Alberto Peña

1236 timestamped statements across 8 collections — auto-found in recorded discussions, each timestamp jumps to the exact moment.

Abdominal Wall Defects · guest expert Colorectal / ARM & Hirschsprung · guest expert

Featured diaries

▶ Ep 18 · 36:54
I 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.
quote · Colorectal / ARM & Hirschsprung
▶ Ep 18 · 36:54
I 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.
quote · Colorectal / ARM & Hirschsprung
▶ Ep 3 · 36:54
I 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.
quote · Enterocolitis
▶ Ep 6 · 36:54
I 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.
quote · Hirschsprung disease
▶ Ep 6 · 36:54
I 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.
quote · Hirschsprung disease
▶ Ep 2 · 17:29
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.
quote · Cloaca

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Abdominal Wall Defects 140 entries

Cloacal Exstrophy with Dr. Alberto Peña

▶ Ep 19 · 1:31
clinical Cloacal exstrophy is a spectrum of congenital malformations affecting the gastrointestinal/colorectal area, urogenital tract, spine and cord, and sometimes lower extremity motion. ↗
▶ Ep 19 · 1:31
clinical Cloacal exstrophy is a spectrum of congenital malformations affecting the gastrointestinal/colorectal area, urogenital tract, spine and cord, and sometimes lower extremity motion. ↗
▶ Ep 19 · 2:09
clinical Babies with cloacal exstrophy are born with an omphalocele, bladder exstrophy (two separated hemibladders), open cecum between the hemibladders, and separated pubic bones. ↗
▶ Ep 19 · 2:09
clinical Babies with cloacal exstrophy are born with an omphalocele, bladder exstrophy (two separated hemibladders), open cecum between the hemibladders, and separated pubic bones. ↗
▶ Ep 19 · 3:08
clinical The small bowel can become exstrophic through the ileocecal valve, creating an 'elephant trunk' appearance. ↗
▶ Ep 19 · 3:08
clinical The small bowel can become exstrophic through the ileocecal valve, creating an 'elephant trunk' appearance. ↗
▶ Ep 19 · 3:32
clinical Male patients have two separated hemiphalluses with normal gonads; female patients have two hemivaginas below the exstrophic bladder leading to two hemiuteri. ↗
▶ Ep 19 · 3:32
clinical Male patients have two separated hemiphalluses with normal gonads; female patients have two hemivaginas below the exstrophic bladder leading to two hemiuteri. ↗
▶ Ep 19 · 4:20
clinical Cloacal exstrophy represents a spectrum of colonic anatomy from normal colon to almost absent or completely absent colon, sometimes with two ceca or two appendices and bizarre blood supply. ↗
▶ Ep 19 · 4:20
clinical Cloacal exstrophy represents a spectrum of colonic anatomy from normal colon to almost absent or completely absent colon, sometimes with two ceca or two appendices and bizarre blood supply. ↗
▶ Ep 19 · 4:57
clinical The amount of colon present at birth has very important implications for the patient's management and outcomes. ↗
▶ Ep 19 · 4:57
clinical The amount of colon present at birth has very important implications for the patient's management and outcomes. ↗
▶ Ep 19 · 5:44
clinical A variant exists where babies are born with intact abdominal skin (no omphalocele, no bladder exstrophy externally) but have a completely open bladder inside with no bladder neck and a single large perineal orifice. ↗
▶ Ep 19 · 5:44
clinical A variant exists where babies are born with intact abdominal skin (no omphalocele, no bladder exstrophy externally) but have a completely open bladder inside with no bladder neck and a single large perineal orifice. ↗
▶ Ep 19 · 7:02
clinical While surgical techniques, intensive care, parenteral nutrition, and metabolic management have improved, functional outcomes (bowel control, urinary control, sexual function, spinal abnormalities) remain severely limited and cannot be made normal. ↗
▶ Ep 19 · 7:02
clinical While surgical techniques, intensive care, parenteral nutrition, and metabolic management have improved, functional outcomes (bowel control, urinary control, sexual function, spinal abnormalities) remain severely limited and cannot be made normal. ↗
▶ Ep 19 · 8:31
clinical Historical practice was to perform bilateral gonadectomy, remove hemiphalluses, create a vagina with bowel, and assign female gender to XY patients with cloacal exstrophy. ↗
▶ Ep 19 · 8:31
clinical Historical practice was to perform bilateral gonadectomy, remove hemiphalluses, create a vagina with bowel, and assign female gender to XY patients with cloacal exstrophy. ↗
▶ Ep 19 · 9:16
clinical Patients raised as female despite XY chromosomes exhibited male attitudes and behavior, and many became upset upon learning their chromosomal sex and that gonads were removed without their consent. ↗
▶ Ep 19 · 9:16
clinical Patients raised as female despite XY chromosomes exhibited male attitudes and behavior, and many became upset upon learning their chromosomal sex and that gonads were removed without their consent. ↗
▶ Ep 19 · 9:59
opinion Patients argued that sex is not the most important aspect of being male, that they wanted their gonads back for fertility (modern techniques allow fertilization and children), and that being male is much more than having a phallus. ↗
▶ Ep 19 · 9:59
quote 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. ↗
▶ Ep 19 · 9:59
opinion Patients argued that sex is not the most important aspect of being male, that they wanted their gonads back for fertility (modern techniques allow fertilization and children), and that being male is much more than having a phallus. ↗
▶ Ep 19 · 9:59
quote 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. ↗
▶ Ep 19 · 10:29
quote being a male is much more than having a phallus ↗
▶ Ep 19 · 10:29
quote being a male is much more than having a phallus ↗
▶ Ep 19 · 10:34
guideline Current consensus is that XY patients should be raised as male, with pediatric urologists and plastic surgeons working on phallus reconstruction techniques. ↗
▶ Ep 19 · 10:34
guideline Current consensus is that XY patients should be raised as male, with pediatric urologists and plastic surgeons working on phallus reconstruction techniques. ↗
▶ Ep 19 · 12:13
clinical When a prominent pediatric urologist dominates management, patients receive good urologic attention but inadequate gastrointestinal care; the reverse occurs when pediatric surgeons dominate. ↗
▶ Ep 19 · 12:13
clinical When a prominent pediatric urologist dominates management, patients receive good urologic attention but inadequate gastrointestinal care; the reverse occurs when pediatric surgeons dominate. ↗
▶ Ep 19 · 13:03
clinical The pediatric surgeon's role in the initial operation is to close the omphalocele (if possible), separate urothelium from intestinal mucosa by placing stitches at the edges and making an incision, allowing the urologist to bring hemibladders together. ↗
▶ Ep 19 · 13:03
clinical The pediatric surgeon's role in the initial operation is to close the omphalocele (if possible), separate urothelium from intestinal mucosa by placing stitches at the edges and making an incision, allowing the urologist to bring hemibladders together. ↗
▶ Ep 19 · 14:55
clinical It is very common but very harmful for pediatric surgeons to simply create an ileostomy, leaving all colon distally attached to the urinary tract. ↗
▶ Ep 19 · 14:55
clinical It is very common but very harmful for pediatric surgeons to simply create an ileostomy, leaving all colon distally attached to the urinary tract. ↗
▶ Ep 19 · 15:11
quote That is a very bad thing to do, and the patient will suffer several consequences because of that ↗
▶ Ep 19 · 15:11
quote That is a very bad thing to do, and the patient will suffer several consequences because of that ↗
▶ Ep 19 · 15:31
clinical Leaving colon attached to the urinary tract creates a congenital bladder augmentation that causes hyperchloremic acidosis from urine absorption, interfering with growth and development. ↗
▶ Ep 19 · 15:31
clinical Leaving colon attached to the urinary tract creates a congenital bladder augmentation that causes hyperchloremic acidosis from urine absorption, interfering with growth and development. ↗
▶ Ep 19 · 15:40
quote Isn't that beautiful from your point of view? ↗
▶ Ep 19 · 15:40
quote Isn't that beautiful from your point of view? ↗
▶ Ep 19 · 16:03
clinical Defunctionalized colonic tissue left distally will not grow; colon requires passage of fecal matter through its lumen to grow. ↗
▶ Ep 19 · 16:03
clinical Defunctionalized colonic tissue left distally will not grow; colon requires passage of fecal matter through its lumen to grow. ↗
▶ Ep 19 · 16:23
clinical The pediatric surgeon must incorporate all gastrointestinal tissue into the fecal stream and create a true end colostomy to ensure fecal matter passes through all colonic tissue. ↗
▶ Ep 19 · 16:23
clinical The pediatric surgeon must incorporate all gastrointestinal tissue into the fecal stream and create a true end colostomy to ensure fecal matter passes through all colonic tissue. ↗
▶ Ep 19 · 16:41
clinical Patients who received ileostomy with defunctionalized colon present at 2-3 years with poor growth, hyperchloremic acidosis managed by nephrologists, and large ileostomy losses. ↗
▶ Ep 19 · 16:41
clinical Patients who received ileostomy with defunctionalized colon present at 2-3 years with poor growth, hyperchloremic acidosis managed by nephrologists, and large ileostomy losses. ↗
▶ Ep 19 · 17:03
clinical The rescue operation involves taking down the ileostomy, finding and incorporating colonic tissue into the GI tract, and creating an end colostomy; acidosis disappears the next day. ↗
▶ Ep 19 · 17:03
clinical The rescue operation involves taking down the ileostomy, finding and incorporating colonic tissue into the GI tract, and creating an end colostomy; acidosis disappears the next day. ↗
▶ Ep 19 · 19:13
opinion Surgeons must accept that cloacal exstrophy is a spectrum and be prepared to deal with complex, variable colonic anatomy rather than taking the easy way out with an ileostomy. ↗
▶ Ep 19 · 19:13
opinion Surgeons must accept that cloacal exstrophy is a spectrum and be prepared to deal with complex, variable colonic anatomy rather than taking the easy way out with an ileostomy. ↗
▶ Ep 19 · 20:07
quote Do not leave gastrointestinal tract inside the functionalized. That's the main, main thing. ↗
▶ Ep 19 · 20:07
quote Do not leave gastrointestinal tract inside the functionalized. That's the main, main thing. ↗
▶ Ep 19 · 21:00
clinical Some institutions routinely perform pelvic osteotomy at the initial operation to facilitate bladder and omphalocele reconstruction, while others wait 2-3 months to do it separately. ↗
▶ Ep 19 · 21:00
clinical Some institutions routinely perform pelvic osteotomy at the initial operation to facilitate bladder and omphalocele reconstruction, while others wait 2-3 months to do it separately. ↗
▶ Ep 19 · 21:09
clinical Even after osteotomy, it is very difficult to see a cloacal exstrophy patient with pubic bones completely together; they usually remain separated. ↗
▶ Ep 19 · 21:09
clinical Even after osteotomy, it is very difficult to see a cloacal exstrophy patient with pubic bones completely together; they usually remain separated. ↗
▶ Ep 19 · 22:06
clinical Even a technically correct end colostomy often has poor motility, and babies may not pass stool easily, sometimes developing bacterial overgrowth similar to Hirschsprung disease. ↗
▶ Ep 19 · 22:06
clinical Even a technically correct end colostomy often has poor motility, and babies may not pass stool easily, sometimes developing bacterial overgrowth similar to Hirschsprung disease. ↗
▶ Ep 19 · 23:00
clinical Families must be taught to irrigate the colostomy with small volumes of saline through a tube to evacuate fecal material when peristalsis is inadequate. ↗
▶ Ep 19 · 23:00
clinical Families must be taught to irrigate the colostomy with small volumes of saline through a tube to evacuate fecal material when peristalsis is inadequate. ↗
▶ Ep 19 · 23:30
clinical Decision-making for bowel control begins around age 3 when parents want to send the child to school clean and dry (no stool or urine in diaper). ↗
▶ Ep 19 · 23:30
clinical Decision-making for bowel control begins around age 3 when parents want to send the child to school clean and dry (no stool or urine in diaper). ↗
▶ Ep 19 · 23:54
clinical Most cloacal exstrophy patients have an inadequate, tiny bladder requiring bladder augmentation with gastrointestinal tract, necessitating coordination between pediatric surgery and urology. ↗
▶ Ep 19 · 23:54
clinical Most cloacal exstrophy patients have an inadequate, tiny bladder requiring bladder augmentation with gastrointestinal tract, necessitating coordination between pediatric surgery and urology. ↗
▶ Ep 19 · 24:42
clinical Patients born with no colon are candidates for permanent colostomy and should never have terminal ileum pulled through, even if sphincter evidence exists, because they will never have bowel control. ↗
▶ Ep 19 · 24:42
clinical Patients born with no colon are candidates for permanent colostomy and should never have terminal ileum pulled through, even if sphincter evidence exists, because they will never have bowel control. ↗
▶ Ep 19 · 25:04
clinical Pull-through is only considered for patients with capacity to form solid stool (adequate colon), as bowel management only works with solid stool. ↗
▶ Ep 19 · 25:04
clinical Pull-through is only considered for patients with capacity to form solid stool (adequate colon), as bowel management only works with solid stool. ↗
▶ Ep 19 · 25:20
clinical It is extremely unusual for cloacal exstrophy patients to have spontaneous bowel control; the overwhelming majority need a bowel management program (enema administration to keep patient clean). ↗
▶ Ep 19 · 25:20
clinical It is extremely unusual for cloacal exstrophy patients to have spontaneous bowel control; the overwhelming majority need a bowel management program (enema administration to keep patient clean). ↗
▶ Ep 19 · 25:47
clinical Pediatric 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. ↗
▶ Ep 19 · 25:47
clinical Pediatric 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. ↗
▶ Ep 19 · 26:07
clinical Annual contrast studies through the colostomy (retrograde injection) are performed to assess colonic growth. ↗
▶ Ep 19 · 26:07
clinical Annual contrast studies through the colostomy (retrograde injection) are performed to assess colonic growth. ↗
▶ Ep 19 · 26:41
clinical Before 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. ↗
▶ Ep 19 · 26:41
clinical Before 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. ↗
▶ Ep 19 · 27:35
clinical Bowel management trial through colostomy is typically started after age 3 when families consider avoiding the stoma for school. ↗
▶ Ep 19 · 27:35
clinical Bowel management trial through colostomy is typically started after age 3 when families consider avoiding the stoma for school. ↗
▶ Ep 19 · 28:07
clinical If 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. ↗
▶ Ep 19 · 28:07
clinical If 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. ↗
▶ Ep 19 · 28:54
clinical The 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. ↗
▶ Ep 19 · 28:54
clinical The 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. ↗
▶ Ep 19 · 29:56
clinical Contrast enema through the colostomy can distinguish true liquid stool from paradoxical diarrhea (liquid stool around solid fecal impaction). ↗
▶ Ep 19 · 29:56
clinical Contrast enema through the colostomy can distinguish true liquid stool from paradoxical diarrhea (liquid stool around solid fecal impaction). ↗
▶ Ep 19 · 31:04
clinical Pull-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). ↗
▶ Ep 19 · 31:04
clinical Pull-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). ↗
▶ Ep 19 · 31:28
clinical During 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. ↗
▶ Ep 19 · 31:28
clinical During 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. ↗
▶ Ep 19 · 31:57
clinical Urologists 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. ↗
▶ Ep 19 · 31:57
clinical Urologists 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. ↗
▶ Ep 19 · 32:18
clinical Occasionally, 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. ↗
▶ Ep 19 · 32:18
clinical Occasionally, 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. ↗
▶ Ep 19 · 33:13
clinical Midline abdominal incision from xiphoid to pubis is used for pull-through to preserve the flanks and quadrants for potential future stomas. ↗
▶ Ep 19 · 33:13
clinical Midline abdominal incision from xiphoid to pubis is used for pull-through to preserve the flanks and quadrants for potential future stomas. ↗
▶ Ep 19 · 33:56
clinical The 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. ↗
▶ Ep 19 · 33:56
clinical The 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. ↗
▶ Ep 19 · 34:39
clinical Careful 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. ↗
▶ Ep 19 · 34:39
clinical Careful 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. ↗
▶ Ep 19 · 35:53
clinical The 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. ↗
▶ Ep 19 · 35:53
clinical The 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. ↗
▶ Ep 19 · 37:33
clinical Cloacal 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). ↗
▶ Ep 19 · 37:33
clinical Cloacal 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). ↗
▶ Ep 19 · 37:38
clinical Some 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. ↗
▶ Ep 19 · 37:38
clinical Some 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. ↗
▶ Ep 19 · 38:50
clinical During pull-through, vaginal reconstruction is attempted by approximating the hemivaginas as much as possible; the degree of separation varies on the spectrum. ↗
▶ Ep 19 · 38:50
clinical During pull-through, vaginal reconstruction is attempted by approximating the hemivaginas as much as possible; the degree of separation varies on the spectrum. ↗
▶ Ep 19 · 39:07
clinical When 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. ↗
▶ Ep 19 · 39:07
clinical When 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. ↗
▶ Ep 19 · 39:53
clinical Patients with functional hemiuterus may become pregnant, but it is high-risk pregnancy requiring specialized pediatric gynecology follow-up; in general, pregnancy is not advised. ↗
▶ Ep 19 · 39:53
clinical Patients with functional hemiuterus may become pregnant, but it is high-risk pregnancy requiring specialized pediatric gynecology follow-up; in general, pregnancy is not advised. ↗
▶ Ep 19 · 40:10
clinical Hemiuterus has a great tendency to produce miscarriages and premature labor; delivery must be by cesarean section due to limited abdominal space. ↗
▶ Ep 19 · 40:10
clinical Hemiuterus has a great tendency to produce miscarriages and premature labor; delivery must be by cesarean section due to limited abdominal space. ↗
▶ Ep 19 · 40:37
clinical Bladder 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. ↗
▶ Ep 19 · 40:37
clinical Bladder 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. ↗
▶ Ep 19 · 41:07
clinical Augmented 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. ↗
▶ Ep 19 · 41:07
clinical Augmented 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. ↗
▶ Ep 19 · 41:33
clinical Bladder irrigation is sometimes performed with gentamicin to ensure all mucus is removed. ↗
▶ Ep 19 · 41:33
clinical Bladder irrigation is sometimes performed with gentamicin to ensure all mucus is removed. ↗
▶ Ep 19 · 41:45
clinical Lifelong 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. ↗
▶ Ep 19 · 41:45
clinical Lifelong 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. ↗
▶ Ep 19 · 42:29
clinical Patients 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. ↗
▶ Ep 19 · 42:29
clinical Patients 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. ↗
▶ Ep 19 · 43:03
opinion Dr. 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. ↗
▶ Ep 19 · 43:03
opinion Dr. 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. ↗
▶ Ep 19 · 43:42
clinical The 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. ↗
▶ Ep 19 · 43:42
clinical The 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. ↗
▶ Ep 19 · 44:48
opinion Colorectal 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. ↗
▶ Ep 19 · 44:48
opinion Colorectal 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. ↗
▶ Ep 19 · 46:00
clinical The 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). ↗
▶ Ep 19 · 46:00
clinical The 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). ↗
▶ Ep 19 · 46:54
clinical Between the initial operation and pull-through, colostomy irrigation is often needed to manage poor colonic motility. ↗
▶ Ep 19 · 46:54
clinical Between the initial operation and pull-through, colostomy irrigation is often needed to manage poor colonic motility. ↗
▶ Ep 19 · 47:58
clinical Prenatal 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. ↗
▶ Ep 19 · 47:58
clinical Prenatal 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. ↗
▶ Ep 19 · 48:48
clinical Absent bladder on prenatal ultrasound (due to exstrophy) is a bad sign and can be detected as early as week 20 of pregnancy. ↗
▶ Ep 19 · 48:48
clinical Absent bladder on prenatal ultrasound (due to exstrophy) is a bad sign and can be detected as early as week 20 of pregnancy. ↗
▶ Ep 19 · 49:38
clinical Prenatal diagnosis allows families to decide about pregnancy continuation and, if continuing, to deliver at a center with a multidisciplinary team experienced in these malformations. ↗
▶ Ep 19 · 49:38
clinical Prenatal diagnosis allows families to decide about pregnancy continuation and, if continuing, to deliver at a center with a multidisciplinary team experienced in these malformations. ↗
▶ Ep 19 · 50:04
opinion Certain 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. ↗
▶ Ep 19 · 50:04
opinion Certain 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. ↗
Anorectal Malformation 4 entries

Cloaca - Prental Imaging & Diagnosis - Counseling

▶ Ep 10 · 30:26
quote Not everybody gets the privilege to get a specialized radiologist is dedicated to the prenatal diagnosis of this malformation that we have. ↗
▶ Ep 10 · 30:42
clinical Dr. 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 hydrocolpos ↗
▶ Ep 10 · 31:37
quote The images were there. What failed was the index of suspicion in the those radiologist. ↗
▶ Ep 10 · 32:15
clinical If 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 diagnosis ↗
Cloaca 19 entries

Cloaca - Workup & Evaluation

▶ Ep 2 · 8:46
quote I 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. ↗
▶ Ep 2 · 8:46
clinical The measurements from 3D cloacography are accurate to within millimeters; when the radiologist reports 3.5 cm, the intraoperative measurement is typically 3.5 cm. ↗
▶ Ep 2 · 17:26
opinion In neonatal cloacas, trying to make a very accurate anatomic diagnosis during the newborn period is useless and may actually hurt the baby. ↗
▶ Ep 2 · 17:29
clinical In 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. ↗
▶ Ep 2 · 17:29
quote 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. ↗
▶ Ep 2 · 18:09
opinion Knowing whether a neonate with cloaca has reflux is not crucial; the priority is decompressing the gastrointestinal and urogenital tracts. ↗
▶ Ep 2 · 18:09
quote 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. ↗
▶ Ep 2 · 18:39
quote Not everybody has the capacity to do it and don't force the endoscope in a tiny structure because you may end up hurting. ↗
▶ Ep 2 · 18:39
clinical Forcing an endoscope into a tiny neonatal cloacal structure can cause harm. ↗
▶ Ep 2 · 19:04
quote Many 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. ↗
▶ Ep 2 · 19:04
guideline Nephrostomy, ureterostomy, and vesicostomy are not indicated in most neonatal cloacas. ↗
▶ Ep 2 · 19:14
quote Our 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. ↗
▶ Ep 2 · 19:14
guideline The 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. ↗
▶ Ep 2 · 19:25
clinical Some 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. ↗
▶ Ep 2 · 19:34
quote By by simply decompressing the hydrocorpus, the, the picture completely changes. ↗

Cloaca - Prental Imaging & Diagnosis - Counseling

▶ Ep 1 · 30:26
quote Not everybody gets the privilege to get a specialized radiologist is dedicated to the prenatal diagnosis of this malformation that we have. ↗
▶ Ep 1 · 30:42
clinical Dr. 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 hydrocolpos ↗
▶ Ep 1 · 31:37
quote The images were there. What failed was the index of suspicion in the those radiologist. ↗
▶ Ep 1 · 32:15
clinical If 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 diagnosis ↗
Colorectal / ARM & Hirschsprung 503 entries

Evaluation & Management Of Hirschsprung's Disease

▶ Ep 10 · 32:44
opinion Dr. Pena would perform rectal biopsy in a case of apparent small left colon because he cannot distinguish it from Hirschsprung disease. ↗
▶ Ep 10 · 32:44
opinion Dr. Pena would perform rectal biopsy in a case of apparent small left colon because he cannot distinguish it from Hirschsprung disease. ↗
▶ Ep 10 · 36:54
quote I 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. ↗
▶ Ep 10 · 36:54
opinion Dr. 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. ↗
▶ Ep 10 · 36:54
opinion Dr. 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. ↗
▶ Ep 10 · 36:54
quote I 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. ↗
▶ Ep 10 · 37:30
quote I wonder if all the patients that developed this traditional characteristic pouch of the duja male are actually patients that never had Hirk disease ↗
▶ Ep 10 · 37:30
quote I wonder if all the patients that developed this traditional characteristic pouch of the duja male are actually patients that never had Hirk disease ↗
▶ Ep 10 · 37:41
opinion Dr. 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. ↗
▶ Ep 10 · 37:41
opinion Dr. 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. ↗
▶ Ep 10 · 40:28
quote if 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. ↗
▶ Ep 10 · 40:28
opinion Dr. 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. ↗
▶ Ep 10 · 40:28
quote if 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. ↗
▶ Ep 10 · 40:28
opinion Dr. 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. ↗
▶ Ep 10 · 40:39
opinion Dr. 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. ↗
▶ Ep 10 · 40:39
opinion Dr. 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. ↗
▶ Ep 10 · 41:32
clinical There 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. ↗
▶ Ep 10 · 41:32
quote there 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. ↗
▶ Ep 10 · 41:32
clinical There 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. ↗
▶ Ep 10 · 41:32
quote there 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. ↗
▶ Ep 10 · 42:05
clinical Someone could take a biopsy in the normal physiologic aganglionic area and get a result of no ganglion cells, which does not indicate Hirschsprung disease. ↗
▶ Ep 10 · 42:05
clinical Someone could take a biopsy in the normal physiologic aganglionic area and get a result of no ganglion cells, which does not indicate Hirschsprung disease. ↗
▶ Ep 10 · 42:14
quote the 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 ↗
▶ Ep 10 · 42:14
opinion The 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. ↗
▶ Ep 10 · 42:14
opinion The 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. ↗
▶ Ep 10 · 42:14
quote the 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 ↗
▶ Ep 10 · 42:45
clinical If the internal sphincter thickening exists, nobody has determined its exact boundaries at different ages. ↗
▶ Ep 10 · 42:45
clinical If the internal sphincter thickening exists, nobody has determined its exact boundaries at different ages. ↗
▶ Ep 10 · 43:16
opinion Internal sphincter achalasia is a manometric concept, not an anatomic concept, similar to the lower esophageal sphincter. ↗
▶ Ep 10 · 43:16
quote alacia of alacia of the internal sphincter is a manometric concept, not an anatomic concept ↗
▶ Ep 10 · 43:16
opinion Dr. 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. ↗
▶ Ep 10 · 43:16
opinion Dr. 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. ↗
▶ Ep 10 · 43:16
opinion Internal sphincter achalasia is a manometric concept, not an anatomic concept, similar to the lower esophageal sphincter. ↗
▶ Ep 10 · 43:16
quote alacia of alacia of the internal sphincter is a manometric concept, not an anatomic concept ↗

Cloaca - Workup & Evaluation

▶ Ep 16 · 8:46
quote I 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. ↗
▶ Ep 16 · 8:46
clinical The measurements from 3D cloacography are accurate to within millimeters; when the radiologist reports 3.5 cm, the intraoperative measurement is typically 3.5 cm. ↗
▶ Ep 16 · 8:46
clinical The measurements from 3D cloacography are accurate to within millimeters; when the radiologist reports 3.5 cm, the intraoperative measurement is typically 3.5 cm. ↗
▶ Ep 16 · 8:46
quote I 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. ↗
▶ Ep 16 · 17:26
opinion In neonatal cloacas, trying to make a very accurate anatomic diagnosis during the newborn period is useless and may actually hurt the baby. ↗
▶ Ep 16 · 17:26
opinion In neonatal cloacas, trying to make a very accurate anatomic diagnosis during the newborn period is useless and may actually hurt the baby. ↗
▶ Ep 16 · 17:29
quote 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. ↗
▶ Ep 16 · 17:29
clinical In 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. ↗
▶ Ep 16 · 17:29
quote 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. ↗
▶ Ep 16 · 17:29
clinical In 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. ↗
▶ Ep 16 · 18:09
opinion Knowing whether a neonate with cloaca has reflux is not crucial; the priority is decompressing the gastrointestinal and urogenital tracts. ↗
▶ Ep 16 · 18:09
quote 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. ↗
▶ Ep 16 · 18:09
opinion Knowing whether a neonate with cloaca has reflux is not crucial; the priority is decompressing the gastrointestinal and urogenital tracts. ↗
▶ Ep 16 · 18:09
quote 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. ↗
▶ Ep 16 · 18:39
quote Not everybody has the capacity to do it and don't force the endoscope in a tiny structure because you may end up hurting. ↗
▶ Ep 16 · 18:39
clinical Forcing an endoscope into a tiny neonatal cloacal structure can cause harm. ↗
▶ Ep 16 · 18:39
clinical Forcing an endoscope into a tiny neonatal cloacal structure can cause harm. ↗
▶ Ep 16 · 18:39
quote Not everybody has the capacity to do it and don't force the endoscope in a tiny structure because you may end up hurting. ↗
▶ Ep 16 · 19:04
guideline Nephrostomy, ureterostomy, and vesicostomy are not indicated in most neonatal cloacas. ↗
▶ Ep 16 · 19:04
quote Many 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. ↗
▶ Ep 16 · 19:04
guideline Nephrostomy, ureterostomy, and vesicostomy are not indicated in most neonatal cloacas. ↗
▶ Ep 16 · 19:04
quote Many 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. ↗
▶ Ep 16 · 19:14
quote Our 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. ↗
▶ Ep 16 · 19:14
guideline The 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. ↗
▶ Ep 16 · 19:14
quote Our 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. ↗
▶ Ep 16 · 19:14
guideline The 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. ↗
▶ Ep 16 · 19:25
clinical Some 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. ↗
▶ Ep 16 · 19:25
clinical Some 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. ↗
▶ Ep 16 · 19:34
quote By by simply decompressing the hydrocorpus, the, the picture completely changes. ↗
▶ Ep 16 · 19:34
quote By by simply decompressing the hydrocorpus, the, the picture completely changes. ↗

Cloaca - Prental Imaging & Diagnosis - Counseling

▶ Ep 17 · 30:26
quote Not everybody gets the privilege to get a specialized radiologist is dedicated to the prenatal diagnosis of this malformation that we have. ↗
▶ Ep 17 · 30:26
quote Not everybody gets the privilege to get a specialized radiologist is dedicated to the prenatal diagnosis of this malformation that we have. ↗
▶ Ep 17 · 30:42
clinical Dr. 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 hydrocolpos ↗
▶ Ep 17 · 30:42
clinical Dr. 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 hydrocolpos ↗
▶ Ep 17 · 31:37
quote The images were there. What failed was the index of suspicion in the those radiologist. ↗
▶ Ep 17 · 31:37
quote The images were there. What failed was the index of suspicion in the those radiologist. ↗
▶ Ep 17 · 32:15
clinical If 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 diagnosis ↗
▶ Ep 17 · 32:15
clinical If 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 diagnosis ↗

Radiology and Image Diagnosis of Hirschsprung Disease

▶ Ep 18 · 32:37
clinical Dr. 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. ↗
▶ Ep 18 · 32:37
clinical Dr. 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. ↗
▶ Ep 18 · 36:54
opinion Dr. 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. ↗
▶ Ep 18 · 36:54
opinion Dr. 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. ↗
▶ Ep 18 · 36:54
quote I 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. ↗
▶ Ep 18 · 36:54
quote I 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. ↗
▶ Ep 18 · 37:30
opinion Dr. 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. ↗
▶ Ep 18 · 37:30
opinion Dr. 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. ↗
▶ Ep 18 · 37:41
quote I suspect that this patient never had Hodgkin disease. ↗
▶ Ep 18 · 37:41
quote I suspect that this patient never had Hodgkin disease. ↗
▶ Ep 18 · 40:26
opinion Dr. 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. ↗
▶ Ep 18 · 40:26
quote if 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. ↗
▶ Ep 18 · 40:26
opinion Dr. 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. ↗
▶ Ep 18 · 40:26
quote if 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. ↗
▶ Ep 18 · 40:44
quote this patient has no Hirshman disease ↗
▶ Ep 18 · 40:44
quote this patient has no Hirshman disease ↗
▶ Ep 18 · 41:00
quote there is no way to differentiate the so-called ultra short with idiopathic constipation ↗
▶ Ep 18 · 41:00
opinion Dr. Peña states there is no way to differentiate so-called ultra-short segment Hirschsprung from idiopathic constipation. ↗
▶ Ep 18 · 41:00
opinion Dr. Peña states there is no way to differentiate so-called ultra-short segment Hirschsprung from idiopathic constipation. ↗
▶ Ep 18 · 41:00
quote there is no way to differentiate the so-called ultra short with idiopathic constipation ↗
▶ Ep 18 · 41:32
quote there 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. ↗
▶ Ep 18 · 41:32
clinical The 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. ↗
▶ Ep 18 · 41:32
quote there 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. ↗
▶ Ep 18 · 41:32
clinical The 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. ↗
▶ Ep 18 · 41:42
opinion There 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. ↗
▶ Ep 18 · 41:42
opinion There 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. ↗
▶ Ep 18 · 41:56
quote That's another challenge for young pediatric surgeons. If you want to make a contribution. studies, this is a real challenge. ↗
▶ Ep 18 · 41:56
quote That's another challenge for young pediatric surgeons. If you want to make a contribution. studies, this is a real challenge. ↗
▶ Ep 18 · 42:05
opinion Somebody could take a biopsy in the normal aganglionic area and get a result of no ganglion cells, but it does not mean anything clinically. ↗
▶ Ep 18 · 42:05
quote conceivably 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. ↗
▶ Ep 18 · 42:05
quote conceivably 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. ↗
▶ Ep 18 · 42:05
opinion Somebody could take a biopsy in the normal aganglionic area and get a result of no ganglion cells, but it does not mean anything clinically. ↗
▶ Ep 18 · 42:14
opinion The 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. ↗
▶ Ep 18 · 42:14
quote the 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. ↗
▶ Ep 18 · 42:14
opinion The 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. ↗
▶ Ep 18 · 42:14
quote the 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. ↗
▶ Ep 18 · 42:45
opinion If 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. ↗
▶ Ep 18 · 42:45
opinion If 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. ↗
▶ Ep 18 · 43:16
opinion Internal 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. ↗
▶ Ep 18 · 43:16
quote alacia of alacia of the internal sphincter is a manometric concept, not an anatomic concept ↗
▶ Ep 18 · 43:16
quote alacia of alacia of the internal sphincter is a manometric concept, not an anatomic concept ↗
▶ Ep 18 · 43:16
opinion Internal 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. ↗
▶ Ep 18 · 43:36
quote 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 ↗
▶ Ep 18 · 43:36
opinion Dr. 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. ↗
▶ Ep 18 · 43:36
opinion Dr. 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. ↗
▶ Ep 18 · 43:36
quote 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 ↗
▶ Ep 18 · 43:48
opinion Botox and similar injections paralyze whatever muscle is there and facilitate passing of stool, but they are not curing a condition of unknown origin. ↗
▶ Ep 18 · 43:48
opinion Botox and similar injections paralyze whatever muscle is there and facilitate passing of stool, but they are not curing a condition of unknown origin. ↗

History of Hirschsprung Disease

▶ Ep 19 · 2:44
quote What 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. ↗
▶ Ep 19 · 2:44
quote What 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. ↗
▶ Ep 19 · 3:00
quote We 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. ↗
▶ Ep 19 · 3:00
quote We 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. ↗
▶ Ep 19 · 3:13
quote We are particularly grateful with Dr. Ova Swenson for his seminal contribution, and, and we still followed his principles in the management of this condition. ↗
▶ Ep 19 · 3:13
quote We are particularly grateful with Dr. Ova Swenson for his seminal contribution, and, and we still followed his principles in the management of this condition. ↗
▶ Ep 19 · 4:05
opinion The 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. ↗
▶ Ep 19 · 4:05
opinion The 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. ↗
▶ Ep 19 · 4:20
opinion Hirschsprung disease is not only about ganglion cells or no ganglion cells; it is a much more complex condition. ↗
▶ Ep 19 · 4:20
opinion Hirschsprung disease is not only about ganglion cells or no ganglion cells; it is a much more complex condition. ↗
▶ Ep 19 · 11:37
clinical Electric 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. ↗
▶ Ep 19 · 11:37
clinical Electric 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. ↗

Surgical Procedures for Hirschsprung Disease

▶ Ep 21 · 3:42
clinical Between 75 and 80% of Hirschsprung cases can be completed transanally, reaching normal ganglionic bowel from below. ↗
▶ Ep 21 · 3:42
clinical Between 75 and 80% of Hirschsprung cases can be completed transanally, reaching normal ganglionic bowel from below. ↗
▶ Ep 21 · 6:28
opinion If you start transanally and cannot reach ganglionic bowel, you simply open the abdomen and continue the resection—this is not a complication. ↗
▶ Ep 21 · 6:28
opinion If you start transanally and cannot reach ganglionic bowel, you simply open the abdomen and continue the resection—this is not a complication. ↗
▶ Ep 21 · 11:10
quote The 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. ↗
▶ Ep 21 · 11:10
quote The 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. ↗
▶ Ep 21 · 11:10
opinion The main problems in Hirschsprung surgery are related to surgeon inexperience and technical incapacity, not the approach (laparoscopic vs. transanal vs. open). ↗
▶ Ep 21 · 11:10
opinion The main problems in Hirschsprung surgery are related to surgeon inexperience and technical incapacity, not the approach (laparoscopic vs. transanal vs. open). ↗
▶ Ep 21 · 11:37
opinion A bad surgeon will damage the patient both ways—laparoscopically or transanally. ↗
▶ Ep 21 · 11:37
opinion A bad surgeon will damage the patient both ways—laparoscopically or transanally. ↗
▶ Ep 21 · 11:45
clinical The basic goal of Hirschsprung surgery is not to damage the sphincter mechanism, which has been damaged by both laparoscopic and non-laparoscopic techniques. ↗
▶ Ep 21 · 11:45
clinical The basic goal of Hirschsprung surgery is not to damage the sphincter mechanism, which has been damaged by both laparoscopic and non-laparoscopic techniques. ↗
▶ Ep 21 · 12:09
quote I 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. ↗
▶ Ep 21 · 12:09
quote I 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. ↗
▶ Ep 21 · 12:17
opinion Patients complain about fecal incontinence, not the size of the scar. ↗
▶ Ep 21 · 12:17
opinion Patients complain about fecal incontinence, not the size of the scar. ↗
▶ Ep 21 · 14:13
clinical When doing transanal dissection, the Lone Star retractor hooks should be placed at the pectinate line to protect the entire anal canal. ↗
▶ Ep 21 · 14:13
clinical When doing transanal dissection, the Lone Star retractor hooks should be placed at the pectinate line to protect the entire anal canal. ↗
▶ Ep 21 · 14:55
clinical Surgeons must be careful not to stretch the anus too much during transanal dissection, as excessive stretch damages the sphincter mechanism and causes fecal incontinence. ↗
▶ Ep 21 · 14:55
clinical Surgeons must be careful not to stretch the anus too much during transanal dissection, as excessive stretch damages the sphincter mechanism and causes fecal incontinence. ↗
▶ Ep 21 · 15:21
clinical The dissection should start 2 centimeters deep inside the rectum from the pectinate line, using multiple silk stitches to distribute tension and avoid tissue damage. ↗
▶ Ep 21 · 15:21
clinical The dissection should start 2 centimeters deep inside the rectum from the pectinate line, using multiple silk stitches to distribute tension and avoid tissue damage. ↗
▶ Ep 21 · 15:48
clinical Dr. de la Torre originally started the transanal operation submucosally and rectally; Dr. Peña prefers full-thickness dissection like Dr. Swenson used to do. ↗
▶ Ep 21 · 15:48
clinical Dr. de la Torre originally started the transanal operation submucosally and rectally; Dr. Peña prefers full-thickness dissection like Dr. Swenson used to do. ↗
▶ Ep 21 · 16:08
clinical Biopsies should be taken every 5 centimeters during transanal dissection until normal ganglionic bowel is found, then go 5 centimeters higher. ↗
▶ Ep 21 · 16:08
clinical Biopsies should be taken every 5 centimeters during transanal dissection until normal ganglionic bowel is found, then go 5 centimeters higher. ↗
▶ Ep 21 · 16:31
clinical A 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. ↗
▶ Ep 21 · 16:31
clinical A 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. ↗
▶ Ep 21 · 17:12
clinical Keys 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. ↗
▶ Ep 21 · 17:12
clinical Keys 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. ↗
▶ Ep 21 · 17:22
opinion Prone 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. ↗
▶ Ep 21 · 17:22
opinion Prone 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. ↗
▶ Ep 21 · 18:30
opinion Leaving 1-2 centimeters of aganglionic bowel does not explain why patients don't behave well postoperatively. ↗
▶ Ep 21 · 18:30
opinion Leaving 1-2 centimeters of aganglionic bowel does not explain why patients don't behave well postoperatively. ↗
▶ Ep 21 · 18:42
clinical Some patients operated with exactly the same technique do beautifully like normal individuals, while others have symptoms of enterocolitis, and we don't know why. ↗
▶ Ep 21 · 18:42
clinical Some patients operated with exactly the same technique do beautifully like normal individuals, while others have symptoms of enterocolitis, and we don't know why. ↗
▶ Ep 21 · 21:33
clinical The 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. ↗
▶ Ep 21 · 21:33
clinical The 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. ↗
▶ Ep 21 · 21:57
clinical A few patients do have an obvious piece of aganglionic bowel left, but usually it's much more than 2 centimeters. ↗
▶ Ep 21 · 21:57
clinical A few patients do have an obvious piece of aganglionic bowel left, but usually it's much more than 2 centimeters. ↗
▶ Ep 21 · 22:06
clinical When 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. ↗
▶ Ep 21 · 22:06
clinical When 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. ↗
▶ Ep 21 · 22:21
opinion The real concern about transanal surgery is fecal incontinence, which happens when the anal canal is damaged. ↗
▶ Ep 21 · 22:21
opinion The real concern about transanal surgery is fecal incontinence, which happens when the anal canal is damaged. ↗
▶ Ep 21 · 22:39
quote When 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. ↗
▶ Ep 21 · 22:39
quote When 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. ↗
▶ Ep 21 · 22:39
clinical When 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. ↗
▶ Ep 21 · 22:39
clinical When 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. ↗
▶ Ep 21 · 22:50
clinical Even adult ulcerative colitis patients with perfect operations and intact anal canals have problems with bowel control—they have accidents at night. ↗
▶ Ep 21 · 22:50
clinical Even adult ulcerative colitis patients with perfect operations and intact anal canals have problems with bowel control—they have accidents at night. ↗
▶ Ep 21 · 23:08
clinical After 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. ↗
▶ Ep 21 · 23:08
clinical After 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. ↗
▶ Ep 21 · 23:22
clinical Removing the rectum in a child results in passing stool constantly, requiring an intact anal canal, sensation, intact sphincter, and cooperation for bowel control. ↗
▶ Ep 21 · 23:22
clinical Removing the rectum in a child results in passing stool constantly, requiring an intact anal canal, sensation, intact sphincter, and cooperation for bowel control. ↗
▶ Ep 21 · 23:35
clinical Even in patients with a very well-preserved anal canal, some children have different degrees of fecal incontinence after Hirschsprung surgery. ↗
▶ Ep 21 · 23:35
clinical Even in patients with a very well-preserved anal canal, some children have different degrees of fecal incontinence after Hirschsprung surgery. ↗
▶ Ep 21 · 23:44
opinion Fecal incontinence after Hirschsprung surgery is much more common than we believe, and we have not been discussing it enough in pediatric surgical meetings. ↗
▶ Ep 21 · 23:44
quote We have not been discussing enough in our pediatric surgical meetings the problem of fecal incontinence that is much more common than we believe. ↗
▶ Ep 21 · 23:44
quote We have not been discussing enough in our pediatric surgical meetings the problem of fecal incontinence that is much more common than we believe. ↗
▶ Ep 21 · 23:44
opinion Fecal incontinence after Hirschsprung surgery is much more common than we believe, and we have not been discussing it enough in pediatric surgical meetings. ↗
▶ Ep 21 · 23:53
clinical Most patients with symptoms of enterocolitis and constipation after pull-through do not have residual aganglionic bowel. ↗
▶ Ep 21 · 23:53
clinical Most patients with symptoms of enterocolitis and constipation after pull-through do not have residual aganglionic bowel. ↗
▶ Ep 21 · 25:09
quote Patients are born with bowel control. We provoke the fecal incontinence. ↗
▶ Ep 21 · 25:09
opinion Patients are born with bowel control; we provoke fecal incontinence through surgical technique. ↗
▶ Ep 21 · 25:09
quote Patients are born with bowel control. We provoke the fecal incontinence. ↗
▶ Ep 21 · 25:09
opinion Patients are born with bowel control; we provoke fecal incontinence through surgical technique. ↗
▶ Ep 21 · 25:34
clinical Until the time of the video, Dr. Peña's group had performed 125 transanal operations: 56 primary Hirschsprung, 42 redo Hirschsprung, 21 for idiopathic constipation. ↗
▶ Ep 21 · 25:34
clinical Until the time of the video, Dr. Peña's group had performed 125 transanal operations: 56 primary Hirschsprung, 42 redo Hirschsprung, 21 for idiopathic constipation. ↗
▶ Ep 21 · 25:51
opinion Transanal pull-through for idiopathic constipation is not a good operation. ↗
▶ Ep 21 · 25:51
opinion Transanal pull-through for idiopathic constipation is not a good operation. ↗
▶ Ep 21 · 26:24
clinical When 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. ↗
▶ Ep 21 · 26:24
clinical When 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. ↗
▶ Ep 21 · 26:45
opinion Cases with fistulas to the vagina or urinary tract after Hirschsprung surgery are unacceptable complications. ↗
▶ Ep 21 · 26:45
opinion Cases with fistulas to the vagina or urinary tract after Hirschsprung surgery are unacceptable complications. ↗
▶ Ep 21 · 27:04
clinical During 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. ↗
▶ Ep 21 · 27:04
clinical During 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. ↗
▶ Ep 21 · 27:23
clinical If you stay right on the bowel wall during dissection, you will not provoke denervation of the urinary tract, as Dr. Swenson emphasized. ↗
▶ Ep 21 · 27:23
host_summary If you stay right on the bowel wall during dissection, you will not provoke denervation of the urinary tract, as Dr. Swenson emphasized. ↗
▶ Ep 21 · 27:50
clinical The outer layer of sutures fixes the rectum in the right position and releases tension from the inner layer. ↗
▶ Ep 21 · 27:50
clinical The outer layer of sutures fixes the rectum in the right position and releases tension from the inner layer. ↗
▶ Ep 21 · 29:10
clinical Avoid using big retractors pulling in different directions during transanal surgery because that stretches the sphincter too much. ↗
▶ Ep 21 · 29:10
clinical Avoid using big retractors pulling in different directions during transanal surgery because that stretches the sphincter too much. ↗
▶ Ep 21 · 1:18:20
opinion Pediatric surgeons compete with each other trying to close stomas as early as possible, but this may not be best for the patient. ↗
▶ Ep 21 · 1:18:20
opinion Pediatric surgeons compete with each other trying to close stomas as early as possible, but this may not be best for the patient. ↗
▶ Ep 21 · 1:18:43
clinical In Hirschsprung disease with or without total colonic aganglionosis, we resect the natural reservoir and connect high-motility bowel to the anal canal. ↗
▶ Ep 21 · 1:18:43
clinical In Hirschsprung disease with or without total colonic aganglionosis, we resect the natural reservoir and connect high-motility bowel to the anal canal. ↗
▶ Ep 21 · 1:19:09
clinical Adults who receive ileoanal anastomosis for ulcerative colitis live the rest of their lives trying to avoid accidents, with terrible diarrhea. ↗
▶ Ep 21 · 1:19:09
clinical Adults who receive ileoanal anastomosis for ulcerative colitis live the rest of their lives trying to avoid accidents, with terrible diarrhea. ↗
▶ Ep 21 · 1:19:46
clinical Creating intentional stasis in the bowel leads to bacterial proliferation, colitis, inflammatory changes, secretory diarrhea, and worsening symptoms. ↗
▶ Ep 21 · 1:19:46
clinical Creating intentional stasis in the bowel leads to bacterial proliferation, colitis, inflammatory changes, secretory diarrhea, and worsening symptoms. ↗
▶ Ep 21 · 1:19:52
quote The baby is happy with the ileostomy. The only unhappy people are others, but not the baby. ↗
▶ Ep 21 · 1:19:52
quote The baby is happy with the ileostomy. The only unhappy people are others, but not the baby. ↗
▶ Ep 21 · 1:19:52
clinical If 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. ↗
▶ Ep 21 · 1:19:52
clinical If 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. ↗
▶ Ep 21 · 1:20:23
clinical Patients with total colonic aganglionosis have a high incidence of enterocolitis and will most likely need rectal irrigation. ↗
▶ Ep 21 · 1:20:23
clinical Patients with total colonic aganglionosis have a high incidence of enterocolitis and will most likely need rectal irrigation. ↗
▶ Ep 21 · 1:20:46
clinical It's not easy to do rectal irrigations in a 3-year-old with severe diaper rash who doesn't want anything near the anus. ↗
▶ Ep 21 · 1:20:46
clinical It's not easy to do rectal irrigations in a 3-year-old with severe diaper rash who doesn't want anything near the anus. ↗
▶ Ep 21 · 1:38:31
clinical For 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. ↗
▶ Ep 21 · 1:38:31
clinical For 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. ↗
▶ Ep 21 · 1:38:53
clinical A good contrast study for Hirschsprung requires multiple films, including lateral views, to see if dilation goes all the way down to the pubococcygeal line. ↗
▶ Ep 21 · 1:38:53
clinical A good contrast study for Hirschsprung requires multiple films, including lateral views, to see if dilation goes all the way down to the pubococcygeal line. ↗
▶ Ep 21 · 1:38:58
clinical If dilation on contrast enema goes all the way down to the pubococcygeal line, that is not Hirschsprung, even if the biopsy says something different. ↗
▶ Ep 21 · 1:38:58
clinical If dilation on contrast enema goes all the way down to the pubococcygeal line, that is not Hirschsprung, even if the biopsy says something different. ↗
▶ Ep 21 · 1:50:52
clinical For 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. ↗
▶ Ep 21 · 1:50:52
clinical For 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. ↗
▶ Ep 21 · 2:00:08
opinion There 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. ↗
▶ Ep 21 · 2:00:08
opinion There 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. ↗
▶ Ep 21 · 2:03:21
clinical Congenital central hypoventilation syndrome is associated with Ondine's curse, where patients stop breathing when they fall asleep. ↗
▶ Ep 21 · 2:03:21
host_summary Congenital central hypoventilation syndrome is associated with Ondine's curse, where patients stop breathing when they fall asleep. ↗

CinciHirsch - Pathology of Hirschprung Disease

▶ Ep 23 · 7:22
opinion After 6 months of age, the clinical picture of Hirschsprung disease is often so obvious that suction biopsy becomes less relevant. ↗
▶ Ep 23 · 7:22
opinion After 6 months of age, the clinical picture of Hirschsprung disease is often so obvious that suction biopsy becomes less relevant. ↗
▶ Ep 23 · 7:44
quote I 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. ↗
▶ Ep 23 · 7:44
quote I 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. ↗
▶ Ep 23 · 7:54
opinion The suction rectal biopsy is not the main element for diagnosis but one extra piece of information, particularly useful in newborn babies. ↗
▶ Ep 23 · 7:54
opinion The suction rectal biopsy is not the main element for diagnosis but one extra piece of information, particularly useful in newborn babies. ↗
▶ Ep 23 · 37:45
quote There 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. ↗
▶ Ep 23 · 37:45
clinical There is not a single topographic study of intestinal neuronal dysplasia describing the anatomical extent of the histological abnormality. ↗
▶ Ep 23 · 37:45
clinical There is not a single topographic study of intestinal neuronal dysplasia describing the anatomical extent of the histological abnormality. ↗
▶ Ep 23 · 37:45
quote There 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. ↗
▶ Ep 23 · 37:56
opinion Without topographic studies, there is no basis for resecting histologically abnormal portions of colon in IND, unlike in Hirschsprung disease where pathology determines resection extent. ↗
▶ Ep 23 · 37:56
opinion Without topographic studies, there is no basis for resecting histologically abnormal portions of colon in IND, unlike in Hirschsprung disease where pathology determines resection extent. ↗
▶ Ep 23 · 38:27
opinion The 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. ↗
▶ Ep 23 · 38:27
opinion The 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. ↗
▶ Ep 23 · 38:37
quote The 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. ↗
▶ Ep 23 · 38:37
quote The 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. ↗

Hirschsprung Disease: Surgical Procedures

▶ Ep 39 · 3:42
clinical Between 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. ↗
▶ Ep 39 · 6:28
opinion If you start transanally and cannot mobilize more bowel, you simply go into the abdomen and continue the resection—this is not considered a complication. ↗
▶ Ep 39 · 6:41
quote I 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. ↗
▶ Ep 39 · 11:10
quote The 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. ↗
▶ Ep 39 · 11:10
opinion The 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. ↗
▶ Ep 39 · 12:09
quote I 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. ↗
▶ Ep 39 · 12:17
opinion Patients complain about fecal incontinence, not about the size of the scar—that is the real problem. ↗
▶ Ep 39 · 14:23
clinical The Lone Star retractor hooks should be placed at the pectinate line itself, not in the anal canal, to protect the anal canal by definition. ↗
▶ Ep 39 · 14:55
clinical Surgeons should be careful not to stretch too much during transanal dissection because excessive stretching damages the sphincter mechanism by definition, potentially causing fecal incontinence. ↗
▶ Ep 39 · 14:55
quote 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. ↗
▶ Ep 39 · 16:42
clinical The 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. ↗
▶ Ep 39 · 17:29
opinion Prone 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. ↗
▶ Ep 39 · 18:30
opinion Leaving 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. ↗
▶ Ep 39 · 18:30
quote I 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. ↗
▶ Ep 39 · 19:00
quote We 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. ↗
▶ Ep 39 · 21:41
clinical The 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. ↗
▶ Ep 39 · 22:39
clinical When 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. ↗
▶ Ep 39 · 22:39
quote When 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. ↗
▶ Ep 39 · 22:50
clinical Even 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. ↗
▶ Ep 39 · 23:32
clinical Even in patients with a very well preserved anal canal, some children have different degrees of fecal incontinence after Hirschsprung surgery. ↗
▶ Ep 39 · 23:44
opinion Fecal incontinence in Hirschsprung disease is much more common than we believe, and we have not been discussing it enough in pediatric surgical meetings. ↗
▶ Ep 39 · 23:44
quote We have not been discussing enough in our pediatric surgical meetings the problem of fecal incontinence that is much more common than we believe. ↗
▶ Ep 39 · 24:43
clinical If you preserve the pectinate line, by definition you are preserving this crucial part of the bowel. ↗
▶ Ep 39 · 25:09
quote The patients are born with bowel control. We provoke the fecal incontinence. ↗
▶ Ep 39 · 25:09
opinion Patients are born with bowel control—we provoke the fecal incontinence through surgical technique. ↗
▶ Ep 39 · 26:24
clinical The 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. ↗
▶ Ep 39 · 27:06
clinical If 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. ↗
▶ Ep 39 · 27:23
clinical If you stay right on the bowel wall during dissection, you will not provoke denervation of the urinary tract. ↗
▶ Ep 39 · 27:23
quote If you stay right in the bowel wall, you will not provoke the innervation of the urinary tract. ↗
▶ Ep 39 · 1:19:52
clinical Waiting 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. ↗
▶ Ep 39 · 1:20:03
quote The baby is happy with the ileostomy. The only unhappy people are others, but not the baby. ↗
▶ Ep 39 · 1:20:23
quote 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. ↗
▶ Ep 39 · 1:20:23
clinical If 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. ↗
▶ Ep 39 · 1:20:46
clinical Patients 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. ↗
▶ Ep 39 · 2:00:20
opinion There 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. ↗

Panel Discussion and Case Presentation Part II: Pediatric Bowel Management 2013

▶ Ep 48 · 2:26
clinical In Spain, de la Torre technique is used; for total colonic aganglionosis, Lester Martin procedure is used. ↗
▶ Ep 48 · 2:26
clinical In Spain, de la Torre technique is used; for total colonic aganglionosis, Lester Martin procedure is used. ↗
▶ Ep 48 · 6:52
clinical When 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. ↗
▶ Ep 48 · 6:52
clinical When 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. ↗
▶ Ep 48 · 7:11
clinical A permanent stoma is indicated when the anal canal is destroyed and the patient has total colonic aganglionosis. ↗
▶ Ep 48 · 7:11
clinical A permanent stoma is indicated when the anal canal is destroyed and the patient has total colonic aganglionosis. ↗
▶ Ep 48 · 7:27
clinical Sometimes 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. ↗
▶ Ep 48 · 7:27
clinical Sometimes 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. ↗
▶ Ep 48 · 12:56
opinion Most pediatricians worldwide do not know the difference between enterocolitis and gastroenteritis; they do not understand the entity called post-Hirschsprung enterocolitis. ↗
▶ Ep 48 · 12:56
quote Most pediatricians all over the world don't know the difference between enterocolitis and gastroenteritis. ↗
▶ Ep 48 · 12:56
opinion Most pediatricians worldwide do not know the difference between enterocolitis and gastroenteritis; they do not understand the entity called post-Hirschsprung enterocolitis. ↗
▶ Ep 48 · 12:56
quote Most pediatricians all over the world don't know the difference between enterocolitis and gastroenteritis. ↗
▶ Ep 48 · 13:14
opinion Pediatricians 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. ↗
▶ Ep 48 · 13:14
opinion Pediatricians 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. ↗
▶ Ep 48 · 14:25
clinical When 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. ↗
▶ Ep 48 · 14:25
clinical When 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. ↗
▶ Ep 48 · 14:53
opinion Dr. Pena does not believe that leaving 1-2 cm of rectal mucosa is the simple cause of enterocolitis. ↗
▶ Ep 48 · 14:53
opinion Dr. Pena does not believe that leaving 1-2 cm of rectal mucosa is the simple cause of enterocolitis. ↗
▶ Ep 48 · 15:12
clinical A group in New York doing neonatal Soave primary procedures reported zero enterocolitis; when Dr. Pena followed some of those patients, many had fecal incontinence. ↗
▶ Ep 48 · 15:12
clinical A group in New York doing neonatal Soave primary procedures reported zero enterocolitis; when Dr. Pena followed some of those patients, many had fecal incontinence. ↗
▶ Ep 48 · 15:48
quote If you produce fecal incontinence in the patient, the enterocolitis is zero. ↗
▶ Ep 48 · 15:48
clinical If 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. ↗
▶ Ep 48 · 15:48
clinical If 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. ↗
▶ Ep 48 · 15:48
quote If you produce fecal incontinence in the patient, the enterocolitis is zero. ↗
▶ Ep 48 · 16:05
clinical A good operation preserving the sphincter and anal canal creates sphincter closure, which creates stasis, and stasis produces enterocolitis. ↗
▶ Ep 48 · 16:05
clinical A good operation preserving the sphincter and anal canal creates sphincter closure, which creates stasis, and stasis produces enterocolitis. ↗
▶ Ep 48 · 16:19
quote We 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. ↗
▶ Ep 48 · 16:19
quote We 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. ↗
▶ Ep 48 · 16:30
quote I prefer to deal with enterocolitis than with fecal incontinence. Fecal incontinence is for life. ↗
▶ Ep 48 · 16:30
opinion Dr. Pena prefers to deal with enterocolitis rather than fecal incontinence; fecal incontinence is for life. ↗
▶ Ep 48 · 16:30
opinion Dr. Pena prefers to deal with enterocolitis rather than fecal incontinence; fecal incontinence is for life. ↗
▶ Ep 48 · 16:30
quote I prefer to deal with enterocolitis than with fecal incontinence. Fecal incontinence is for life. ↗
▶ Ep 48 · 19:16
epidemiological Dr. Pena's incidence of enterocolitis in pull-through patients is about 30%. ↗
▶ Ep 48 · 19:16
epidemiological Dr. Pena's incidence of enterocolitis in pull-through patients is about 30%. ↗
▶ Ep 48 · 23:02
clinical Resecting the rectosigmoid introduces a major pathophysiological change; children with perfect pull-throughs preserving the anal canal sometimes have toilet-training problems without explanation. ↗
▶ Ep 48 · 23:02
clinical Resecting the rectosigmoid introduces a major pathophysiological change; children with perfect pull-throughs preserving the anal canal sometimes have toilet-training problems without explanation. ↗
▶ Ep 48 · 23:30
clinical Hyperactive 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. ↗
▶ Ep 48 · 23:30
clinical Hyperactive 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. ↗
▶ Ep 48 · 24:01
clinical Even 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. ↗
▶ Ep 48 · 24:01
clinical Even 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. ↗
▶ Ep 48 · 24:59
opinion Dr. Pena does not find rectal manometry useful in Hirschsprung disease, constipation, or anorectal malformations after many years of experience. ↗
▶ Ep 48 · 24:59
opinion Dr. Pena does not find rectal manometry useful in Hirschsprung disease, constipation, or anorectal malformations after many years of experience. ↗
▶ Ep 48 · 26:25
clinical Patients are discharged with three irrigations per day and metronidazole (Flagyl); every month the number of irrigations is decreased. ↗
▶ Ep 48 · 26:25
clinical Patients are discharged with three irrigations per day and metronidazole (Flagyl); every month the number of irrigations is decreased. ↗
▶ Ep 48 · 26:46
clinical If 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. ↗
▶ Ep 48 · 26:46
clinical If 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. ↗
▶ Ep 48 · 27:07
clinical For refractory enterocolitis, another option is further resection of normal ganglionic colon to remove more. ↗
▶ Ep 48 · 27:07
clinical For refractory enterocolitis, another option is further resection of normal ganglionic colon to remove more. ↗
▶ Ep 48 · 27:17
opinion Hirschsprung 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. ↗
▶ Ep 48 · 27:17
opinion Hirschsprung 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. ↗
▶ Ep 48 · 27:45
clinical '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. ↗
▶ Ep 48 · 27:45
clinical '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. ↗
▶ Ep 48 · 28:13
epidemiological In 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. ↗
▶ Ep 48 · 28:13
epidemiological In 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. ↗
▶ Ep 48 · 28:30
quote There 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. ↗
▶ Ep 48 · 28:30
opinion There 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. ↗
▶ Ep 48 · 28:30
opinion There 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. ↗
▶ Ep 48 · 28:30
quote There 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. ↗
▶ Ep 48 · 28:46
opinion Some 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. ↗
▶ Ep 48 · 28:46
opinion Some 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. ↗
▶ Ep 48 · 31:25
opinion Dr. 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. ↗
▶ Ep 48 · 31:25
opinion Dr. 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. ↗
▶ Ep 48 · 32:02
clinical Dr. 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. ↗
▶ Ep 48 · 32:02
clinical Dr. 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. ↗
▶ Ep 48 · 32:48
clinical Patients 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. ↗
▶ Ep 48 · 32:48
clinical Patients 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. ↗
▶ Ep 48 · 33:11
clinical Patients subjected to myotomies, myectomies, or repeated Botox injections eventually develop more severe fecal incontinence. ↗
▶ Ep 48 · 33:11
clinical Patients subjected to myotomies, myectomies, or repeated Botox injections eventually develop more severe fecal incontinence. ↗
▶ Ep 48 · 33:24
opinion Dr. Pena does not believe in myectomy/myotomy/Botox procedures and does not use Botox. ↗
▶ Ep 48 · 33:24
opinion Dr. Pena does not believe in myectomy/myotomy/Botox procedures and does not use Botox. ↗

Cloacal Exstrophy with Dr. Alberto Peña

▶ Ep 80 · 1:31
clinical Cloacal exstrophy is a spectrum of congenital malformations affecting the gastrointestinal/colorectal area, urogenital tract, spine and cord, and sometimes lower extremity motion. ↗
▶ Ep 80 · 1:31
clinical Cloacal exstrophy is a spectrum of congenital malformations affecting the gastrointestinal/colorectal area, urogenital tract, spine and cord, and sometimes lower extremity motion. ↗
▶ Ep 80 · 2:09
clinical Babies with cloacal exstrophy are born with an omphalocele, bladder exstrophy (two separated hemibladders), open cecum between the hemibladders, and separated pubic bones. ↗
▶ Ep 80 · 2:09
clinical Babies with cloacal exstrophy are born with an omphalocele, bladder exstrophy (two separated hemibladders), open cecum between the hemibladders, and separated pubic bones. ↗
▶ Ep 80 · 3:08
clinical The small bowel can become exstrophic through the ileocecal valve, creating an 'elephant trunk' appearance. ↗
▶ Ep 80 · 3:08
clinical The small bowel can become exstrophic through the ileocecal valve, creating an 'elephant trunk' appearance. ↗
▶ Ep 80 · 3:32
clinical Male patients have two separated hemiphalluses with normal gonads; female patients have two hemivaginas below the exstrophic bladder leading to two hemiuteri. ↗
▶ Ep 80 · 3:32
clinical Male patients have two separated hemiphalluses with normal gonads; female patients have two hemivaginas below the exstrophic bladder leading to two hemiuteri. ↗
▶ Ep 80 · 4:20
clinical Cloacal exstrophy represents a spectrum of colonic anatomy from normal colon to almost absent or completely absent colon, sometimes with two ceca or two appendices and bizarre blood supply. ↗
▶ Ep 80 · 4:20
clinical Cloacal exstrophy represents a spectrum of colonic anatomy from normal colon to almost absent or completely absent colon, sometimes with two ceca or two appendices and bizarre blood supply. ↗
▶ Ep 80 · 4:57
clinical The amount of colon present at birth has very important implications for the patient's management and outcomes. ↗
▶ Ep 80 · 4:57
clinical The amount of colon present at birth has very important implications for the patient's management and outcomes. ↗
▶ Ep 80 · 5:44
clinical A variant exists where babies are born with intact abdominal skin (no omphalocele, no bladder exstrophy externally) but have a completely open bladder inside with no bladder neck and a single large perineal orifice. ↗
▶ Ep 80 · 5:44
clinical A variant exists where babies are born with intact abdominal skin (no omphalocele, no bladder exstrophy externally) but have a completely open bladder inside with no bladder neck and a single large perineal orifice. ↗
▶ Ep 80 · 7:02
clinical While surgical techniques, intensive care, parenteral nutrition, and metabolic management have improved, functional outcomes (bowel control, urinary control, sexual function, spinal abnormalities) remain severely limited and cannot be made normal. ↗
▶ Ep 80 · 7:02
clinical While surgical techniques, intensive care, parenteral nutrition, and metabolic management have improved, functional outcomes (bowel control, urinary control, sexual function, spinal abnormalities) remain severely limited and cannot be made normal. ↗
▶ Ep 80 · 8:31
clinical Historical practice was to perform bilateral gonadectomy, remove hemiphalluses, create a vagina with bowel, and assign female gender to XY patients with cloacal exstrophy. ↗
▶ Ep 80 · 8:31
clinical Historical practice was to perform bilateral gonadectomy, remove hemiphalluses, create a vagina with bowel, and assign female gender to XY patients with cloacal exstrophy. ↗
▶ Ep 80 · 9:16
clinical Patients raised as female despite XY chromosomes exhibited male attitudes and behavior, and many became upset upon learning their chromosomal sex and that gonads were removed without their consent. ↗
▶ Ep 80 · 9:16
clinical Patients raised as female despite XY chromosomes exhibited male attitudes and behavior, and many became upset upon learning their chromosomal sex and that gonads were removed without their consent. ↗
▶ Ep 80 · 9:59
opinion Patients argued that sex is not the most important aspect of being male, that they wanted their gonads back for fertility (modern techniques allow fertilization and children), and that being male is much more than having a phallus. ↗
▶ Ep 80 · 9:59
quote 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. ↗
▶ Ep 80 · 9:59
quote 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. ↗
▶ Ep 80 · 9:59
opinion Patients argued that sex is not the most important aspect of being male, that they wanted their gonads back for fertility (modern techniques allow fertilization and children), and that being male is much more than having a phallus. ↗
▶ Ep 80 · 10:29
quote being a male is much more than having a phallus ↗
▶ Ep 80 · 10:29
quote being a male is much more than having a phallus ↗
▶ Ep 80 · 10:34
guideline Current consensus is that XY patients should be raised as male, with pediatric urologists and plastic surgeons working on phallus reconstruction techniques. ↗
▶ Ep 80 · 10:34
guideline Current consensus is that XY patients should be raised as male, with pediatric urologists and plastic surgeons working on phallus reconstruction techniques. ↗
▶ Ep 80 · 12:13
clinical When a prominent pediatric urologist dominates management, patients receive good urologic attention but inadequate gastrointestinal care; the reverse occurs when pediatric surgeons dominate. ↗
▶ Ep 80 · 12:13
clinical When a prominent pediatric urologist dominates management, patients receive good urologic attention but inadequate gastrointestinal care; the reverse occurs when pediatric surgeons dominate. ↗
▶ Ep 80 · 13:03
clinical The pediatric surgeon's role in the initial operation is to close the omphalocele (if possible), separate urothelium from intestinal mucosa by placing stitches at the edges and making an incision, allowing the urologist to bring hemibladders together. ↗
▶ Ep 80 · 13:03
clinical The pediatric surgeon's role in the initial operation is to close the omphalocele (if possible), separate urothelium from intestinal mucosa by placing stitches at the edges and making an incision, allowing the urologist to bring hemibladders together. ↗
▶ Ep 80 · 14:55
clinical It is very common but very harmful for pediatric surgeons to simply create an ileostomy, leaving all colon distally attached to the urinary tract. ↗
▶ Ep 80 · 14:55
clinical It is very common but very harmful for pediatric surgeons to simply create an ileostomy, leaving all colon distally attached to the urinary tract. ↗
▶ Ep 80 · 15:11
quote That is a very bad thing to do, and the patient will suffer several consequences because of that ↗
▶ Ep 80 · 15:11
quote That is a very bad thing to do, and the patient will suffer several consequences because of that ↗
▶ Ep 80 · 15:31
clinical Leaving colon attached to the urinary tract creates a congenital bladder augmentation that causes hyperchloremic acidosis from urine absorption, interfering with growth and development. ↗
▶ Ep 80 · 15:31
clinical Leaving colon attached to the urinary tract creates a congenital bladder augmentation that causes hyperchloremic acidosis from urine absorption, interfering with growth and development. ↗
▶ Ep 80 · 15:40
quote Isn't that beautiful from your point of view? ↗
▶ Ep 80 · 15:40
quote Isn't that beautiful from your point of view? ↗
▶ Ep 80 · 16:03
clinical Defunctionalized colonic tissue left distally will not grow; colon requires passage of fecal matter through its lumen to grow. ↗
▶ Ep 80 · 16:03
clinical Defunctionalized colonic tissue left distally will not grow; colon requires passage of fecal matter through its lumen to grow. ↗
▶ Ep 80 · 16:23
clinical The pediatric surgeon must incorporate all gastrointestinal tissue into the fecal stream and create a true end colostomy to ensure fecal matter passes through all colonic tissue. ↗
▶ Ep 80 · 16:23
clinical The pediatric surgeon must incorporate all gastrointestinal tissue into the fecal stream and create a true end colostomy to ensure fecal matter passes through all colonic tissue. ↗
▶ Ep 80 · 16:41
clinical Patients who received ileostomy with defunctionalized colon present at 2-3 years with poor growth, hyperchloremic acidosis managed by nephrologists, and large ileostomy losses. ↗
▶ Ep 80 · 16:41
clinical Patients who received ileostomy with defunctionalized colon present at 2-3 years with poor growth, hyperchloremic acidosis managed by nephrologists, and large ileostomy losses. ↗
▶ Ep 80 · 17:03
clinical The rescue operation involves taking down the ileostomy, finding and incorporating colonic tissue into the GI tract, and creating an end colostomy; acidosis disappears the next day. ↗
▶ Ep 80 · 17:03
clinical The rescue operation involves taking down the ileostomy, finding and incorporating colonic tissue into the GI tract, and creating an end colostomy; acidosis disappears the next day. ↗
▶ Ep 80 · 19:13
opinion Surgeons must accept that cloacal exstrophy is a spectrum and be prepared to deal with complex, variable colonic anatomy rather than taking the easy way out with an ileostomy. ↗
▶ Ep 80 · 19:13
opinion Surgeons must accept that cloacal exstrophy is a spectrum and be prepared to deal with complex, variable colonic anatomy rather than taking the easy way out with an ileostomy. ↗
▶ Ep 80 · 20:07
quote Do not leave gastrointestinal tract inside the functionalized. That's the main, main thing. ↗
▶ Ep 80 · 20:07
quote Do not leave gastrointestinal tract inside the functionalized. That's the main, main thing. ↗
▶ Ep 80 · 21:00
clinical Some institutions routinely perform pelvic osteotomy at the initial operation to facilitate bladder and omphalocele reconstruction, while others wait 2-3 months to do it separately. ↗
▶ Ep 80 · 21:00
clinical Some institutions routinely perform pelvic osteotomy at the initial operation to facilitate bladder and omphalocele reconstruction, while others wait 2-3 months to do it separately. ↗
▶ Ep 80 · 21:09
clinical Even after osteotomy, it is very difficult to see a cloacal exstrophy patient with pubic bones completely together; they usually remain separated. ↗
▶ Ep 80 · 21:09
clinical Even after osteotomy, it is very difficult to see a cloacal exstrophy patient with pubic bones completely together; they usually remain separated. ↗
▶ Ep 80 · 22:06
clinical Even a technically correct end colostomy often has poor motility, and babies may not pass stool easily, sometimes developing bacterial overgrowth similar to Hirschsprung disease. ↗
▶ Ep 80 · 22:06
clinical Even a technically correct end colostomy often has poor motility, and babies may not pass stool easily, sometimes developing bacterial overgrowth similar to Hirschsprung disease. ↗
▶ Ep 80 · 23:00
clinical Families must be taught to irrigate the colostomy with small volumes of saline through a tube to evacuate fecal material when peristalsis is inadequate. ↗
▶ Ep 80 · 23:00
clinical Families must be taught to irrigate the colostomy with small volumes of saline through a tube to evacuate fecal material when peristalsis is inadequate. ↗
▶ Ep 80 · 23:30
clinical Decision-making for bowel control begins around age 3 when parents want to send the child to school clean and dry (no stool or urine in diaper). ↗
▶ Ep 80 · 23:30
clinical Decision-making for bowel control begins around age 3 when parents want to send the child to school clean and dry (no stool or urine in diaper). ↗
▶ Ep 80 · 23:54
clinical Most cloacal exstrophy patients have an inadequate, tiny bladder requiring bladder augmentation with gastrointestinal tract, necessitating coordination between pediatric surgery and urology. ↗
▶ Ep 80 · 23:54
clinical Most cloacal exstrophy patients have an inadequate, tiny bladder requiring bladder augmentation with gastrointestinal tract, necessitating coordination between pediatric surgery and urology. ↗
▶ Ep 80 · 24:42
clinical Patients born with no colon are candidates for permanent colostomy and should never have terminal ileum pulled through, even if sphincter evidence exists, because they will never have bowel control. ↗
▶ Ep 80 · 24:42
clinical Patients born with no colon are candidates for permanent colostomy and should never have terminal ileum pulled through, even if sphincter evidence exists, because they will never have bowel control. ↗
▶ Ep 80 · 25:04
clinical Pull-through is only considered for patients with capacity to form solid stool (adequate colon), as bowel management only works with solid stool. ↗
▶ Ep 80 · 25:04
clinical Pull-through is only considered for patients with capacity to form solid stool (adequate colon), as bowel management only works with solid stool. ↗
▶ Ep 80 · 25:20
clinical It is extremely unusual for cloacal exstrophy patients to have spontaneous bowel control; the overwhelming majority need a bowel management program (enema administration to keep patient clean). ↗
▶ Ep 80 · 25:20
clinical It is extremely unusual for cloacal exstrophy patients to have spontaneous bowel control; the overwhelming majority need a bowel management program (enema administration to keep patient clean). ↗
▶ Ep 80 · 25:47
clinical Pediatric 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. ↗
▶ Ep 80 · 25:47
clinical Pediatric 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. ↗
▶ Ep 80 · 26:07
clinical Annual contrast studies through the colostomy (retrograde injection) are performed to assess colonic growth. ↗
▶ Ep 80 · 26:07
clinical Annual contrast studies through the colostomy (retrograde injection) are performed to assess colonic growth. ↗
▶ Ep 80 · 26:41
clinical Before 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. ↗
▶ Ep 80 · 26:41
clinical Before 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. ↗
▶ Ep 80 · 27:35
clinical Bowel management trial through colostomy is typically started after age 3 when families consider avoiding the stoma for school. ↗
▶ Ep 80 · 27:35
clinical Bowel management trial through colostomy is typically started after age 3 when families consider avoiding the stoma for school. ↗
▶ Ep 80 · 28:07
clinical If 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. ↗
▶ Ep 80 · 28:07
clinical If 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. ↗
▶ Ep 80 · 28:54
clinical The 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. ↗
▶ Ep 80 · 28:54
clinical The 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. ↗
▶ Ep 80 · 29:56
clinical Contrast enema through the colostomy can distinguish true liquid stool from paradoxical diarrhea (liquid stool around solid fecal impaction). ↗
▶ Ep 80 · 29:56
clinical Contrast enema through the colostomy can distinguish true liquid stool from paradoxical diarrhea (liquid stool around solid fecal impaction). ↗
▶ Ep 80 · 31:04
clinical Pull-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). ↗
▶ Ep 80 · 31:04
clinical Pull-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). ↗
▶ Ep 80 · 31:28
clinical During 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. ↗
▶ Ep 80 · 31:28
clinical During 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. ↗
▶ Ep 80 · 31:57
clinical Urologists 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. ↗
▶ Ep 80 · 31:57
clinical Urologists 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. ↗
▶ Ep 80 · 32:18
clinical Occasionally, 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. ↗
▶ Ep 80 · 32:18
clinical Occasionally, 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. ↗
▶ Ep 80 · 33:13
clinical Midline abdominal incision from xiphoid to pubis is used for pull-through to preserve the flanks and quadrants for potential future stomas. ↗
▶ Ep 80 · 33:13
clinical Midline abdominal incision from xiphoid to pubis is used for pull-through to preserve the flanks and quadrants for potential future stomas. ↗
▶ Ep 80 · 33:56
clinical The 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. ↗
▶ Ep 80 · 33:56
clinical The 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. ↗
▶ Ep 80 · 34:39
clinical Careful 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. ↗
▶ Ep 80 · 34:39
clinical Careful 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. ↗
▶ Ep 80 · 35:53
clinical The 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. ↗
▶ Ep 80 · 35:53
clinical The 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. ↗
▶ Ep 80 · 37:33
clinical Cloacal 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). ↗
▶ Ep 80 · 37:33
clinical Cloacal 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). ↗
▶ Ep 80 · 37:38
clinical Some 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. ↗
▶ Ep 80 · 37:38
clinical Some 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. ↗
▶ Ep 80 · 38:50
clinical During pull-through, vaginal reconstruction is attempted by approximating the hemivaginas as much as possible; the degree of separation varies on the spectrum. ↗
▶ Ep 80 · 38:50
clinical During pull-through, vaginal reconstruction is attempted by approximating the hemivaginas as much as possible; the degree of separation varies on the spectrum. ↗
▶ Ep 80 · 39:07
clinical When 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. ↗
▶ Ep 80 · 39:07
clinical When 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. ↗
▶ Ep 80 · 39:53
clinical Patients with functional hemiuterus may become pregnant, but it is high-risk pregnancy requiring specialized pediatric gynecology follow-up; in general, pregnancy is not advised. ↗
▶ Ep 80 · 39:53
clinical Patients with functional hemiuterus may become pregnant, but it is high-risk pregnancy requiring specialized pediatric gynecology follow-up; in general, pregnancy is not advised. ↗
▶ Ep 80 · 40:10
clinical Hemiuterus has a great tendency to produce miscarriages and premature labor; delivery must be by cesarean section due to limited abdominal space. ↗
▶ Ep 80 · 40:10
clinical Hemiuterus has a great tendency to produce miscarriages and premature labor; delivery must be by cesarean section due to limited abdominal space. ↗
▶ Ep 80 · 40:37
clinical Bladder 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. ↗
▶ Ep 80 · 40:37
clinical Bladder 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. ↗
▶ Ep 80 · 41:07
clinical Augmented 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. ↗
▶ Ep 80 · 41:07
clinical Augmented 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. ↗
▶ Ep 80 · 41:33
clinical Bladder irrigation is sometimes performed with gentamicin to ensure all mucus is removed. ↗
▶ Ep 80 · 41:33
clinical Bladder irrigation is sometimes performed with gentamicin to ensure all mucus is removed. ↗
▶ Ep 80 · 41:45
clinical Lifelong 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. ↗
▶ Ep 80 · 41:45
clinical Lifelong 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. ↗
▶ Ep 80 · 42:29
clinical Patients 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. ↗
▶ Ep 80 · 42:29
clinical Patients 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. ↗
▶ Ep 80 · 43:03
opinion Dr. 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. ↗
▶ Ep 80 · 43:03
opinion Dr. 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. ↗
▶ Ep 80 · 43:42
clinical The 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. ↗
▶ Ep 80 · 43:42
clinical The 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. ↗
▶ Ep 80 · 44:48
opinion Colorectal 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. ↗
▶ Ep 80 · 44:48
opinion Colorectal 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. ↗
▶ Ep 80 · 46:00
clinical The 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). ↗
▶ Ep 80 · 46:00
clinical The 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). ↗
▶ Ep 80 · 46:54
clinical Between the initial operation and pull-through, colostomy irrigation is often needed to manage poor colonic motility. ↗
▶ Ep 80 · 46:54
clinical Between the initial operation and pull-through, colostomy irrigation is often needed to manage poor colonic motility. ↗
▶ Ep 80 · 47:58
clinical Prenatal 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. ↗
▶ Ep 80 · 47:58
clinical Prenatal 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. ↗
▶ Ep 80 · 48:48
clinical Absent bladder on prenatal ultrasound (due to exstrophy) is a bad sign and can be detected as early as week 20 of pregnancy. ↗
▶ Ep 80 · 48:48
clinical Absent bladder on prenatal ultrasound (due to exstrophy) is a bad sign and can be detected as early as week 20 of pregnancy. ↗
▶ Ep 80 · 49:38
clinical Prenatal diagnosis allows families to decide about pregnancy continuation and, if continuing, to deliver at a center with a multidisciplinary team experienced in these malformations. ↗
▶ Ep 80 · 49:38
clinical Prenatal diagnosis allows families to decide about pregnancy continuation and, if continuing, to deliver at a center with a multidisciplinary team experienced in these malformations. ↗
▶ Ep 80 · 50:04
opinion Certain 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. ↗
▶ Ep 80 · 50:04
opinion Certain 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. ↗
Enterocolitis 41 entries

Evaluation & Management Of Hirschsprung's Disease

▶ Ep 1 · 32:44
opinion Dr. Pena would perform rectal biopsy in a case of apparent small left colon because he cannot distinguish it from Hirschsprung disease. ↗
▶ Ep 1 · 36:54
quote I 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. ↗
▶ Ep 1 · 36:54
opinion Dr. 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. ↗
▶ Ep 1 · 37:30
quote I wonder if all the patients that developed this traditional characteristic pouch of the duja male are actually patients that never had Hirk disease ↗
▶ Ep 1 · 37:41
opinion Dr. 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. ↗
▶ Ep 1 · 40:28
quote if 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. ↗
▶ Ep 1 · 40:28
opinion Dr. 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. ↗
▶ Ep 1 · 40:39
opinion Dr. 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. ↗
▶ Ep 1 · 41:32
clinical There 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. ↗
▶ Ep 1 · 41:32
quote there 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. ↗
▶ Ep 1 · 42:05
clinical Someone could take a biopsy in the normal physiologic aganglionic area and get a result of no ganglion cells, which does not indicate Hirschsprung disease. ↗
▶ Ep 1 · 42:14
opinion The 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. ↗
▶ Ep 1 · 42:14
quote the 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 ↗
▶ Ep 1 · 42:45
clinical If the internal sphincter thickening exists, nobody has determined its exact boundaries at different ages. ↗
▶ Ep 1 · 43:16
quote alacia of alacia of the internal sphincter is a manometric concept, not an anatomic concept ↗
▶ Ep 1 · 43:16
opinion Internal sphincter achalasia is a manometric concept, not an anatomic concept, similar to the lower esophageal sphincter. ↗
▶ Ep 1 · 43:16
opinion Dr. 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. ↗

Radiology and Image Diagnosis of Hirschsprung Disease

▶ Ep 3 · 32:37
clinical Dr. 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. ↗
▶ Ep 3 · 36:54
quote I 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. ↗
▶ Ep 3 · 36:54
opinion Dr. 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. ↗
▶ Ep 3 · 37:30
opinion Dr. 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. ↗
▶ Ep 3 · 37:41
quote I suspect that this patient never had Hodgkin disease. ↗
▶ Ep 3 · 40:26
quote if 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. ↗
▶ Ep 3 · 40:26
opinion Dr. 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. ↗
▶ Ep 3 · 40:44
quote this patient has no Hirshman disease ↗
▶ Ep 3 · 41:00
quote there is no way to differentiate the so-called ultra short with idiopathic constipation ↗
▶ Ep 3 · 41:00
opinion Dr. Peña states there is no way to differentiate so-called ultra-short segment Hirschsprung from idiopathic constipation. ↗
▶ Ep 3 · 41:32
quote there 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. ↗
▶ Ep 3 · 41:32
clinical The 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. ↗
▶ Ep 3 · 41:42
opinion There 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. ↗
▶ Ep 3 · 41:56
quote That's another challenge for young pediatric surgeons. If you want to make a contribution. studies, this is a real challenge. ↗
▶ Ep 3 · 42:05
quote conceivably 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. ↗
▶ Ep 3 · 42:05
opinion Somebody could take a biopsy in the normal aganglionic area and get a result of no ganglion cells, but it does not mean anything clinically. ↗
▶ Ep 3 · 42:14
opinion The 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. ↗
▶ Ep 3 · 42:14
quote the 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. ↗
▶ Ep 3 · 42:45
opinion If 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. ↗
▶ Ep 3 · 43:16
opinion Internal 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. ↗
▶ Ep 3 · 43:16
quote alacia of alacia of the internal sphincter is a manometric concept, not an anatomic concept ↗
▶ Ep 3 · 43:36
quote 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 ↗
▶ Ep 3 · 43:36
opinion Dr. 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. ↗
▶ Ep 3 · 43:48
opinion Botox and similar injections paralyze whatever muscle is there and facilitate passing of stool, but they are not curing a condition of unknown origin. ↗
Hirschsprung disease 334 entries

Evaluation & Management Of Hirschsprung's Disease

▶ Ep 2 · 32:44
opinion Dr. Pena would perform rectal biopsy in a case of apparent small left colon because he cannot distinguish it from Hirschsprung disease. ↗
▶ Ep 2 · 32:44
opinion Dr. Pena would perform rectal biopsy in a case of apparent small left colon because he cannot distinguish it from Hirschsprung disease. ↗
▶ Ep 2 · 36:54
quote I 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. ↗
▶ Ep 2 · 36:54
opinion Dr. 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. ↗
▶ Ep 2 · 36:54
quote I 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. ↗
▶ Ep 2 · 36:54
opinion Dr. 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. ↗
▶ Ep 2 · 37:30
quote I wonder if all the patients that developed this traditional characteristic pouch of the duja male are actually patients that never had Hirk disease ↗
▶ Ep 2 · 37:30
quote I wonder if all the patients that developed this traditional characteristic pouch of the duja male are actually patients that never had Hirk disease ↗
▶ Ep 2 · 37:41
opinion Dr. 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. ↗
▶ Ep 2 · 37:41
opinion Dr. 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. ↗
▶ Ep 2 · 40:28
quote if 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. ↗
▶ Ep 2 · 40:28
opinion Dr. 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. ↗
▶ Ep 2 · 40:28
quote if 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. ↗
▶ Ep 2 · 40:28
opinion Dr. 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. ↗
▶ Ep 2 · 40:39
opinion Dr. 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. ↗
▶ Ep 2 · 40:39
opinion Dr. 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. ↗
▶ Ep 2 · 41:32
quote there 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. ↗
▶ Ep 2 · 41:32
clinical There 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. ↗
▶ Ep 2 · 41:32
quote there 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. ↗
▶ Ep 2 · 41:32
clinical There 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. ↗
▶ Ep 2 · 42:05
clinical Someone could take a biopsy in the normal physiologic aganglionic area and get a result of no ganglion cells, which does not indicate Hirschsprung disease. ↗
▶ Ep 2 · 42:05
clinical Someone could take a biopsy in the normal physiologic aganglionic area and get a result of no ganglion cells, which does not indicate Hirschsprung disease. ↗
▶ Ep 2 · 42:14
opinion The 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. ↗
▶ Ep 2 · 42:14
quote the 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 ↗
▶ Ep 2 · 42:14
quote the 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 ↗
▶ Ep 2 · 42:14
opinion The 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. ↗
▶ Ep 2 · 42:45
clinical If the internal sphincter thickening exists, nobody has determined its exact boundaries at different ages. ↗
▶ Ep 2 · 42:45
clinical If the internal sphincter thickening exists, nobody has determined its exact boundaries at different ages. ↗
▶ Ep 2 · 43:16
opinion Internal sphincter achalasia is a manometric concept, not an anatomic concept, similar to the lower esophageal sphincter. ↗
▶ Ep 2 · 43:16
quote alacia of alacia of the internal sphincter is a manometric concept, not an anatomic concept ↗
▶ Ep 2 · 43:16
opinion Dr. 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. ↗
▶ Ep 2 · 43:16
quote alacia of alacia of the internal sphincter is a manometric concept, not an anatomic concept ↗
▶ Ep 2 · 43:16
opinion Internal sphincter achalasia is a manometric concept, not an anatomic concept, similar to the lower esophageal sphincter. ↗
▶ Ep 2 · 43:16
opinion Dr. 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. ↗

Radiology and Image Diagnosis of Hirschsprung Disease

▶ Ep 6 · 32:37
clinical Dr. 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. ↗
▶ Ep 6 · 32:37
clinical Dr. 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. ↗
▶ Ep 6 · 36:54
quote I 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. ↗
▶ Ep 6 · 36:54
opinion Dr. 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. ↗
▶ Ep 6 · 36:54
opinion Dr. 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. ↗
▶ Ep 6 · 36:54
quote I 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. ↗
▶ Ep 6 · 37:30
opinion Dr. 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. ↗
▶ Ep 6 · 37:30
opinion Dr. 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. ↗
▶ Ep 6 · 37:41
quote I suspect that this patient never had Hodgkin disease. ↗
▶ Ep 6 · 37:41
quote I suspect that this patient never had Hodgkin disease. ↗
▶ Ep 6 · 40:26
quote if 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. ↗
▶ Ep 6 · 40:26
opinion Dr. 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. ↗
▶ Ep 6 · 40:26
quote if 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. ↗
▶ Ep 6 · 40:26
opinion Dr. 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. ↗
▶ Ep 6 · 40:44
quote this patient has no Hirshman disease ↗
▶ Ep 6 · 40:44
quote this patient has no Hirshman disease ↗
▶ Ep 6 · 41:00
quote there is no way to differentiate the so-called ultra short with idiopathic constipation ↗
▶ Ep 6 · 41:00
opinion Dr. Peña states there is no way to differentiate so-called ultra-short segment Hirschsprung from idiopathic constipation. ↗
▶ Ep 6 · 41:00
opinion Dr. Peña states there is no way to differentiate so-called ultra-short segment Hirschsprung from idiopathic constipation. ↗
▶ Ep 6 · 41:00
quote there is no way to differentiate the so-called ultra short with idiopathic constipation ↗
▶ Ep 6 · 41:32
clinical The 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. ↗
▶ Ep 6 · 41:32
clinical The 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. ↗
▶ Ep 6 · 41:32
quote there 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. ↗
▶ Ep 6 · 41:32
quote there 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. ↗
▶ Ep 6 · 41:42
opinion There 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. ↗
▶ Ep 6 · 41:42
opinion There 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. ↗
▶ Ep 6 · 41:56
quote That's another challenge for young pediatric surgeons. If you want to make a contribution. studies, this is a real challenge. ↗
▶ Ep 6 · 41:56
quote That's another challenge for young pediatric surgeons. If you want to make a contribution. studies, this is a real challenge. ↗
▶ Ep 6 · 42:05
quote conceivably 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. ↗
▶ Ep 6 · 42:05
opinion Somebody could take a biopsy in the normal aganglionic area and get a result of no ganglion cells, but it does not mean anything clinically. ↗
▶ Ep 6 · 42:05
opinion Somebody could take a biopsy in the normal aganglionic area and get a result of no ganglion cells, but it does not mean anything clinically. ↗
▶ Ep 6 · 42:05
quote conceivably 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. ↗
▶ Ep 6 · 42:14
quote the 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. ↗
▶ Ep 6 · 42:14
opinion The 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. ↗
▶ Ep 6 · 42:14
opinion The 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. ↗
▶ Ep 6 · 42:14
quote the 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. ↗
▶ Ep 6 · 42:45
opinion If 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. ↗
▶ Ep 6 · 42:45
opinion If 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. ↗
▶ Ep 6 · 43:16
opinion Internal 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. ↗
▶ Ep 6 · 43:16
opinion Internal 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. ↗
▶ Ep 6 · 43:16
quote alacia of alacia of the internal sphincter is a manometric concept, not an anatomic concept ↗
▶ Ep 6 · 43:16
quote alacia of alacia of the internal sphincter is a manometric concept, not an anatomic concept ↗
▶ Ep 6 · 43:36
quote 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 ↗
▶ Ep 6 · 43:36
quote 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 ↗
▶ Ep 6 · 43:36
opinion Dr. 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. ↗
▶ Ep 6 · 43:36
opinion Dr. 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. ↗
▶ Ep 6 · 43:48
opinion Botox and similar injections paralyze whatever muscle is there and facilitate passing of stool, but they are not curing a condition of unknown origin. ↗
▶ Ep 6 · 43:48
opinion Botox and similar injections paralyze whatever muscle is there and facilitate passing of stool, but they are not curing a condition of unknown origin. ↗

History of Hirschsprung Disease

▶ Ep 7 · 2:44
quote What 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. ↗
▶ Ep 7 · 3:00
quote We 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. ↗
▶ Ep 7 · 3:13
quote We are particularly grateful with Dr. Ova Swenson for his seminal contribution, and, and we still followed his principles in the management of this condition. ↗
▶ Ep 7 · 4:05
opinion The 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. ↗
▶ Ep 7 · 4:20
opinion Hirschsprung disease is not only about ganglion cells or no ganglion cells; it is a much more complex condition. ↗
▶ Ep 7 · 11:37
clinical Electric 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. ↗

Surgical Procedures for Hirschsprung Disease

▶ Ep 8 · 3:42
clinical Between 75 and 80% of Hirschsprung cases can be completed transanally, reaching normal ganglionic bowel from below. ↗
▶ Ep 8 · 6:28
opinion If you start transanally and cannot reach ganglionic bowel, you simply open the abdomen and continue the resection—this is not a complication. ↗
▶ Ep 8 · 11:10
quote The 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. ↗
▶ Ep 8 · 11:10
opinion The main problems in Hirschsprung surgery are related to surgeon inexperience and technical incapacity, not the approach (laparoscopic vs. transanal vs. open). ↗
▶ Ep 8 · 11:37
opinion A bad surgeon will damage the patient both ways—laparoscopically or transanally. ↗
▶ Ep 8 · 11:45
clinical The basic goal of Hirschsprung surgery is not to damage the sphincter mechanism, which has been damaged by both laparoscopic and non-laparoscopic techniques. ↗
▶ Ep 8 · 12:09
quote I 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. ↗
▶ Ep 8 · 12:17
opinion Patients complain about fecal incontinence, not the size of the scar. ↗
▶ Ep 8 · 14:13
clinical When doing transanal dissection, the Lone Star retractor hooks should be placed at the pectinate line to protect the entire anal canal. ↗
▶ Ep 8 · 14:55
clinical Surgeons must be careful not to stretch the anus too much during transanal dissection, as excessive stretch damages the sphincter mechanism and causes fecal incontinence. ↗
▶ Ep 8 · 15:21
clinical The dissection should start 2 centimeters deep inside the rectum from the pectinate line, using multiple silk stitches to distribute tension and avoid tissue damage. ↗
▶ Ep 8 · 15:48
clinical Dr. de la Torre originally started the transanal operation submucosally and rectally; Dr. Peña prefers full-thickness dissection like Dr. Swenson used to do. ↗
▶ Ep 8 · 16:08
clinical Biopsies should be taken every 5 centimeters during transanal dissection until normal ganglionic bowel is found, then go 5 centimeters higher. ↗
▶ Ep 8 · 16:31
clinical A 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. ↗
▶ Ep 8 · 17:12
clinical Keys 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. ↗
▶ Ep 8 · 17:22
opinion Prone 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. ↗
▶ Ep 8 · 18:30
opinion Leaving 1-2 centimeters of aganglionic bowel does not explain why patients don't behave well postoperatively. ↗
▶ Ep 8 · 18:42
clinical Some patients operated with exactly the same technique do beautifully like normal individuals, while others have symptoms of enterocolitis, and we don't know why. ↗
▶ Ep 8 · 21:33
clinical The 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. ↗
▶ Ep 8 · 21:57
clinical A few patients do have an obvious piece of aganglionic bowel left, but usually it's much more than 2 centimeters. ↗
▶ Ep 8 · 22:06
clinical When 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. ↗
▶ Ep 8 · 22:21
opinion The real concern about transanal surgery is fecal incontinence, which happens when the anal canal is damaged. ↗
▶ Ep 8 · 22:39
clinical When 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. ↗
▶ Ep 8 · 22:39
quote When 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. ↗
▶ Ep 8 · 22:50
clinical Even adult ulcerative colitis patients with perfect operations and intact anal canals have problems with bowel control—they have accidents at night. ↗
▶ Ep 8 · 23:08
clinical After 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. ↗
▶ Ep 8 · 23:22
clinical Removing the rectum in a child results in passing stool constantly, requiring an intact anal canal, sensation, intact sphincter, and cooperation for bowel control. ↗
▶ Ep 8 · 23:35
clinical Even in patients with a very well-preserved anal canal, some children have different degrees of fecal incontinence after Hirschsprung surgery. ↗
▶ Ep 8 · 23:44
opinion Fecal incontinence after Hirschsprung surgery is much more common than we believe, and we have not been discussing it enough in pediatric surgical meetings. ↗
▶ Ep 8 · 23:44
quote We have not been discussing enough in our pediatric surgical meetings the problem of fecal incontinence that is much more common than we believe. ↗
▶ Ep 8 · 23:53
clinical Most patients with symptoms of enterocolitis and constipation after pull-through do not have residual aganglionic bowel. ↗
▶ Ep 8 · 25:09
opinion Patients are born with bowel control; we provoke fecal incontinence through surgical technique. ↗
▶ Ep 8 · 25:09
quote Patients are born with bowel control. We provoke the fecal incontinence. ↗
▶ Ep 8 · 25:34
clinical Until the time of the video, Dr. Peña's group had performed 125 transanal operations: 56 primary Hirschsprung, 42 redo Hirschsprung, 21 for idiopathic constipation. ↗
▶ Ep 8 · 25:51
opinion Transanal pull-through for idiopathic constipation is not a good operation. ↗
▶ Ep 8 · 26:24
clinical When 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. ↗
▶ Ep 8 · 26:45
opinion Cases with fistulas to the vagina or urinary tract after Hirschsprung surgery are unacceptable complications. ↗
▶ Ep 8 · 27:04
clinical During 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. ↗
▶ Ep 8 · 27:23
clinical If you stay right on the bowel wall during dissection, you will not provoke denervation of the urinary tract, as Dr. Swenson emphasized. ↗
▶ Ep 8 · 27:50
clinical The outer layer of sutures fixes the rectum in the right position and releases tension from the inner layer. ↗
▶ Ep 8 · 29:10
clinical Avoid using big retractors pulling in different directions during transanal surgery because that stretches the sphincter too much. ↗
▶ Ep 8 · 1:18:20
opinion Pediatric surgeons compete with each other trying to close stomas as early as possible, but this may not be best for the patient. ↗
▶ Ep 8 · 1:18:43
clinical In Hirschsprung disease with or without total colonic aganglionosis, we resect the natural reservoir and connect high-motility bowel to the anal canal. ↗
▶ Ep 8 · 1:19:09
clinical Adults who receive ileoanal anastomosis for ulcerative colitis live the rest of their lives trying to avoid accidents, with terrible diarrhea. ↗
▶ Ep 8 · 1:19:46
clinical Creating intentional stasis in the bowel leads to bacterial proliferation, colitis, inflammatory changes, secretory diarrhea, and worsening symptoms. ↗
▶ Ep 8 · 1:19:52
quote The baby is happy with the ileostomy. The only unhappy people are others, but not the baby. ↗
▶ Ep 8 · 1:19:52
clinical If 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. ↗
▶ Ep 8 · 1:20:23
clinical Patients with total colonic aganglionosis have a high incidence of enterocolitis and will most likely need rectal irrigation. ↗
▶ Ep 8 · 1:20:46
clinical It's not easy to do rectal irrigations in a 3-year-old with severe diaper rash who doesn't want anything near the anus. ↗
▶ Ep 8 · 1:38:31
clinical For 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. ↗
▶ Ep 8 · 1:38:53
clinical A good contrast study for Hirschsprung requires multiple films, including lateral views, to see if dilation goes all the way down to the pubococcygeal line. ↗
▶ Ep 8 · 1:38:58
clinical If dilation on contrast enema goes all the way down to the pubococcygeal line, that is not Hirschsprung, even if the biopsy says something different. ↗
▶ Ep 8 · 1:50:52
clinical For 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. ↗
▶ Ep 8 · 2:00:08
opinion There 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. ↗
▶ Ep 8 · 2:03:21
clinical Congenital central hypoventilation syndrome is associated with Ondine's curse, where patients stop breathing when they fall asleep. ↗

CinciHirsch - Pathology of Hirschprung Disease

▶ Ep 9 · 7:22
opinion After 6 months of age, the clinical picture of Hirschsprung disease is often so obvious that suction biopsy becomes less relevant. ↗
▶ Ep 9 · 7:22
opinion After 6 months of age, the clinical picture of Hirschsprung disease is often so obvious that suction biopsy becomes less relevant. ↗
▶ Ep 9 · 7:44
quote I 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. ↗
▶ Ep 9 · 7:44
quote I 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. ↗
▶ Ep 9 · 7:54
opinion The suction rectal biopsy is not the main element for diagnosis but one extra piece of information, particularly useful in newborn babies. ↗
▶ Ep 9 · 7:54
opinion The suction rectal biopsy is not the main element for diagnosis but one extra piece of information, particularly useful in newborn babies. ↗
▶ Ep 9 · 37:45
quote There 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. ↗
▶ Ep 9 · 37:45
clinical There is not a single topographic study of intestinal neuronal dysplasia describing the anatomical extent of the histological abnormality. ↗
▶ Ep 9 · 37:45
quote There 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. ↗
▶ Ep 9 · 37:45
clinical There is not a single topographic study of intestinal neuronal dysplasia describing the anatomical extent of the histological abnormality. ↗
▶ Ep 9 · 37:56
opinion Without topographic studies, there is no basis for resecting histologically abnormal portions of colon in IND, unlike in Hirschsprung disease where pathology determines resection extent. ↗
▶ Ep 9 · 37:56
opinion Without topographic studies, there is no basis for resecting histologically abnormal portions of colon in IND, unlike in Hirschsprung disease where pathology determines resection extent. ↗
▶ Ep 9 · 38:27
opinion The 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. ↗
▶ Ep 9 · 38:27
opinion The 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. ↗
▶ Ep 9 · 38:37
quote The 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. ↗
▶ Ep 9 · 38:37
quote The 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. ↗

Hirschsprung Disease: Surgical Procedures

▶ Ep 19 · 3:42
clinical Between 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. ↗
▶ Ep 19 · 6:28
opinion If you start transanally and cannot mobilize more bowel, you simply go into the abdomen and continue the resection—this is not considered a complication. ↗
▶ Ep 19 · 6:41
quote I 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. ↗
▶ Ep 19 · 11:10
quote The 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. ↗
▶ Ep 19 · 11:10
opinion The 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. ↗
▶ Ep 19 · 12:09
quote I 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. ↗
▶ Ep 19 · 12:17
opinion Patients complain about fecal incontinence, not about the size of the scar—that is the real problem. ↗
▶ Ep 19 · 14:23
clinical The Lone Star retractor hooks should be placed at the pectinate line itself, not in the anal canal, to protect the anal canal by definition. ↗
▶ Ep 19 · 14:55
clinical Surgeons should be careful not to stretch too much during transanal dissection because excessive stretching damages the sphincter mechanism by definition, potentially causing fecal incontinence. ↗
▶ Ep 19 · 14:55
quote 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. ↗
▶ Ep 19 · 16:42
clinical The 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. ↗
▶ Ep 19 · 17:29
opinion Prone 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. ↗
▶ Ep 19 · 18:30
opinion Leaving 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. ↗
▶ Ep 19 · 18:30
quote I 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. ↗
▶ Ep 19 · 19:00
quote We 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. ↗
▶ Ep 19 · 21:41
clinical The 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. ↗
▶ Ep 19 · 22:39
clinical When 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. ↗
▶ Ep 19 · 22:39
quote When 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. ↗
▶ Ep 19 · 22:50
clinical Even 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. ↗
▶ Ep 19 · 23:32
clinical Even in patients with a very well preserved anal canal, some children have different degrees of fecal incontinence after Hirschsprung surgery. ↗
▶ Ep 19 · 23:44
opinion Fecal incontinence in Hirschsprung disease is much more common than we believe, and we have not been discussing it enough in pediatric surgical meetings. ↗
▶ Ep 19 · 23:44
quote We have not been discussing enough in our pediatric surgical meetings the problem of fecal incontinence that is much more common than we believe. ↗
▶ Ep 19 · 24:43
clinical If you preserve the pectinate line, by definition you are preserving this crucial part of the bowel. ↗
▶ Ep 19 · 25:09
opinion Patients are born with bowel control—we provoke the fecal incontinence through surgical technique. ↗
▶ Ep 19 · 25:09
quote The patients are born with bowel control. We provoke the fecal incontinence. ↗
▶ Ep 19 · 26:24
clinical The 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. ↗
▶ Ep 19 · 27:06
clinical If 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. ↗
▶ Ep 19 · 27:23
clinical If you stay right on the bowel wall during dissection, you will not provoke denervation of the urinary tract. ↗
▶ Ep 19 · 27:23
quote If you stay right in the bowel wall, you will not provoke the innervation of the urinary tract. ↗
▶ Ep 19 · 1:19:52
clinical Waiting 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. ↗
▶ Ep 19 · 1:20:03
quote The baby is happy with the ileostomy. The only unhappy people are others, but not the baby. ↗
▶ Ep 19 · 1:20:23
quote 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. ↗
▶ Ep 19 · 1:20:23
clinical If 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. ↗
▶ Ep 19 · 1:20:46
clinical Patients 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. ↗
▶ Ep 19 · 2:00:20
opinion There 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. ↗

Panel Discussion and Case Presentation Part II: Pediatric Bowel Management 2013

▶ Ep 25 · 2:26
clinical In Spain, de la Torre technique is used; for total colonic aganglionosis, Lester Martin procedure is used. ↗
▶ Ep 25 · 2:26
clinical In Spain, de la Torre technique is used; for total colonic aganglionosis, Lester Martin procedure is used. ↗
▶ Ep 25 · 6:52
clinical When 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. ↗
▶ Ep 25 · 6:52
clinical When 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. ↗
▶ Ep 25 · 7:11
clinical A permanent stoma is indicated when the anal canal is destroyed and the patient has total colonic aganglionosis. ↗
▶ Ep 25 · 7:11
clinical A permanent stoma is indicated when the anal canal is destroyed and the patient has total colonic aganglionosis. ↗
▶ Ep 25 · 7:27
clinical Sometimes 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. ↗
▶ Ep 25 · 7:27
clinical Sometimes 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. ↗
▶ Ep 25 · 12:56
opinion Most pediatricians worldwide do not know the difference between enterocolitis and gastroenteritis; they do not understand the entity called post-Hirschsprung enterocolitis. ↗
▶ Ep 25 · 12:56
opinion Most pediatricians worldwide do not know the difference between enterocolitis and gastroenteritis; they do not understand the entity called post-Hirschsprung enterocolitis. ↗
▶ Ep 25 · 12:56
quote Most pediatricians all over the world don't know the difference between enterocolitis and gastroenteritis. ↗
▶ Ep 25 · 12:56
quote Most pediatricians all over the world don't know the difference between enterocolitis and gastroenteritis. ↗
▶ Ep 25 · 13:14
opinion Pediatricians 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. ↗
▶ Ep 25 · 13:14
opinion Pediatricians 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. ↗
▶ Ep 25 · 14:25
clinical When 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. ↗
▶ Ep 25 · 14:25
clinical When 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. ↗
▶ Ep 25 · 14:53
opinion Dr. Pena does not believe that leaving 1-2 cm of rectal mucosa is the simple cause of enterocolitis. ↗
▶ Ep 25 · 14:53
opinion Dr. Pena does not believe that leaving 1-2 cm of rectal mucosa is the simple cause of enterocolitis. ↗
▶ Ep 25 · 15:12
clinical A group in New York doing neonatal Soave primary procedures reported zero enterocolitis; when Dr. Pena followed some of those patients, many had fecal incontinence. ↗
▶ Ep 25 · 15:12
clinical A group in New York doing neonatal Soave primary procedures reported zero enterocolitis; when Dr. Pena followed some of those patients, many had fecal incontinence. ↗
▶ Ep 25 · 15:48
clinical If 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. ↗
▶ Ep 25 · 15:48
clinical If 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. ↗
▶ Ep 25 · 15:48
quote If you produce fecal incontinence in the patient, the enterocolitis is zero. ↗
▶ Ep 25 · 15:48
quote If you produce fecal incontinence in the patient, the enterocolitis is zero. ↗
▶ Ep 25 · 16:05
clinical A good operation preserving the sphincter and anal canal creates sphincter closure, which creates stasis, and stasis produces enterocolitis. ↗
▶ Ep 25 · 16:05
clinical A good operation preserving the sphincter and anal canal creates sphincter closure, which creates stasis, and stasis produces enterocolitis. ↗
▶ Ep 25 · 16:19
quote We 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. ↗
▶ Ep 25 · 16:19
quote We 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. ↗
▶ Ep 25 · 16:30
quote I prefer to deal with enterocolitis than with fecal incontinence. Fecal incontinence is for life. ↗
▶ Ep 25 · 16:30
quote I prefer to deal with enterocolitis than with fecal incontinence. Fecal incontinence is for life. ↗
▶ Ep 25 · 16:30
opinion Dr. Pena prefers to deal with enterocolitis rather than fecal incontinence; fecal incontinence is for life. ↗
▶ Ep 25 · 16:30
opinion Dr. Pena prefers to deal with enterocolitis rather than fecal incontinence; fecal incontinence is for life. ↗
▶ Ep 25 · 19:16
epidemiological Dr. Pena's incidence of enterocolitis in pull-through patients is about 30%. ↗
▶ Ep 25 · 19:16
epidemiological Dr. Pena's incidence of enterocolitis in pull-through patients is about 30%. ↗
▶ Ep 25 · 23:02
clinical Resecting the rectosigmoid introduces a major pathophysiological change; children with perfect pull-throughs preserving the anal canal sometimes have toilet-training problems without explanation. ↗
▶ Ep 25 · 23:02
clinical Resecting the rectosigmoid introduces a major pathophysiological change; children with perfect pull-throughs preserving the anal canal sometimes have toilet-training problems without explanation. ↗
▶ Ep 25 · 23:30
clinical Hyperactive 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. ↗
▶ Ep 25 · 23:30
clinical Hyperactive 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. ↗
▶ Ep 25 · 24:01
clinical Even 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. ↗
▶ Ep 25 · 24:01
clinical Even 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. ↗
▶ Ep 25 · 24:59
opinion Dr. Pena does not find rectal manometry useful in Hirschsprung disease, constipation, or anorectal malformations after many years of experience. ↗
▶ Ep 25 · 24:59
opinion Dr. Pena does not find rectal manometry useful in Hirschsprung disease, constipation, or anorectal malformations after many years of experience. ↗
▶ Ep 25 · 26:25
clinical Patients are discharged with three irrigations per day and metronidazole (Flagyl); every month the number of irrigations is decreased. ↗
▶ Ep 25 · 26:25
clinical Patients are discharged with three irrigations per day and metronidazole (Flagyl); every month the number of irrigations is decreased. ↗
▶ Ep 25 · 26:46
clinical If 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. ↗
▶ Ep 25 · 26:46
clinical If 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. ↗
▶ Ep 25 · 27:07
clinical For refractory enterocolitis, another option is further resection of normal ganglionic colon to remove more. ↗
▶ Ep 25 · 27:07
clinical For refractory enterocolitis, another option is further resection of normal ganglionic colon to remove more. ↗
▶ Ep 25 · 27:17
opinion Hirschsprung 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. ↗
▶ Ep 25 · 27:17
opinion Hirschsprung 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. ↗
▶ Ep 25 · 27:45
clinical '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. ↗
▶ Ep 25 · 27:45
clinical '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. ↗
▶ Ep 25 · 28:13
epidemiological In 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. ↗
▶ Ep 25 · 28:13
epidemiological In 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. ↗
▶ Ep 25 · 28:30
opinion There 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. ↗
▶ Ep 25 · 28:30
quote There 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. ↗
▶ Ep 25 · 28:30
opinion There 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. ↗
▶ Ep 25 · 28:30
quote There 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. ↗
▶ Ep 25 · 28:46
opinion Some 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. ↗
▶ Ep 25 · 28:46
opinion Some 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. ↗
▶ Ep 25 · 31:25
opinion Dr. 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. ↗
▶ Ep 25 · 31:25
opinion Dr. 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. ↗
▶ Ep 25 · 32:02
clinical Dr. 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. ↗
▶ Ep 25 · 32:02
clinical Dr. 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. ↗
▶ Ep 25 · 32:48
clinical Patients 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. ↗
▶ Ep 25 · 32:48
clinical Patients 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. ↗
▶ Ep 25 · 33:11
clinical Patients subjected to myotomies, myectomies, or repeated Botox injections eventually develop more severe fecal incontinence. ↗
▶ Ep 25 · 33:11
clinical Patients subjected to myotomies, myectomies, or repeated Botox injections eventually develop more severe fecal incontinence. ↗
▶ Ep 25 · 33:24
opinion Dr. Pena does not believe in myectomy/myotomy/Botox procedures and does not use Botox. ↗
▶ Ep 25 · 33:24
opinion Dr. Pena does not believe in myectomy/myotomy/Botox procedures and does not use Botox. ↗

Cloacal Exstrophy with Dr. Alberto Peña

▶ Ep 33 · 1:31
clinical Cloacal exstrophy is a spectrum of congenital malformations affecting the gastrointestinal/colorectal area, urogenital tract, spine and cord, and sometimes lower extremity motion. ↗
▶ Ep 33 · 2:09
clinical Babies with cloacal exstrophy are born with an omphalocele, bladder exstrophy (two separated hemibladders), open cecum between the hemibladders, and separated pubic bones. ↗
▶ Ep 33 · 3:08
clinical The small bowel can become exstrophic through the ileocecal valve, creating an 'elephant trunk' appearance. ↗
▶ Ep 33 · 3:32
clinical Male patients have two separated hemiphalluses with normal gonads; female patients have two hemivaginas below the exstrophic bladder leading to two hemiuteri. ↗
▶ Ep 33 · 4:20
clinical Cloacal exstrophy represents a spectrum of colonic anatomy from normal colon to almost absent or completely absent colon, sometimes with two ceca or two appendices and bizarre blood supply. ↗
▶ Ep 33 · 4:57
clinical The amount of colon present at birth has very important implications for the patient's management and outcomes. ↗
▶ Ep 33 · 5:44
clinical A variant exists where babies are born with intact abdominal skin (no omphalocele, no bladder exstrophy externally) but have a completely open bladder inside with no bladder neck and a single large perineal orifice. ↗
▶ Ep 33 · 7:02
clinical While surgical techniques, intensive care, parenteral nutrition, and metabolic management have improved, functional outcomes (bowel control, urinary control, sexual function, spinal abnormalities) remain severely limited and cannot be made normal. ↗
▶ Ep 33 · 8:31
clinical Historical practice was to perform bilateral gonadectomy, remove hemiphalluses, create a vagina with bowel, and assign female gender to XY patients with cloacal exstrophy. ↗
▶ Ep 33 · 9:16
clinical Patients raised as female despite XY chromosomes exhibited male attitudes and behavior, and many became upset upon learning their chromosomal sex and that gonads were removed without their consent. ↗
▶ Ep 33 · 9:59
opinion Patients argued that sex is not the most important aspect of being male, that they wanted their gonads back for fertility (modern techniques allow fertilization and children), and that being male is much more than having a phallus. ↗
▶ Ep 33 · 9:59
quote 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. ↗
▶ Ep 33 · 10:29
quote being a male is much more than having a phallus ↗
▶ Ep 33 · 10:34
guideline Current consensus is that XY patients should be raised as male, with pediatric urologists and plastic surgeons working on phallus reconstruction techniques. ↗
▶ Ep 33 · 12:13
clinical When a prominent pediatric urologist dominates management, patients receive good urologic attention but inadequate gastrointestinal care; the reverse occurs when pediatric surgeons dominate. ↗
▶ Ep 33 · 13:03
clinical The pediatric surgeon's role in the initial operation is to close the omphalocele (if possible), separate urothelium from intestinal mucosa by placing stitches at the edges and making an incision, allowing the urologist to bring hemibladders together. ↗
▶ Ep 33 · 14:55
clinical It is very common but very harmful for pediatric surgeons to simply create an ileostomy, leaving all colon distally attached to the urinary tract. ↗
▶ Ep 33 · 15:11
quote That is a very bad thing to do, and the patient will suffer several consequences because of that ↗
▶ Ep 33 · 15:31
clinical Leaving colon attached to the urinary tract creates a congenital bladder augmentation that causes hyperchloremic acidosis from urine absorption, interfering with growth and development. ↗
▶ Ep 33 · 15:40
quote Isn't that beautiful from your point of view? ↗
▶ Ep 33 · 16:03
clinical Defunctionalized colonic tissue left distally will not grow; colon requires passage of fecal matter through its lumen to grow. ↗
▶ Ep 33 · 16:23
clinical The pediatric surgeon must incorporate all gastrointestinal tissue into the fecal stream and create a true end colostomy to ensure fecal matter passes through all colonic tissue. ↗
▶ Ep 33 · 16:41
clinical Patients who received ileostomy with defunctionalized colon present at 2-3 years with poor growth, hyperchloremic acidosis managed by nephrologists, and large ileostomy losses. ↗
▶ Ep 33 · 17:03
clinical The rescue operation involves taking down the ileostomy, finding and incorporating colonic tissue into the GI tract, and creating an end colostomy; acidosis disappears the next day. ↗
▶ Ep 33 · 19:13
opinion Surgeons must accept that cloacal exstrophy is a spectrum and be prepared to deal with complex, variable colonic anatomy rather than taking the easy way out with an ileostomy. ↗
▶ Ep 33 · 20:07
quote Do not leave gastrointestinal tract inside the functionalized. That's the main, main thing. ↗
▶ Ep 33 · 21:00
clinical Some institutions routinely perform pelvic osteotomy at the initial operation to facilitate bladder and omphalocele reconstruction, while others wait 2-3 months to do it separately. ↗
▶ Ep 33 · 21:09
clinical Even after osteotomy, it is very difficult to see a cloacal exstrophy patient with pubic bones completely together; they usually remain separated. ↗
▶ Ep 33 · 22:06
clinical Even a technically correct end colostomy often has poor motility, and babies may not pass stool easily, sometimes developing bacterial overgrowth similar to Hirschsprung disease. ↗
▶ Ep 33 · 23:00
clinical Families must be taught to irrigate the colostomy with small volumes of saline through a tube to evacuate fecal material when peristalsis is inadequate. ↗
▶ Ep 33 · 23:30
clinical Decision-making for bowel control begins around age 3 when parents want to send the child to school clean and dry (no stool or urine in diaper). ↗
▶ Ep 33 · 23:54
clinical Most cloacal exstrophy patients have an inadequate, tiny bladder requiring bladder augmentation with gastrointestinal tract, necessitating coordination between pediatric surgery and urology. ↗
▶ Ep 33 · 24:42
clinical Patients born with no colon are candidates for permanent colostomy and should never have terminal ileum pulled through, even if sphincter evidence exists, because they will never have bowel control. ↗
▶ Ep 33 · 25:04
clinical Pull-through is only considered for patients with capacity to form solid stool (adequate colon), as bowel management only works with solid stool. ↗
▶ Ep 33 · 25:20
clinical It is extremely unusual for cloacal exstrophy patients to have spontaneous bowel control; the overwhelming majority need a bowel management program (enema administration to keep patient clean). ↗
▶ Ep 33 · 25:47
clinical Pediatric 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. ↗
▶ Ep 33 · 26:07
clinical Annual contrast studies through the colostomy (retrograde injection) are performed to assess colonic growth. ↗
▶ Ep 33 · 26:41
clinical Before 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. ↗
▶ Ep 33 · 27:35
clinical Bowel management trial through colostomy is typically started after age 3 when families consider avoiding the stoma for school. ↗
▶ Ep 33 · 28:07
clinical If 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. ↗
▶ Ep 33 · 28:54
clinical The 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. ↗
▶ Ep 33 · 29:56
clinical Contrast enema through the colostomy can distinguish true liquid stool from paradoxical diarrhea (liquid stool around solid fecal impaction). ↗
▶ Ep 33 · 31:04
clinical Pull-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). ↗
▶ Ep 33 · 31:28
clinical During 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. ↗
▶ Ep 33 · 31:57
clinical Urologists 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. ↗
▶ Ep 33 · 32:18
clinical Occasionally, 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. ↗
▶ Ep 33 · 33:13
clinical Midline abdominal incision from xiphoid to pubis is used for pull-through to preserve the flanks and quadrants for potential future stomas. ↗
▶ Ep 33 · 33:56
clinical The 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. ↗
▶ Ep 33 · 34:39
clinical Careful 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. ↗
▶ Ep 33 · 35:53
clinical The 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. ↗
▶ Ep 33 · 37:33
clinical Cloacal 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). ↗
▶ Ep 33 · 37:38
clinical Some 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. ↗
▶ Ep 33 · 38:50
clinical During pull-through, vaginal reconstruction is attempted by approximating the hemivaginas as much as possible; the degree of separation varies on the spectrum. ↗
▶ Ep 33 · 39:07
clinical When 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. ↗
▶ Ep 33 · 39:53
clinical Patients with functional hemiuterus may become pregnant, but it is high-risk pregnancy requiring specialized pediatric gynecology follow-up; in general, pregnancy is not advised. ↗
▶ Ep 33 · 40:10
clinical Hemiuterus has a great tendency to produce miscarriages and premature labor; delivery must be by cesarean section due to limited abdominal space. ↗
▶ Ep 33 · 40:37
clinical Bladder 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. ↗
▶ Ep 33 · 41:07
clinical Augmented 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. ↗
▶ Ep 33 · 41:33
clinical Bladder irrigation is sometimes performed with gentamicin to ensure all mucus is removed. ↗
▶ Ep 33 · 41:45
clinical Lifelong 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. ↗
▶ Ep 33 · 42:29
clinical Patients 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. ↗
▶ Ep 33 · 43:03
opinion Dr. 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. ↗
▶ Ep 33 · 43:42
clinical The 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. ↗
▶ Ep 33 · 44:48
opinion Colorectal 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. ↗
▶ Ep 33 · 46:00
clinical The 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). ↗
▶ Ep 33 · 46:54
clinical Between the initial operation and pull-through, colostomy irrigation is often needed to manage poor colonic motility. ↗
▶ Ep 33 · 47:58
clinical Prenatal 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. ↗
▶ Ep 33 · 48:48
clinical Absent bladder on prenatal ultrasound (due to exstrophy) is a bad sign and can be detected as early as week 20 of pregnancy. ↗
▶ Ep 33 · 49:38
clinical Prenatal diagnosis allows families to decide about pregnancy continuation and, if continuing, to deliver at a center with a multidisciplinary team experienced in these malformations. ↗
▶ Ep 33 · 50:04
opinion Certain 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. ↗
Omphalocele 70 entries

Cloacal Exstrophy with Dr. Alberto Peña

▶ Ep 7 · 1:31
clinical Cloacal exstrophy is a spectrum of congenital malformations affecting the gastrointestinal/colorectal area, urogenital tract, spine and cord, and sometimes lower extremity motion. ↗
▶ Ep 7 · 2:09
clinical Babies with cloacal exstrophy are born with an omphalocele, bladder exstrophy (two separated hemibladders), open cecum between the hemibladders, and separated pubic bones. ↗
▶ Ep 7 · 3:08
clinical The small bowel can become exstrophic through the ileocecal valve, creating an 'elephant trunk' appearance. ↗
▶ Ep 7 · 3:32
clinical Male patients have two separated hemiphalluses with normal gonads; female patients have two hemivaginas below the exstrophic bladder leading to two hemiuteri. ↗
▶ Ep 7 · 4:20
clinical Cloacal exstrophy represents a spectrum of colonic anatomy from normal colon to almost absent or completely absent colon, sometimes with two ceca or two appendices and bizarre blood supply. ↗
▶ Ep 7 · 4:57
clinical The amount of colon present at birth has very important implications for the patient's management and outcomes. ↗
▶ Ep 7 · 5:44
clinical A variant exists where babies are born with intact abdominal skin (no omphalocele, no bladder exstrophy externally) but have a completely open bladder inside with no bladder neck and a single large perineal orifice. ↗
▶ Ep 7 · 7:02
clinical While surgical techniques, intensive care, parenteral nutrition, and metabolic management have improved, functional outcomes (bowel control, urinary control, sexual function, spinal abnormalities) remain severely limited and cannot be made normal. ↗
▶ Ep 7 · 8:31
clinical Historical practice was to perform bilateral gonadectomy, remove hemiphalluses, create a vagina with bowel, and assign female gender to XY patients with cloacal exstrophy. ↗
▶ Ep 7 · 9:16
clinical Patients raised as female despite XY chromosomes exhibited male attitudes and behavior, and many became upset upon learning their chromosomal sex and that gonads were removed without their consent. ↗
▶ Ep 7 · 9:59
quote 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. ↗
▶ Ep 7 · 9:59
opinion Patients argued that sex is not the most important aspect of being male, that they wanted their gonads back for fertility (modern techniques allow fertilization and children), and that being male is much more than having a phallus. ↗
▶ Ep 7 · 10:29
quote being a male is much more than having a phallus ↗
▶ Ep 7 · 10:34
guideline Current consensus is that XY patients should be raised as male, with pediatric urologists and plastic surgeons working on phallus reconstruction techniques. ↗
▶ Ep 7 · 12:13
clinical When a prominent pediatric urologist dominates management, patients receive good urologic attention but inadequate gastrointestinal care; the reverse occurs when pediatric surgeons dominate. ↗
▶ Ep 7 · 13:03
clinical The pediatric surgeon's role in the initial operation is to close the omphalocele (if possible), separate urothelium from intestinal mucosa by placing stitches at the edges and making an incision, allowing the urologist to bring hemibladders together. ↗
▶ Ep 7 · 14:55
clinical It is very common but very harmful for pediatric surgeons to simply create an ileostomy, leaving all colon distally attached to the urinary tract. ↗
▶ Ep 7 · 15:11
quote That is a very bad thing to do, and the patient will suffer several consequences because of that ↗
▶ Ep 7 · 15:31
clinical Leaving colon attached to the urinary tract creates a congenital bladder augmentation that causes hyperchloremic acidosis from urine absorption, interfering with growth and development. ↗
▶ Ep 7 · 15:40
quote Isn't that beautiful from your point of view? ↗
▶ Ep 7 · 16:03
clinical Defunctionalized colonic tissue left distally will not grow; colon requires passage of fecal matter through its lumen to grow. ↗
▶ Ep 7 · 16:23
clinical The pediatric surgeon must incorporate all gastrointestinal tissue into the fecal stream and create a true end colostomy to ensure fecal matter passes through all colonic tissue. ↗
▶ Ep 7 · 16:41
clinical Patients who received ileostomy with defunctionalized colon present at 2-3 years with poor growth, hyperchloremic acidosis managed by nephrologists, and large ileostomy losses. ↗
▶ Ep 7 · 17:03
clinical The rescue operation involves taking down the ileostomy, finding and incorporating colonic tissue into the GI tract, and creating an end colostomy; acidosis disappears the next day. ↗
▶ Ep 7 · 19:13
opinion Surgeons must accept that cloacal exstrophy is a spectrum and be prepared to deal with complex, variable colonic anatomy rather than taking the easy way out with an ileostomy. ↗
▶ Ep 7 · 20:07
quote Do not leave gastrointestinal tract inside the functionalized. That's the main, main thing. ↗
▶ Ep 7 · 21:00
clinical Some institutions routinely perform pelvic osteotomy at the initial operation to facilitate bladder and omphalocele reconstruction, while others wait 2-3 months to do it separately. ↗
▶ Ep 7 · 21:09
clinical Even after osteotomy, it is very difficult to see a cloacal exstrophy patient with pubic bones completely together; they usually remain separated. ↗
▶ Ep 7 · 22:06
clinical Even a technically correct end colostomy often has poor motility, and babies may not pass stool easily, sometimes developing bacterial overgrowth similar to Hirschsprung disease. ↗
▶ Ep 7 · 23:00
clinical Families must be taught to irrigate the colostomy with small volumes of saline through a tube to evacuate fecal material when peristalsis is inadequate. ↗
▶ Ep 7 · 23:30
clinical Decision-making for bowel control begins around age 3 when parents want to send the child to school clean and dry (no stool or urine in diaper). ↗
▶ Ep 7 · 23:54
clinical Most cloacal exstrophy patients have an inadequate, tiny bladder requiring bladder augmentation with gastrointestinal tract, necessitating coordination between pediatric surgery and urology. ↗
▶ Ep 7 · 24:42
clinical Patients born with no colon are candidates for permanent colostomy and should never have terminal ileum pulled through, even if sphincter evidence exists, because they will never have bowel control. ↗
▶ Ep 7 · 25:04
clinical Pull-through is only considered for patients with capacity to form solid stool (adequate colon), as bowel management only works with solid stool. ↗
▶ Ep 7 · 25:20
clinical It is extremely unusual for cloacal exstrophy patients to have spontaneous bowel control; the overwhelming majority need a bowel management program (enema administration to keep patient clean). ↗
▶ Ep 7 · 25:47
clinical Pediatric 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. ↗
▶ Ep 7 · 26:07
clinical Annual contrast studies through the colostomy (retrograde injection) are performed to assess colonic growth. ↗
▶ Ep 7 · 26:41
clinical Before 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. ↗
▶ Ep 7 · 27:35
clinical Bowel management trial through colostomy is typically started after age 3 when families consider avoiding the stoma for school. ↗
▶ Ep 7 · 28:07
clinical If 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. ↗
▶ Ep 7 · 28:54
clinical The 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. ↗
▶ Ep 7 · 29:56
clinical Contrast enema through the colostomy can distinguish true liquid stool from paradoxical diarrhea (liquid stool around solid fecal impaction). ↗
▶ Ep 7 · 31:04
clinical Pull-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). ↗
▶ Ep 7 · 31:28
clinical During 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. ↗
▶ Ep 7 · 31:57
clinical Urologists 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. ↗
▶ Ep 7 · 32:18
clinical Occasionally, 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. ↗
▶ Ep 7 · 33:13
clinical Midline abdominal incision from xiphoid to pubis is used for pull-through to preserve the flanks and quadrants for potential future stomas. ↗
▶ Ep 7 · 33:56
clinical The 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. ↗
▶ Ep 7 · 34:39
clinical Careful 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. ↗
▶ Ep 7 · 35:53
clinical The 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. ↗
▶ Ep 7 · 37:33
clinical Cloacal 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). ↗
▶ Ep 7 · 37:38
clinical Some 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. ↗
▶ Ep 7 · 38:50
clinical During pull-through, vaginal reconstruction is attempted by approximating the hemivaginas as much as possible; the degree of separation varies on the spectrum. ↗
▶ Ep 7 · 39:07
clinical When 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. ↗
▶ Ep 7 · 39:53
clinical Patients with functional hemiuterus may become pregnant, but it is high-risk pregnancy requiring specialized pediatric gynecology follow-up; in general, pregnancy is not advised. ↗
▶ Ep 7 · 40:10
clinical Hemiuterus has a great tendency to produce miscarriages and premature labor; delivery must be by cesarean section due to limited abdominal space. ↗
▶ Ep 7 · 40:37
clinical Bladder 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. ↗
▶ Ep 7 · 41:07
clinical Augmented 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. ↗
▶ Ep 7 · 41:33
clinical Bladder irrigation is sometimes performed with gentamicin to ensure all mucus is removed. ↗
▶ Ep 7 · 41:45
clinical Lifelong 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. ↗
▶ Ep 7 · 42:29
clinical Patients 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. ↗
▶ Ep 7 · 43:03
opinion Dr. 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. ↗
▶ Ep 7 · 43:42
clinical The 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. ↗
▶ Ep 7 · 44:48
opinion Colorectal 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. ↗
▶ Ep 7 · 46:00
clinical The 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). ↗
▶ Ep 7 · 46:54
clinical Between the initial operation and pull-through, colostomy irrigation is often needed to manage poor colonic motility. ↗
▶ Ep 7 · 47:58
clinical Prenatal 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. ↗
▶ Ep 7 · 48:48
clinical Absent bladder on prenatal ultrasound (due to exstrophy) is a bad sign and can be detected as early as week 20 of pregnancy. ↗
▶ Ep 7 · 49:38
clinical Prenatal diagnosis allows families to decide about pregnancy continuation and, if continuing, to deliver at a center with a multidisciplinary team experienced in these malformations. ↗
▶ Ep 7 · 50:04
opinion Certain 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. ↗
Total Colonic Aganglionosis 125 entries

Surgical Procedures for Hirschsprung Disease

▶ Ep 2 · 3:42
clinical Between 75 and 80% of Hirschsprung cases can be completed transanally, reaching normal ganglionic bowel from below. ↗
▶ Ep 2 · 6:28
opinion If you start transanally and cannot reach ganglionic bowel, you simply open the abdomen and continue the resection—this is not a complication. ↗
▶ Ep 2 · 11:10
opinion The main problems in Hirschsprung surgery are related to surgeon inexperience and technical incapacity, not the approach (laparoscopic vs. transanal vs. open). ↗
▶ Ep 2 · 11:10
quote The 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. ↗
▶ Ep 2 · 11:37
opinion A bad surgeon will damage the patient both ways—laparoscopically or transanally. ↗
▶ Ep 2 · 11:45
clinical The basic goal of Hirschsprung surgery is not to damage the sphincter mechanism, which has been damaged by both laparoscopic and non-laparoscopic techniques. ↗
▶ Ep 2 · 12:09
quote I 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. ↗
▶ Ep 2 · 12:17
opinion Patients complain about fecal incontinence, not the size of the scar. ↗
▶ Ep 2 · 14:13
clinical When doing transanal dissection, the Lone Star retractor hooks should be placed at the pectinate line to protect the entire anal canal. ↗
▶ Ep 2 · 14:55
clinical Surgeons must be careful not to stretch the anus too much during transanal dissection, as excessive stretch damages the sphincter mechanism and causes fecal incontinence. ↗
▶ Ep 2 · 15:21
clinical The dissection should start 2 centimeters deep inside the rectum from the pectinate line, using multiple silk stitches to distribute tension and avoid tissue damage. ↗
▶ Ep 2 · 15:48
clinical Dr. de la Torre originally started the transanal operation submucosally and rectally; Dr. Peña prefers full-thickness dissection like Dr. Swenson used to do. ↗
▶ Ep 2 · 16:08
clinical Biopsies should be taken every 5 centimeters during transanal dissection until normal ganglionic bowel is found, then go 5 centimeters higher. ↗
▶ Ep 2 · 16:31
clinical A 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. ↗
▶ Ep 2 · 17:12
clinical Keys 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. ↗
▶ Ep 2 · 17:22
opinion Prone 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. ↗
▶ Ep 2 · 18:30
opinion Leaving 1-2 centimeters of aganglionic bowel does not explain why patients don't behave well postoperatively. ↗
▶ Ep 2 · 18:42
clinical Some patients operated with exactly the same technique do beautifully like normal individuals, while others have symptoms of enterocolitis, and we don't know why. ↗
▶ Ep 2 · 21:33
clinical The 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. ↗
▶ Ep 2 · 21:57
clinical A few patients do have an obvious piece of aganglionic bowel left, but usually it's much more than 2 centimeters. ↗
▶ Ep 2 · 22:06
clinical When 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. ↗
▶ Ep 2 · 22:21
opinion The real concern about transanal surgery is fecal incontinence, which happens when the anal canal is damaged. ↗
▶ Ep 2 · 22:39
clinical When 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. ↗
▶ Ep 2 · 22:39
quote When 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. ↗
▶ Ep 2 · 22:50
clinical Even adult ulcerative colitis patients with perfect operations and intact anal canals have problems with bowel control—they have accidents at night. ↗
▶ Ep 2 · 23:08
clinical After 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. ↗
▶ Ep 2 · 23:22
clinical Removing the rectum in a child results in passing stool constantly, requiring an intact anal canal, sensation, intact sphincter, and cooperation for bowel control. ↗
▶ Ep 2 · 23:35
clinical Even in patients with a very well-preserved anal canal, some children have different degrees of fecal incontinence after Hirschsprung surgery. ↗
▶ Ep 2 · 23:44
quote We have not been discussing enough in our pediatric surgical meetings the problem of fecal incontinence that is much more common than we believe. ↗
▶ Ep 2 · 23:44
opinion Fecal incontinence after Hirschsprung surgery is much more common than we believe, and we have not been discussing it enough in pediatric surgical meetings. ↗
▶ Ep 2 · 23:53
clinical Most patients with symptoms of enterocolitis and constipation after pull-through do not have residual aganglionic bowel. ↗
▶ Ep 2 · 25:09
quote Patients are born with bowel control. We provoke the fecal incontinence. ↗
▶ Ep 2 · 25:09
opinion Patients are born with bowel control; we provoke fecal incontinence through surgical technique. ↗
▶ Ep 2 · 25:34
clinical Until the time of the video, Dr. Peña's group had performed 125 transanal operations: 56 primary Hirschsprung, 42 redo Hirschsprung, 21 for idiopathic constipation. ↗
▶ Ep 2 · 25:51
opinion Transanal pull-through for idiopathic constipation is not a good operation. ↗
▶ Ep 2 · 26:24
clinical When 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. ↗
▶ Ep 2 · 26:45
opinion Cases with fistulas to the vagina or urinary tract after Hirschsprung surgery are unacceptable complications. ↗
▶ Ep 2 · 27:04
clinical During 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. ↗
▶ Ep 2 · 27:23
host_summary If you stay right on the bowel wall during dissection, you will not provoke denervation of the urinary tract, as Dr. Swenson emphasized. ↗
▶ Ep 2 · 27:50
clinical The outer layer of sutures fixes the rectum in the right position and releases tension from the inner layer. ↗
▶ Ep 2 · 29:10
clinical Avoid using big retractors pulling in different directions during transanal surgery because that stretches the sphincter too much. ↗
▶ Ep 2 · 1:18:20
opinion Pediatric surgeons compete with each other trying to close stomas as early as possible, but this may not be best for the patient. ↗
▶ Ep 2 · 1:18:43
clinical In Hirschsprung disease with or without total colonic aganglionosis, we resect the natural reservoir and connect high-motility bowel to the anal canal. ↗
▶ Ep 2 · 1:19:09
clinical Adults who receive ileoanal anastomosis for ulcerative colitis live the rest of their lives trying to avoid accidents, with terrible diarrhea. ↗
▶ Ep 2 · 1:19:46
clinical Creating intentional stasis in the bowel leads to bacterial proliferation, colitis, inflammatory changes, secretory diarrhea, and worsening symptoms. ↗
▶ Ep 2 · 1:19:52
clinical If 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. ↗
▶ Ep 2 · 1:19:52
quote The baby is happy with the ileostomy. The only unhappy people are others, but not the baby. ↗
▶ Ep 2 · 1:20:23
clinical Patients with total colonic aganglionosis have a high incidence of enterocolitis and will most likely need rectal irrigation. ↗
▶ Ep 2 · 1:20:46
clinical It's not easy to do rectal irrigations in a 3-year-old with severe diaper rash who doesn't want anything near the anus. ↗
▶ Ep 2 · 1:38:31
clinical For 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. ↗
▶ Ep 2 · 1:38:53
clinical A good contrast study for Hirschsprung requires multiple films, including lateral views, to see if dilation goes all the way down to the pubococcygeal line. ↗
▶ Ep 2 · 1:38:58
clinical If dilation on contrast enema goes all the way down to the pubococcygeal line, that is not Hirschsprung, even if the biopsy says something different. ↗
▶ Ep 2 · 1:50:52
clinical For 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. ↗
▶ Ep 2 · 2:00:08
opinion There 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. ↗
▶ Ep 2 · 2:03:21
host_summary Congenital central hypoventilation syndrome is associated with Ondine's curse, where patients stop breathing when they fall asleep. ↗

Hirschsprung Disease: Surgical Procedures

▶ Ep 3 · 3:42
clinical Between 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. ↗
▶ Ep 3 · 6:28
opinion If you start transanally and cannot mobilize more bowel, you simply go into the abdomen and continue the resection—this is not considered a complication. ↗
▶ Ep 3 · 6:41
quote I 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. ↗
▶ Ep 3 · 11:10
quote The 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. ↗
▶ Ep 3 · 11:10
opinion The 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. ↗
▶ Ep 3 · 12:09
quote I 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. ↗
▶ Ep 3 · 12:17
opinion Patients complain about fecal incontinence, not about the size of the scar—that is the real problem. ↗
▶ Ep 3 · 14:23
clinical The Lone Star retractor hooks should be placed at the pectinate line itself, not in the anal canal, to protect the anal canal by definition. ↗
▶ Ep 3 · 14:55
quote 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. ↗
▶ Ep 3 · 14:55
clinical Surgeons should be careful not to stretch too much during transanal dissection because excessive stretching damages the sphincter mechanism by definition, potentially causing fecal incontinence. ↗
▶ Ep 3 · 16:42
clinical The 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. ↗
▶ Ep 3 · 17:29
opinion Prone 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. ↗
▶ Ep 3 · 18:30
quote I 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. ↗
▶ Ep 3 · 18:30
opinion Leaving 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. ↗
▶ Ep 3 · 19:00
quote We 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. ↗
▶ Ep 3 · 21:41
clinical The 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. ↗
▶ Ep 3 · 22:39
clinical When 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. ↗
▶ Ep 3 · 22:39
quote When 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. ↗
▶ Ep 3 · 22:50
clinical Even 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. ↗
▶ Ep 3 · 23:32
clinical Even in patients with a very well preserved anal canal, some children have different degrees of fecal incontinence after Hirschsprung surgery. ↗
▶ Ep 3 · 23:44
opinion Fecal incontinence in Hirschsprung disease is much more common than we believe, and we have not been discussing it enough in pediatric surgical meetings. ↗
▶ Ep 3 · 23:44
quote We have not been discussing enough in our pediatric surgical meetings the problem of fecal incontinence that is much more common than we believe. ↗
▶ Ep 3 · 24:43
clinical If you preserve the pectinate line, by definition you are preserving this crucial part of the bowel. ↗
▶ Ep 3 · 25:09
quote The patients are born with bowel control. We provoke the fecal incontinence. ↗
▶ Ep 3 · 25:09
opinion Patients are born with bowel control—we provoke the fecal incontinence through surgical technique. ↗
▶ Ep 3 · 26:24
clinical The 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. ↗
▶ Ep 3 · 27:06
clinical If 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. ↗
▶ Ep 3 · 27:23
clinical If you stay right on the bowel wall during dissection, you will not provoke denervation of the urinary tract. ↗
▶ Ep 3 · 27:23
quote If you stay right in the bowel wall, you will not provoke the innervation of the urinary tract. ↗
▶ Ep 3 · 1:19:52
clinical Waiting 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. ↗
▶ Ep 3 · 1:20:03
quote The baby is happy with the ileostomy. The only unhappy people are others, but not the baby. ↗
▶ Ep 3 · 1:20:23
quote 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. ↗
▶ Ep 3 · 1:20:23
clinical If 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. ↗
▶ Ep 3 · 1:20:46
clinical Patients 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. ↗
▶ Ep 3 · 2:00:20
opinion There 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. ↗

Panel Discussion and Case Presentation Part II: Pediatric Bowel Management 2013

▶ Ep 5 · 2:26
clinical In Spain, de la Torre technique is used; for total colonic aganglionosis, Lester Martin procedure is used. ↗
▶ Ep 5 · 6:52
clinical When 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. ↗
▶ Ep 5 · 7:11
clinical A permanent stoma is indicated when the anal canal is destroyed and the patient has total colonic aganglionosis. ↗
▶ Ep 5 · 7:27
clinical Sometimes 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. ↗
▶ Ep 5 · 12:56
opinion Most pediatricians worldwide do not know the difference between enterocolitis and gastroenteritis; they do not understand the entity called post-Hirschsprung enterocolitis. ↗
▶ Ep 5 · 12:56
quote Most pediatricians all over the world don't know the difference between enterocolitis and gastroenteritis. ↗
▶ Ep 5 · 13:14
opinion Pediatricians 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. ↗
▶ Ep 5 · 14:25
clinical When 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. ↗
▶ Ep 5 · 14:53
opinion Dr. Pena does not believe that leaving 1-2 cm of rectal mucosa is the simple cause of enterocolitis. ↗
▶ Ep 5 · 15:12
clinical A group in New York doing neonatal Soave primary procedures reported zero enterocolitis; when Dr. Pena followed some of those patients, many had fecal incontinence. ↗
▶ Ep 5 · 15:48
quote If you produce fecal incontinence in the patient, the enterocolitis is zero. ↗
▶ Ep 5 · 15:48
clinical If 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. ↗
▶ Ep 5 · 16:05
clinical A good operation preserving the sphincter and anal canal creates sphincter closure, which creates stasis, and stasis produces enterocolitis. ↗
▶ Ep 5 · 16:19
quote We 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. ↗
▶ Ep 5 · 16:30
opinion Dr. Pena prefers to deal with enterocolitis rather than fecal incontinence; fecal incontinence is for life. ↗
▶ Ep 5 · 16:30
quote I prefer to deal with enterocolitis than with fecal incontinence. Fecal incontinence is for life. ↗
▶ Ep 5 · 19:16
epidemiological Dr. Pena's incidence of enterocolitis in pull-through patients is about 30%. ↗
▶ Ep 5 · 23:02
clinical Resecting the rectosigmoid introduces a major pathophysiological change; children with perfect pull-throughs preserving the anal canal sometimes have toilet-training problems without explanation. ↗
▶ Ep 5 · 23:30
clinical Hyperactive 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. ↗
▶ Ep 5 · 24:01
clinical Even 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. ↗
▶ Ep 5 · 24:59
opinion Dr. Pena does not find rectal manometry useful in Hirschsprung disease, constipation, or anorectal malformations after many years of experience. ↗
▶ Ep 5 · 26:25
clinical Patients are discharged with three irrigations per day and metronidazole (Flagyl); every month the number of irrigations is decreased. ↗
▶ Ep 5 · 26:46
clinical If 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. ↗
▶ Ep 5 · 27:07
clinical For refractory enterocolitis, another option is further resection of normal ganglionic colon to remove more. ↗
▶ Ep 5 · 27:17
opinion Hirschsprung 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. ↗
▶ Ep 5 · 27:45
clinical '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. ↗
▶ Ep 5 · 28:13
epidemiological In 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. ↗
▶ Ep 5 · 28:30
quote There 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. ↗
▶ Ep 5 · 28:30
opinion There 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. ↗
▶ Ep 5 · 28:46
opinion Some 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. ↗
▶ Ep 5 · 31:25
opinion Dr. 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. ↗
▶ Ep 5 · 32:02
clinical Dr. 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. ↗
▶ Ep 5 · 32:48
clinical Patients 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. ↗
▶ Ep 5 · 33:11
clinical Patients subjected to myotomies, myectomies, or repeated Botox injections eventually develop more severe fecal incontinence. ↗
▶ Ep 5 · 33:24
opinion Dr. Pena does not believe in myectomy/myotomy/Botox procedures and does not use Botox. ↗