Andrea Bischoff

470 timestamped statements across 5 collections — auto-found in recorded discussions, each timestamp jumps to the exact moment.

Colorectal / ARM & Hirschsprung · guest expert

Featured diaries

▶ Ep 10 · 33:08
There's never a good timing to give the bad news, but at least they have time to digest and then during the birth of the child. They can actually enjoy the birth of the child and know that 24 hours from now that baby will go to a surgery that might represent being in a different hospital from the mother so the family has time to plan for that.
quote · Anorectal Malformation
▶ Ep 1 · 33:08
There's never a good timing to give the bad news, but at least they have time to digest and then during the birth of the child. They can actually enjoy the birth of the child and know that 24 hours from now that baby will go to a surgery that might represent being in a different hospital from the mother so the family has time to plan for that.
quote · Cloaca
▶ Ep 17 · 33:08
There's never a good timing to give the bad news, but at least they have time to digest and then during the birth of the child. They can actually enjoy the birth of the child and know that 24 hours from now that baby will go to a surgery that might represent being in a different hospital from the mother so the family has time to plan for that.
quote · Colorectal / ARM & Hirschsprung
▶ Ep 17 · 33:08
There's never a good timing to give the bad news, but at least they have time to digest and then during the birth of the child. They can actually enjoy the birth of the child and know that 24 hours from now that baby will go to a surgery that might represent being in a different hospital from the mother so the family has time to plan for that.
quote · Colorectal / ARM & Hirschsprung
▶ Ep 14 · 1:52
Patients with anorectal malformation with good prognosis for bowel control, they will have a well-formed buttocks with a good midline groove and a good anal dimple. Patients with malformations with bad prognosis for bowel control will have a flat bottom and not clear delineation of the anal dimple.
quote · Anorectal Malformation
▶ Ep 19 · 1:16:10
You should not do an ileoanal until the patient is toilet trained for urine because the patient needs to know how to go to the bathroom and evacuate in the toilet, otherwise you will have the worst unmanageable diaper rash that you've ever seen.
quote · Hirschsprung disease

Nothing matches these filters — clear the search or widen the filters.

Anorectal Malformation 148 entries

Cloaca - Prental Imaging & Diagnosis - Counseling

▶ Ep 10 · 32:46
opinion Advantages of prenatal diagnosis include transferring the patient to a specialized center for proper colostomy and hydrocolpos drainage, and preparing the mother so she has time to digest the news and can enjoy the birth knowing the baby will go to surgery 24 hours later ↗
▶ Ep 10 · 33:08
quote There's never a good timing to give the bad news, but at least they have time to digest and then during the birth of the child. They can actually enjoy the birth of the child and know that 24 hours from now that baby will go to a surgery that might represent being in a different hospital from the mother so the family has time to plan for that. ↗
▶ Ep 10 · 33:30
clinical For proper prenatal counseling of cloaca patients, surgeons want to know the common channel length and sacrum status, but this information is still limited in prenatal imaging ↗

Anorectal Malformations with Dr. Andrea Bischoff

▶ Ep 14 · 1:52
quote Patients with anorectal malformation with good prognosis for bowel control, they will have a well-formed buttocks with a good midline groove and a good anal dimple. Patients with malformations with bad prognosis for bowel control will have a flat bottom and not clear delineation of the anal dimple. ↗
▶ Ep 14 · 1:52
clinical Patients with anorectal malformation with good prognosis for bowel control will have well-formed buttocks with a good midline groove and a good anal dimple, while patients with bad prognosis will have a flat bottom and no clear delineation of the anal dimple. ↗
▶ Ep 14 · 2:31
clinical In a newborn baby, a normal caliber anus should accommodate a number 12 Hegar dilator. ↗
▶ Ep 14 · 3:16
quote We don't even say anterior because we don't like the term anterior anus because it goes with what's the definition of an anus. An anus has to have a normal caliber. ↗
▶ Ep 14 · 3:42
clinical In a rectal perineal fistula, the sphincter mechanism is in a horseshoe shape with posterior and lateral portions having sphincter but the anterior portion lacking sphincter. ↗
▶ Ep 14 · 5:01
epidemiological 8% of patients with anorectal malformation will have esophageal atresia. ↗
▶ Ep 14 · 5:27
epidemiological 30% of patients with anorectal malformation will have cardiac anomalies, but in only 10% of them are these anomalies hemodynamically significant. ↗
▶ Ep 14 · 5:54
epidemiological 50% of patients with anorectal malformation have associated urological defects. ↗
▶ Ep 14 · 6:02
epidemiological 25% of patients with anorectal malformation have tethered cord. ↗
▶ Ep 14 · 6:31
clinical If no fistula is identified on initial examination, the patient should be re-examined after 24 hours because it takes time for air to travel distally and for meconium to pass through a tiny fistula. ↗
▶ Ep 14 · 7:04
clinical Cross-table lateral film with pelvis elevated should never be done before 24 hours of life because it will give the false impression of a high malformation due to muscle tone. ↗
▶ Ep 14 · 8:22
clinical Spinal ultrasound is adequate to detect tethered cord in babies less than 3 months of age; after 3 months, MRI is needed due to ossification. ↗
▶ Ep 14 · 8:47
clinical Tethered cord has more influence on the urinary tract rather than the gastrointestinal tract in terms of prognosis. ↗
▶ Ep 14 · 9:53
clinical Presacral masses are most commonly found in malformations with good prognosis such as rectal perineal fistula, rectal vestibular fistula, and rectal atresia, but when present, the prognosis changes. ↗
▶ Ep 14 · 11:55
clinical The advantage of primary newborn repair is that bowel preparation is not required since meconium is considered sterile. ↗
▶ Ep 14 · 12:20
quote It is actually better to open a colostomy and have a perfect operation than to do a primary repair, have a complication such as the hissing retraction that requires a re-operation. ↗
▶ Ep 14 · 12:20
opinion It is better to open a colostomy and have a perfect operation than to do a primary repair and have a complication such as dehiscence retraction that requires re-operation. ↗
▶ Ep 14 · 12:33
clinical Patients with anorectal malformation have one chance to have the right operation; secondary operations or re-operations usually change the prognosis for bowel control. ↗
▶ Ep 14 · 12:33
quote We like to say that patients with an anorectal malformation have one chance to have the right operation. Secondary operations or re-operations usually change the prognosis for bowel control in these patients. ↗
▶ Ep 14 · 13:30
clinical An 8 French feeding tube should be used to catheterize a suspected vestibular fistula if it cannot be visualized. ↗
▶ Ep 14 · 13:36
epidemiological Vestibular fistula is the most common type of anorectal anomaly in females. ↗
▶ Ep 14 · 17:58
clinical The ideal colostomy should be totally diverting, located in the descending colon to leave enough distal bowel for pull-through and avoid prolapse. ↗
▶ Ep 14 · 18:23
clinical The proximal stoma should be located in the center of a triangle formed by the left rib, umbilicus, and iliac crest to ensure it is surrounded by normal skin for stoma bag application. ↗
▶ Ep 14 · 18:59
clinical During colostomy creation, all distal bowel should be irrigated with plenty of normal saline to remove all meconium. ↗
▶ Ep 14 · 19:55
clinical For high pressure distal colostogram, a number 8 French Foley catheter is normally used in the mucous fistula. ↗
▶ Ep 14 · 20:50
clinical During distal colostogram, the patient should be turned to perfect lateral position with knees at 90 degrees and one femur exactly in front of the other, and the radiologist must show all reference points including mucous fistula, sacrum, and anal marker. ↗
▶ Ep 14 · 22:45
clinical In rectal urethral bulbar fistula, the most important portion of the operation is separation between the rectum and the long common wall with the urethra, with minimal rectal mobilization needed. ↗
▶ Ep 14 · 23:11
clinical In rectal urethral prostatic fistula, the common wall with urethra is shorter than bulbar fistula but more rectal dissection is needed to gain length. ↗
▶ Ep 14 · 23:45
clinical In rectal bladder neck fistula, the rectum joins the urinary tract in a T fashion and the challenge is gaining enough length to reach the perineum while preserving good blood supply through selective ligation of mesenteric vessels. ↗
▶ Ep 14 · 24:54
quote If I can make a plea to pediatric surgeons, I would ask them to disagree strongly when the neonatologist labels a cloaca patient as a disorder of sexual differentiation. ↗
▶ Ep 14 · 25:23
clinical Cloaca patients have never been seen with disorder of sexual differentiation; they are all females with normal ovaries. ↗
▶ Ep 14 · 25:23
quote If the patient has a single orifice, you palpate the pseudophalo and it is skin. There's no corpora. This is a cloaca, and we have never seen a cloaca with disorder of sexual differentiation. They are all females. ↗
▶ Ep 14 · 26:56
clinical Cloaca with common channel less than 3 centimeters can be repaired posterior sagittally with total urogenital mobilization. ↗
▶ Ep 14 · 27:43
quote We actually believe that parents should have this information about how the future is going to be in terms of prognosis for bowel control as early as possible in life to correctly adjust their expectations. ↗
▶ Ep 14 · 28:08
clinical Rectal perineal fistula patients with normal sacrum and no tethered cord have 100% chance of bowel control. ↗
▶ Ep 14 · 28:18
clinical Malformations with better prognosis for bowel control will suffer from more constipation. ↗
▶ Ep 14 · 28:40
clinical Rectal vestibular fistula patients with normal sacrum and no tethered cord have 95% chance of bowel control. ↗
▶ Ep 14 · 28:57
clinical Rectal urethral bulbar fistula has 85% chance of bowel control. ↗
▶ Ep 14 · 29:02
clinical Anorectal malformation without fistula has 80% chance of bowel control. ↗
▶ Ep 14 · 29:08
clinical Rectal urethral prostatic fistula has 60% chance of bowel control. ↗
▶ Ep 14 · 29:14
clinical Rectal bladder neck fistula has 20% chance of bowel control. ↗
▶ Ep 14 · 29:24
clinical Cloaca with common channel less than 3 centimeters and normal sacrum has generally about 70% chance of bowel control. ↗
▶ Ep 14 · 29:57
opinion The only indication to keep a colostomy is incapacity to form solid stool; most patients with anorectal malformation have normal colon and can form solid stool. ↗
▶ Ep 14 · 29:57
quote We think that the only indication to keep a colostomy would be incapacity to form solid stool. ↗
▶ Ep 14 · 30:20
opinion Patients prefer the quality of life of a pull-through with bowel management rather than with a colostomy. ↗
▶ Ep 14 · 30:43
quote We believe that all children with anorectal malformations, they should be out of diapers at the same age that other children are normally out of diapers. Here in the United States, it's usually at 3 years of age. ↗
▶ Ep 14 · 30:43
guideline All children with anorectal malformations should be out of diapers at the same age that other children are normally out of diapers, which in the United States is usually at 3 years of age. ↗
▶ Ep 14 · 31:13
guideline After 3 years of age, if the child has not potty trained, formal bowel management should start: enemas for children with bad prognosis, enemas on temporary basis for borderline bowel control, and laxatives for children with bowel control and constipation. ↗
▶ Ep 14 · 31:41
clinical Bowel management for fecal incontinence consists of finding the enema that completely cleans the colon and allows the child to be clean in underwear for 24 hours. ↗
▶ Ep 14 · 32:55
clinical Determining which enema works for each child is a trial and error process that usually takes about one week. ↗
▶ Ep 14 · 33:17
clinical Contrast enema without bowel preparation can divide fecally incontinent patients into two groups: those with dilated colon and tendency for constipation, and those with non-dilated colon and tendency for diarrhea. ↗
▶ Ep 14 · 33:32
clinical For dilated hypomotile colon, a large and concentrated enema is needed to clean the colon, but after cleaning there is natural tendency not to produce bowel movements. ↗
▶ Ep 14 · 33:54
clinical For non-dilated hypermotile colon, a small enema (sometimes just normal saline) is needed, but the challenge is keeping the colon from moving between enemas, usually requiring constipating diet and medication to slow the colon. ↗
▶ Ep 14 · 34:33
clinical Enema base is normal saline 200 to 1000 mLs, with additives to increase concentration: liquid glycerin 10-40 mLs, Castile soap 9-27 mLs, or Fleet sodium phosphate (33 mLs for ages 2-4, 66 mLs for ages 7-10, 133 mLs for over 10 years). ↗
▶ Ep 14 · 38:33
clinical The enema should be done at the same time every day and the entire process (administration, holding, evacuation) should last one hour. ↗
▶ Ep 14 · 39:12
clinical Enema adjustments are made based on parent/patient report and abdominal radiograph; if real stool accidents occur and radiograph is not clean, concentration should be increased. ↗
▶ Ep 14 · 39:36
clinical Enema frequency is always once daily; concentration is adjusted rather than volume or frequency. ↗
▶ Ep 14 · 40:32
clinical The Malone procedure (appendicostomy) is not the treatment for fecal incontinence; the treatment is finding the enema that works. The appendicostomy provides independence. ↗
▶ Ep 14 · 40:32
quote The Malone procedure or appendicostomy is not the treatment for the fecal incontinence. The treatment for the fecal incontinence is finding the enema that works for the child. The appendicostomy will just give More independence. ↗
▶ Ep 14 · 40:50
clinical Appendicostomy is offered when the child wants to do the enema independently or for patients with borderline bowel control who remain enema-dependent after yearly trials off enemas. ↗
▶ Ep 14 · 42:19
clinical For constipation management, disimpaction is required before determining laxative dosage to avoid severe cramping. ↗
▶ Ep 14 · 42:35
clinical Disimpaction protocol consists of 3 enemas per day for 3 days; most children are disimpacted after day 3. ↗
▶ Ep 14 · 42:52
clinical If still impacted after 3 days of enemas, admit for nasogastric tube with GoLYTELY for 2 days plus enemas; disimpaction under anesthesia is extremely rare (approximately 4 cases in 8 years). ↗
▶ Ep 14 · 43:30
clinical Senna-based laxative is preferred, given at 6 PM to produce bowel movement the next day, with dosage range from 8.8 mg to 175 mg. ↗
▶ Ep 14 · 43:37
clinical Laxative dosage is adjusted daily based on bowel movement frequency, consistency, and abdominal radiograph showing stool burden; if no bowel movements in 24 hours, give enema and increase laxative dose. ↗

Error Traps and Culture of Safety in Anorectal Malformations

▶ Ep 30 · 0:31
clinical Five error traps have been identified in the management of anorectal malformations. ↗
▶ Ep 30 · 0:31
quote We have identified 5 error traps. ↗
▶ Ep 30 · 0:34
quote The first one is the creation of a colostomy 2 distal in the sigmoid column. Our recommendation is for a colostomy at the descending column. ↗
▶ Ep 30 · 0:34
clinical The first error trap is the creation of a colostomy too distal in the sigmoid colon. ↗
▶ Ep 30 · 0:40
guideline The recommendation is for a colostomy at the descending colon. ↗
▶ Ep 30 · 0:45
clinical The second error trap is having an inaccurate distal colostogram. ↗
▶ Ep 30 · 0:51
guideline A correctly done distal colostogram should show the site of the mucous fistula, the amount of bowel length available for the pull-through, the end of the rectum or the connection to the urinary tract, whenever possible the bladder and the urethra, the tip of the sacrum, and the anal marker. ↗
▶ Ep 30 · 1:16
clinical The third error trap is operating on a male patient without a Foley catheter and ignoring the hazards of the separation between the anterior rectal wall and the genitourinary tract. ↗
▶ Ep 30 · 1:32
clinical The fourth error trap is attempting to dilate a true rectal stricture. ↗
▶ Ep 30 · 1:39
guideline Anal dilations are not for real strictures. ↗
▶ Ep 30 · 1:39
quote Anal dilations are not for real strictures ↗
▶ Ep 30 · 1:39
clinical The fifth error trap is not offering long-term follow-up to these patients. ↗
▶ Ep 30 · 1:49
guideline Patients with anorectal malformations need long-term follow-up in colorectal, urology, and gynecology. ↗
▶ Ep 30 · 1:49
quote Those patients need long-term follow-up in colorectal, urology, and gynecology. ↗
▶ Ep 30 · 1:56
opinion By avoiding these common error traps, patients will receive better care. ↗

Anorectal Malformations with Dr. Andrea Bischoff

▶ Ep 32 · 1:52
clinical Patients with anorectal malformation with good prognosis for bowel control will have well-formed buttocks with a good midline groove and a good anal dimple, while patients with bad prognosis will have a flat bottom and no clear delineation of the anal dimple. ↗
▶ Ep 32 · 1:52
quote Patients with anorectal malformation with good prognosis for bowel control, they will have a well-formed buttocks with a good midline groove and a good anal dimple. Patients with malformations with bad prognosis for bowel control will have a flat bottom and not clear delineation of the anal dimple. ↗
▶ Ep 32 · 2:31
clinical In a newborn baby, a normal caliber anus should accommodate a number 12 Hegar dilator. ↗
▶ Ep 32 · 3:16
quote We don't even say anterior because we don't like the term anterior anus because it goes with what's the definition of an anus. An anus has to have a normal caliber. ↗
▶ Ep 32 · 3:42
clinical In a rectal perineal fistula, the sphincter mechanism is in a horseshoe shape with posterior and lateral portions having sphincter but the anterior portion lacking sphincter. ↗
▶ Ep 32 · 5:01
epidemiological 8% of patients with anorectal malformation will have esophageal atresia. ↗
▶ Ep 32 · 5:27
epidemiological 30% of patients with anorectal malformation will have cardiac anomalies, but in only 10% of them are these anomalies hemodynamically significant. ↗
▶ Ep 32 · 5:54
epidemiological 50% of patients with anorectal malformation have associated urological defects. ↗
▶ Ep 32 · 6:02
epidemiological 25% of patients with anorectal malformation have tethered cord. ↗
▶ Ep 32 · 6:31
clinical If no fistula is identified on initial examination, the patient should be re-examined after 24 hours because it takes time for air to travel distally and for meconium to pass through a tiny fistula. ↗
▶ Ep 32 · 7:04
clinical Cross-table lateral film with pelvis elevated should never be done before 24 hours of life because it will give the false impression of a high malformation due to muscle tone. ↗
▶ Ep 32 · 8:22
clinical Spinal ultrasound is adequate to detect tethered cord in babies less than 3 months of age; after 3 months, MRI is needed due to ossification. ↗
▶ Ep 32 · 8:47
clinical Tethered cord has more influence on the urinary tract rather than the gastrointestinal tract in terms of prognosis. ↗
▶ Ep 32 · 9:53
clinical Presacral masses are most commonly found in malformations with good prognosis such as rectal perineal fistula, rectal vestibular fistula, and rectal atresia, but when present, the prognosis changes. ↗
▶ Ep 32 · 11:55
clinical The advantage of primary newborn repair is that bowel preparation is not required since meconium is considered sterile. ↗
▶ Ep 32 · 12:20
opinion It is better to open a colostomy and have a perfect operation than to do a primary repair and have a complication such as dehiscence retraction that requires re-operation. ↗
▶ Ep 32 · 12:20
quote It is actually better to open a colostomy and have a perfect operation than to do a primary repair, have a complication such as the hissing retraction that requires a re-operation. ↗
▶ Ep 32 · 12:33
quote We like to say that patients with an anorectal malformation have one chance to have the right operation. Secondary operations or re-operations usually change the prognosis for bowel control in these patients. ↗
▶ Ep 32 · 12:33
clinical Patients with anorectal malformation have one chance to have the right operation; secondary operations or re-operations usually change the prognosis for bowel control. ↗
▶ Ep 32 · 13:30
clinical An 8 French feeding tube should be used to catheterize a suspected vestibular fistula if it cannot be visualized. ↗
▶ Ep 32 · 13:36
epidemiological Vestibular fistula is the most common type of anorectal anomaly in females. ↗
▶ Ep 32 · 17:58
clinical The ideal colostomy should be totally diverting, located in the descending colon to leave enough distal bowel for pull-through and avoid prolapse. ↗
▶ Ep 32 · 18:23
clinical The proximal stoma should be located in the center of a triangle formed by the left rib, umbilicus, and iliac crest to ensure it is surrounded by normal skin for stoma bag application. ↗
▶ Ep 32 · 18:59
clinical During colostomy creation, all distal bowel should be irrigated with plenty of normal saline to remove all meconium. ↗
▶ Ep 32 · 19:55
clinical For high pressure distal colostogram, a number 8 French Foley catheter is normally used in the mucous fistula. ↗
▶ Ep 32 · 20:50
clinical During distal colostogram, the patient should be turned to perfect lateral position with knees at 90 degrees and one femur exactly in front of the other, and the radiologist must show all reference points including mucous fistula, sacrum, and anal marker. ↗
▶ Ep 32 · 22:45
clinical In rectal urethral bulbar fistula, the most important portion of the operation is separation between the rectum and the long common wall with the urethra, with minimal rectal mobilization needed. ↗
▶ Ep 32 · 23:11
clinical In rectal urethral prostatic fistula, the common wall with urethra is shorter than bulbar fistula but more rectal dissection is needed to gain length. ↗
▶ Ep 32 · 23:45
clinical In rectal bladder neck fistula, the rectum joins the urinary tract in a T fashion and the challenge is gaining enough length to reach the perineum while preserving good blood supply through selective ligation of mesenteric vessels. ↗
▶ Ep 32 · 24:54
quote If I can make a plea to pediatric surgeons, I would ask them to disagree strongly when the neonatologist labels a cloaca patient as a disorder of sexual differentiation. ↗
▶ Ep 32 · 25:23
clinical Cloaca patients have never been seen with disorder of sexual differentiation; they are all females with normal ovaries. ↗
▶ Ep 32 · 25:23
quote If the patient has a single orifice, you palpate the pseudophalo and it is skin. There's no corpora. This is a cloaca, and we have never seen a cloaca with disorder of sexual differentiation. They are all females. ↗
▶ Ep 32 · 26:56
clinical Cloaca with common channel less than 3 centimeters can be repaired posterior sagittally with total urogenital mobilization. ↗
▶ Ep 32 · 27:43
quote We actually believe that parents should have this information about how the future is going to be in terms of prognosis for bowel control as early as possible in life to correctly adjust their expectations. ↗
▶ Ep 32 · 28:08
clinical Rectal perineal fistula patients with normal sacrum and no tethered cord have 100% chance of bowel control. ↗
▶ Ep 32 · 28:18
clinical Malformations with better prognosis for bowel control will suffer from more constipation. ↗
▶ Ep 32 · 28:40
clinical Rectal vestibular fistula patients with normal sacrum and no tethered cord have 95% chance of bowel control. ↗
▶ Ep 32 · 28:57
clinical Rectal urethral bulbar fistula has 85% chance of bowel control. ↗
▶ Ep 32 · 29:02
clinical Anorectal malformation without fistula has 80% chance of bowel control. ↗
▶ Ep 32 · 29:08
clinical Rectal urethral prostatic fistula has 60% chance of bowel control. ↗
▶ Ep 32 · 29:14
clinical Rectal bladder neck fistula has 20% chance of bowel control. ↗
▶ Ep 32 · 29:24
clinical Cloaca with common channel less than 3 centimeters and normal sacrum has generally about 70% chance of bowel control. ↗
▶ Ep 32 · 29:57
quote We think that the only indication to keep a colostomy would be incapacity to form solid stool. ↗
▶ Ep 32 · 29:57
opinion The only indication to keep a colostomy is incapacity to form solid stool; most patients with anorectal malformation have normal colon and can form solid stool. ↗
▶ Ep 32 · 30:20
opinion Patients prefer the quality of life of a pull-through with bowel management rather than with a colostomy. ↗
▶ Ep 32 · 30:43
quote We believe that all children with anorectal malformations, they should be out of diapers at the same age that other children are normally out of diapers. Here in the United States, it's usually at 3 years of age. ↗
▶ Ep 32 · 30:43
guideline All children with anorectal malformations should be out of diapers at the same age that other children are normally out of diapers, which in the United States is usually at 3 years of age. ↗
▶ Ep 32 · 31:13
guideline After 3 years of age, if the child has not potty trained, formal bowel management should start: enemas for children with bad prognosis, enemas on temporary basis for borderline bowel control, and laxatives for children with bowel control and constipation. ↗
▶ Ep 32 · 31:41
clinical Bowel management for fecal incontinence consists of finding the enema that completely cleans the colon and allows the child to be clean in underwear for 24 hours. ↗
▶ Ep 32 · 32:55
clinical Determining which enema works for each child is a trial and error process that usually takes about one week. ↗
▶ Ep 32 · 33:17
clinical Contrast enema without bowel preparation can divide fecally incontinent patients into two groups: those with dilated colon and tendency for constipation, and those with non-dilated colon and tendency for diarrhea. ↗
▶ Ep 32 · 33:32
clinical For dilated hypomotile colon, a large and concentrated enema is needed to clean the colon, but after cleaning there is natural tendency not to produce bowel movements. ↗
▶ Ep 32 · 33:54
clinical For non-dilated hypermotile colon, a small enema (sometimes just normal saline) is needed, but the challenge is keeping the colon from moving between enemas, usually requiring constipating diet and medication to slow the colon. ↗
▶ Ep 32 · 34:33
clinical Enema base is normal saline 200 to 1000 mLs, with additives to increase concentration: liquid glycerin 10-40 mLs, Castile soap 9-27 mLs, or Fleet sodium phosphate (33 mLs for ages 2-4, 66 mLs for ages 7-10, 133 mLs for over 10 years). ↗
▶ Ep 32 · 38:33
clinical The enema should be done at the same time every day and the entire process (administration, holding, evacuation) should last one hour. ↗
▶ Ep 32 · 39:12
clinical Enema adjustments are made based on parent/patient report and abdominal radiograph; if real stool accidents occur and radiograph is not clean, concentration should be increased. ↗
▶ Ep 32 · 39:36
clinical Enema frequency is always once daily; concentration is adjusted rather than volume or frequency. ↗
▶ Ep 32 · 40:32
quote The Malone procedure or appendicostomy is not the treatment for the fecal incontinence. The treatment for the fecal incontinence is finding the enema that works for the child. The appendicostomy will just give More independence. ↗
▶ Ep 32 · 40:32
clinical The Malone procedure (appendicostomy) is not the treatment for fecal incontinence; the treatment is finding the enema that works. The appendicostomy provides independence. ↗
▶ Ep 32 · 40:50
clinical Appendicostomy is offered when the child wants to do the enema independently or for patients with borderline bowel control who remain enema-dependent after yearly trials off enemas. ↗
▶ Ep 32 · 42:19
clinical For constipation management, disimpaction is required before determining laxative dosage to avoid severe cramping. ↗
▶ Ep 32 · 42:35
clinical Disimpaction protocol consists of 3 enemas per day for 3 days; most children are disimpacted after day 3. ↗
▶ Ep 32 · 42:52
clinical If still impacted after 3 days of enemas, admit for nasogastric tube with GoLYTELY for 2 days plus enemas; disimpaction under anesthesia is extremely rare (approximately 4 cases in 8 years). ↗
▶ Ep 32 · 43:30
clinical Senna-based laxative is preferred, given at 6 PM to produce bowel movement the next day, with dosage range from 8.8 mg to 175 mg. ↗
▶ Ep 32 · 43:37
clinical Laxative dosage is adjusted daily based on bowel movement frequency, consistency, and abdominal radiograph showing stool burden; if no bowel movements in 24 hours, give enema and increase laxative dose. ↗
Cloaca 3 entries

Cloaca - Prental Imaging & Diagnosis - Counseling

▶ Ep 1 · 32:46
opinion Advantages of prenatal diagnosis include transferring the patient to a specialized center for proper colostomy and hydrocolpos drainage, and preparing the mother so she has time to digest the news and can enjoy the birth knowing the baby will go to surgery 24 hours later ↗
▶ Ep 1 · 33:08
quote There's never a good timing to give the bad news, but at least they have time to digest and then during the birth of the child. They can actually enjoy the birth of the child and know that 24 hours from now that baby will go to a surgery that might represent being in a different hospital from the mother so the family has time to plan for that. ↗
▶ Ep 1 · 33:30
clinical For proper prenatal counseling of cloaca patients, surgeons want to know the common channel length and sacrum status, but this information is still limited in prenatal imaging ↗
Colorectal / ARM & Hirschsprung 243 entries

Cloaca - Prental Imaging & Diagnosis - Counseling

▶ Ep 17 · 32:46
opinion Advantages of prenatal diagnosis include transferring the patient to a specialized center for proper colostomy and hydrocolpos drainage, and preparing the mother so she has time to digest the news and can enjoy the birth knowing the baby will go to surgery 24 hours later ↗
▶ Ep 17 · 32:46
opinion Advantages of prenatal diagnosis include transferring the patient to a specialized center for proper colostomy and hydrocolpos drainage, and preparing the mother so she has time to digest the news and can enjoy the birth knowing the baby will go to surgery 24 hours later ↗
▶ Ep 17 · 33:08
quote There's never a good timing to give the bad news, but at least they have time to digest and then during the birth of the child. They can actually enjoy the birth of the child and know that 24 hours from now that baby will go to a surgery that might represent being in a different hospital from the mother so the family has time to plan for that. ↗
▶ Ep 17 · 33:08
quote There's never a good timing to give the bad news, but at least they have time to digest and then during the birth of the child. They can actually enjoy the birth of the child and know that 24 hours from now that baby will go to a surgery that might represent being in a different hospital from the mother so the family has time to plan for that. ↗
▶ Ep 17 · 33:30
clinical For proper prenatal counseling of cloaca patients, surgeons want to know the common channel length and sacrum status, but this information is still limited in prenatal imaging ↗
▶ Ep 17 · 33:30
clinical For proper prenatal counseling of cloaca patients, surgeons want to know the common channel length and sacrum status, but this information is still limited in prenatal imaging ↗

History of Hirschsprung Disease

▶ Ep 19 · 4:52
quote I think if we don't know history, we are always at risk to repeat the same mistakes. ↗
▶ Ep 19 · 4:52
quote I think if we don't know history, we are always at risk to repeat the same mistakes. ↗
▶ Ep 19 · 5:02
clinical Harald Hirschsprung presented a paper on constipation in newborns due to dilation and hypertrophy of the colon in 1886 at the Society of Pediatrics in Berlin. ↗
▶ Ep 19 · 5:02
host_summary Harald Hirschsprung presented a paper on constipation in newborns due to dilation and hypertrophy of the colon in 1886 at the Society of Pediatrics in Berlin. ↗
▶ Ep 19 · 5:54
host_summary Harald Hirschsprung was a pediatrician who developed the hydrostatic reduction of ileocolonic intussusception. ↗
▶ Ep 19 · 5:54
clinical Harald Hirschsprung was a pediatrician who developed the hydrostatic reduction of ileocolonic intussusception. ↗
▶ Ep 19 · 7:15
clinical Early theories attempting to explain Hirschsprung disease etiology were all wrong because everyone was obsessed that the dilated portion was the diseased one, trying to explain why the dilated portion was the cause rather than the consequence of the disease. ↗
▶ Ep 19 · 7:15
host_summary Early theories attempting to explain Hirschsprung disease etiology were all wrong because everyone was obsessed that the dilated portion was the diseased one, trying to explain why the dilated portion was the cause rather than the consequence of the disease. ↗
▶ Ep 19 · 8:52
host_summary William Osler, one of the four founding professors of Johns Hopkins and creator of the residency, proposed colostomy or rectal tube and irrigation as possible treatments for Hirschsprung disease. ↗
▶ Ep 19 · 8:52
clinical William Osler, one of the four founding professors of Johns Hopkins and creator of the residency, proposed colostomy or rectal tube and irrigation as possible treatments for Hirschsprung disease. ↗
▶ Ep 19 · 10:08
host_summary In 1946, Orvar Swenson finally recognized the finding of no ganglion cells in the narrow rectal sigmoid as the cause of Hirschsprung disease, whereas others before him had noted absent ganglion cells but thought it was an acquired condition. ↗
▶ Ep 19 · 10:08
clinical In 1946, Orvar Swenson finally recognized the finding of no ganglion cells in the narrow rectal sigmoid as the cause of Hirschsprung disease, whereas others before him had noted absent ganglion cells but thought it was an acquired condition. ↗
▶ Ep 19 · 12:38
clinical Resection of the distal non-dilated portion was not used as a treatment for Hirschsprung disease prior to 1946. ↗
▶ Ep 19 · 12:38
host_summary Resection of the distal non-dilated portion was not used as a treatment for Hirschsprung disease prior to 1946. ↗
▶ Ep 19 · 13:23
host_summary Barium enema technique became the standard diagnostic test for Hirschsprung disease in 1948, with Dr. Swenson involved in this publication. ↗
▶ Ep 19 · 13:23
clinical Barium enema technique became the standard diagnostic test for Hirschsprung disease in 1948, with Dr. Swenson involved in this publication. ↗
▶ Ep 19 · 15:33
host_summary Barry Shandling, who worked in Canada, proposed punch biopsies for newborns that required no closure nor anesthesia. ↗
▶ Ep 19 · 15:33
clinical Barry Shandling, who worked in Canada, proposed punch biopsies for newborns that required no closure nor anesthesia. ↗
▶ Ep 19 · 16:43
clinical Dr. Syndergaard from Sweden performed the first successful operation for total colonic aganglionosis in 1953, doing a colon resection with an ileo-anal anastomosis. ↗
▶ Ep 19 · 16:43
host_summary Dr. Syndergaard from Sweden performed the first successful operation for total colonic aganglionosis in 1953, doing a colon resection with an ileo-anal anastomosis. ↗
▶ Ep 19 · 17:35
host_summary Dr. Swenson observed that when patients had a colostomy the obstruction was relieved, but after closing the colostomy the disease returned. ↗
▶ Ep 19 · 17:35
clinical Dr. Swenson observed that when patients had a colostomy the obstruction was relieved, but after closing the colostomy the disease returned. ↗
▶ Ep 19 · 17:57
clinical Dr. Swenson scoped from the rectum when the patient had a colostomy and saw there was no true obstruction. ↗
▶ Ep 19 · 17:57
host_summary Dr. Swenson scoped from the rectum when the patient had a colostomy and saw there was no true obstruction. ↗
▶ Ep 19 · 18:09
host_summary Dr. Swenson used a probe on the proximal stoma and saw normal peristalsis, but when he put a probe in the distal stoma he saw there was no peristalsis. ↗
▶ Ep 19 · 18:09
clinical Dr. Swenson used a probe on the proximal stoma and saw normal peristalsis, but when he put a probe in the distal stoma he saw there was no peristalsis. ↗
▶ Ep 19 · 18:22
clinical Dr. Swenson performed a contrast study and observed a non-dilated portion followed by a dilated portion, leading him to conclude that the distal portion was the diseased one. ↗
▶ Ep 19 · 18:22
host_summary Dr. Swenson performed a contrast study and observed a non-dilated portion followed by a dilated portion, leading him to conclude that the distal portion was the diseased one. ↗

Surgical Procedures for Hirschsprung Disease

▶ Ep 21 · 8:59
clinical Transanal approach results in absolutely no scar, and patients have minimal postoperative pain. ↗
▶ Ep 21 · 8:59
clinical Transanal approach results in absolutely no scar, and patients have minimal postoperative pain. ↗
▶ Ep 21 · 1:10:05
clinical Patients with total colonic aganglionosis have suboptimal long-term results and a high incidence of complications. ↗
▶ Ep 21 · 1:10:05
clinical Patients with total colonic aganglionosis have suboptimal long-term results and a high incidence of complications. ↗
▶ Ep 21 · 1:10:18
clinical The most common complication in total colonic aganglionosis is ileostomy prolapse, which can be avoided by tacking the bowel proximal to the stoma to the abdominal wall. ↗
▶ Ep 21 · 1:10:18
clinical The most common complication in total colonic aganglionosis is ileostomy prolapse, which can be avoided by tacking the bowel proximal to the stoma to the abdominal wall. ↗
▶ Ep 21 · 1:10:44
opinion Pouch pull-through is not recommended for patients with Hirschsprung disease due to obstructive symptoms. ↗
▶ Ep 21 · 1:10:44
opinion Pouch pull-through is not recommended for patients with Hirschsprung disease due to obstructive symptoms. ↗
▶ Ep 21 · 1:11:12
clinical Severe diaper rash in total colonic aganglionosis can happen if the anal canal is destroyed (fecal incontinence) or if the pull-through is performed too early. ↗
▶ Ep 21 · 1:11:12
clinical Severe diaper rash in total colonic aganglionosis can happen if the anal canal is destroyed (fecal incontinence) or if the pull-through is performed too early. ↗
▶ Ep 21 · 1:11:43
clinical For total colonic aganglionosis diagnosed in a healthy newborn, perform colectomy with straight ileoanal anastomosis and ileostomy at presentation. ↗
▶ Ep 21 · 1:11:43
clinical For total colonic aganglionosis diagnosed in a healthy newborn, perform colectomy with straight ileoanal anastomosis and ileostomy at presentation. ↗
▶ Ep 21 · 1:12:01
clinical Close the ileostomy in total colonic aganglionosis only when the child is toilet-trained for urine and willing to accept rectal irrigation. ↗
▶ Ep 21 · 1:12:01
clinical Close the ileostomy in total colonic aganglionosis only when the child is toilet-trained for urine and willing to accept rectal irrigation. ↗
▶ Ep 21 · 1:12:13
clinical Patients with total colonic aganglionosis have a higher risk for enterocolitis, and the best treatment for enterocolitis is rectal irrigation. ↗
▶ Ep 21 · 1:12:13
clinical Patients with total colonic aganglionosis have a higher risk for enterocolitis, and the best treatment for enterocolitis is rectal irrigation. ↗
▶ Ep 21 · 1:16:29
clinical In total colonic aganglionosis, if ganglion cells are only present 45 cm proximal to the ileocecal valve, performing an ileoanal anastomosis and more proximal ileostomy would put the child in danger of decompensating a compensated situation. ↗
▶ Ep 21 · 1:16:29
clinical In total colonic aganglionosis, if ganglion cells are only present 45 cm proximal to the ileocecal valve, performing an ileoanal anastomosis and more proximal ileostomy would put the child in danger of decompensating a compensated situation. ↗
▶ Ep 21 · 1:16:55
clinical Leaving unused colon in total colonic aganglionosis can lead to enterocolitis from mucus accumulation and infection, and it's difficult to irrigate. ↗
▶ Ep 21 · 1:16:55
clinical Leaving unused colon in total colonic aganglionosis can lead to enterocolitis from mucus accumulation and infection, and it's difficult to irrigate. ↗
▶ Ep 21 · 2:01:44
clinical The PHOX2B gene provides instructions for making a protein that acts early in development to promote nerve cell formation and regulate neuron maturation. ↗
▶ Ep 21 · 2:01:44
host_summary The PHOX2B gene provides instructions for making a protein that acts early in development to promote nerve cell formation and regulate neuron maturation. ↗
▶ Ep 21 · 2:01:58
clinical The PHOX2B protein is active in the neural crest, and neural crest cells migrate to form parts of the autonomic nervous system, which controls breathing, blood pressure, heart rate, and digestion. ↗
▶ Ep 21 · 2:01:58
host_summary The PHOX2B protein is active in the neural crest, and neural crest cells migrate to form parts of the autonomic nervous system, which controls breathing, blood pressure, heart rate, and digestion. ↗
▶ Ep 21 · 2:02:15
clinical PHOX2B mutation is associated with congenital central hypoventilation syndrome, neuroblastoma, and Hirschsprung disease. ↗
▶ Ep 21 · 2:02:15
clinical PHOX2B mutation is associated with congenital central hypoventilation syndrome, neuroblastoma, and Hirschsprung disease. ↗
▶ Ep 21 · 2:04:17
clinical Patients with congenital central hypoventilation syndrome need tracheostomy and assisted ventilation during sleep. ↗
▶ Ep 21 · 2:04:17
clinical Patients with congenital central hypoventilation syndrome need tracheostomy and assisted ventilation during sleep. ↗
▶ Ep 21 · 2:06:22
clinical The association of Hirschsprung disease and anorectal malformation is very bad because every patient will be fecally incontinent. ↗
▶ Ep 21 · 2:06:22
clinical The association of Hirschsprung disease and anorectal malformation is very bad because every patient will be fecally incontinent. ↗
▶ Ep 21 · 2:06:31
clinical Patients with anorectal malformation have no anal canal by definition. ↗
▶ Ep 21 · 2:06:31
clinical Patients with anorectal malformation have no anal canal by definition. ↗
▶ Ep 21 · 2:06:38
clinical Due to Hirschsprung disease, we will resect the natural reservoir (rectosigmoid), so patients with both conditions will be fecally incontinent. ↗
▶ Ep 21 · 2:06:38
clinical Due to Hirschsprung disease, we will resect the natural reservoir (rectosigmoid), so patients with both conditions will be fecally incontinent. ↗
▶ Ep 21 · 2:06:47
clinical It is very important to discuss guaranteed fecal incontinence with parents prior to surgery for combined Hirschsprung and anorectal malformation. ↗
▶ Ep 21 · 2:06:47
clinical It is very important to discuss guaranteed fecal incontinence with parents prior to surgery for combined Hirschsprung and anorectal malformation. ↗

Anorectal Malformations with Dr. Andrea Bischoff

▶ Ep 30 · 1:52
quote Patients with anorectal malformation with good prognosis for bowel control, they will have a well-formed buttocks with a good midline groove and a good anal dimple. Patients with malformations with bad prognosis for bowel control will have a flat bottom and not clear delineation of the anal dimple. ↗
▶ Ep 30 · 1:52
clinical Patients with anorectal malformation with good prognosis for bowel control will have well-formed buttocks with a good midline groove and a good anal dimple, while patients with bad prognosis will have a flat bottom and no clear delineation of the anal dimple. ↗
▶ Ep 30 · 2:31
clinical In a newborn baby, a normal caliber anus should accommodate a number 12 Hegar dilator. ↗
▶ Ep 30 · 3:16
quote We don't even say anterior because we don't like the term anterior anus because it goes with what's the definition of an anus. An anus has to have a normal caliber. ↗
▶ Ep 30 · 3:42
clinical In a rectal perineal fistula, the sphincter mechanism is in a horseshoe shape with posterior and lateral portions having sphincter but the anterior portion lacking sphincter. ↗
▶ Ep 30 · 5:01
epidemiological 8% of patients with anorectal malformation will have esophageal atresia. ↗
▶ Ep 30 · 5:27
epidemiological 30% of patients with anorectal malformation will have cardiac anomalies, but in only 10% of them are these anomalies hemodynamically significant. ↗
▶ Ep 30 · 5:54
epidemiological 50% of patients with anorectal malformation have associated urological defects. ↗
▶ Ep 30 · 6:02
epidemiological 25% of patients with anorectal malformation have tethered cord. ↗
▶ Ep 30 · 6:31
clinical If no fistula is identified on initial examination, the patient should be re-examined after 24 hours because it takes time for air to travel distally and for meconium to pass through a tiny fistula. ↗
▶ Ep 30 · 7:04
clinical Cross-table lateral film with pelvis elevated should never be done before 24 hours of life because it will give the false impression of a high malformation due to muscle tone. ↗
▶ Ep 30 · 8:22
clinical Spinal ultrasound is adequate to detect tethered cord in babies less than 3 months of age; after 3 months, MRI is needed due to ossification. ↗
▶ Ep 30 · 8:47
clinical Tethered cord has more influence on the urinary tract rather than the gastrointestinal tract in terms of prognosis. ↗
▶ Ep 30 · 9:53
clinical Presacral masses are most commonly found in malformations with good prognosis such as rectal perineal fistula, rectal vestibular fistula, and rectal atresia, but when present, the prognosis changes. ↗
▶ Ep 30 · 11:55
clinical The advantage of primary newborn repair is that bowel preparation is not required since meconium is considered sterile. ↗
▶ Ep 30 · 12:20
quote It is actually better to open a colostomy and have a perfect operation than to do a primary repair, have a complication such as the hissing retraction that requires a re-operation. ↗
▶ Ep 30 · 12:20
opinion It is better to open a colostomy and have a perfect operation than to do a primary repair and have a complication such as dehiscence retraction that requires re-operation. ↗
▶ Ep 30 · 12:33
quote We like to say that patients with an anorectal malformation have one chance to have the right operation. Secondary operations or re-operations usually change the prognosis for bowel control in these patients. ↗
▶ Ep 30 · 12:33
clinical Patients with anorectal malformation have one chance to have the right operation; secondary operations or re-operations usually change the prognosis for bowel control. ↗
▶ Ep 30 · 13:30
clinical An 8 French feeding tube should be used to catheterize a suspected vestibular fistula if it cannot be visualized. ↗
▶ Ep 30 · 13:36
epidemiological Vestibular fistula is the most common type of anorectal anomaly in females. ↗
▶ Ep 30 · 17:58
clinical The ideal colostomy should be totally diverting, located in the descending colon to leave enough distal bowel for pull-through and avoid prolapse. ↗
▶ Ep 30 · 18:23
clinical The proximal stoma should be located in the center of a triangle formed by the left rib, umbilicus, and iliac crest to ensure it is surrounded by normal skin for stoma bag application. ↗
▶ Ep 30 · 18:59
clinical During colostomy creation, all distal bowel should be irrigated with plenty of normal saline to remove all meconium. ↗
▶ Ep 30 · 19:55
clinical For high pressure distal colostogram, a number 8 French Foley catheter is normally used in the mucous fistula. ↗
▶ Ep 30 · 20:50
clinical During distal colostogram, the patient should be turned to perfect lateral position with knees at 90 degrees and one femur exactly in front of the other, and the radiologist must show all reference points including mucous fistula, sacrum, and anal marker. ↗
▶ Ep 30 · 22:45
clinical In rectal urethral bulbar fistula, the most important portion of the operation is separation between the rectum and the long common wall with the urethra, with minimal rectal mobilization needed. ↗
▶ Ep 30 · 23:11
clinical In rectal urethral prostatic fistula, the common wall with urethra is shorter than bulbar fistula but more rectal dissection is needed to gain length. ↗
▶ Ep 30 · 23:45
clinical In rectal bladder neck fistula, the rectum joins the urinary tract in a T fashion and the challenge is gaining enough length to reach the perineum while preserving good blood supply through selective ligation of mesenteric vessels. ↗
▶ Ep 30 · 24:54
quote If I can make a plea to pediatric surgeons, I would ask them to disagree strongly when the neonatologist labels a cloaca patient as a disorder of sexual differentiation. ↗
▶ Ep 30 · 25:23
clinical Cloaca patients have never been seen with disorder of sexual differentiation; they are all females with normal ovaries. ↗
▶ Ep 30 · 25:23
quote If the patient has a single orifice, you palpate the pseudophalo and it is skin. There's no corpora. This is a cloaca, and we have never seen a cloaca with disorder of sexual differentiation. They are all females. ↗
▶ Ep 30 · 26:56
clinical Cloaca with common channel less than 3 centimeters can be repaired posterior sagittally with total urogenital mobilization. ↗
▶ Ep 30 · 27:43
quote We actually believe that parents should have this information about how the future is going to be in terms of prognosis for bowel control as early as possible in life to correctly adjust their expectations. ↗
▶ Ep 30 · 28:08
clinical Rectal perineal fistula patients with normal sacrum and no tethered cord have 100% chance of bowel control. ↗
▶ Ep 30 · 28:18
clinical Malformations with better prognosis for bowel control will suffer from more constipation. ↗
▶ Ep 30 · 28:40
clinical Rectal vestibular fistula patients with normal sacrum and no tethered cord have 95% chance of bowel control. ↗
▶ Ep 30 · 28:57
clinical Rectal urethral bulbar fistula has 85% chance of bowel control. ↗
▶ Ep 30 · 29:02
clinical Anorectal malformation without fistula has 80% chance of bowel control. ↗
▶ Ep 30 · 29:08
clinical Rectal urethral prostatic fistula has 60% chance of bowel control. ↗
▶ Ep 30 · 29:14
clinical Rectal bladder neck fistula has 20% chance of bowel control. ↗
▶ Ep 30 · 29:24
clinical Cloaca with common channel less than 3 centimeters and normal sacrum has generally about 70% chance of bowel control. ↗
▶ Ep 30 · 29:57
quote We think that the only indication to keep a colostomy would be incapacity to form solid stool. ↗
▶ Ep 30 · 29:57
opinion The only indication to keep a colostomy is incapacity to form solid stool; most patients with anorectal malformation have normal colon and can form solid stool. ↗
▶ Ep 30 · 30:20
opinion Patients prefer the quality of life of a pull-through with bowel management rather than with a colostomy. ↗
▶ Ep 30 · 30:43
guideline All children with anorectal malformations should be out of diapers at the same age that other children are normally out of diapers, which in the United States is usually at 3 years of age. ↗
▶ Ep 30 · 30:43
quote We believe that all children with anorectal malformations, they should be out of diapers at the same age that other children are normally out of diapers. Here in the United States, it's usually at 3 years of age. ↗
▶ Ep 30 · 31:13
guideline After 3 years of age, if the child has not potty trained, formal bowel management should start: enemas for children with bad prognosis, enemas on temporary basis for borderline bowel control, and laxatives for children with bowel control and constipation. ↗
▶ Ep 30 · 31:41
clinical Bowel management for fecal incontinence consists of finding the enema that completely cleans the colon and allows the child to be clean in underwear for 24 hours. ↗
▶ Ep 30 · 32:55
clinical Determining which enema works for each child is a trial and error process that usually takes about one week. ↗
▶ Ep 30 · 33:17
clinical Contrast enema without bowel preparation can divide fecally incontinent patients into two groups: those with dilated colon and tendency for constipation, and those with non-dilated colon and tendency for diarrhea. ↗
▶ Ep 30 · 33:32
clinical For dilated hypomotile colon, a large and concentrated enema is needed to clean the colon, but after cleaning there is natural tendency not to produce bowel movements. ↗
▶ Ep 30 · 33:54
clinical For non-dilated hypermotile colon, a small enema (sometimes just normal saline) is needed, but the challenge is keeping the colon from moving between enemas, usually requiring constipating diet and medication to slow the colon. ↗
▶ Ep 30 · 34:33
clinical Enema base is normal saline 200 to 1000 mLs, with additives to increase concentration: liquid glycerin 10-40 mLs, Castile soap 9-27 mLs, or Fleet sodium phosphate (33 mLs for ages 2-4, 66 mLs for ages 7-10, 133 mLs for over 10 years). ↗
▶ Ep 30 · 38:33
clinical The enema should be done at the same time every day and the entire process (administration, holding, evacuation) should last one hour. ↗
▶ Ep 30 · 39:12
clinical Enema adjustments are made based on parent/patient report and abdominal radiograph; if real stool accidents occur and radiograph is not clean, concentration should be increased. ↗
▶ Ep 30 · 39:36
clinical Enema frequency is always once daily; concentration is adjusted rather than volume or frequency. ↗
▶ Ep 30 · 40:32
clinical The Malone procedure (appendicostomy) is not the treatment for fecal incontinence; the treatment is finding the enema that works. The appendicostomy provides independence. ↗
▶ Ep 30 · 40:32
quote The Malone procedure or appendicostomy is not the treatment for the fecal incontinence. The treatment for the fecal incontinence is finding the enema that works for the child. The appendicostomy will just give More independence. ↗
▶ Ep 30 · 40:50
clinical Appendicostomy is offered when the child wants to do the enema independently or for patients with borderline bowel control who remain enema-dependent after yearly trials off enemas. ↗
▶ Ep 30 · 42:19
clinical For constipation management, disimpaction is required before determining laxative dosage to avoid severe cramping. ↗
▶ Ep 30 · 42:35
clinical Disimpaction protocol consists of 3 enemas per day for 3 days; most children are disimpacted after day 3. ↗
▶ Ep 30 · 42:52
clinical If still impacted after 3 days of enemas, admit for nasogastric tube with GoLYTELY for 2 days plus enemas; disimpaction under anesthesia is extremely rare (approximately 4 cases in 8 years). ↗
▶ Ep 30 · 43:30
clinical Senna-based laxative is preferred, given at 6 PM to produce bowel movement the next day, with dosage range from 8.8 mg to 175 mg. ↗
▶ Ep 30 · 43:37
clinical Laxative dosage is adjusted daily based on bowel movement frequency, consistency, and abdominal radiograph showing stool burden; if no bowel movements in 24 hours, give enema and increase laxative dose. ↗

Hirschsprung Disease: Surgical Procedures

▶ Ep 39 · 8:59
clinical Transanal approach results in absolutely no scar and minimal postoperative pain. ↗
▶ Ep 39 · 1:10:18
clinical Most common complications in total colonic aganglionosis include ileostomy prolapse, obstructive symptoms following pouch pull-through, wrong pathological diagnosis, anastomotic stricture or acquired atresia, severe diaper rash, and enterocolitis. ↗
▶ Ep 39 · 1:10:27
clinical To avoid ileostomy prolapse, tack the bowel proximal to the stoma to the abdominal wall—whenever you open a stoma in a mobile portion of colon or intestine, you are at risk of prolapse. ↗
▶ Ep 39 · 1:11:53
clinical For total colonic aganglionosis in a healthy newborn, perform colectomy with straight ileoanal anastomosis and ileostomy at presentation, then close the ileostomy only when the child is toilet trained for urine and willing to accept rectal irrigation. ↗
▶ Ep 39 · 1:12:13
clinical Patients with total colonic aganglionosis have higher risk for enterocolitis, and the best treatment for enterocolitis is rectal irrigation. ↗
▶ Ep 39 · 1:14:45
clinical If urinary sodium is less than 20 millimoles per liter in a patient with ileostomy, start oral sodium replacement. ↗
▶ Ep 39 · 1:16:10
quote You should not do an ileoanal until the patient is toilet trained for urine because the patient needs to know how to go to the bathroom and evacuate in the toilet, otherwise you will have the worst unmanageable diaper rash that you've ever seen. ↗
▶ Ep 39 · 1:16:10
clinical You should not do an ileoanal anastomosis until the patient is toilet trained for urine because the patient needs to know how to go to the bathroom and evacuate in the toilet, otherwise you will have the worst unmanageable diaper rash. ↗
▶ Ep 39 · 2:01:44
clinical The PHOX2B gene provides instructions for making a protein that acts early in development to help promote nerve cell formation and regulate neuron maturation, and is active in the neural crest cells that form parts of the autonomic nervous system controlling breathing, blood pressure, heart rate, and digestion. ↗
▶ Ep 39 · 2:02:24
clinical PHOX2B mutation is associated with congenital central hypoventilation syndrome, neuroblastoma, and Hirschsprung disease. ↗
▶ Ep 39 · 2:04:17
clinical Patients with congenital central hypoventilation syndrome (Ondine's curse) need tracheostomy and assisted ventilation during sleep because they stop breathing when they fall asleep. ↗
▶ Ep 39 · 2:06:22
quote The association of HR and anorectal malformation is a very bad association because every patient will be fecally incontinent. ↗
▶ Ep 39 · 2:06:22
clinical The association of Hirschsprung disease and anorectal malformation is very bad because every patient will be fecally incontinent—the patient has no anal canal by definition, and resection of the rectosigmoid removes the natural reservoir. ↗

Error Traps and Culture of Safety in Anorectal Malformations

▶ Ep 68 · 0:31
clinical Five error traps have been identified in the management of anorectal malformations. ↗
▶ Ep 68 · 0:31
quote We have identified 5 error traps. ↗
▶ Ep 68 · 0:31
quote We have identified 5 error traps. ↗
▶ Ep 68 · 0:31
clinical Five error traps have been identified in the management of anorectal malformations. ↗
▶ Ep 68 · 0:34
clinical The first error trap is the creation of a colostomy too distal in the sigmoid colon. ↗
▶ Ep 68 · 0:34
quote The first one is the creation of a colostomy 2 distal in the sigmoid column. Our recommendation is for a colostomy at the descending column. ↗
▶ Ep 68 · 0:34
quote The first one is the creation of a colostomy 2 distal in the sigmoid column. Our recommendation is for a colostomy at the descending column. ↗
▶ Ep 68 · 0:34
clinical The first error trap is the creation of a colostomy too distal in the sigmoid colon. ↗
▶ Ep 68 · 0:40
guideline The recommendation is for a colostomy at the descending colon. ↗
▶ Ep 68 · 0:40
guideline The recommendation is for a colostomy at the descending colon. ↗
▶ Ep 68 · 0:45
clinical The second error trap is having an inaccurate distal colostogram. ↗
▶ Ep 68 · 0:45
clinical The second error trap is having an inaccurate distal colostogram. ↗
▶ Ep 68 · 0:51
guideline A correctly done distal colostogram should show the site of the mucous fistula, the amount of bowel length available for the pull-through, the end of the rectum or the connection to the urinary tract, whenever possible the bladder and the urethra, the tip of the sacrum, and the anal marker. ↗
▶ Ep 68 · 0:51
guideline A correctly done distal colostogram should show the site of the mucous fistula, the amount of bowel length available for the pull-through, the end of the rectum or the connection to the urinary tract, whenever possible the bladder and the urethra, the tip of the sacrum, and the anal marker. ↗
▶ Ep 68 · 1:16
clinical The third error trap is operating on a male patient without a Foley catheter and ignoring the hazards of the separation between the anterior rectal wall and the genitourinary tract. ↗
▶ Ep 68 · 1:16
clinical The third error trap is operating on a male patient without a Foley catheter and ignoring the hazards of the separation between the anterior rectal wall and the genitourinary tract. ↗
▶ Ep 68 · 1:32
clinical The fourth error trap is attempting to dilate a true rectal stricture. ↗
▶ Ep 68 · 1:32
clinical The fourth error trap is attempting to dilate a true rectal stricture. ↗
▶ Ep 68 · 1:39
guideline Anal dilations are not for real strictures. ↗
▶ Ep 68 · 1:39
clinical The fifth error trap is not offering long-term follow-up to these patients. ↗
▶ Ep 68 · 1:39
quote Anal dilations are not for real strictures ↗
▶ Ep 68 · 1:39
clinical The fifth error trap is not offering long-term follow-up to these patients. ↗
▶ Ep 68 · 1:39
guideline Anal dilations are not for real strictures. ↗
▶ Ep 68 · 1:39
quote Anal dilations are not for real strictures ↗
▶ Ep 68 · 1:49
quote Those patients need long-term follow-up in colorectal, urology, and gynecology. ↗
▶ Ep 68 · 1:49
guideline Patients with anorectal malformations need long-term follow-up in colorectal, urology, and gynecology. ↗
▶ Ep 68 · 1:49
quote Those patients need long-term follow-up in colorectal, urology, and gynecology. ↗
▶ Ep 68 · 1:49
guideline Patients with anorectal malformations need long-term follow-up in colorectal, urology, and gynecology. ↗
▶ Ep 68 · 1:56
opinion By avoiding these common error traps, patients will receive better care. ↗
▶ Ep 68 · 1:56
opinion By avoiding these common error traps, patients will receive better care. ↗

Anorectal Malformations with Dr. Andrea Bischoff

▶ Ep 81 · 1:52
quote Patients with anorectal malformation with good prognosis for bowel control, they will have a well-formed buttocks with a good midline groove and a good anal dimple. Patients with malformations with bad prognosis for bowel control will have a flat bottom and not clear delineation of the anal dimple. ↗
▶ Ep 81 · 1:52
clinical Patients with anorectal malformation with good prognosis for bowel control will have well-formed buttocks with a good midline groove and a good anal dimple, while patients with bad prognosis will have a flat bottom and no clear delineation of the anal dimple. ↗
▶ Ep 81 · 2:31
clinical In a newborn baby, a normal caliber anus should accommodate a number 12 Hegar dilator. ↗
▶ Ep 81 · 3:16
quote We don't even say anterior because we don't like the term anterior anus because it goes with what's the definition of an anus. An anus has to have a normal caliber. ↗
▶ Ep 81 · 3:42
clinical In a rectal perineal fistula, the sphincter mechanism is in a horseshoe shape with posterior and lateral portions having sphincter but the anterior portion lacking sphincter. ↗
▶ Ep 81 · 5:01
epidemiological 8% of patients with anorectal malformation will have esophageal atresia. ↗
▶ Ep 81 · 5:27
epidemiological 30% of patients with anorectal malformation will have cardiac anomalies, but in only 10% of them are these anomalies hemodynamically significant. ↗
▶ Ep 81 · 5:54
epidemiological 50% of patients with anorectal malformation have associated urological defects. ↗
▶ Ep 81 · 6:02
epidemiological 25% of patients with anorectal malformation have tethered cord. ↗
▶ Ep 81 · 6:31
clinical If no fistula is identified on initial examination, the patient should be re-examined after 24 hours because it takes time for air to travel distally and for meconium to pass through a tiny fistula. ↗
▶ Ep 81 · 7:04
clinical Cross-table lateral film with pelvis elevated should never be done before 24 hours of life because it will give the false impression of a high malformation due to muscle tone. ↗
▶ Ep 81 · 8:22
clinical Spinal ultrasound is adequate to detect tethered cord in babies less than 3 months of age; after 3 months, MRI is needed due to ossification. ↗
▶ Ep 81 · 8:47
clinical Tethered cord has more influence on the urinary tract rather than the gastrointestinal tract in terms of prognosis. ↗
▶ Ep 81 · 9:53
clinical Presacral masses are most commonly found in malformations with good prognosis such as rectal perineal fistula, rectal vestibular fistula, and rectal atresia, but when present, the prognosis changes. ↗
▶ Ep 81 · 11:55
clinical The advantage of primary newborn repair is that bowel preparation is not required since meconium is considered sterile. ↗
▶ Ep 81 · 12:20
quote It is actually better to open a colostomy and have a perfect operation than to do a primary repair, have a complication such as the hissing retraction that requires a re-operation. ↗
▶ Ep 81 · 12:20
opinion It is better to open a colostomy and have a perfect operation than to do a primary repair and have a complication such as dehiscence retraction that requires re-operation. ↗
▶ Ep 81 · 12:33
clinical Patients with anorectal malformation have one chance to have the right operation; secondary operations or re-operations usually change the prognosis for bowel control. ↗
▶ Ep 81 · 12:33
quote We like to say that patients with an anorectal malformation have one chance to have the right operation. Secondary operations or re-operations usually change the prognosis for bowel control in these patients. ↗
▶ Ep 81 · 13:30
clinical An 8 French feeding tube should be used to catheterize a suspected vestibular fistula if it cannot be visualized. ↗
▶ Ep 81 · 13:36
epidemiological Vestibular fistula is the most common type of anorectal anomaly in females. ↗
▶ Ep 81 · 17:58
clinical The ideal colostomy should be totally diverting, located in the descending colon to leave enough distal bowel for pull-through and avoid prolapse. ↗
▶ Ep 81 · 18:23
clinical The proximal stoma should be located in the center of a triangle formed by the left rib, umbilicus, and iliac crest to ensure it is surrounded by normal skin for stoma bag application. ↗
▶ Ep 81 · 18:59
clinical During colostomy creation, all distal bowel should be irrigated with plenty of normal saline to remove all meconium. ↗
▶ Ep 81 · 19:55
clinical For high pressure distal colostogram, a number 8 French Foley catheter is normally used in the mucous fistula. ↗
▶ Ep 81 · 20:50
clinical During distal colostogram, the patient should be turned to perfect lateral position with knees at 90 degrees and one femur exactly in front of the other, and the radiologist must show all reference points including mucous fistula, sacrum, and anal marker. ↗
▶ Ep 81 · 22:45
clinical In rectal urethral bulbar fistula, the most important portion of the operation is separation between the rectum and the long common wall with the urethra, with minimal rectal mobilization needed. ↗
▶ Ep 81 · 23:11
clinical In rectal urethral prostatic fistula, the common wall with urethra is shorter than bulbar fistula but more rectal dissection is needed to gain length. ↗
▶ Ep 81 · 23:45
clinical In rectal bladder neck fistula, the rectum joins the urinary tract in a T fashion and the challenge is gaining enough length to reach the perineum while preserving good blood supply through selective ligation of mesenteric vessels. ↗
▶ Ep 81 · 24:54
quote If I can make a plea to pediatric surgeons, I would ask them to disagree strongly when the neonatologist labels a cloaca patient as a disorder of sexual differentiation. ↗
▶ Ep 81 · 25:23
clinical Cloaca patients have never been seen with disorder of sexual differentiation; they are all females with normal ovaries. ↗
▶ Ep 81 · 25:23
quote If the patient has a single orifice, you palpate the pseudophalo and it is skin. There's no corpora. This is a cloaca, and we have never seen a cloaca with disorder of sexual differentiation. They are all females. ↗
▶ Ep 81 · 26:56
clinical Cloaca with common channel less than 3 centimeters can be repaired posterior sagittally with total urogenital mobilization. ↗
▶ Ep 81 · 27:43
quote We actually believe that parents should have this information about how the future is going to be in terms of prognosis for bowel control as early as possible in life to correctly adjust their expectations. ↗
▶ Ep 81 · 28:08
clinical Rectal perineal fistula patients with normal sacrum and no tethered cord have 100% chance of bowel control. ↗
▶ Ep 81 · 28:18
clinical Malformations with better prognosis for bowel control will suffer from more constipation. ↗
▶ Ep 81 · 28:40
clinical Rectal vestibular fistula patients with normal sacrum and no tethered cord have 95% chance of bowel control. ↗
▶ Ep 81 · 28:57
clinical Rectal urethral bulbar fistula has 85% chance of bowel control. ↗
▶ Ep 81 · 29:02
clinical Anorectal malformation without fistula has 80% chance of bowel control. ↗
▶ Ep 81 · 29:08
clinical Rectal urethral prostatic fistula has 60% chance of bowel control. ↗
▶ Ep 81 · 29:14
clinical Rectal bladder neck fistula has 20% chance of bowel control. ↗
▶ Ep 81 · 29:24
clinical Cloaca with common channel less than 3 centimeters and normal sacrum has generally about 70% chance of bowel control. ↗
▶ Ep 81 · 29:57
opinion The only indication to keep a colostomy is incapacity to form solid stool; most patients with anorectal malformation have normal colon and can form solid stool. ↗
▶ Ep 81 · 29:57
quote We think that the only indication to keep a colostomy would be incapacity to form solid stool. ↗
▶ Ep 81 · 30:20
opinion Patients prefer the quality of life of a pull-through with bowel management rather than with a colostomy. ↗
▶ Ep 81 · 30:43
guideline All children with anorectal malformations should be out of diapers at the same age that other children are normally out of diapers, which in the United States is usually at 3 years of age. ↗
▶ Ep 81 · 30:43
quote We believe that all children with anorectal malformations, they should be out of diapers at the same age that other children are normally out of diapers. Here in the United States, it's usually at 3 years of age. ↗
▶ Ep 81 · 31:13
guideline After 3 years of age, if the child has not potty trained, formal bowel management should start: enemas for children with bad prognosis, enemas on temporary basis for borderline bowel control, and laxatives for children with bowel control and constipation. ↗
▶ Ep 81 · 31:41
clinical Bowel management for fecal incontinence consists of finding the enema that completely cleans the colon and allows the child to be clean in underwear for 24 hours. ↗
▶ Ep 81 · 32:55
clinical Determining which enema works for each child is a trial and error process that usually takes about one week. ↗
▶ Ep 81 · 33:17
clinical Contrast enema without bowel preparation can divide fecally incontinent patients into two groups: those with dilated colon and tendency for constipation, and those with non-dilated colon and tendency for diarrhea. ↗
▶ Ep 81 · 33:32
clinical For dilated hypomotile colon, a large and concentrated enema is needed to clean the colon, but after cleaning there is natural tendency not to produce bowel movements. ↗
▶ Ep 81 · 33:54
clinical For non-dilated hypermotile colon, a small enema (sometimes just normal saline) is needed, but the challenge is keeping the colon from moving between enemas, usually requiring constipating diet and medication to slow the colon. ↗
▶ Ep 81 · 34:33
clinical Enema base is normal saline 200 to 1000 mLs, with additives to increase concentration: liquid glycerin 10-40 mLs, Castile soap 9-27 mLs, or Fleet sodium phosphate (33 mLs for ages 2-4, 66 mLs for ages 7-10, 133 mLs for over 10 years). ↗
▶ Ep 81 · 38:33
clinical The enema should be done at the same time every day and the entire process (administration, holding, evacuation) should last one hour. ↗
▶ Ep 81 · 39:12
clinical Enema adjustments are made based on parent/patient report and abdominal radiograph; if real stool accidents occur and radiograph is not clean, concentration should be increased. ↗
▶ Ep 81 · 39:36
clinical Enema frequency is always once daily; concentration is adjusted rather than volume or frequency. ↗
▶ Ep 81 · 40:32
quote The Malone procedure or appendicostomy is not the treatment for the fecal incontinence. The treatment for the fecal incontinence is finding the enema that works for the child. The appendicostomy will just give More independence. ↗
▶ Ep 81 · 40:32
clinical The Malone procedure (appendicostomy) is not the treatment for fecal incontinence; the treatment is finding the enema that works. The appendicostomy provides independence. ↗
▶ Ep 81 · 40:50
clinical Appendicostomy is offered when the child wants to do the enema independently or for patients with borderline bowel control who remain enema-dependent after yearly trials off enemas. ↗
▶ Ep 81 · 42:19
clinical For constipation management, disimpaction is required before determining laxative dosage to avoid severe cramping. ↗
▶ Ep 81 · 42:35
clinical Disimpaction protocol consists of 3 enemas per day for 3 days; most children are disimpacted after day 3. ↗
▶ Ep 81 · 42:52
clinical If still impacted after 3 days of enemas, admit for nasogastric tube with GoLYTELY for 2 days plus enemas; disimpaction under anesthesia is extremely rare (approximately 4 cases in 8 years). ↗
▶ Ep 81 · 43:30
clinical Senna-based laxative is preferred, given at 6 PM to produce bowel movement the next day, with dosage range from 8.8 mg to 175 mg. ↗
▶ Ep 81 · 43:37
clinical Laxative dosage is adjusted daily based on bowel movement frequency, consistency, and abdominal radiograph showing stool burden; if no bowel movements in 24 hours, give enema and increase laxative dose. ↗
Hirschsprung disease 45 entries

History of Hirschsprung Disease

▶ Ep 7 · 4:52
quote I think if we don't know history, we are always at risk to repeat the same mistakes. ↗
▶ Ep 7 · 5:02
clinical Harald Hirschsprung presented a paper on constipation in newborns due to dilation and hypertrophy of the colon in 1886 at the Society of Pediatrics in Berlin. ↗
▶ Ep 7 · 5:54
clinical Harald Hirschsprung was a pediatrician who developed the hydrostatic reduction of ileocolonic intussusception. ↗
▶ Ep 7 · 7:15
clinical Early theories attempting to explain Hirschsprung disease etiology were all wrong because everyone was obsessed that the dilated portion was the diseased one, trying to explain why the dilated portion was the cause rather than the consequence of the disease. ↗
▶ Ep 7 · 8:52
clinical William Osler, one of the four founding professors of Johns Hopkins and creator of the residency, proposed colostomy or rectal tube and irrigation as possible treatments for Hirschsprung disease. ↗
▶ Ep 7 · 10:08
clinical In 1946, Orvar Swenson finally recognized the finding of no ganglion cells in the narrow rectal sigmoid as the cause of Hirschsprung disease, whereas others before him had noted absent ganglion cells but thought it was an acquired condition. ↗
▶ Ep 7 · 12:38
clinical Resection of the distal non-dilated portion was not used as a treatment for Hirschsprung disease prior to 1946. ↗
▶ Ep 7 · 13:23
clinical Barium enema technique became the standard diagnostic test for Hirschsprung disease in 1948, with Dr. Swenson involved in this publication. ↗
▶ Ep 7 · 15:33
clinical Barry Shandling, who worked in Canada, proposed punch biopsies for newborns that required no closure nor anesthesia. ↗
▶ Ep 7 · 16:43
clinical Dr. Syndergaard from Sweden performed the first successful operation for total colonic aganglionosis in 1953, doing a colon resection with an ileo-anal anastomosis. ↗
▶ Ep 7 · 17:35
clinical Dr. Swenson observed that when patients had a colostomy the obstruction was relieved, but after closing the colostomy the disease returned. ↗
▶ Ep 7 · 17:57
clinical Dr. Swenson scoped from the rectum when the patient had a colostomy and saw there was no true obstruction. ↗
▶ Ep 7 · 18:09
clinical Dr. Swenson used a probe on the proximal stoma and saw normal peristalsis, but when he put a probe in the distal stoma he saw there was no peristalsis. ↗
▶ Ep 7 · 18:22
clinical Dr. Swenson performed a contrast study and observed a non-dilated portion followed by a dilated portion, leading him to conclude that the distal portion was the diseased one. ↗

Surgical Procedures for Hirschsprung Disease

▶ Ep 8 · 8:59
clinical Transanal approach results in absolutely no scar, and patients have minimal postoperative pain. ↗
▶ Ep 8 · 1:10:05
clinical Patients with total colonic aganglionosis have suboptimal long-term results and a high incidence of complications. ↗
▶ Ep 8 · 1:10:18
clinical The most common complication in total colonic aganglionosis is ileostomy prolapse, which can be avoided by tacking the bowel proximal to the stoma to the abdominal wall. ↗
▶ Ep 8 · 1:10:44
opinion Pouch pull-through is not recommended for patients with Hirschsprung disease due to obstructive symptoms. ↗
▶ Ep 8 · 1:11:12
clinical Severe diaper rash in total colonic aganglionosis can happen if the anal canal is destroyed (fecal incontinence) or if the pull-through is performed too early. ↗
▶ Ep 8 · 1:11:43
clinical For total colonic aganglionosis diagnosed in a healthy newborn, perform colectomy with straight ileoanal anastomosis and ileostomy at presentation. ↗
▶ Ep 8 · 1:12:01
clinical Close the ileostomy in total colonic aganglionosis only when the child is toilet-trained for urine and willing to accept rectal irrigation. ↗
▶ Ep 8 · 1:12:13
clinical Patients with total colonic aganglionosis have a higher risk for enterocolitis, and the best treatment for enterocolitis is rectal irrigation. ↗
▶ Ep 8 · 1:16:29
clinical In total colonic aganglionosis, if ganglion cells are only present 45 cm proximal to the ileocecal valve, performing an ileoanal anastomosis and more proximal ileostomy would put the child in danger of decompensating a compensated situation. ↗
▶ Ep 8 · 1:16:55
clinical Leaving unused colon in total colonic aganglionosis can lead to enterocolitis from mucus accumulation and infection, and it's difficult to irrigate. ↗
▶ Ep 8 · 2:01:44
clinical The PHOX2B gene provides instructions for making a protein that acts early in development to promote nerve cell formation and regulate neuron maturation. ↗
▶ Ep 8 · 2:01:58
clinical The PHOX2B protein is active in the neural crest, and neural crest cells migrate to form parts of the autonomic nervous system, which controls breathing, blood pressure, heart rate, and digestion. ↗
▶ Ep 8 · 2:02:15
clinical PHOX2B mutation is associated with congenital central hypoventilation syndrome, neuroblastoma, and Hirschsprung disease. ↗
▶ Ep 8 · 2:04:17
clinical Patients with congenital central hypoventilation syndrome need tracheostomy and assisted ventilation during sleep. ↗
▶ Ep 8 · 2:06:22
clinical The association of Hirschsprung disease and anorectal malformation is very bad because every patient will be fecally incontinent. ↗
▶ Ep 8 · 2:06:31
clinical Patients with anorectal malformation have no anal canal by definition. ↗
▶ Ep 8 · 2:06:38
clinical Due to Hirschsprung disease, we will resect the natural reservoir (rectosigmoid), so patients with both conditions will be fecally incontinent. ↗
▶ Ep 8 · 2:06:47
clinical It is very important to discuss guaranteed fecal incontinence with parents prior to surgery for combined Hirschsprung and anorectal malformation. ↗

Hirschsprung Disease: Surgical Procedures

▶ Ep 19 · 8:59
clinical Transanal approach results in absolutely no scar and minimal postoperative pain. ↗
▶ Ep 19 · 1:10:18
clinical Most common complications in total colonic aganglionosis include ileostomy prolapse, obstructive symptoms following pouch pull-through, wrong pathological diagnosis, anastomotic stricture or acquired atresia, severe diaper rash, and enterocolitis. ↗
▶ Ep 19 · 1:10:27
clinical To avoid ileostomy prolapse, tack the bowel proximal to the stoma to the abdominal wall—whenever you open a stoma in a mobile portion of colon or intestine, you are at risk of prolapse. ↗
▶ Ep 19 · 1:11:53
clinical For total colonic aganglionosis in a healthy newborn, perform colectomy with straight ileoanal anastomosis and ileostomy at presentation, then close the ileostomy only when the child is toilet trained for urine and willing to accept rectal irrigation. ↗
▶ Ep 19 · 1:12:13
clinical Patients with total colonic aganglionosis have higher risk for enterocolitis, and the best treatment for enterocolitis is rectal irrigation. ↗
▶ Ep 19 · 1:14:45
clinical If urinary sodium is less than 20 millimoles per liter in a patient with ileostomy, start oral sodium replacement. ↗
▶ Ep 19 · 1:16:10
quote You should not do an ileoanal until the patient is toilet trained for urine because the patient needs to know how to go to the bathroom and evacuate in the toilet, otherwise you will have the worst unmanageable diaper rash that you've ever seen. ↗
▶ Ep 19 · 1:16:10
clinical You should not do an ileoanal anastomosis until the patient is toilet trained for urine because the patient needs to know how to go to the bathroom and evacuate in the toilet, otherwise you will have the worst unmanageable diaper rash. ↗
▶ Ep 19 · 2:01:44
clinical The PHOX2B gene provides instructions for making a protein that acts early in development to help promote nerve cell formation and regulate neuron maturation, and is active in the neural crest cells that form parts of the autonomic nervous system controlling breathing, blood pressure, heart rate, and digestion. ↗
▶ Ep 19 · 2:02:24
clinical PHOX2B mutation is associated with congenital central hypoventilation syndrome, neuroblastoma, and Hirschsprung disease. ↗
▶ Ep 19 · 2:04:17
clinical Patients with congenital central hypoventilation syndrome (Ondine's curse) need tracheostomy and assisted ventilation during sleep because they stop breathing when they fall asleep. ↗
▶ Ep 19 · 2:06:22
clinical The association of Hirschsprung disease and anorectal malformation is very bad because every patient will be fecally incontinent—the patient has no anal canal by definition, and resection of the rectosigmoid removes the natural reservoir. ↗
▶ Ep 19 · 2:06:22
quote The association of HR and anorectal malformation is a very bad association because every patient will be fecally incontinent. ↗
Total Colonic Aganglionosis 31 entries

Surgical Procedures for Hirschsprung Disease

▶ Ep 2 · 8:59
clinical Transanal approach results in absolutely no scar, and patients have minimal postoperative pain. ↗
▶ Ep 2 · 1:10:05
clinical Patients with total colonic aganglionosis have suboptimal long-term results and a high incidence of complications. ↗
▶ Ep 2 · 1:10:18
clinical The most common complication in total colonic aganglionosis is ileostomy prolapse, which can be avoided by tacking the bowel proximal to the stoma to the abdominal wall. ↗
▶ Ep 2 · 1:10:44
opinion Pouch pull-through is not recommended for patients with Hirschsprung disease due to obstructive symptoms. ↗
▶ Ep 2 · 1:11:12
clinical Severe diaper rash in total colonic aganglionosis can happen if the anal canal is destroyed (fecal incontinence) or if the pull-through is performed too early. ↗
▶ Ep 2 · 1:11:43
clinical For total colonic aganglionosis diagnosed in a healthy newborn, perform colectomy with straight ileoanal anastomosis and ileostomy at presentation. ↗
▶ Ep 2 · 1:12:01
clinical Close the ileostomy in total colonic aganglionosis only when the child is toilet-trained for urine and willing to accept rectal irrigation. ↗
▶ Ep 2 · 1:12:13
clinical Patients with total colonic aganglionosis have a higher risk for enterocolitis, and the best treatment for enterocolitis is rectal irrigation. ↗
▶ Ep 2 · 1:16:29
clinical In total colonic aganglionosis, if ganglion cells are only present 45 cm proximal to the ileocecal valve, performing an ileoanal anastomosis and more proximal ileostomy would put the child in danger of decompensating a compensated situation. ↗
▶ Ep 2 · 1:16:55
clinical Leaving unused colon in total colonic aganglionosis can lead to enterocolitis from mucus accumulation and infection, and it's difficult to irrigate. ↗
▶ Ep 2 · 2:01:44
host_summary The PHOX2B gene provides instructions for making a protein that acts early in development to promote nerve cell formation and regulate neuron maturation. ↗
▶ Ep 2 · 2:01:58
host_summary The PHOX2B protein is active in the neural crest, and neural crest cells migrate to form parts of the autonomic nervous system, which controls breathing, blood pressure, heart rate, and digestion. ↗
▶ Ep 2 · 2:02:15
clinical PHOX2B mutation is associated with congenital central hypoventilation syndrome, neuroblastoma, and Hirschsprung disease. ↗
▶ Ep 2 · 2:04:17
clinical Patients with congenital central hypoventilation syndrome need tracheostomy and assisted ventilation during sleep. ↗
▶ Ep 2 · 2:06:22
clinical The association of Hirschsprung disease and anorectal malformation is very bad because every patient will be fecally incontinent. ↗
▶ Ep 2 · 2:06:31
clinical Patients with anorectal malformation have no anal canal by definition. ↗
▶ Ep 2 · 2:06:38
clinical Due to Hirschsprung disease, we will resect the natural reservoir (rectosigmoid), so patients with both conditions will be fecally incontinent. ↗
▶ Ep 2 · 2:06:47
clinical It is very important to discuss guaranteed fecal incontinence with parents prior to surgery for combined Hirschsprung and anorectal malformation. ↗

Hirschsprung Disease: Surgical Procedures

▶ Ep 3 · 8:59
clinical Transanal approach results in absolutely no scar and minimal postoperative pain. ↗
▶ Ep 3 · 1:10:18
clinical Most common complications in total colonic aganglionosis include ileostomy prolapse, obstructive symptoms following pouch pull-through, wrong pathological diagnosis, anastomotic stricture or acquired atresia, severe diaper rash, and enterocolitis. ↗
▶ Ep 3 · 1:10:27
clinical To avoid ileostomy prolapse, tack the bowel proximal to the stoma to the abdominal wall—whenever you open a stoma in a mobile portion of colon or intestine, you are at risk of prolapse. ↗
▶ Ep 3 · 1:11:53
clinical For total colonic aganglionosis in a healthy newborn, perform colectomy with straight ileoanal anastomosis and ileostomy at presentation, then close the ileostomy only when the child is toilet trained for urine and willing to accept rectal irrigation. ↗
▶ Ep 3 · 1:12:13
clinical Patients with total colonic aganglionosis have higher risk for enterocolitis, and the best treatment for enterocolitis is rectal irrigation. ↗
▶ Ep 3 · 1:14:45
clinical If urinary sodium is less than 20 millimoles per liter in a patient with ileostomy, start oral sodium replacement. ↗
▶ Ep 3 · 1:16:10
quote You should not do an ileoanal until the patient is toilet trained for urine because the patient needs to know how to go to the bathroom and evacuate in the toilet, otherwise you will have the worst unmanageable diaper rash that you've ever seen. ↗
▶ Ep 3 · 1:16:10
clinical You should not do an ileoanal anastomosis until the patient is toilet trained for urine because the patient needs to know how to go to the bathroom and evacuate in the toilet, otherwise you will have the worst unmanageable diaper rash. ↗
▶ Ep 3 · 2:01:44
host_summary The PHOX2B gene provides instructions for making a protein that acts early in development to help promote nerve cell formation and regulate neuron maturation, and is active in the neural crest cells that form parts of the autonomic nervous system controlling breathing, blood pressure, heart rate, and digestion. ↗
▶ Ep 3 · 2:02:24
host_summary PHOX2B mutation is associated with congenital central hypoventilation syndrome, neuroblastoma, and Hirschsprung disease. ↗
▶ Ep 3 · 2:04:17
clinical Patients with congenital central hypoventilation syndrome (Ondine's curse) need tracheostomy and assisted ventilation during sleep because they stop breathing when they fall asleep. ↗
▶ Ep 3 · 2:06:22
clinical The association of Hirschsprung disease and anorectal malformation is very bad because every patient will be fecally incontinent—the patient has no anal canal by definition, and resection of the rectosigmoid removes the natural reservoir. ↗
▶ Ep 3 · 2:06:22
quote The association of HR and anorectal malformation is a very bad association because every patient will be fecally incontinent. ↗