedures paralyze muscle to facilitate stool passage rather than curing a condition of unknown origin.Open
Quotationalacia of alacia of the internal sphincter is a manometric concept, not an anatomic conceptOpen
StatementInternal sphincter achalasia is a manometric concept, not an anatomic concept, similar to the lower esophageal sphincter.Open
StatementDr. Pena does not perform or recommend myectomies, myotomies, or botulinum toxin injections for internal sphincter achalasia because the target muscle area is undefined and these procedures paralyze muscle to facilitate stool passage rather than curing a condition of unknown origin.Open
Radiology and Image Diagnosis of Hirschsprung Disease
StatementDr. Peña would not do biopsy if sure it is meconium ileus, but would do biopsy for a picture of small left colon because he does not know how to distinguish it from Hirschsprung.Open
StatementDr. Peña would not do biopsy if sure it is meconium ileus, but would do biopsy for a picture of small left colon because he does not know how to distinguish it from Hirschsprung.Open
QuotationI have been wondering why patients with Duhamel get this image. If you think about it, we have seen patients with his disease that are 1012, 1415 years old. We have never seen a dilated rectum in a patient with Hirp disease. By definition, Hirp disease is a condition in which the egg ganglionic segment doesn't get distended. Even if you go 10 years, 15 years, and yet in these patients we see the rectum, the rectum was opposed to this angryonic, very stretched with fecally impacted.Open
StatementDr. Peña wonders why Duhamel patients get dilated rectum; by definition Hirschsprung disease is a condition where the aganglionic segment does not get distended even after 10-15 years, yet in these patients the rectum is very stretched and fecally impacted.Open
StatementDr. Peña wonders why Duhamel patients get dilated rectum; by definition Hirschsprung disease is a condition where the aganglionic segment does not get distended even after 10-15 years, yet in these patients the rectum is very stretched and fecally impacted.Open
QuotationI have been wondering why patients with Duhamel get this image. If you think about it, we have seen patients with his disease that are 1012, 1415 years old. We have never seen a dilated rectum in a patient with Hirp disease. By definition, Hirp disease is a condition in which the egg ganglionic segment doesn't get distended. Even if you go 10 years, 15 years, and yet in these patients we see the rectum, the rectum was opposed to this angryonic, very stretched with fecally impacted.Open
StatementDr. Peña suspects that all patients who developed the traditional Duhamel pouch may never have had Hirschsprung disease; he invites surgeons to orient the resected pouch and have pathologists study whether there are ganglion cells in that rectum.Open
StatementDr. Peña suspects that all patients who developed the traditional Duhamel pouch may never have had Hirschsprung disease; he invites surgeons to orient the resected pouch and have pathologists study whether there are ganglion cells in that rectum.Open
QuotationI suspect that this patient never had Hodgkin disease.Open
QuotationI suspect that this patient never had Hodgkin disease.Open
Quotationif I see a patient with this contrast enema, first of all, I will not take a rectal biopsy. I will not take your time because it's a waste of time from my point of view.Open
Quotationif I see a patient with this contrast enema, first of all, I will not take a rectal biopsy. I will not take your time because it's a waste of time from my point of view.Open
StatementDr. Peña states that if he sees a patient with a contrast enema showing redundant stool-filled colon with normal rectum, he will not take a rectal biopsy because it is a waste of time and the patient has no Hirschsprung disease.Open
StatementDr. Peña states that if he sees a patient with a contrast enema showing redundant stool-filled colon with normal rectum, he will not take a rectal biopsy because it is a waste of time and the patient has no Hirschsprung disease.Open
StatementDr. Peña states there is no way to differentiate so-called ultra-short segment Hirschsprung from idiopathic constipation.Open
Quotationthere is no way to differentiate the so-called ultra short with idiopathic constipationOpen
Quotationthere is no way to differentiate the so-called ultra short with idiopathic constipationOpen
StatementDr. Peña states there is no way to differentiate so-called ultra-short segment Hirschsprung from idiopathic constipation.Open
Quotationthere is one area. And the length of that area with no ganglion cells has never been accurately determined at different ages in the human being.Open
Quotationthere is one area. And the length of that area with no ganglion cells has never been accurately determined at different ages in the human being.Open
StatementThe rectum has ganglion cells in normal ganglionic bowel, then there is one area with no ganglion cells, but the length of that normal aganglionic segment has never been accurately determined at different ages in humans.Open
StatementThe rectum has ganglion cells in normal ganglionic bowel, then there is one area with no ganglion cells, but the length of that normal aganglionic segment has never been accurately determined at different ages in humans.Open
StatementThere is no accurate study of the normal aganglionic segment length in a preemie, full-term baby, or 6-month-old baby; this is a challenge for young pediatric surgeons to contribute.Open
StatementThere is no accurate study of the normal aganglionic segment length in a preemie, full-term baby, or 6-month-old baby; this is a challenge for young pediatric surgeons to contribute.Open
QuotationThat's another challenge for young pediatric surgeons. If you want to make a contribution. studies, this is a real challenge.Open
QuotationThat's another challenge for young pediatric surgeons. If you want to make a contribution. studies, this is a real challenge.Open
StatementSomebody could take a biopsy in the normal aganglionic area and get a result of no ganglion cells, but it does not mean anything clinically.Open
Quotationconceivably somebody could take a biopsy in that area and come up with the result of no ganglion cells. It doesn't mean anything for us.Open
Quotationconceivably somebody could take a biopsy in that area and come up with the result of no ganglion cells. It doesn't mean anything for us.Open
StatementSomebody could take a biopsy in the normal aganglionic area and get a result of no ganglion cells, but it does not mean anything clinically.Open
Quotationthe internal sphincter has been defined as a thickening, as you can see in this diagram. A thickening of the circular layer of the normal smooth muscle bowel. In other words, and I have never seen that thickening personally, and I have opened these normal rectums in different, different ages. I have been looking at that. I don't have a microscope, but I have never seen that thickening.Open
StatementThe internal sphincter has been defined as a thickening of the circular layer of normal smooth muscle bowel, but Dr. Peña has never personally seen that thickening despite opening normal rectums at different ages.Open
StatementThe internal sphincter has been defined as a thickening of the circular layer of normal smooth muscle bowel, but Dr. Peña has never personally seen that thickening despite opening normal rectums at different ages.Open
Quotationthe internal sphincter has been defined as a thickening, as you can see in this diagram. A thickening of the circular layer of the normal smooth muscle bowel. In other words, and I have never seen that thickening personally, and I have opened these normal rectums in different, different ages. I have been looking at that. I don't have a microscope, but I have never seen that thickening.Open
StatementIf the internal sphincter thickening exists, nobody has determined the exact limit of that thickening at different ages, so it becomes a kind of magic or witchery type of diagnosis.Open
StatementIf the internal sphincter thickening exists, nobody has determined the exact limit of that thickening at different ages, so it becomes a kind of magic or witchery type of diagnosis.Open
Quotationalacia of alacia of the internal sphincter is a manometric concept, not an anatomic conceptOpen
StatementInternal anal sphincter achalasia is a manometric concept, not an anatomic concept, similar to the lower esophageal sphincter and other sphincters that have been defined but never anatomically seen.Open
StatementInternal anal sphincter achalasia is a manometric concept, not an anatomic concept, similar to the lower esophageal sphincter and other sphincters that have been defined but never anatomically seen.Open
Quotationalacia of alacia of the internal sphincter is a manometric concept, not an anatomic conceptOpen
QuotationI don't pay attention to that and I don't perform myectomies and I don't recommend those myectomies and myotomies and buttock and those concepts because we don't know where where you inject. What area of the muscle, nobody knowsOpen
StatementDr. Peña does not perform myectomies or myotomies and does not recommend those procedures because we do not know where to inject or what area of muscle to target.Open
StatementDr. Peña does not perform myectomies or myotomies and does not recommend those procedures because we do not know where to inject or what area of muscle to target.Open
QuotationI don't pay attention to that and I don't perform myectomies and I don't recommend those myectomies and myotomies and buttock and those concepts because we don't know where where you inject. What area of the muscle, nobody knowsOpen
StatementBotox and similar injections paralyze whatever muscle is there and facilitate passing of stool, but they are not curing a condition of unknown origin.Open
StatementBotox and similar injections paralyze whatever muscle is there and facilitate passing of stool, but they are not curing a condition of unknown origin.Open
History of Hirschsprung Disease
QuotationWhat would happen if Harold Hib could be here today? Contemplating these fantastic advances in technology and hearing, repeating and repeating his name are related with a condition that he described so many years ago.Open
QuotationWe are in depth with all those through history that contribute to the progress in the management of these serious condition that affects so many children.Open
QuotationWe are particularly grateful with Dr. Ova Swenson for his seminal contribution, and, and we still followed his principles in the management of this condition.Open
StatementThe most serious challenge in Hirschsprung disease is the basic science approach to solve the problem of enterocolitis and many other problems affecting children with the disease.Open
StatementHirschsprung disease is not only about ganglion cells or no ganglion cells; it is a much more complex condition.Open
StatementElectric enemas were described around 1908, involving passing a tube through the rectum with an electrode inside and another electrode on the abdomen, delivering about 40 milliamps of interrupted cycling current to facilitate expulsion of saline solution from the colon.Open
Surgical Procedures for Hirschsprung Disease
StatementBetween 75 and 80% of Hirschsprung cases can be completed transanally, reaching normal ganglionic bowel from below.Open
StatementIf you start transanally and cannot reach ganglionic bowel, you simply open the abdomen and continue the resection—this is not a complication.Open
StatementThe main problems in Hirschsprung surgery are related to surgeon inexperience and technical incapacity, not the approach (laparoscopic vs. transanal vs. open).Open
QuotationThe main problems that we have seen in Hirschsprung disease are not related with the approach that you use—are related with the inexperience and technical incapacity of the surgeon that damaged the baby on a permanent basis both ways, laparoscopically or transanally. Those are the real problems that we see.Open
StatementA bad surgeon will damage the patient both ways—laparoscopically or transanally.Open
StatementThe basic goal of Hirschsprung surgery is not to damage the sphincter mechanism, which has been damaged by both laparoscopic and non-laparoscopic techniques.Open
QuotationI never heard people complaining about the scar or not the scar. I see patients complaining about fecal incontinence remaining—that's the real problem, not the size of the scar.Open
StatementPatients complain about fecal incontinence, not the size of the scar.Open
StatementWhen doing transanal dissection, the Lone Star retractor hooks should be placed at the pectinate line to protect the entire anal canal.Open
StatementSurgeons must be careful not to stretch the anus too much during transanal dissection, as excessive stretch damages the sphincter mechanism and causes fecal incontinence.Open
StatementThe dissection should start 2 centimeters deep inside the rectum from the pectinate line, using multiple silk stitches to distribute tension and avoid tissue damage.Open
StatementDr. de la Torre originally started the transanal operation submucosally and rectally; Dr. Peña prefers full-thickness dissection like Dr. Swenson used to do.Open
StatementBiopsies should be taken every 5 centimeters during transanal dissection until normal ganglionic bowel is found, then go 5 centimeters higher.Open
StatementA two-layer anastomosis is performed: the first layer takes seromuscular of the bowel and tissue above the divided rectum; the second layer is mucosa-to-mucosa.Open
StatementKeys to successful transanal surgery: respect the pectinate line and anal canal, don't stretch the anus too much, mobilize rectum to ensure ganglionic bowel with good blood supply, and perform anastomosis with no tension.Open
StatementProne position is preferred over lithotomy for transanal surgery because the surgeon is not the only one who can see, the field is not vertical, and instruments are not lost.Open
StatementLeaving 1-2 centimeters of aganglionic bowel does not explain why patients don't behave well postoperatively.Open
StatementSome patients operated with exactly the same technique do beautifully like normal individuals, while others have symptoms of enterocolitis, and we don't know why.Open
StatementThe majority of patients who come with symptoms of retention (enterocolitis or constipation) after pull-through do NOT have a portion of aganglionic bowel left—they simply behave that way.Open
StatementA few patients do have an obvious piece of aganglionic bowel left, but usually it's much more than 2 centimeters.Open
StatementWhen you finish the operation, the 2 centimeters of bowel you left above the pectinate line are already damaged, so you are very near the pectinate line.Open
StatementThe real concern about transanal surgery is fecal incontinence, which happens when the anal canal is damaged.Open
StatementWhen we remove the rectum of a human being, we are already seriously affecting the mechanisms of bowel control because we are removing the natural reservoir.Open
QuotationWhen we remove the rectum of a human being, we are already affecting seriously the mechanisms of bowel control because we are removing the natural reservoir of the patient.Open
StatementEven adult ulcerative colitis patients with perfect operations and intact anal canals have problems with bowel control—they have accidents at night.Open
StatementAfter removing the rectum, we connect a piece of colon that is constantly moving with peristalsis, whereas the rectum normally rests and only moves when it wants to empty.Open
StatementRemoving the rectum in a child results in passing stool constantly, requiring an intact anal canal, sensation, intact sphincter, and cooperation for bowel control.Open
StatementEven in patients with a very well-preserved anal canal, some children have different degrees of fecal incontinence after Hirschsprung surgery.Open
StatementFecal incontinence after Hirschsprung surgery is much more common than we believe, and we have not been discussing it enough in pediatric surgical meetings.Open
QuotationWe have not been discussing enough in our pediatric surgical meetings the problem of fecal incontinence that is much more common than we believe.Open
StatementMost patients with symptoms of enterocolitis and constipation after pull-through do not have residual aganglionic bowel.Open
QuotationPatients are born with bowel control. We provoke the fecal incontinence.Open
StatementPatients are born with bowel control; we provoke fecal incontinence through surgical technique.Open
StatementUntil the time of the video, Dr. Peña's group had performed 125 transanal operations: 56 primary Hirschsprung, 42 redo Hirschsprung, 21 for idiopathic constipation.Open
StatementTransanal pull-through for idiopathic constipation is not a good operation.Open
StatementWhen dissecting the anterior rectal wall transanally, the dissection must be conducted very meticulously because the rectum has a common wall with the vagina and prostatic urethra.Open
StatementCases with fistulas to the vagina or urinary tract after Hirschsprung surgery are unacceptable complications.Open
StatementDuring full-thickness transanal dissection, if you see fat around the rectum, you can get closer to the rectum because that means you are not in the real rectal wall.Open
StatementIf you stay right on the bowel wall during dissection, you will not provoke denervation of the urinary tract, as Dr. Swenson emphasized.Open
StatementThe outer layer of sutures fixes the rectum in the right position and releases tension from the inner layer.Open
StatementAvoid using big retractors pulling in different directions during transanal surgery because that stretches the sphincter too much.Open
StatementPediatric surgeons compete with each other trying to close stomas as early as possible, but this may not be best for the patient.Open
StatementIn Hirschsprung disease with or without total colonic aganglionosis, we resect the natural reservoir and connect high-motility bowel to the anal canal.Open
StatementAdults who receive ileoanal anastomosis for ulcerative colitis live the rest of their lives trying to avoid accidents, with terrible diarrhea.Open
StatementCreating intentional stasis in the bowel leads to bacterial proliferation, colitis, inflammatory changes, secretory diarrhea, and worsening symptoms.Open
QuotationThe baby is happy with the ileostomy. The only unhappy people are others, but not the baby.Open
StatementIf you wait until the patient is toilet-trained for urine (usually around 3 years), then close the ileostomy, the baby becomes totally trained for stool soon afterward, provided you did a correct operation and preserved the anal canal.Open
StatementPatients with total colonic aganglionosis have a high incidence of enterocolitis and will most likely need rectal irrigation.Open
StatementIt's not easy to do rectal irrigations in a 3-year-old with severe diaper rash who doesn't want anything near the anus.Open
StatementFor contrast enema in Hirschsprung, do not overfill the colon—some technicians open the contrast and fill the entire colon, even the small bowel, and patients vomit.Open
StatementA good contrast study for Hirschsprung requires multiple films, including lateral views, to see if dilation goes all the way down to the pubococcygeal line.Open
StatementIf dilation on contrast enema goes all the way down to the pubococcygeal line, that is not Hirschsprung, even if the biopsy says something different.Open
StatementFor newborn babies with obvious Hirschsprung on contrast enema, keep NPO, place nasogastric tube, place central line, give parenteral nutrition, and do irrigations until abdomen is completely flat and you obtain bile through irrigations—then the baby is ready for surgery.Open
StatementThere are two types of Hirschsprung disease: a benign type where patients never have enterocolitis and can go for years with huge megacolon, and a bad group with manifestations during the newborn period who have great tendency to suffer enterocolitis even with good operation.Open
StatementCongenital central hypoventilation syndrome is associated with Ondine's curse, where patients stop breathing when they fall asleep.Open
CinciHirsch - Pathology of Hirschprung Disease
StatementAfter 6 months of age, the clinical picture of Hirschsprung disease is often so obvious that suction biopsy becomes less relevant.Open
StatementAfter 6 months of age, the clinical picture of Hirschsprung disease is often so obvious that suction biopsy becomes less relevant.Open
QuotationI don't believe that the suction rectal biopsy is the main element for the diagnosis, and it's it's one extra piece of information.Open
QuotationI don't believe that the suction rectal biopsy is the main element for the diagnosis, and it's it's one extra piece of information.Open
StatementThe suction rectal biopsy is not the main element for diagnosis but one extra piece of information, particularly useful in newborn babies.Open
StatementThe suction rectal biopsy is not the main element for diagnosis but one extra piece of information, particularly useful in newborn babies.Open
StatementThere is not a single topographic study of intestinal neuronal dysplasia describing the anatomical extent of the histological abnormality.Open
QuotationThere is not a single topographic study of so-called neuronal intestinal dysplasia, and yet people are doing resections, treating with laxatives, treating with enemas, and but they don't, they don't describe that they resected the the histologically abnormal portion of the colon.Open
QuotationThere is not a single topographic study of so-called neuronal intestinal dysplasia, and yet people are doing resections, treating with laxatives, treating with enemas, and but they don't, they don't describe that they resected the the histologically abnormal portion of the colon.Open
StatementThere is not a single topographic study of intestinal neuronal dysplasia describing the anatomical extent of the histological abnormality.Open
StatementWithout topographic studies, there is no basis for resecting histologically abnormal portions of colon in IND, unlike in Hirschsprung disease where pathology determines resection extent.Open
StatementWithout topographic studies, there is no basis for resecting histologically abnormal portions of colon in IND, unlike in Hirschsprung disease where pathology determines resection extent.Open
StatementThe concept of IND is currently not clinically relevant, though there may be secrets in histological characterization of motility disorders to be studied in the future.Open
StatementThe concept of IND is currently not clinically relevant, though there may be secrets in histological characterization of motility disorders to be studied in the future.Open
QuotationThe concept is still irrelevant. I'm sure that there are many secrets in the in histological characterization of the many motility disorders that have to be studied in the future.Open
QuotationThe concept is still irrelevant. I'm sure that there are many secrets in the in histological characterization of the many motility disorders that have to be studied in the future.Open
Hirschsprung Disease: Surgical Procedures
StatementBetween 75 and 80% of the time, transanal dissection with sequential biopsies reaches normal ganglionic bowel; in the other 20%, conversion to abdominal approach is needed.Open
StatementIf you start transanally and cannot mobilize more bowel, you simply go into the abdomen and continue the resection—this is not considered a complication.Open
QuotationI don't consider that a complication. I don't consider that a problem. You see they go and do what, what you was, what you were going to do in the first place.Open
QuotationThe main problems that we have seen in history from disease are not related with the approach that you got are related with the inexperience and technical incapacity of the surgeon that damaged the baby on a permanent basis both ways laparoscopically or transenally, a bad surgeon will damage the patient.Open
StatementThe main problems in Hirschsprung disease are not related to the surgical approach (laparoscopic vs. transanal) but to inexperience and technical incapacity of the surgeon that damages the patient permanently.Open
QuotationI never heard people complaining about the scar or not the scar. I see patients complaining about fecal incontinence remaining that's the real problem, not the size of the scar.Open
StatementPatients complain about fecal incontinence, not about the size of the scar—that is the real problem.Open
StatementThe Lone Star retractor hooks should be placed at the pectinate line itself, not in the anal canal, to protect the anal canal by definition.Open
StatementSurgeons should be careful not to stretch too much during transanal dissection because excessive stretching damages the sphincter mechanism by definition, potentially causing fecal incontinence.Open
QuotationThe surgeons should be careful not to stretch too much because you start the dissection and you forget you want to be comfortable. You put retractors and I walk into the operating room and see sometimes a big hole that what used to be the anus becomes a huge hole and that is damaging by definition the sphincter mechanism.Open
StatementThe keys to avoiding strictures and retractions are: respecting the pectinate line and anal canal, not stretching the anus too much, mobilizing the rectum to ensure normal ganglionic bowel with good blood supply, and performing anastomosis with no tension.Open
StatementProne position is preferred over lithotomy position for transanal procedures because in lithotomy the surgeon is the only one who can see, the field is vertical causing instrument loss, and the scrub nurse has difficulty seeing.Open
StatementLeaving 1-2 centimeters of aganglionosis does not explain why patients don't behave well—some patients operated with exactly the same technique do beautifully while others have enterocolitis symptoms, and we don't know why.Open
QuotationI don't blame on the 1 centimeter or 1.5 centimeters or 2 centimeters of ganglionnosis to explain why a patient doesn't behave well.Open
QuotationWe have been obsessed with the idea of leaving 1 centimeter or two a ganglionnosis, and that explains symptoms. That simply is not true from my point of view.Open
StatementThe majority of patients who come with symptoms of retention (enterocolitis or constipation) after pull-through do not have a portion of ganglionic bowel left—they simply behave like that and we do not know why.Open
StatementWhen we remove the rectum of a human being, we are already seriously affecting the mechanisms of bowel control because we are removing the natural reservoir of the patient.Open
QuotationWhen we remove the rectum of a human being, we are already affecting seriously the mechanisms of bowel control because we are removing the natural reservoir of the patient.Open
StatementEven adult ulcerative colitis patients with perfect operations have problems with bowel control—they have accidents at night—because we are connecting a piece of colon that is constantly moving to where the rectum normally rests.Open
StatementEven in patients with a very well preserved anal canal, some children have different degrees of fecal incontinence after Hirschsprung surgery.Open
StatementFecal incontinence in Hirschsprung disease is much more common than we believe, and we have not been discussing it enough in pediatric surgical meetings.Open
QuotationWe have not been discussing enough in our pediatric surgical meetings the problem of fecal incontinence that is much more common than we believe.Open
StatementIf you preserve the pectinate line, by definition you are preserving this crucial part of the bowel.Open
QuotationThe patients are born with bowel control. We provoke the fecal incontinence.Open
StatementPatients are born with bowel control—we provoke the fecal incontinence through surgical technique.Open
StatementThe anterior dissection of the rectal wall must be conducted in a very meticulous way because the rectum has a common wall with the vagina and the prostatic urethra—fistulas to the vagina or urinary tract are unacceptable complications.Open
StatementIf you see fat around the rectum during full-thickness dissection, you can get closer to the rectum because that means you are not in the real rectal wall.Open
StatementIf you stay right on the bowel wall during dissection, you will not provoke denervation of the urinary tract.Open
QuotationIf you stay right in the bowel wall, you will not provoke the innervation of the urinary tract.Open
StatementWaiting until the patient is toilet trained for urine (usually around 3 years of age) before closing the ileostomy results in the baby becoming totally trained for stool soon after closure, provided you did a correct operation and preserved the anal canal.Open
QuotationThe baby is happy with the ileostomy. The only unhappy people are others, but not the baby.Open
StatementIf you take a child that has been traumatized with rectal maneuvers and try to do rectal irrigations, there will be problems, whereas if a patient has an ileostomy and the mother does rectal irrigations once in a while with a soft catheter, the baby will accept that.Open
QuotationIf you take a child that has been traumatized with rectal maneuvers and you try to do rectal irrigations, it's going to be a lot of problems, whereas if a patient has an ileostomy and the mother does rectal irrigations once in a while with a soft catheter, the baby will accept that.Open
StatementPatients with total colonic aganglionosis have a high incidence of enterocolitis and will most likely need rectal irrigation, which is difficult to do in a 3-year-old with severe diaper rash who doesn't want anything near the anus.Open
StatementThere are two big types of Hirschsprung disease: a benign type where patients never have enterocolitis and can go for years with huge megacolon, and a bad group with manifestations during the newborn period who have great tendency to suffer enterocolitis even with a good operation.Open
Panel Discussion and Case Presentation Part II: Pediatric Bowel Management 2013
StatementIn Spain, de la Torre technique is used; for total colonic aganglionosis, Lester Martin procedure is used.Open
StatementIn Spain, de la Torre technique is used; for total colonic aganglionosis, Lester Martin procedure is used.Open
StatementWhen the anal canal is destroyed, the patient will not have bowel control; with total colonic aganglionosis producing liquid stool, there is no bowel management possible.Open
StatementWhen the anal canal is destroyed, the patient will not have bowel control; with total colonic aganglionosis producing liquid stool, there is no bowel management possible.Open
StatementA permanent stoma is indicated when the anal canal is destroyed and the patient has total colonic aganglionosis.Open
StatementA permanent stoma is indicated when the anal canal is destroyed and the patient has total colonic aganglionosis.Open
StatementSometimes constipating diet and fiber are tried to convince parents that there is no other option except permanent stoma; this is one of the few indications for permanent stoma.Open
StatementSometimes constipating diet and fiber are tried to convince parents that there is no other option except permanent stoma; this is one of the few indications for permanent stoma.Open
QuotationMost pediatricians all over the world don't know the difference between enterocolitis and gastroenteritis.Open
StatementMost pediatricians worldwide do not know the difference between enterocolitis and gastroenteritis; they do not understand the entity called post-Hirschsprung enterocolitis.Open
StatementMost pediatricians worldwide do not know the difference between enterocolitis and gastroenteritis; they do not understand the entity called post-Hirschsprung enterocolitis.Open
QuotationMost pediatricians all over the world don't know the difference between enterocolitis and gastroenteritis.Open
StatementPediatricians do not understand why irrigations are necessary; they see dilated bowel on X-ray and think it is intestinal obstruction, not recognizing the entity itself.Open
StatementPediatricians do not understand why irrigations are necessary; they see dilated bowel on X-ray and think it is intestinal obstruction, not recognizing the entity itself.Open
StatementWhen starting dissection 2 cm above the dentate line and pulling bowel through, the upper mucosa is often damaged, and the anastomosis ends up about 1 cm above the dentate line.Open
StatementWhen starting dissection 2 cm above the dentate line and pulling bowel through, the upper mucosa is often damaged, and the anastomosis ends up about 1 cm above the dentate line.Open
StatementDr. Pena does not believe that leaving 1-2 cm of rectal mucosa is the simple cause of enterocolitis.Open
StatementDr. Pena does not believe that leaving 1-2 cm of rectal mucosa is the simple cause of enterocolitis.Open
StatementA group in New York doing neonatal Soave primary procedures reported zero enterocolitis; when Dr. Pena followed some of those patients, many had fecal incontinence.Open
StatementA group in New York doing neonatal Soave primary procedures reported zero enterocolitis; when Dr. Pena followed some of those patients, many had fecal incontinence.Open
StatementIf you produce fecal incontinence in a patient, enterocolitis is zero; a patient with destroyed anal canal is equivalent to a stoma, and patients with stomas rarely have enterocolitis.Open
QuotationIf you produce fecal incontinence in the patient, the enterocolitis is zero.Open
QuotationIf you produce fecal incontinence in the patient, the enterocolitis is zero.Open
StatementIf you produce fecal incontinence in a patient, enterocolitis is zero; a patient with destroyed anal canal is equivalent to a stoma, and patients with stomas rarely have enterocolitis.Open
StatementA good operation preserving the sphincter and anal canal creates sphincter closure, which creates stasis, and stasis produces enterocolitis.Open
StatementA good operation preserving the sphincter and anal canal creates sphincter closure, which creates stasis, and stasis produces enterocolitis.Open
QuotationWe don't know why the patients have enterocolitis, but if you produce, if you have very little enterocolitis, chances are that you are damaging the anal canal.Open
QuotationWe don't know why the patients have enterocolitis, but if you produce, if you have very little enterocolitis, chances are that you are damaging the anal canal.Open
QuotationI prefer to deal with enterocolitis than with fecal incontinence. Fecal incontinence is for life.Open
StatementDr. Pena prefers to deal with enterocolitis rather than fecal incontinence; fecal incontinence is for life.Open
QuotationI prefer to deal with enterocolitis than with fecal incontinence. Fecal incontinence is for life.Open
StatementDr. Pena prefers to deal with enterocolitis rather than fecal incontinence; fecal incontinence is for life.Open
StatementDr. Pena's incidence of enterocolitis in pull-through patients is about 30%.Open
StatementDr. Pena's incidence of enterocolitis in pull-through patients is about 30%.Open
StatementResecting the rectosigmoid introduces a major pathophysiological change; children with perfect pull-throughs preserving the anal canal sometimes have toilet-training problems without explanation.Open
StatementResecting the rectosigmoid introduces a major pathophysiological change; children with perfect pull-throughs preserving the anal canal sometimes have toilet-training problems without explanation.Open
StatementHyperactive children with attention deficit disorder have more toilet-training problems because they have a piece of colon that does not act like a reservoir, connected to the rectum and moving constantly; significant cooperation from the child is required.Open
StatementHyperactive children with attention deficit disorder have more toilet-training problems because they have a piece of colon that does not act like a reservoir, connected to the rectum and moving constantly; significant cooperation from the child is required.Open
StatementEven with a perfect operation, patients may have certain toilet-training problems; if the anal canal is destroyed, they will be totally incontinent; partial anal canal destruction causes more problems.Open
StatementEven with a perfect operation, patients may have certain toilet-training problems; if the anal canal is destroyed, they will be totally incontinent; partial anal canal destruction causes more problems.Open
StatementDr. Pena does not find rectal manometry useful in Hirschsprung disease, constipation, or anorectal malformations after many years of experience.Open
StatementDr. Pena does not find rectal manometry useful in Hirschsprung disease, constipation, or anorectal malformations after many years of experience.Open
StatementPatients are discharged with three irrigations per day and metronidazole (Flagyl); every month the number of irrigations is decreased.Open
StatementPatients are discharged with three irrigations per day and metronidazole (Flagyl); every month the number of irrigations is decreased.Open
StatementIf decreasing irrigations causes recurrent enterocolitis and the patient does not tolerate lack of irrigation, and by 6 months post-op the patient is still on irrigations, parents become very nervous; at that point, other options are discussed.Open
StatementIf decreasing irrigations causes recurrent enterocolitis and the patient does not tolerate lack of irrigation, and by 6 months post-op the patient is still on irrigations, parents become very nervous; at that point, other options are discussed.Open
StatementFor refractory enterocolitis, another option is further resection of normal ganglionic colon to remove more.Open
StatementFor refractory enterocolitis, another option is further resection of normal ganglionic colon to remove more.Open
StatementHirschsprung disease is much more than ganglion cells vs. no ganglion cells; we do not know why some patients never have enterocolitis and toilet-train early, behaving like normal children, while others have severe enterocolitis from day one.Open
StatementHirschsprung disease is much more than ganglion cells vs. no ganglion cells; we do not know why some patients never have enterocolitis and toilet-train early, behaving like normal children, while others have severe enterocolitis from day one.Open
Statement'Benign Hirschsprung disease' patients present at 8-10 years old with classic imaging and abdominal distention but never had enterocolitis, grew and developed normally, and do very well after surgery.Open
Statement'Benign Hirschsprung disease' patients present at 8-10 years old with classic imaging and abdominal distention but never had enterocolitis, grew and developed normally, and do very well after surgery.Open
StatementIn the United States, earlier diagnosis is being made of patients with 'bad Hirschsprung'—enterocolitis from day one, very sick, and high incidence of enterocolitis after surgery.Open
StatementIn the United States, earlier diagnosis is being made of patients with 'bad Hirschsprung'—enterocolitis from day one, very sick, and high incidence of enterocolitis after surgery.Open
QuotationThere is a lot of things that we don't know about Higp disease. Some bowel has no the story is much more than absent ganglion cells.Open
QuotationThere is a lot of things that we don't know about Higp disease. Some bowel has no the story is much more than absent ganglion cells.Open
StatementThere is much we do not know about Hirschsprung disease; the story is much more than absent ganglion cells, and taking bowel with normal ganglion cells down does not mean that bowel is 100% normal.Open
StatementThere is much we do not know about Hirschsprung disease; the story is much more than absent ganglion cells, and taking bowel with normal ganglion cells down does not mean that bowel is 100% normal.Open
StatementSome believe that ganglionic bowel may have neuronal intestinal dysplasia, but this is a very controversial histopathological diagnosis; we do not know what is wrong and must learn much more.Open
StatementSome believe that ganglionic bowel may have neuronal intestinal dysplasia, but this is a very controversial histopathological diagnosis; we do not know what is wrong and must learn much more.Open
StatementDr. Pena is skeptical that the cuff produces obstruction; to believe it, he would need to see the cuff producing real obstruction manifested by very dilated colon above the cuff, which is very unusual.Open
StatementDr. Pena is skeptical that the cuff produces obstruction; to believe it, he would need to see the cuff producing real obstruction manifested by very dilated colon above the cuff, which is very unusual.Open
StatementDr. Mark Levitt has experience dealing with obstructive cuffs transanally; laparoscopic approach is not a bad idea but Dr. Pena has never heard of it being done laparoscopically.Open
StatementDr. Mark Levitt has experience dealing with obstructive cuffs transanally; laparoscopic approach is not a bad idea but Dr. Pena has never heard of it being done laparoscopically.Open
StatementPatients with total fecal incontinence have no enterocolitis; all operations moving toward fecal incontinence (myectomies, myotomies, Botox, massive dilatation, putting 3 fingers in the rectum) are temporary or permanent moves toward incontinence.Open
StatementPatients with total fecal incontinence have no enterocolitis; all operations moving toward fecal incontinence (myectomies, myotomies, Botox, massive dilatation, putting 3 fingers in the rectum) are temporary or permanent moves toward incontinence.Open
StatementPatients subjected to myotomies, myectomies, or repeated Botox injections eventually develop more severe fecal incontinence.Open
StatementPatients subjected to myotomies, myectomies, or repeated Botox injections eventually develop more severe fecal incontinence.Open
StatementDr. Pena does not believe in myectomy/myotomy/Botox procedures and does not use Botox.Open
StatementDr. Pena does not believe in myectomy/myotomy/Botox procedures and does not use Botox.Open
Cloacal Exstrophy with Dr. Alberto Peña
StatementCloacal exstrophy is a spectrum of congenital malformations affecting the gastrointestinal/colorectal area, urogenital tract, spine and cord, and sometimes lower extremity motion.Open
StatementBabies with cloacal exstrophy are born with an omphalocele, bladder exstrophy (two separated hemibladders), open cecum between the hemibladders, and separated pubic bones.Open
StatementThe small bowel can become exstrophic through the ileocecal valve, creating an 'elephant trunk' appearance.Open
StatementMale patients have two separated hemiphalluses with normal gonads; female patients have two hemivaginas below the exstrophic bladder leading to two hemiuteri.Open
StatementCloacal 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.Open
StatementThe amount of colon present at birth has very important implications for the patient's management and outcomes.Open
StatementA 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.Open
StatementWhile 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.Open
StatementHistorical practice was to perform bilateral gonadectomy, remove hemiphalluses, create a vagina with bowel, and assign female gender to XY patients with cloacal exstrophy.Open
StatementPatients 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.Open
Quotationwho 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.Open
StatementPatients 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.Open
Quotationbeing a male is much more than having a phallusOpen
StatementCurrent consensus is that XY patients should be raised as male, with pediatric urologists and plastic surgeons working on phallus reconstruction techniques.Open
StatementWhen a prominent pediatric urologist dominates management, patients receive good urologic attention but inadequate gastrointestinal care; the reverse occurs when pediatric surgeons dominate.Open
StatementThe 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.Open
StatementIt is very common but very harmful for pediatric surgeons to simply create an ileostomy, leaving all colon distally attached to the urinary tract.Open
QuotationThat is a very bad thing to do, and the patient will suffer several consequences because of thatOpen
StatementLeaving colon attached to the urinary tract creates a congenital bladder augmentation that causes hyperchloremic acidosis from urine absorption, interfering with growth and development.Open
QuotationIsn't that beautiful from your point of view?Open
StatementDefunctionalized colonic tissue left distally will not grow; colon requires passage of fecal matter through its lumen to grow.Open
StatementThe 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.Open
StatementPatients who received ileostomy with defunctionalized colon present at 2-3 years with poor growth, hyperchloremic acidosis managed by nephrologists, and large ileostomy losses.Open
StatementThe 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.Open
StatementSurgeons 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.Open
QuotationDo not leave gastrointestinal tract inside the functionalized. That's the main, main thing.Open
StatementSome 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.Open
StatementEven after osteotomy, it is very difficult to see a cloacal exstrophy patient with pubic bones completely together; they usually remain separated.Open
StatementEven a technically correct end colostomy often has poor motility, and babies may not pass stool easily, sometimes developing bacterial overgrowth similar to Hirschsprung disease.Open
StatementFamilies must be taught to irrigate the colostomy with small volumes of saline through a tube to evacuate fecal material when peristalsis is inadequate.Open
StatementDecision-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).Open
StatementMost cloacal exstrophy patients have an inadequate, tiny bladder requiring bladder augmentation with gastrointestinal tract, necessitating coordination between pediatric surgery and urology.Open
StatementPatients 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.Open
StatementPull-through is only considered for patients with capacity to form solid stool (adequate colon), as bowel management only works with solid stool.Open
StatementIt 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).Open
StatementPediatric surgeons should not underestimate the growth capacity of tiny colonic pieces during the newborn period; even small segments will grow over three years if fecal stream passes through them.Open
StatementAnnual contrast studies through the colostomy (retrograde injection) are performed to assess colonic growth.Open
StatementBefore committing to pull-through, a trial bowel management program is performed through the colostomy: enemas are given to empty the colonic pouch, and if the patient stays 24 hours without stool in the colostomy bag, pull-through is likely to succeed.Open
StatementBowel management trial through colostomy is typically started after age 3 when families consider avoiding the stoma for school.Open
StatementIf a patient has very little colon and cannot form solid stool, the urologist is free to use bowel for bladder augmentation; if the patient has borderline colon, the urologist must use stomach for augmentation to preserve bowel for fecal function.Open
StatementThe colon to be pulled through is the most posterior structure in the pelvis, with the bladder and augmentation anterior to it; therefore, bladder augmentation must not be done before deciding on pull-through, or accessing the colon will be extremely difficult.Open
StatementContrast enema through the colostomy can distinguish true liquid stool from paradoxical diarrhea (liquid stool around solid fecal impaction).Open
StatementPull-through and bladder augmentation are ideally performed together in a single operation lasting approximately 12 hours, with pediatric surgery going first (posterior dissection) followed by urology (anterior augmentation).Open
StatementDuring pull-through, if the patient has one or two appendices, a Malone appendicostomy can be created for antegrade enema administration, as the appendix remains in the abdomen when colon is pulled down.Open
StatementUrologists almost never use colon for bladder augmentation in cloacal exstrophy because colon is needed to form solid stool; they typically use small bowel or stomach.Open
StatementOccasionally, when a patient has a giant colonic pouch with very poor motility, the poor motility makes it good for bowel management (irrigate once daily, stays clean between irrigations), and a piece can be shared with urology for augmentation.Open
StatementMidline abdominal incision from xiphoid to pubis is used for pull-through to preserve the flanks and quadrants for potential future stomas.Open
StatementThe colostomy is circumferentially dissected and separated from the abdominal wall, then the blood supply is carefully studied because cloacal exstrophy patients have very bizarre, aberrant vascular anatomy.Open
StatementCareful observation of the vascular anatomy allows the surgeon to decide which vessels can be ligated to mobilize the colon to the perineum without compromising blood supply; sometimes no vessels need to be ligated.Open
StatementThe space behind the bladder is easily created, and the bowel is placed posteriorly; patients do not need prone positioning because the exstrophy makes everything anterior, so a supine frog-leg position provides full perineal access.Open
StatementCloacal exstrophy patients are lifelong patients due to orthopedic problems (separated pubic bones causing abnormal gait) and spinal problems (tethered cord requiring neurosurgical follow-up and potential cord release).Open
StatementSome teenagers are unhappy about separated pubic bones causing abnormal gait (feet pointing laterally); some dedicated orthopedic surgeons have been able to bring pubic bones closer together.Open
StatementDuring pull-through, vaginal reconstruction is attempted by approximating the hemivaginas as much as possible; the degree of separation varies on the spectrum.Open
StatementWhen hemivaginas are close with only a septum separating them superiorly, the septum is removed as high as possible; when vaginas run in completely different directions, one hemivagina may be removed, leaving the one with the better-looking cervix.Open
StatementPatients with functional hemiuterus may become pregnant, but it is high-risk pregnancy requiring specialized pediatric gynecology follow-up; in general, pregnancy is not advised.Open
StatementHemiuterus has a great tendency to produce miscarriages and premature labor; delivery must be by cesarean section due to limited abdominal space.Open
StatementBladder augmentation requires a Mitrofanoff conduit (appendix or part of a long appendix, sometimes shared half for urology and half for Malone) for intermittent catheterization to empty the bladder.Open
StatementAugmented bladders produce large amounts of mucus; if mucus is not removed, it forms stones, so families must be taught to irrigate the bladder (not just empty it) to remove mucus and prevent infections.Open
StatementBladder irrigation is sometimes performed with gentamicin to ensure all mucus is removed.Open
StatementLifelong urologic follow-up is needed to monitor for reflux and kidney damage; the Mitrofanoff may stop working or leak urine, requiring revision or valve tightening.Open
StatementPatients transitioning to adult hospitals often feel uncomfortable because adult urologists and orthopedic surgeons lack experience with these congenital malformations; patients prefer to remain in pediatric environments even as adults.Open
StatementDr. Peña observes that cloacal exstrophy patients are particularly charming, intelligent, beautiful, and charismatic when they grow up, and some have energy to help others manage their own problems.Open
StatementThe Pull-Through Network is a national organization (similar to cystic fibrosis or breast cancer organizations) for parents and patients with anorectal malformations, Hirschsprung disease, or bowel/urinary control problems; it has over 1000 members, holds annual meetings, and invites doctors to give talks.Open
StatementColorectal and urogenital problems have been left behind in terms of scientific approach and research funding because they are not 'elegant' problems—they involve stool, urine, and sex—and institutions are not eager to receive these patients.Open
StatementThe initial operation includes omphalocele closure (sometimes requiring staged closure), bowel-bladder separation, end colostomy creation, bladder closure attempt (sometimes requiring multiple stages), and in some institutions, pelvic osteotomy (either at initial operation or 2-3 months later).Open
StatementBetween the initial operation and pull-through, colostomy irrigation is often needed to manage poor colonic motility.Open
StatementPrenatal diagnosis of anorectal and urogenital malformations is easier for complex defects (like cloacal exstrophy) than simple defects because complex cases have associated findings (spinal problems, absent bladder) visible on imaging.Open
StatementAbsent bladder on prenatal ultrasound (due to exstrophy) is a bad sign and can be detected as early as week 20 of pregnancy.Open
StatementPrenatal diagnosis allows families to decide about pregnancy continuation and, if continuing, to deliver at a center with a multidisciplinary team experienced in these malformations.Open
StatementCertain malformations require centers of excellence where surgeons focus and sacrifice other areas of practice; attempting to train all surgeons superficially in complex conditions results in damaged children and no one becoming truly trained.Open
Alberto's statements about Omphalocele70 statements
StatementCloacal exstrophy is a spectrum of congenital malformations affecting the gastrointestinal/colorectal area, urogenital tract, spine and cord, and sometimes lower extremity motion.Open
StatementBabies with cloacal exstrophy are born with an omphalocele, bladder exstrophy (two separated hemibladders), open cecum between the hemibladders, and separated pubic bones.Open
StatementThe small bowel can become exstrophic through the ileocecal valve, creating an 'elephant trunk' appearance.Open
StatementMale patients have two separated hemiphalluses with normal gonads; female patients have two hemivaginas below the exstrophic bladder leading to two hemiuteri.Open
StatementCloacal 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.Open
StatementThe amount of colon present at birth has very important implications for the patient's management and outcomes.Open
StatementA 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.Open
StatementWhile 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.Open
StatementHistorical practice was to perform bilateral gonadectomy, remove hemiphalluses, create a vagina with bowel, and assign female gender to XY patients with cloacal exstrophy.Open
StatementPatients 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.Open
Quotationwho 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.Open
StatementPatients 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.Open
Quotationbeing a male is much more than having a phallusOpen
StatementCurrent consensus is that XY patients should be raised as male, with pediatric urologists and plastic surgeons working on phallus reconstruction techniques.Open
StatementWhen a prominent pediatric urologist dominates management, patients receive good urologic attention but inadequate gastrointestinal care; the reverse occurs when pediatric surgeons dominate.Open
StatementThe 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.Open
StatementIt is very common but very harmful for pediatric surgeons to simply create an ileostomy, leaving all colon distally attached to the urinary tract.Open
QuotationThat is a very bad thing to do, and the patient will suffer several consequences because of thatOpen
StatementLeaving colon attached to the urinary tract creates a congenital bladder augmentation that causes hyperchloremic acidosis from urine absorption, interfering with growth and development.Open
QuotationIsn't that beautiful from your point of view?Open
StatementDefunctionalized colonic tissue left distally will not grow; colon requires passage of fecal matter through its lumen to grow.Open
StatementThe 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.Open
StatementPatients who received ileostomy with defunctionalized colon present at 2-3 years with poor growth, hyperchloremic acidosis managed by nephrologists, and large ileostomy losses.Open
StatementThe 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.Open
StatementSurgeons 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.Open
QuotationDo not leave gastrointestinal tract inside the functionalized. That's the main, main thing.Open
StatementSome 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.Open
StatementEven after osteotomy, it is very difficult to see a cloacal exstrophy patient with pubic bones completely together; they usually remain separated.Open
StatementEven a technically correct end colostomy often has poor motility, and babies may not pass stool easily, sometimes developing bacterial overgrowth similar to Hirschsprung disease.Open
StatementFamilies must be taught to irrigate the colostomy with small volumes of saline through a tube to evacuate fecal material when peristalsis is inadequate.Open
StatementDecision-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).Open
StatementMost cloacal exstrophy patients have an inadequate, tiny bladder requiring bladder augmentation with gastrointestinal tract, necessitating coordination between pediatric surgery and urology.Open
StatementPatients 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.Open
StatementPull-through is only considered for patients with capacity to form solid stool (adequate colon), as bowel management only works with solid stool.Open
StatementIt 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).Open
StatementPediatric surgeons should not underestimate the growth capacity of tiny colonic pieces during the newborn period; even small segments will grow over three years if fecal stream passes through them.Open
StatementAnnual contrast studies through the colostomy (retrograde injection) are performed to assess colonic growth.Open
StatementBefore committing to pull-through, a trial bowel management program is performed through the colostomy: enemas are given to empty the colonic pouch, and if the patient stays 24 hours without stool in the colostomy bag, pull-through is likely to succeed.Open
StatementBowel management trial through colostomy is typically started after age 3 when families consider avoiding the stoma for school.Open
StatementIf a patient has very little colon and cannot form solid stool, the urologist is free to use bowel for bladder augmentation; if the patient has borderline colon, the urologist must use stomach for augmentation to preserve bowel for fecal function.Open
StatementThe colon to be pulled through is the most posterior structure in the pelvis, with the bladder and augmentation anterior to it; therefore, bladder augmentation must not be done before deciding on pull-through, or accessing the colon will be extremely difficult.Open
StatementContrast enema through the colostomy can distinguish true liquid stool from paradoxical diarrhea (liquid stool around solid fecal impaction).Open
StatementPull-through and bladder augmentation are ideally performed together in a single operation lasting approximately 12 hours, with pediatric surgery going first (posterior dissection) followed by urology (anterior augmentation).Open
StatementDuring pull-through, if the patient has one or two appendices, a Malone appendicostomy can be created for antegrade enema administration, as the appendix remains in the abdomen when colon is pulled down.Open
StatementUrologists almost never use colon for bladder augmentation in cloacal exstrophy because colon is needed to form solid stool; they typically use small bowel or stomach.Open
StatementOccasionally, when a patient has a giant colonic pouch with very poor motility, the poor motility makes it good for bowel management (irrigate once daily, stays clean between irrigations), and a piece can be shared with urology for augmentation.Open
StatementMidline abdominal incision from xiphoid to pubis is used for pull-through to preserve the flanks and quadrants for potential future stomas.Open
StatementThe colostomy is circumferentially dissected and separated from the abdominal wall, then the blood supply is carefully studied because cloacal exstrophy patients have very bizarre, aberrant vascular anatomy.Open
StatementCareful observation of the vascular anatomy allows the surgeon to decide which vessels can be ligated to mobilize the colon to the perineum without compromising blood supply; sometimes no vessels need to be ligated.Open
StatementThe space behind the bladder is easily created, and the bowel is placed posteriorly; patients do not need prone positioning because the exstrophy makes everything anterior, so a supine frog-leg position provides full perineal access.Open
StatementCloacal exstrophy patients are lifelong patients due to orthopedic problems (separated pubic bones causing abnormal gait) and spinal problems (tethered cord requiring neurosurgical follow-up and potential cord release).Open
StatementSome teenagers are unhappy about separated pubic bones causing abnormal gait (feet pointing laterally); some dedicated orthopedic surgeons have been able to bring pubic bones closer together.Open
StatementDuring pull-through, vaginal reconstruction is attempted by approximating the hemivaginas as much as possible; the degree of separation varies on the spectrum.Open
StatementWhen hemivaginas are close with only a septum separating them superiorly, the septum is removed as high as possible; when vaginas run in completely different directions, one hemivagina may be removed, leaving the one with the better-looking cervix.Open
StatementPatients with functional hemiuterus may become pregnant, but it is high-risk pregnancy requiring specialized pediatric gynecology follow-up; in general, pregnancy is not advised.Open
StatementHemiuterus has a great tendency to produce miscarriages and premature labor; delivery must be by cesarean section due to limited abdominal space.Open
StatementBladder augmentation requires a Mitrofanoff conduit (appendix or part of a long appendix, sometimes shared half for urology and half for Malone) for intermittent catheterization to empty the bladder.Open
StatementAugmented bladders produce large amounts of mucus; if mucus is not removed, it forms stones, so families must be taught to irrigate the bladder (not just empty it) to remove mucus and prevent infections.Open
StatementBladder irrigation is sometimes performed with gentamicin to ensure all mucus is removed.Open
StatementLifelong urologic follow-up is needed to monitor for reflux and kidney damage; the Mitrofanoff may stop working or leak urine, requiring revision or valve tightening.Open
StatementPatients transitioning to adult hospitals often feel uncomfortable because adult urologists and orthopedic surgeons lack experience with these congenital malformations; patients prefer to remain in pediatric environments even as adults.Open
StatementDr. Peña observes that cloacal exstrophy patients are particularly charming, intelligent, beautiful, and charismatic when they grow up, and some have energy to help others manage their own problems.Open
StatementThe Pull-Through Network is a national organization (similar to cystic fibrosis or breast cancer organizations) for parents and patients with anorectal malformations, Hirschsprung disease, or bowel/urinary control problems; it has over 1000 members, holds annual meetings, and invites doctors to give talks.Open
StatementColorectal and urogenital problems have been left behind in terms of scientific approach and research funding because they are not 'elegant' problems—they involve stool, urine, and sex—and institutions are not eager to receive these patients.Open
StatementThe initial operation includes omphalocele closure (sometimes requiring staged closure), bowel-bladder separation, end colostomy creation, bladder closure attempt (sometimes requiring multiple stages), and in some institutions, pelvic osteotomy (either at initial operation or 2-3 months later).Open
StatementBetween the initial operation and pull-through, colostomy irrigation is often needed to manage poor colonic motility.Open
StatementPrenatal diagnosis of anorectal and urogenital malformations is easier for complex defects (like cloacal exstrophy) than simple defects because complex cases have associated findings (spinal problems, absent bladder) visible on imaging.Open
StatementAbsent bladder on prenatal ultrasound (due to exstrophy) is a bad sign and can be detected as early as week 20 of pregnancy.Open
StatementPrenatal diagnosis allows families to decide about pregnancy continuation and, if continuing, to deliver at a center with a multidisciplinary team experienced in these malformations.Open
StatementCertain malformations require centers of excellence where surgeons focus and sacrifice other areas of practice; attempting to train all surgeons superficially in complex conditions results in damaged children and no one becoming truly trained.Open
Alberto's statements about Total Colonic Aganglionosis123 statements
StatementBetween 75 and 80% of Hirschsprung cases can be completed transanally, reaching normal ganglionic bowel from below.Open
StatementIf you start transanally and cannot reach ganglionic bowel, you simply open the abdomen and continue the resection—this is not a complication.Open
QuotationThe main problems that we have seen in Hirschsprung disease are not related with the approach that you use—are related with the inexperience and technical incapacity of the surgeon that damaged the baby on a permanent basis both ways, laparoscopically or transanally. Those are the real problems that we see.Open
StatementThe main problems in Hirschsprung surgery are related to surgeon inexperience and technical incapacity, not the approach (laparoscopic vs. transanal vs. open).Open
StatementA bad surgeon will damage the patient both ways—laparoscopically or transanally.Open
StatementThe basic goal of Hirschsprung surgery is not to damage the sphincter mechanism, which has been damaged by both laparoscopic and non-laparoscopic techniques.Open
QuotationI never heard people complaining about the scar or not the scar. I see patients complaining about fecal incontinence remaining—that's the real problem, not the size of the scar.Open
StatementPatients complain about fecal incontinence, not the size of the scar.Open
StatementWhen doing transanal dissection, the Lone Star retractor hooks should be placed at the pectinate line to protect the entire anal canal.Open
StatementSurgeons must be careful not to stretch the anus too much during transanal dissection, as excessive stretch damages the sphincter mechanism and causes fecal incontinence.Open
StatementThe dissection should start 2 centimeters deep inside the rectum from the pectinate line, using multiple silk stitches to distribute tension and avoid tissue damage.Open
StatementDr. de la Torre originally started the transanal operation submucosally and rectally; Dr. Peña prefers full-thickness dissection like Dr. Swenson used to do.Open
StatementBiopsies should be taken every 5 centimeters during transanal dissection until normal ganglionic bowel is found, then go 5 centimeters higher.Open
StatementA two-layer anastomosis is performed: the first layer takes seromuscular of the bowel and tissue above the divided rectum; the second layer is mucosa-to-mucosa.Open
StatementKeys to successful transanal surgery: respect the pectinate line and anal canal, don't stretch the anus too much, mobilize rectum to ensure ganglionic bowel with good blood supply, and perform anastomosis with no tension.Open
StatementProne position is preferred over lithotomy for transanal surgery because the surgeon is not the only one who can see, the field is not vertical, and instruments are not lost.Open
StatementLeaving 1-2 centimeters of aganglionic bowel does not explain why patients don't behave well postoperatively.Open
StatementSome patients operated with exactly the same technique do beautifully like normal individuals, while others have symptoms of enterocolitis, and we don't know why.Open
StatementThe majority of patients who come with symptoms of retention (enterocolitis or constipation) after pull-through do NOT have a portion of aganglionic bowel left—they simply behave that way.Open
StatementA few patients do have an obvious piece of aganglionic bowel left, but usually it's much more than 2 centimeters.Open
StatementWhen you finish the operation, the 2 centimeters of bowel you left above the pectinate line are already damaged, so you are very near the pectinate line.Open
StatementThe real concern about transanal surgery is fecal incontinence, which happens when the anal canal is damaged.Open
StatementWhen we remove the rectum of a human being, we are already seriously affecting the mechanisms of bowel control because we are removing the natural reservoir.Open
QuotationWhen we remove the rectum of a human being, we are already affecting seriously the mechanisms of bowel control because we are removing the natural reservoir of the patient.Open
StatementEven adult ulcerative colitis patients with perfect operations and intact anal canals have problems with bowel control—they have accidents at night.Open
StatementAfter removing the rectum, we connect a piece of colon that is constantly moving with peristalsis, whereas the rectum normally rests and only moves when it wants to empty.Open
StatementRemoving the rectum in a child results in passing stool constantly, requiring an intact anal canal, sensation, intact sphincter, and cooperation for bowel control.Open
StatementEven in patients with a very well-preserved anal canal, some children have different degrees of fecal incontinence after Hirschsprung surgery.Open
QuotationWe have not been discussing enough in our pediatric surgical meetings the problem of fecal incontinence that is much more common than we believe.Open
StatementFecal incontinence after Hirschsprung surgery is much more common than we believe, and we have not been discussing it enough in pediatric surgical meetings.Open
StatementMost patients with symptoms of enterocolitis and constipation after pull-through do not have residual aganglionic bowel.Open
StatementPatients are born with bowel control; we provoke fecal incontinence through surgical technique.Open
QuotationPatients are born with bowel control. We provoke the fecal incontinence.Open
StatementUntil the time of the video, Dr. Peña's group had performed 125 transanal operations: 56 primary Hirschsprung, 42 redo Hirschsprung, 21 for idiopathic constipation.Open
StatementTransanal pull-through for idiopathic constipation is not a good operation.Open
StatementWhen dissecting the anterior rectal wall transanally, the dissection must be conducted very meticulously because the rectum has a common wall with the vagina and prostatic urethra.Open
StatementCases with fistulas to the vagina or urinary tract after Hirschsprung surgery are unacceptable complications.Open
StatementDuring full-thickness transanal dissection, if you see fat around the rectum, you can get closer to the rectum because that means you are not in the real rectal wall.Open
StatementThe outer layer of sutures fixes the rectum in the right position and releases tension from the inner layer.Open
StatementAvoid using big retractors pulling in different directions during transanal surgery because that stretches the sphincter too much.Open
StatementPediatric surgeons compete with each other trying to close stomas as early as possible, but this may not be best for the patient.Open
StatementIn Hirschsprung disease with or without total colonic aganglionosis, we resect the natural reservoir and connect high-motility bowel to the anal canal.Open
StatementAdults who receive ileoanal anastomosis for ulcerative colitis live the rest of their lives trying to avoid accidents, with terrible diarrhea.Open
StatementCreating intentional stasis in the bowel leads to bacterial proliferation, colitis, inflammatory changes, secretory diarrhea, and worsening symptoms.Open
QuotationThe baby is happy with the ileostomy. The only unhappy people are others, but not the baby.Open
StatementIf you wait until the patient is toilet-trained for urine (usually around 3 years), then close the ileostomy, the baby becomes totally trained for stool soon afterward, provided you did a correct operation and preserved the anal canal.Open
StatementPatients with total colonic aganglionosis have a high incidence of enterocolitis and will most likely need rectal irrigation.Open
StatementIt's not easy to do rectal irrigations in a 3-year-old with severe diaper rash who doesn't want anything near the anus.Open
StatementFor contrast enema in Hirschsprung, do not overfill the colon—some technicians open the contrast and fill the entire colon, even the small bowel, and patients vomit.Open
StatementA good contrast study for Hirschsprung requires multiple films, including lateral views, to see if dilation goes all the way down to the pubococcygeal line.Open
StatementIf dilation on contrast enema goes all the way down to the pubococcygeal line, that is not Hirschsprung, even if the biopsy says something different.Open
StatementFor newborn babies with obvious Hirschsprung on contrast enema, keep NPO, place nasogastric tube, place central line, give parenteral nutrition, and do irrigations until abdomen is completely flat and you obtain bile through irrigations—then the baby is ready for surgery.Open
StatementThere are two types of Hirschsprung disease: a benign type where patients never have enterocolitis and can go for years with huge megacolon, and a bad group with manifestations during the newborn period who have great tendency to suffer enterocolitis even with good operation.Open
Hirschsprung Disease: Surgical Procedures
StatementBetween 75 and 80% of the time, transanal dissection with sequential biopsies reaches normal ganglionic bowel; in the other 20%, conversion to abdominal approach is needed.Open
StatementIf you start transanally and cannot mobilize more bowel, you simply go into the abdomen and continue the resection—this is not considered a complication.Open
QuotationI don't consider that a complication. I don't consider that a problem. You see they go and do what, what you was, what you were going to do in the first place.Open
QuotationThe main problems that we have seen in history from disease are not related with the approach that you got are related with the inexperience and technical incapacity of the surgeon that damaged the baby on a permanent basis both ways laparoscopically or transenally, a bad surgeon will damage the patient.Open
StatementThe main problems in Hirschsprung disease are not related to the surgical approach (laparoscopic vs. transanal) but to inexperience and technical incapacity of the surgeon that damages the patient permanently.Open
QuotationI never heard people complaining about the scar or not the scar. I see patients complaining about fecal incontinence remaining that's the real problem, not the size of the scar.Open
StatementPatients complain about fecal incontinence, not about the size of the scar—that is the real problem.Open
StatementThe Lone Star retractor hooks should be placed at the pectinate line itself, not in the anal canal, to protect the anal canal by definition.Open
QuotationThe surgeons should be careful not to stretch too much because you start the dissection and you forget you want to be comfortable. You put retractors and I walk into the operating room and see sometimes a big hole that what used to be the anus becomes a huge hole and that is damaging by definition the sphincter mechanism.Open
StatementSurgeons should be careful not to stretch too much during transanal dissection because excessive stretching damages the sphincter mechanism by definition, potentially causing fecal incontinence.Open
StatementThe keys to avoiding strictures and retractions are: respecting the pectinate line and anal canal, not stretching the anus too much, mobilizing the rectum to ensure normal ganglionic bowel with good blood supply, and performing anastomosis with no tension.Open
StatementProne position is preferred over lithotomy position for transanal procedures because in lithotomy the surgeon is the only one who can see, the field is vertical causing instrument loss, and the scrub nurse has difficulty seeing.Open
StatementLeaving 1-2 centimeters of aganglionosis does not explain why patients don't behave well—some patients operated with exactly the same technique do beautifully while others have enterocolitis symptoms, and we don't know why.Open
QuotationI don't blame on the 1 centimeter or 1.5 centimeters or 2 centimeters of ganglionnosis to explain why a patient doesn't behave well.Open
QuotationWe have been obsessed with the idea of leaving 1 centimeter or two a ganglionnosis, and that explains symptoms. That simply is not true from my point of view.Open
StatementThe majority of patients who come with symptoms of retention (enterocolitis or constipation) after pull-through do not have a portion of ganglionic bowel left—they simply behave like that and we do not know why.Open
StatementWhen we remove the rectum of a human being, we are already seriously affecting the mechanisms of bowel control because we are removing the natural reservoir of the patient.Open
QuotationWhen we remove the rectum of a human being, we are already affecting seriously the mechanisms of bowel control because we are removing the natural reservoir of the patient.Open
StatementEven adult ulcerative colitis patients with perfect operations have problems with bowel control—they have accidents at night—because we are connecting a piece of colon that is constantly moving to where the rectum normally rests.Open
StatementEven in patients with a very well preserved anal canal, some children have different degrees of fecal incontinence after Hirschsprung surgery.Open
StatementFecal incontinence in Hirschsprung disease is much more common than we believe, and we have not been discussing it enough in pediatric surgical meetings.Open
QuotationWe have not been discussing enough in our pediatric surgical meetings the problem of fecal incontinence that is much more common than we believe.Open
StatementIf you preserve the pectinate line, by definition you are preserving this crucial part of the bowel.Open
QuotationThe patients are born with bowel control. We provoke the fecal incontinence.Open
StatementPatients are born with bowel control—we provoke the fecal incontinence through surgical technique.Open
StatementThe anterior dissection of the rectal wall must be conducted in a very meticulous way because the rectum has a common wall with the vagina and the prostatic urethra—fistulas to the vagina or urinary tract are unacceptable complications.Open
StatementIf you see fat around the rectum during full-thickness dissection, you can get closer to the rectum because that means you are not in the real rectal wall.Open
QuotationIf you stay right in the bowel wall, you will not provoke the innervation of the urinary tract.Open
StatementIf you stay right on the bowel wall during dissection, you will not provoke denervation of the urinary tract.Open
StatementWaiting until the patient is toilet trained for urine (usually around 3 years of age) before closing the ileostomy results in the baby becoming totally trained for stool soon after closure, provided you did a correct operation and preserved the anal canal.Open
QuotationThe baby is happy with the ileostomy. The only unhappy people are others, but not the baby.Open
StatementIf you take a child that has been traumatized with rectal maneuvers and try to do rectal irrigations, there will be problems, whereas if a patient has an ileostomy and the mother does rectal irrigations once in a while with a soft catheter, the baby will accept that.Open
QuotationIf you take a child that has been traumatized with rectal maneuvers and you try to do rectal irrigations, it's going to be a lot of problems, whereas if a patient has an ileostomy and the mother does rectal irrigations once in a while with a soft catheter, the baby will accept that.Open
StatementPatients with total colonic aganglionosis have a high incidence of enterocolitis and will most likely need rectal irrigation, which is difficult to do in a 3-year-old with severe diaper rash who doesn't want anything near the anus.Open
StatementThere are two big types of Hirschsprung disease: a benign type where patients never have enterocolitis and can go for years with huge megacolon, and a bad group with manifestations during the newborn period who have great tendency to suffer enterocolitis even with a good operation.Open
Panel Discussion and Case Presentation Part II: Pediatric Bowel Management 2013
StatementIn Spain, de la Torre technique is used; for total colonic aganglionosis, Lester Martin procedure is used.Open
StatementWhen the anal canal is destroyed, the patient will not have bowel control; with total colonic aganglionosis producing liquid stool, there is no bowel management possible.Open
StatementA permanent stoma is indicated when the anal canal is destroyed and the patient has total colonic aganglionosis.Open
StatementSometimes constipating diet and fiber are tried to convince parents that there is no other option except permanent stoma; this is one of the few indications for permanent stoma.Open
QuotationMost pediatricians all over the world don't know the difference between enterocolitis and gastroenteritis.Open
StatementMost pediatricians worldwide do not know the difference between enterocolitis and gastroenteritis; they do not understand the entity called post-Hirschsprung enterocolitis.Open
StatementPediatricians do not understand why irrigations are necessary; they see dilated bowel on X-ray and think it is intestinal obstruction, not recognizing the entity itself.Open
StatementWhen starting dissection 2 cm above the dentate line and pulling bowel through, the upper mucosa is often damaged, and the anastomosis ends up about 1 cm above the dentate line.Open
StatementDr. Pena does not believe that leaving 1-2 cm of rectal mucosa is the simple cause of enterocolitis.Open
StatementA group in New York doing neonatal Soave primary procedures reported zero enterocolitis; when Dr. Pena followed some of those patients, many had fecal incontinence.Open
QuotationIf you produce fecal incontinence in the patient, the enterocolitis is zero.Open
StatementIf you produce fecal incontinence in a patient, enterocolitis is zero; a patient with destroyed anal canal is equivalent to a stoma, and patients with stomas rarely have enterocolitis.Open
StatementA good operation preserving the sphincter and anal canal creates sphincter closure, which creates stasis, and stasis produces enterocolitis.Open
QuotationWe don't know why the patients have enterocolitis, but if you produce, if you have very little enterocolitis, chances are that you are damaging the anal canal.Open
QuotationI prefer to deal with enterocolitis than with fecal incontinence. Fecal incontinence is for life.Open
StatementDr. Pena prefers to deal with enterocolitis rather than fecal incontinence; fecal incontinence is for life.Open
StatementDr. Pena's incidence of enterocolitis in pull-through patients is about 30%.Open
StatementResecting the rectosigmoid introduces a major pathophysiological change; children with perfect pull-throughs preserving the anal canal sometimes have toilet-training problems without explanation.Open
StatementHyperactive children with attention deficit disorder have more toilet-training problems because they have a piece of colon that does not act like a reservoir, connected to the rectum and moving constantly; significant cooperation from the child is required.Open
StatementEven with a perfect operation, patients may have certain toilet-training problems; if the anal canal is destroyed, they will be totally incontinent; partial anal canal destruction causes more problems.Open
StatementDr. Pena does not find rectal manometry useful in Hirschsprung disease, constipation, or anorectal malformations after many years of experience.Open
StatementPatients are discharged with three irrigations per day and metronidazole (Flagyl); every month the number of irrigations is decreased.Open
StatementIf decreasing irrigations causes recurrent enterocolitis and the patient does not tolerate lack of irrigation, and by 6 months post-op the patient is still on irrigations, parents become very nervous; at that point, other options are discussed.Open
StatementFor refractory enterocolitis, another option is further resection of normal ganglionic colon to remove more.Open
StatementHirschsprung disease is much more than ganglion cells vs. no ganglion cells; we do not know why some patients never have enterocolitis and toilet-train early, behaving like normal children, while others have severe enterocolitis from day one.Open
Statement'Benign Hirschsprung disease' patients present at 8-10 years old with classic imaging and abdominal distention but never had enterocolitis, grew and developed normally, and do very well after surgery.Open
StatementIn the United States, earlier diagnosis is being made of patients with 'bad Hirschsprung'—enterocolitis from day one, very sick, and high incidence of enterocolitis after surgery.Open
QuotationThere is a lot of things that we don't know about Higp disease. Some bowel has no the story is much more than absent ganglion cells.Open
StatementThere is much we do not know about Hirschsprung disease; the story is much more than absent ganglion cells, and taking bowel with normal ganglion cells down does not mean that bowel is 100% normal.Open
StatementSome believe that ganglionic bowel may have neuronal intestinal dysplasia, but this is a very controversial histopathological diagnosis; we do not know what is wrong and must learn much more.Open
StatementDr. Pena is skeptical that the cuff produces obstruction; to believe it, he would need to see the cuff producing real obstruction manifested by very dilated colon above the cuff, which is very unusual.Open
StatementDr. Mark Levitt has experience dealing with obstructive cuffs transanally; laparoscopic approach is not a bad idea but Dr. Pena has never heard of it being done laparoscopically.Open
StatementPatients with total fecal incontinence have no enterocolitis; all operations moving toward fecal incontinence (myectomies, myotomies, Botox, massive dilatation, putting 3 fingers in the rectum) are temporary or permanent moves toward incontinence.Open
StatementPatients subjected to myotomies, myectomies, or repeated Botox injections eventually develop more severe fecal incontinence.Open
StatementDr. Pena does not believe in myectomy/myotomy/Botox procedures and does not use Botox.Open
Summaries Alberto gave as host
· 18 summaries
Recaps of other experts' statements, not Alberto's own clinical position.
Summaries Alberto gave as host · Abdominal Wall Defects2 summaries
Host summaryAlberto Peña summarizing a resource: 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.Open
Host summaryAlberto Peña summarizing a resource: being a male is much more than having a phallusOpen
Summaries Alberto gave as host · Cloacal Exstrophy4 summaries
Host summaryAlberto Peña summarizing a resource: who told you that sex is the most important thing? I want, I wanted my go nuts backOpen
Host summaryAlberto Peña summarizing a resource: being a male is much more than having a phallusOpen
Cloacal Exstrophy with Dr. Alberto Peña
Host summaryAlberto Peña summarizing a resource: 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.Open
Host summaryAlberto Peña summarizing a resource: being a male is much more than having a phallusOpen
Summaries Alberto gave as host · Colorectal / ARM & Hirschsprung7 summaries
Outcomes and Complications in Hirschsprung Disease
Host summaryAlberto Peña summarizing the discussion: Constipation is partially preventable; if a very dilated piece of bowel is left, even if ganglionic, patients will likely suffer from constipation because very dilated colon suffers from hypomotility.Open
Host summaryAlberto Peña summarizing the discussion: The very dilated colon suffers for hypomotility, poor motility, and therefore the patients suffer from constipation.Open
Host summaryAlberto Peña summarizing the discussion: Prophylactic postoperative irrigations (3 times per day for the first month with oral Flagyl, tapering over 3 months) can prevent enterocolitis after pull-through; a Salt Lake City study demonstrated this approach.Open
Surgical Procedures for Hirschsprung Disease
Host summaryAlberto Peña summarizing the discussion: If you stay right on the bowel wall during dissection, you will not provoke denervation of the urinary tract, as Dr. Swenson emphasized.Open
Host summaryAlberto Peña summarizing the discussion: Congenital central hypoventilation syndrome is associated with Ondine's curse, where patients stop breathing when they fall asleep.Open
Cloacal Exstrophy with Dr. Alberto Peña
Host summaryAlberto Peña summarizing a resource: 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.Open
Host summaryAlberto Peña summarizing a resource: being a male is much more than having a phallusOpen
Summaries Alberto gave as host · Enterocolitis3 summaries
Outcomes and Complications in Hirschsprung Disease
Host summaryAlberto Peña summarizing the discussion: Constipation is partially preventable; if a very dilated piece of bowel is left, even if ganglionic, patients will likely suffer from constipation because very dilated colon suffers from hypomotility.Open
Host summaryAlberto Peña summarizing the discussion: The very dilated colon suffers for hypomotility, poor motility, and therefore the patients suffer from constipation.Open
Host summaryAlberto Peña summarizing the discussion: Prophylactic postoperative irrigations (3 times per day for the first month with oral Flagyl, tapering over 3 months) can prevent enterocolitis after pull-through; a Salt Lake City study demonstrated this approach.Open
Summaries Alberto gave as host · Total Colonic Aganglionosis2 summaries
Host summaryAlberto Peña summarizing the discussion: If you stay right on the bowel wall during dissection, you will not provoke denervation of the urinary tract, as Dr. Swenson emphasized.Open
Host summaryAlberto Peña summarizing the discussion: Congenital central hypoventilation syndrome is associated with Ondine's curse, where patients stop breathing when they fall asleep.Open