I think it's also important to look for cervices and see if you have one vagina, two vaginas. Do you have a cervix, which will sort of be the front door? Is there a uterus behind there?
All anorectal malformation patients should be followed by urology long-term; more complex malformations have more associated urinary tract malformations, but even minor malformations require periodic follow-up to ensure kidney function and proper urinary tract emptying.
Appendicostomy (Malone/MAC/ACE procedure) involves bringing the appendix to the belly button with a valve mechanism to prevent reflux, allowing patients to independently administer flushes through the colon over 10–15 minutes, then sit on toilet for approximately 45 minutes to empty.
In anorectal malformation, rectum can land outside anal canal/sphincter complex on skin just above that area, outside vagina in girls, anywhere along urinary tract in boys (with fistula causing stool to come out urinary tract), or connected to bladder in more complex cases.
Sacral nerve stimulator in adults is used in patients with normal anatomy/development, but in pediatrics it is used in patients with prior pelvic operations, malformations, or disease processes, making study difficult because every patient is different.
Bowel Management Updates & Innovations with Live Q&A: April 2018
▶Ep 2 · 0:15
clinicalThe Colorectal Center patient population includes anorectal malformations (imperforate anus), Hirschsprung's disease, spina bifida, sacrococcygeal teratomas, trauma patients, and refractory constipation.↗
▶Ep 2 · 2:32
clinicalAppendicostomy (Malone/MAC/ACE procedure) involves bringing the appendix to the belly button with a valve mechanism to prevent reflux, allowing patients to independently administer flushes through the colon over 10–15 minutes, then sit on toilet for approximately 45 minutes to empty.↗
▶Ep 2 · 3:42
clinicalSacral nerve stimulator is not FDA approved for children under 18, but is performed off-label; it acts like a heart pacemaker, stimulating the third sacral nerve to improve bowel and urinary continence outcomes.↗
▶Ep 2 · 4:41
clinicalSacral nerve stimulator is a two-stage procedure (test phase and permanent phase) and has been performed at Cincinnati Children's for almost 3 years.↗
▶Ep 2 · 4:53
clinicalSolesta is an injection of hyaluronic acid polymer that fills space in the anal canal, used in patients with patulous (more open) anuses to help gain control.↗
▶Ep 2 · 7:27
clinicalPatients with deformed sacrums have lower likelihood of success with bowel management using medication alone (not on enema program).↗
▶Ep 2 · 7:50
clinicalMalone procedure requires prior success with enemas because it only changes the route of administration; surgical procedure should not be performed on patients unsuccessful with enema program.↗
▶Ep 2 · 8:17
clinicalMalone procedure is typically performed starting at age 5 years and older, most commonly between ages 5 and 10–12 years.↗
▶Ep 2 · 8:53
clinicalVomiting after enema can be caused by high-volume enema with irritant causing colon distention and discomfort.↗
▶Ep 2 · 10:59
guidelineSacral nerve stimulator indication per manufacturer requires 50% improvement on symptoms; patient must have tried non-surgical bowel management first.↗
▶Ep 2 · 11:17
clinicalSacral nerve stimulator requires normal sacrum; it is the least invasive option compared to Malone or Solesta when bowel management is working or needs improvement.↗
▶Ep 2 · 11:49
clinicalUsing hybrid OR with 3D imaging, sacral nerve stimulator can be placed in patients with very abnormal sacrums by identifying anatomy in real-time.↗
▶Ep 2 · 13:45
clinicalFor failed redo Hirschsprung's pull-through, redo surgery can almost always create a channel for stool evacuation via rectum/anus, even after 1–3 prior redos; very few patients end up with ileostomy if goal is rectal evacuation.↗
▶Ep 2 · 17:18
clinicalThe large intestine (colon) primarily absorbs water; in adults it can absorb up to 1 liter of water per day. When colon is removed, stool becomes softer/looser because less water is absorbed.↗
▶Ep 2 · 17:46
clinicalBowel management sometimes requires using Imodium to constipate/harden stool, then using enema to empty it—thickening stool first, then mechanically emptying.↗
▶Ep 2 · 19:12
quoteThere is no perfect surgery for constipation. If we had it, we would all recommend it and do it.↗
▶Ep 2 · 19:12
opinionThere is no perfect surgery for constipation; if there were, all surgeons would recommend and perform it.↗
▶Ep 2 · 19:20
clinicalTransanal colon/rectum resection is minimally invasive but carries risk of post-surgical incontinence in some patients.↗
▶Ep 2 · 19:56
clinicalColon resection for constipation may lessen treatment burden (e.g., reduce laxative dose from 10 to 5 squares of Ex-Lax) but this outcome is not guaranteed.↗
▶Ep 2 · 20:22
clinicalBefore colon resection, offer sacral nerve stimulator and perform manometry (colonic and anorectal) to identify non-functioning colon segments or outlet problems (sphincter dysfunction).↗
▶Ep 2 · 20:55
clinicalAnorectal manometry can reveal paradoxical sphincter function where child squeezes when trying to relax and vice versa; Botox works in younger children, physical therapy and biofeedback in older children for this.↗
▶Ep 2 · 22:31
clinicalColon resection is a big surgery requiring bowel anastomosis with associated risks; once colon is resected, it cannot be replaced. All other treatments (sacral nerve, enemas, laxatives) are reversible.↗
▶Ep 2 · 25:25
opinionSenna laxative is like insulin for a diabetic—a necessary medication to help move bowels in severe constipation; dose may change over time but patients are not 'addicted' to it.↗
▶Ep 2 · 25:29
quoteI sort of say it's like insulin for a diabetic. A diabetic needs insulin to lower their blood blood glucose levels. A patient who has severe constipation needs a laxative stimulant to help move their bowels.↗
▶Ep 2 · 26:48
clinicalStimulant laxatives (Senna, Dulcolax) make the colon squeeze/push stool forward and reduce water absorption time, resulting in softer stool—two mechanisms that benefit the patient population.↗
▶Ep 2 · 27:17
clinicalOnly two stimulant laxatives are available in the United States: Senna (Ex-Lax chocolate squares, tablet, liquid forms) and Dulcolax.↗
▶Ep 2 · 27:35
clinicalMiraLax is not a stimulant laxative; it prevents colon from absorbing water, keeping water in colon and making stool softer.↗
▶Ep 2 · 27:51
clinicalMiraLax is avoided in patients without full continence mechanisms because very loose stool is hard to control when nerves and muscle are partially impaired.↗
▶Ep 2 · 28:15
clinicalIn spinal cord patients (tethered cord, spina bifida), stool is often so formed that MiraLax-type agent is needed to soften stool along with motility agents.↗
▶Ep 2 · 30:09
clinicalBowel rest with colostomy is typically used in patients with anorectal malformation or Hirschsprung's pull-through complications (narrowing, stenosis, tightness) causing dilated colon/rectum upstream.↗
▶Ep 2 · 30:47
clinicalIf colostomy is not working due to constipation, consider ileostomy to totally exclude colon and divert stool before it reaches colon.↗
▶Ep 2 · 35:30
clinicalMichael Helmrath at Cincinnati Children's is growing colons in the lab from single cells, able to lengthen colon in mice and expanding to larger primates.↗
▶Ep 2 · 35:48
clinicalAnal sphincter tissue regeneration has been attempted by many researchers (using muscles from other areas, other sphincters from esophagus/stomach) but none have been proven to work; no successful procedure is currently offered.↗
▶Ep 2 · 37:00
clinicalCincinnati Children's has nearly 3 years of experience with sacral nerve stimulators in children; it works in refractory constipation and combined urinary/stool issues, but pediatric patient heterogeneity makes outcome prediction difficult.↗
▶Ep 2 · 37:05
clinicalSacral nerve stimulator was initially designed for urinary incontinence in postpartum women; during testing it was found to also help fecal constipation and incontinence, leading to later FDA approval for fecal symptoms.↗
▶Ep 2 · 37:30
clinicalSacral nerve stimulator in adults is used in patients with normal anatomy/development, but in pediatrics it is used in patients with prior pelvic operations, malformations, or disease processes, making study difficult because every patient is different.↗
▶Ep 2 · 38:58
clinicalSacral nerve stimulator has a temporary trial stage (1–2 weeks); if it doesn't work, the device is removed without implanting permanent battery.↗
▶Ep 2 · 40:34
clinicalConstipation or fecal impaction puts pressure on urinary tract, causing urinary infections, improper emptying, urgency, and other symptoms; treating stool issues is often first step before urologic investigation.↗
▶Ep 2 · 41:58
guidelineAll anorectal malformation patients should be followed by urology long-term; more complex malformations have more associated urinary tract malformations, but even minor malformations require periodic follow-up to ensure kidney function and proper urinary tract emptying.↗
▶Ep 2 · 42:43
clinicalUrinary tract problems in anorectal malformation patients can cause diminished health with late-appearing signs/symptoms; regular urology checkups (exam, renal ultrasound, possibly further testing) are important even without visible problems.↗
▶Ep 2 · 43:37
clinicalCincinnati Children's has weekly meeting with colorectal, urology, and gynecology teams reviewing all patients to ensure comprehensive (not just focused) evaluation.↗
▶Ep 2 · 44:12
clinicalLong-term MiraLax literature is scant; many patients do fine on it. A class action lawsuit against MiraLax occurred 1–2 years ago but has not been heard about recently.↗
▶Ep 2 · 45:00
clinicalA couple of studies suggested MiraLax may have less-than-ideal neurodevelopmental outcomes, but these have not been reproduced; it is something to be thoughtful about when prescribing.↗
▶Ep 2 · 46:05
clinicalFleet phosphate enema causes the most irritation with long-term use, making colon look like a 'lead pipe' (very stiff, lacking mobility and pliability); it is used intermittently as rescue enema, not for long-term maintenance.↗
▶Ep 2 · 47:14
clinicalSolesta is not FDA approved for children; it is used in patients with patulous (open) anus as a filler to close the anal canal and help with fecal incontinence.↗
▶Ep 2 · 48:13
clinicalSolesta can be used in any age patient; Cincinnati has used it in patients 3–4 years old and older, with mixed results but little side effect (small infection risk).↗
▶Ep 2 · 48:31
clinicalSolesta or sacral nerve stimulator may help patients transition from enemas to laxatives when prior attempts have failed, depending on individual anal canal anatomy and situation.↗
▶Ep 2 · 49:17
clinicalSacral nerve stimulator results in spina bifida patients are mixed; some respond positively, others do not. Literature and research exist on this.↗
▶Ep 2 · 49:36
clinicalSacral nerve stimulator in spina bifida requires adequate sacrum anatomy and presence of third sacral foramina (opening where third sacral nerve exits sacrum to innervate pelvis).↗
▶Ep 2 · 51:56
clinicalBotox is a muscle relaxant that relaxes the anal sphincter; it is used in Hirschsprung's disease patients with high anal resting pressures.↗
▶Ep 2 · 52:19
clinicalSolesta is hyaluronic acid polymer, a gel filling agent that closes a wide-open (patulous) anal canal; it acts very differently from Botox.↗
▶Ep 2 · 55:06
clinicalHirschsprung's patients can potty train around the same age as other children; some will have harder time with continence depending on post-surgical anal canal anatomy and development.↗
▶Ep 2 · 55:30
clinicalHirschsprung's evaluation requires contrast enema to assess surgical anatomy of anal canal and entire colon, plus repeat biopsy because initial frozen section may show adequate nerve cells but later those cells may be less abundant (transition zone).↗
▶Ep 2 · 56:58
clinicalAnorectal malformation is abnormal development of where colon/rectum ends up (where it 'lands' in development); there is a wide spectrum in males and females.↗
▶Ep 2 · 57:13
clinicalIn anorectal malformation, rectum can land outside anal canal/sphincter complex on skin just above that area, outside vagina in girls, anywhere along urinary tract in boys (with fistula causing stool to come out urinary tract), or connected to bladder in more complex cases.↗
▶Ep 2 · 57:53
clinicalAnorectal malformation diagnosis requires good physical exam and sometimes imaging.↗
Jason's statements about Anorectal Malformations & Cloacal Reconstruction10 statements
quoteYeah, so we usually do it at about five to six months.↗
▶Ep 19 · 1:45
clinicalDefinitive diagnostic workup for cloaca is usually performed at about 5-6 months of age↗
▶Ep 19 · 4:34
clinicalDuring cystoscopy it is important to look for cervices to determine if there is one or two vaginas and assess Müllerian development↗
▶Ep 19 · 4:50
quoteI think it's also important to look for cervices and see if you have one vagina, two vaginas. Do you have a cervix, which will sort of be the front door? Is there a uterus behind there?↗
▶Ep 19 · 5:00
clinicalSurgeons should look for ureteral orifices during cystoscopy because in complex malformations there can be anomalous ureteral attachments to the bladder or bladder neck that come in very low and could be treacherous↗
▶Ep 19 · 7:41
opinionEndoscopy performed by general pediatric surgeons has value in determining whether a cloaca is straightforward or complex, helping surgeons decide whether to refer to a specialized center↗
▶Ep 19 · 8:30
clinicalAs recently as 10-15 years ago, there was much more redoing of cloacas required because surgeons attempted repairs without adequate evaluation and realized the cases were more complicated than imagined↗
▶Ep 19 · 8:30
quoteI can tell you, as recently as 10 or 15 years ago, there was a lot more redoing of cloacas that was required because I think surgeons attempted a repair and then realized that it was much more complicated than they had imagined.↗
▶Ep 19 · 9:00
opinionLower confluence cloacas are elegant operations if the surgeon knows how to perform them, while higher confluence cases with vaginal replacements and ectopic ureters should be done at specialized centers↗
▶Ep 19 · 19:00
quoteThe definition is of urethral length because I think some people may consider that from the single orifice all the way to the bladder neck and I don't think that's what you're alluding to.↗
quoteYeah, so we usually do it at about five to six months.↗
▶Ep 31 · 1:45
clinicalDefinitive diagnostic workup for cloaca is usually performed at about 5-6 months of age↗
▶Ep 31 · 4:34
clinicalDuring cystoscopy it is important to look for cervices to determine if there is one or two vaginas and assess Müllerian development↗
▶Ep 31 · 4:50
quoteI think it's also important to look for cervices and see if you have one vagina, two vaginas. Do you have a cervix, which will sort of be the front door? Is there a uterus behind there?↗
▶Ep 31 · 5:00
clinicalSurgeons should look for ureteral orifices during cystoscopy because in complex malformations there can be anomalous ureteral attachments to the bladder or bladder neck that come in very low and could be treacherous↗
▶Ep 31 · 7:41
opinionEndoscopy performed by general pediatric surgeons has value in determining whether a cloaca is straightforward or complex, helping surgeons decide whether to refer to a specialized center↗
▶Ep 31 · 8:30
quoteI can tell you, as recently as 10 or 15 years ago, there was a lot more redoing of cloacas that was required because I think surgeons attempted a repair and then realized that it was much more complicated than they had imagined.↗
▶Ep 31 · 8:30
clinicalAs recently as 10-15 years ago, there was much more redoing of cloacas required because surgeons attempted repairs without adequate evaluation and realized the cases were more complicated than imagined↗
▶Ep 31 · 9:00
opinionLower confluence cloacas are elegant operations if the surgeon knows how to perform them, while higher confluence cases with vaginal replacements and ectopic ureters should be done at specialized centers↗
▶Ep 31 · 19:00
quoteThe definition is of urethral length because I think some people may consider that from the single orifice all the way to the bladder neck and I don't think that's what you're alluding to.↗
Jason's statements about Colorectal / ARM & Hirschsprung72 statements
Bowel Management Updates & Innovations with Live Q&A: April 2018
▶Ep 2 · 0:15
clinicalThe Colorectal Center patient population includes anorectal malformations (imperforate anus), Hirschsprung's disease, spina bifida, sacrococcygeal teratomas, trauma patients, and refractory constipation.↗
▶Ep 2 · 2:32
clinicalAppendicostomy (Malone/MAC/ACE procedure) involves bringing the appendix to the belly button with a valve mechanism to prevent reflux, allowing patients to independently administer flushes through the colon over 10–15 minutes, then sit on toilet for approximately 45 minutes to empty.↗
▶Ep 2 · 3:42
clinicalSacral nerve stimulator is not FDA approved for children under 18, but is performed off-label; it acts like a heart pacemaker, stimulating the third sacral nerve to improve bowel and urinary continence outcomes.↗
▶Ep 2 · 4:41
clinicalSacral nerve stimulator is a two-stage procedure (test phase and permanent phase) and has been performed at Cincinnati Children's for almost 3 years.↗
▶Ep 2 · 4:53
clinicalSolesta is an injection of hyaluronic acid polymer that fills space in the anal canal, used in patients with patulous (more open) anuses to help gain control.↗
▶Ep 2 · 7:27
clinicalPatients with deformed sacrums have lower likelihood of success with bowel management using medication alone (not on enema program).↗
▶Ep 2 · 7:50
clinicalMalone procedure requires prior success with enemas because it only changes the route of administration; surgical procedure should not be performed on patients unsuccessful with enema program.↗
▶Ep 2 · 8:17
clinicalMalone procedure is typically performed starting at age 5 years and older, most commonly between ages 5 and 10–12 years.↗
▶Ep 2 · 8:53
clinicalVomiting after enema can be caused by high-volume enema with irritant causing colon distention and discomfort.↗
▶Ep 2 · 10:59
guidelineSacral nerve stimulator indication per manufacturer requires 50% improvement on symptoms; patient must have tried non-surgical bowel management first.↗
▶Ep 2 · 11:17
clinicalSacral nerve stimulator requires normal sacrum; it is the least invasive option compared to Malone or Solesta when bowel management is working or needs improvement.↗
▶Ep 2 · 11:49
clinicalUsing hybrid OR with 3D imaging, sacral nerve stimulator can be placed in patients with very abnormal sacrums by identifying anatomy in real-time.↗
▶Ep 2 · 13:45
clinicalFor failed redo Hirschsprung's pull-through, redo surgery can almost always create a channel for stool evacuation via rectum/anus, even after 1–3 prior redos; very few patients end up with ileostomy if goal is rectal evacuation.↗
▶Ep 2 · 17:18
clinicalThe large intestine (colon) primarily absorbs water; in adults it can absorb up to 1 liter of water per day. When colon is removed, stool becomes softer/looser because less water is absorbed.↗
▶Ep 2 · 17:46
clinicalBowel management sometimes requires using Imodium to constipate/harden stool, then using enema to empty it—thickening stool first, then mechanically emptying.↗
▶Ep 2 · 19:12
opinionThere is no perfect surgery for constipation; if there were, all surgeons would recommend and perform it.↗
▶Ep 2 · 19:12
quoteThere is no perfect surgery for constipation. If we had it, we would all recommend it and do it.↗
▶Ep 2 · 19:20
clinicalTransanal colon/rectum resection is minimally invasive but carries risk of post-surgical incontinence in some patients.↗
▶Ep 2 · 19:56
clinicalColon resection for constipation may lessen treatment burden (e.g., reduce laxative dose from 10 to 5 squares of Ex-Lax) but this outcome is not guaranteed.↗
▶Ep 2 · 20:22
clinicalBefore colon resection, offer sacral nerve stimulator and perform manometry (colonic and anorectal) to identify non-functioning colon segments or outlet problems (sphincter dysfunction).↗
▶Ep 2 · 20:55
clinicalAnorectal manometry can reveal paradoxical sphincter function where child squeezes when trying to relax and vice versa; Botox works in younger children, physical therapy and biofeedback in older children for this.↗
▶Ep 2 · 22:31
clinicalColon resection is a big surgery requiring bowel anastomosis with associated risks; once colon is resected, it cannot be replaced. All other treatments (sacral nerve, enemas, laxatives) are reversible.↗
▶Ep 2 · 25:25
opinionSenna laxative is like insulin for a diabetic—a necessary medication to help move bowels in severe constipation; dose may change over time but patients are not 'addicted' to it.↗
▶Ep 2 · 25:29
quoteI sort of say it's like insulin for a diabetic. A diabetic needs insulin to lower their blood blood glucose levels. A patient who has severe constipation needs a laxative stimulant to help move their bowels.↗
▶Ep 2 · 26:48
clinicalStimulant laxatives (Senna, Dulcolax) make the colon squeeze/push stool forward and reduce water absorption time, resulting in softer stool—two mechanisms that benefit the patient population.↗
▶Ep 2 · 27:17
clinicalOnly two stimulant laxatives are available in the United States: Senna (Ex-Lax chocolate squares, tablet, liquid forms) and Dulcolax.↗
▶Ep 2 · 27:35
clinicalMiraLax is not a stimulant laxative; it prevents colon from absorbing water, keeping water in colon and making stool softer.↗
▶Ep 2 · 27:51
clinicalMiraLax is avoided in patients without full continence mechanisms because very loose stool is hard to control when nerves and muscle are partially impaired.↗
▶Ep 2 · 28:15
clinicalIn spinal cord patients (tethered cord, spina bifida), stool is often so formed that MiraLax-type agent is needed to soften stool along with motility agents.↗
▶Ep 2 · 30:09
clinicalBowel rest with colostomy is typically used in patients with anorectal malformation or Hirschsprung's pull-through complications (narrowing, stenosis, tightness) causing dilated colon/rectum upstream.↗
▶Ep 2 · 30:47
clinicalIf colostomy is not working due to constipation, consider ileostomy to totally exclude colon and divert stool before it reaches colon.↗
▶Ep 2 · 35:30
clinicalMichael Helmrath at Cincinnati Children's is growing colons in the lab from single cells, able to lengthen colon in mice and expanding to larger primates.↗
▶Ep 2 · 35:48
clinicalAnal sphincter tissue regeneration has been attempted by many researchers (using muscles from other areas, other sphincters from esophagus/stomach) but none have been proven to work; no successful procedure is currently offered.↗
▶Ep 2 · 37:00
clinicalCincinnati Children's has nearly 3 years of experience with sacral nerve stimulators in children; it works in refractory constipation and combined urinary/stool issues, but pediatric patient heterogeneity makes outcome prediction difficult.↗
▶Ep 2 · 37:05
clinicalSacral nerve stimulator was initially designed for urinary incontinence in postpartum women; during testing it was found to also help fecal constipation and incontinence, leading to later FDA approval for fecal symptoms.↗
▶Ep 2 · 37:30
clinicalSacral nerve stimulator in adults is used in patients with normal anatomy/development, but in pediatrics it is used in patients with prior pelvic operations, malformations, or disease processes, making study difficult because every patient is different.↗
▶Ep 2 · 38:58
clinicalSacral nerve stimulator has a temporary trial stage (1–2 weeks); if it doesn't work, the device is removed without implanting permanent battery.↗
▶Ep 2 · 40:34
clinicalConstipation or fecal impaction puts pressure on urinary tract, causing urinary infections, improper emptying, urgency, and other symptoms; treating stool issues is often first step before urologic investigation.↗
▶Ep 2 · 41:58
guidelineAll anorectal malformation patients should be followed by urology long-term; more complex malformations have more associated urinary tract malformations, but even minor malformations require periodic follow-up to ensure kidney function and proper urinary tract emptying.↗
▶Ep 2 · 42:43
clinicalUrinary tract problems in anorectal malformation patients can cause diminished health with late-appearing signs/symptoms; regular urology checkups (exam, renal ultrasound, possibly further testing) are important even without visible problems.↗
▶Ep 2 · 43:37
clinicalCincinnati Children's has weekly meeting with colorectal, urology, and gynecology teams reviewing all patients to ensure comprehensive (not just focused) evaluation.↗
▶Ep 2 · 44:12
clinicalLong-term MiraLax literature is scant; many patients do fine on it. A class action lawsuit against MiraLax occurred 1–2 years ago but has not been heard about recently.↗
▶Ep 2 · 45:00
clinicalA couple of studies suggested MiraLax may have less-than-ideal neurodevelopmental outcomes, but these have not been reproduced; it is something to be thoughtful about when prescribing.↗
▶Ep 2 · 46:05
clinicalFleet phosphate enema causes the most irritation with long-term use, making colon look like a 'lead pipe' (very stiff, lacking mobility and pliability); it is used intermittently as rescue enema, not for long-term maintenance.↗
▶Ep 2 · 47:14
clinicalSolesta is not FDA approved for children; it is used in patients with patulous (open) anus as a filler to close the anal canal and help with fecal incontinence.↗
▶Ep 2 · 48:13
clinicalSolesta can be used in any age patient; Cincinnati has used it in patients 3–4 years old and older, with mixed results but little side effect (small infection risk).↗
▶Ep 2 · 48:31
clinicalSolesta or sacral nerve stimulator may help patients transition from enemas to laxatives when prior attempts have failed, depending on individual anal canal anatomy and situation.↗
▶Ep 2 · 49:17
clinicalSacral nerve stimulator results in spina bifida patients are mixed; some respond positively, others do not. Literature and research exist on this.↗
▶Ep 2 · 49:36
clinicalSacral nerve stimulator in spina bifida requires adequate sacrum anatomy and presence of third sacral foramina (opening where third sacral nerve exits sacrum to innervate pelvis).↗
▶Ep 2 · 51:56
clinicalBotox is a muscle relaxant that relaxes the anal sphincter; it is used in Hirschsprung's disease patients with high anal resting pressures.↗
▶Ep 2 · 52:19
clinicalSolesta is hyaluronic acid polymer, a gel filling agent that closes a wide-open (patulous) anal canal; it acts very differently from Botox.↗
▶Ep 2 · 55:06
clinicalHirschsprung's patients can potty train around the same age as other children; some will have harder time with continence depending on post-surgical anal canal anatomy and development.↗
▶Ep 2 · 55:30
clinicalHirschsprung's evaluation requires contrast enema to assess surgical anatomy of anal canal and entire colon, plus repeat biopsy because initial frozen section may show adequate nerve cells but later those cells may be less abundant (transition zone).↗
▶Ep 2 · 56:58
clinicalAnorectal malformation is abnormal development of where colon/rectum ends up (where it 'lands' in development); there is a wide spectrum in males and females.↗
▶Ep 2 · 57:13
clinicalIn anorectal malformation, rectum can land outside anal canal/sphincter complex on skin just above that area, outside vagina in girls, anywhere along urinary tract in boys (with fistula causing stool to come out urinary tract), or connected to bladder in more complex cases.↗
▶Ep 2 · 57:53
clinicalAnorectal malformation diagnosis requires good physical exam and sometimes imaging.↗
Hirschsprung Disease: Update Course 2013
▶Ep 49 · 8:56
epidemiologicalFemale patients may have higher risk of long-segment disease, with 50-50 incidence of long-segment in girls↗
▶Ep 49 · 32:05
clinicalTransanal resection of massively dilated colon in older children causes enormous sphincter stretch and impairs postoperative continence; laparoscopic dissection to pelvic floor is preferred↗
▶Ep 49 · 33:42
clinicalColonoscopic biopsies can miss Hirschsprung disease; rectal biopsy is more reliable↗
▶Ep 49 · 33:42
quotethat patient was at a well-known institution in Ohio. And had, was seen by GI and had multiple biopsies that were normal. Those were done by colonoscopy↗
▶Ep 49 · 34:04
quotejust caution to watch out what type of biopsy was performed and how Hirschprung's was ruled out↗
The Colorectal Quiz Episode 9: Motility Disorders Part 2
▶Ep 93 · 8:02
clinicalMany patients undergoing colonic manometry have normal results, meaning they have slow transit but normal HAPCs.↗
Colorectal Quiz Episode 18: Cloaca Part 2
▶Ep 227 · 1:45
clinicalDefinitive diagnostic workup for cloaca is usually performed at about 5-6 months of age↗
▶Ep 227 · 1:45
quoteYeah, so we usually do it at about five to six months.↗
▶Ep 227 · 4:34
clinicalDuring cystoscopy it is important to look for cervices to determine if there is one or two vaginas and assess Müllerian development↗
▶Ep 227 · 4:50
quoteI think it's also important to look for cervices and see if you have one vagina, two vaginas. Do you have a cervix, which will sort of be the front door? Is there a uterus behind there?↗
▶Ep 227 · 5:00
clinicalSurgeons should look for ureteral orifices during cystoscopy because in complex malformations there can be anomalous ureteral attachments to the bladder or bladder neck that come in very low and could be treacherous↗
▶Ep 227 · 7:41
opinionEndoscopy performed by general pediatric surgeons has value in determining whether a cloaca is straightforward or complex, helping surgeons decide whether to refer to a specialized center↗
▶Ep 227 · 8:30
quoteI can tell you, as recently as 10 or 15 years ago, there was a lot more redoing of cloacas that was required because I think surgeons attempted a repair and then realized that it was much more complicated than they had imagined.↗
▶Ep 227 · 8:30
clinicalAs recently as 10-15 years ago, there was much more redoing of cloacas required because surgeons attempted repairs without adequate evaluation and realized the cases were more complicated than imagined↗
▶Ep 227 · 9:00
opinionLower confluence cloacas are elegant operations if the surgeon knows how to perform them, while higher confluence cases with vaginal replacements and ectopic ureters should be done at specialized centers↗
▶Ep 227 · 19:00
quoteThe definition is of urethral length because I think some people may consider that from the single orifice all the way to the bladder neck and I don't think that's what you're alluding to.↗
Jason's statements about Enterocolitis5 statements
epidemiologicalFemale patients may have higher risk of long-segment disease, with 50-50 incidence of long-segment in girls↗
▶Ep 11 · 32:05
clinicalTransanal resection of massively dilated colon in older children causes enormous sphincter stretch and impairs postoperative continence; laparoscopic dissection to pelvic floor is preferred↗
▶Ep 11 · 33:42
quotethat patient was at a well-known institution in Ohio. And had, was seen by GI and had multiple biopsies that were normal. Those were done by colonoscopy↗
▶Ep 11 · 33:42
clinicalColonoscopic biopsies can miss Hirschsprung disease; rectal biopsy is more reliable↗
▶Ep 11 · 34:04
quotejust caution to watch out what type of biopsy was performed and how Hirschprung's was ruled out↗
Jason's statements about Enterocolitis5 statements
epidemiologicalFemale patients may have higher risk of long-segment disease, with 50-50 incidence of long-segment in girls↗
▶Ep 11 · 32:05
clinicalTransanal resection of massively dilated colon in older children causes enormous sphincter stretch and impairs postoperative continence; laparoscopic dissection to pelvic floor is preferred↗
▶Ep 11 · 33:42
quotethat patient was at a well-known institution in Ohio. And had, was seen by GI and had multiple biopsies that were normal. Those were done by colonoscopy↗
▶Ep 11 · 33:42
clinicalColonoscopic biopsies can miss Hirschsprung disease; rectal biopsy is more reliable↗
▶Ep 11 · 34:04
quotejust caution to watch out what type of biopsy was performed and how Hirschprung's was ruled out↗
Jason's statements about Hirschsprung disease62 statements
Bowel Management Updates & Innovations with Live Q&A: April 2018
▶Ep 1 · 0:15
clinicalThe Colorectal Center patient population includes anorectal malformations (imperforate anus), Hirschsprung's disease, spina bifida, sacrococcygeal teratomas, trauma patients, and refractory constipation.↗
▶Ep 1 · 2:32
clinicalAppendicostomy (Malone/MAC/ACE procedure) involves bringing the appendix to the belly button with a valve mechanism to prevent reflux, allowing patients to independently administer flushes through the colon over 10–15 minutes, then sit on toilet for approximately 45 minutes to empty.↗
▶Ep 1 · 3:42
clinicalSacral nerve stimulator is not FDA approved for children under 18, but is performed off-label; it acts like a heart pacemaker, stimulating the third sacral nerve to improve bowel and urinary continence outcomes.↗
▶Ep 1 · 4:41
clinicalSacral nerve stimulator is a two-stage procedure (test phase and permanent phase) and has been performed at Cincinnati Children's for almost 3 years.↗
▶Ep 1 · 4:53
clinicalSolesta is an injection of hyaluronic acid polymer that fills space in the anal canal, used in patients with patulous (more open) anuses to help gain control.↗
▶Ep 1 · 7:27
clinicalPatients with deformed sacrums have lower likelihood of success with bowel management using medication alone (not on enema program).↗
▶Ep 1 · 7:50
clinicalMalone procedure requires prior success with enemas because it only changes the route of administration; surgical procedure should not be performed on patients unsuccessful with enema program.↗
▶Ep 1 · 8:17
clinicalMalone procedure is typically performed starting at age 5 years and older, most commonly between ages 5 and 10–12 years.↗
▶Ep 1 · 8:53
clinicalVomiting after enema can be caused by high-volume enema with irritant causing colon distention and discomfort.↗
▶Ep 1 · 10:59
guidelineSacral nerve stimulator indication per manufacturer requires 50% improvement on symptoms; patient must have tried non-surgical bowel management first.↗
▶Ep 1 · 11:17
clinicalSacral nerve stimulator requires normal sacrum; it is the least invasive option compared to Malone or Solesta when bowel management is working or needs improvement.↗
▶Ep 1 · 11:49
clinicalUsing hybrid OR with 3D imaging, sacral nerve stimulator can be placed in patients with very abnormal sacrums by identifying anatomy in real-time.↗
▶Ep 1 · 13:45
clinicalFor failed redo Hirschsprung's pull-through, redo surgery can almost always create a channel for stool evacuation via rectum/anus, even after 1–3 prior redos; very few patients end up with ileostomy if goal is rectal evacuation.↗
▶Ep 1 · 17:18
clinicalThe large intestine (colon) primarily absorbs water; in adults it can absorb up to 1 liter of water per day. When colon is removed, stool becomes softer/looser because less water is absorbed.↗
▶Ep 1 · 17:46
clinicalBowel management sometimes requires using Imodium to constipate/harden stool, then using enema to empty it—thickening stool first, then mechanically emptying.↗
▶Ep 1 · 19:12
opinionThere is no perfect surgery for constipation; if there were, all surgeons would recommend and perform it.↗
▶Ep 1 · 19:12
quoteThere is no perfect surgery for constipation. If we had it, we would all recommend it and do it.↗
▶Ep 1 · 19:20
clinicalTransanal colon/rectum resection is minimally invasive but carries risk of post-surgical incontinence in some patients.↗
▶Ep 1 · 19:56
clinicalColon resection for constipation may lessen treatment burden (e.g., reduce laxative dose from 10 to 5 squares of Ex-Lax) but this outcome is not guaranteed.↗
▶Ep 1 · 20:22
clinicalBefore colon resection, offer sacral nerve stimulator and perform manometry (colonic and anorectal) to identify non-functioning colon segments or outlet problems (sphincter dysfunction).↗
▶Ep 1 · 20:55
clinicalAnorectal manometry can reveal paradoxical sphincter function where child squeezes when trying to relax and vice versa; Botox works in younger children, physical therapy and biofeedback in older children for this.↗
▶Ep 1 · 22:31
clinicalColon resection is a big surgery requiring bowel anastomosis with associated risks; once colon is resected, it cannot be replaced. All other treatments (sacral nerve, enemas, laxatives) are reversible.↗
▶Ep 1 · 25:25
opinionSenna laxative is like insulin for a diabetic—a necessary medication to help move bowels in severe constipation; dose may change over time but patients are not 'addicted' to it.↗
▶Ep 1 · 25:29
quoteI sort of say it's like insulin for a diabetic. A diabetic needs insulin to lower their blood blood glucose levels. A patient who has severe constipation needs a laxative stimulant to help move their bowels.↗
▶Ep 1 · 26:48
clinicalStimulant laxatives (Senna, Dulcolax) make the colon squeeze/push stool forward and reduce water absorption time, resulting in softer stool—two mechanisms that benefit the patient population.↗
▶Ep 1 · 27:17
clinicalOnly two stimulant laxatives are available in the United States: Senna (Ex-Lax chocolate squares, tablet, liquid forms) and Dulcolax.↗
▶Ep 1 · 27:35
clinicalMiraLax is not a stimulant laxative; it prevents colon from absorbing water, keeping water in colon and making stool softer.↗
▶Ep 1 · 27:51
clinicalMiraLax is avoided in patients without full continence mechanisms because very loose stool is hard to control when nerves and muscle are partially impaired.↗
▶Ep 1 · 28:15
clinicalIn spinal cord patients (tethered cord, spina bifida), stool is often so formed that MiraLax-type agent is needed to soften stool along with motility agents.↗
▶Ep 1 · 30:09
clinicalBowel rest with colostomy is typically used in patients with anorectal malformation or Hirschsprung's pull-through complications (narrowing, stenosis, tightness) causing dilated colon/rectum upstream.↗
▶Ep 1 · 30:47
clinicalIf colostomy is not working due to constipation, consider ileostomy to totally exclude colon and divert stool before it reaches colon.↗
▶Ep 1 · 35:30
clinicalMichael Helmrath at Cincinnati Children's is growing colons in the lab from single cells, able to lengthen colon in mice and expanding to larger primates.↗
▶Ep 1 · 35:48
clinicalAnal sphincter tissue regeneration has been attempted by many researchers (using muscles from other areas, other sphincters from esophagus/stomach) but none have been proven to work; no successful procedure is currently offered.↗
▶Ep 1 · 37:00
clinicalCincinnati Children's has nearly 3 years of experience with sacral nerve stimulators in children; it works in refractory constipation and combined urinary/stool issues, but pediatric patient heterogeneity makes outcome prediction difficult.↗
▶Ep 1 · 37:05
clinicalSacral nerve stimulator was initially designed for urinary incontinence in postpartum women; during testing it was found to also help fecal constipation and incontinence, leading to later FDA approval for fecal symptoms.↗
▶Ep 1 · 37:30
clinicalSacral nerve stimulator in adults is used in patients with normal anatomy/development, but in pediatrics it is used in patients with prior pelvic operations, malformations, or disease processes, making study difficult because every patient is different.↗
▶Ep 1 · 38:58
clinicalSacral nerve stimulator has a temporary trial stage (1–2 weeks); if it doesn't work, the device is removed without implanting permanent battery.↗
▶Ep 1 · 40:34
clinicalConstipation or fecal impaction puts pressure on urinary tract, causing urinary infections, improper emptying, urgency, and other symptoms; treating stool issues is often first step before urologic investigation.↗
▶Ep 1 · 41:58
guidelineAll anorectal malformation patients should be followed by urology long-term; more complex malformations have more associated urinary tract malformations, but even minor malformations require periodic follow-up to ensure kidney function and proper urinary tract emptying.↗
▶Ep 1 · 42:43
clinicalUrinary tract problems in anorectal malformation patients can cause diminished health with late-appearing signs/symptoms; regular urology checkups (exam, renal ultrasound, possibly further testing) are important even without visible problems.↗
▶Ep 1 · 43:37
clinicalCincinnati Children's has weekly meeting with colorectal, urology, and gynecology teams reviewing all patients to ensure comprehensive (not just focused) evaluation.↗
▶Ep 1 · 44:12
clinicalLong-term MiraLax literature is scant; many patients do fine on it. A class action lawsuit against MiraLax occurred 1–2 years ago but has not been heard about recently.↗
▶Ep 1 · 45:00
clinicalA couple of studies suggested MiraLax may have less-than-ideal neurodevelopmental outcomes, but these have not been reproduced; it is something to be thoughtful about when prescribing.↗
▶Ep 1 · 46:05
clinicalFleet phosphate enema causes the most irritation with long-term use, making colon look like a 'lead pipe' (very stiff, lacking mobility and pliability); it is used intermittently as rescue enema, not for long-term maintenance.↗
▶Ep 1 · 47:14
clinicalSolesta is not FDA approved for children; it is used in patients with patulous (open) anus as a filler to close the anal canal and help with fecal incontinence.↗
▶Ep 1 · 48:13
clinicalSolesta can be used in any age patient; Cincinnati has used it in patients 3–4 years old and older, with mixed results but little side effect (small infection risk).↗
▶Ep 1 · 48:31
clinicalSolesta or sacral nerve stimulator may help patients transition from enemas to laxatives when prior attempts have failed, depending on individual anal canal anatomy and situation.↗
▶Ep 1 · 49:17
clinicalSacral nerve stimulator results in spina bifida patients are mixed; some respond positively, others do not. Literature and research exist on this.↗
▶Ep 1 · 49:36
clinicalSacral nerve stimulator in spina bifida requires adequate sacrum anatomy and presence of third sacral foramina (opening where third sacral nerve exits sacrum to innervate pelvis).↗
▶Ep 1 · 51:56
clinicalBotox is a muscle relaxant that relaxes the anal sphincter; it is used in Hirschsprung's disease patients with high anal resting pressures.↗
▶Ep 1 · 52:19
clinicalSolesta is hyaluronic acid polymer, a gel filling agent that closes a wide-open (patulous) anal canal; it acts very differently from Botox.↗
▶Ep 1 · 55:06
clinicalHirschsprung's patients can potty train around the same age as other children; some will have harder time with continence depending on post-surgical anal canal anatomy and development.↗
▶Ep 1 · 55:30
clinicalHirschsprung's evaluation requires contrast enema to assess surgical anatomy of anal canal and entire colon, plus repeat biopsy because initial frozen section may show adequate nerve cells but later those cells may be less abundant (transition zone).↗
▶Ep 1 · 56:58
clinicalAnorectal malformation is abnormal development of where colon/rectum ends up (where it 'lands' in development); there is a wide spectrum in males and females.↗
▶Ep 1 · 57:13
clinicalIn anorectal malformation, rectum can land outside anal canal/sphincter complex on skin just above that area, outside vagina in girls, anywhere along urinary tract in boys (with fistula causing stool to come out urinary tract), or connected to bladder in more complex cases.↗
▶Ep 1 · 57:53
clinicalAnorectal malformation diagnosis requires good physical exam and sometimes imaging.↗
Hirschsprung Disease: Update Course 2013
▶Ep 24 · 8:56
epidemiologicalFemale patients may have higher risk of long-segment disease, with 50-50 incidence of long-segment in girls↗
▶Ep 24 · 32:05
clinicalTransanal resection of massively dilated colon in older children causes enormous sphincter stretch and impairs postoperative continence; laparoscopic dissection to pelvic floor is preferred↗
▶Ep 24 · 33:42
quotethat patient was at a well-known institution in Ohio. And had, was seen by GI and had multiple biopsies that were normal. Those were done by colonoscopy↗
▶Ep 24 · 33:42
clinicalColonoscopic biopsies can miss Hirschsprung disease; rectal biopsy is more reliable↗
▶Ep 24 · 34:04
quotejust caution to watch out what type of biopsy was performed and how Hirschprung's was ruled out↗
The Colorectal Quiz Episode 9: Motility Disorders Part 2
▶Ep 41 · 8:02
clinicalMany patients undergoing colonic manometry have normal results, meaning they have slow transit but normal HAPCs.↗
Jason's statements about Intestinal Rehab1 statement
