Andrea Bischoff
511 statements · 6 topics · summaries given as host listed separately
Featured statements
Laxatives do not produce immediate, predictable bowel movements; if we knew that you would take the laxative and you would have the immediate effect, meaning I take the pill and I will immediately go to the to the toilet and have a bowel movement, that would be perfect, but unfortunately we don't have this drug available in the market.
quote ·
Colorectal / ARM & Hirschsprung
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
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 ·
Colorectal / ARM & Hirschsprung
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.
quote ·
Anorectal Malformation
If you take laxative, you'll have many bowel movements at unpredictable time, so if you're giving laxatives to a patient that is fecally incontinent, you're just making this patient worse.
quote ·
Enterocolitis
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.
clinical ·
Hirschsprung disease
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Andrea's statements about Anorectal Malformation 148 statements
Open the Anorectal Malformation collection →
Cloaca - Prental Imaging & Diagnosis - Counseling
Statement
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
Open
Quotation
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.
Open
Statement
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
Open
Anorectal Malformations with Dr. Andrea Bischoff
Quotation
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.
Open
Statement
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.
Open
Statement
In a newborn baby, a normal caliber anus should accommodate a number 12 Hegar dilator.
Open
Quotation
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.
Open
Statement
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.
Open
Statement
30% of patients with anorectal malformation will have cardiac anomalies, but in only 10% of them are these anomalies hemodynamically significant.
Open
Statement
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
Tethered cord has more influence on the urinary tract rather than the gastrointestinal tract in terms of prognosis.
Open
Statement
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.
Open
Statement
The advantage of primary newborn repair is that bowel preparation is not required since meconium is considered sterile.
Open
Statement
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.
Open
Quotation
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.
Open
Quotation
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.
Open
Statement
Patients with anorectal malformation have one chance to have the right operation; secondary operations or re-operations usually change the prognosis for bowel control.
Open
Statement
An 8 French feeding tube should be used to catheterize a suspected vestibular fistula if it cannot be visualized.
Open
Statement
The ideal colostomy should be totally diverting, located in the descending colon to leave enough distal bowel for pull-through and avoid prolapse.
Open
Statement
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.
Open
Statement
During colostomy creation, all distal bowel should be irrigated with plenty of normal saline to remove all meconium.
Open
Statement
For high pressure distal colostogram, a number 8 French Foley catheter is normally used in the mucous fistula.
Open
Statement
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.
Open
Statement
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.
Open
Statement
In rectal urethral prostatic fistula, the common wall with urethra is shorter than bulbar fistula but more rectal dissection is needed to gain length.
Open
Statement
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.
Open
Quotation
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.
Open
Quotation
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.
Open
Statement
Cloaca patients have never been seen with disorder of sexual differentiation; they are all females with normal ovaries.
Open
Statement
Cloaca with common channel less than 3 centimeters can be repaired posterior sagittally with total urogenital mobilization.
Open
Quotation
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.
Open
Statement
Rectal perineal fistula patients with normal sacrum and no tethered cord have 100% chance of bowel control.
Open
Statement
Malformations with better prognosis for bowel control will suffer from more constipation.
Open
Statement
Rectal vestibular fistula patients with normal sacrum and no tethered cord have 95% chance of bowel control.
Open
Statement
Cloaca with common channel less than 3 centimeters and normal sacrum has generally about 70% chance of bowel control.
Open
Statement
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.
Open
Quotation
We think that the only indication to keep a colostomy would be incapacity to form solid stool.
Open
Statement
Patients prefer the quality of life of a pull-through with bowel management rather than with a colostomy.
Open
Statement
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.
Open
Quotation
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
Determining which enema works for each child is a trial and error process that usually takes about one week.
Open
Statement
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
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).
Open
Statement
The enema should be done at the same time every day and the entire process (administration, holding, evacuation) should last one hour.
Open
Statement
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.
Open
Statement
Enema frequency is always once daily; concentration is adjusted rather than volume or frequency.
Open
Statement
The Malone procedure (appendicostomy) is not the treatment for fecal incontinence; the treatment is finding the enema that works. The appendicostomy provides independence.
Open
Quotation
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.
Open
Statement
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.
Open
Statement
For constipation management, disimpaction is required before determining laxative dosage to avoid severe cramping.
Open
Statement
Disimpaction protocol consists of 3 enemas per day for 3 days; most children are disimpacted after day 3.
Open
Statement
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).
Open
Statement
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.
Open
Statement
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.
Open
Error Traps and Culture of Safety in Anorectal Malformations
Quotation
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
Patients with anorectal malformations need long-term follow-up in colorectal, urology, and gynecology.
Open
Anorectal Malformations with Dr. Andrea Bischoff
Statement
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.
Open
Quotation
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.
Open
Statement
In a newborn baby, a normal caliber anus should accommodate a number 12 Hegar dilator.
Open
Quotation
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.
Open
Statement
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.
Open
Statement
30% of patients with anorectal malformation will have cardiac anomalies, but in only 10% of them are these anomalies hemodynamically significant.
Open
Statement
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
Tethered cord has more influence on the urinary tract rather than the gastrointestinal tract in terms of prognosis.
Open
Statement
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.
Open
Statement
The advantage of primary newborn repair is that bowel preparation is not required since meconium is considered sterile.
Open
Statement
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.
Open
Quotation
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.
Open
Quotation
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.
Open
Statement
Patients with anorectal malformation have one chance to have the right operation; secondary operations or re-operations usually change the prognosis for bowel control.
Open
Statement
An 8 French feeding tube should be used to catheterize a suspected vestibular fistula if it cannot be visualized.
Open
Statement
The ideal colostomy should be totally diverting, located in the descending colon to leave enough distal bowel for pull-through and avoid prolapse.
Open
Statement
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.
Open
Statement
During colostomy creation, all distal bowel should be irrigated with plenty of normal saline to remove all meconium.
Open
Statement
For high pressure distal colostogram, a number 8 French Foley catheter is normally used in the mucous fistula.
Open
Statement
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.
Open
Statement
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.
Open
Statement
In rectal urethral prostatic fistula, the common wall with urethra is shorter than bulbar fistula but more rectal dissection is needed to gain length.
Open
Statement
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.
Open
Quotation
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.
Open
Statement
Cloaca patients have never been seen with disorder of sexual differentiation; they are all females with normal ovaries.
Open
Quotation
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.
Open
Statement
Cloaca with common channel less than 3 centimeters can be repaired posterior sagittally with total urogenital mobilization.
Open
Quotation
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.
Open
Statement
Rectal perineal fistula patients with normal sacrum and no tethered cord have 100% chance of bowel control.
Open
Statement
Malformations with better prognosis for bowel control will suffer from more constipation.
Open
Statement
Rectal vestibular fistula patients with normal sacrum and no tethered cord have 95% chance of bowel control.
Open
Statement
Cloaca with common channel less than 3 centimeters and normal sacrum has generally about 70% chance of bowel control.
Open
Statement
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.
Open
Quotation
We think that the only indication to keep a colostomy would be incapacity to form solid stool.
Open
Statement
Patients prefer the quality of life of a pull-through with bowel management rather than with a colostomy.
Open
Quotation
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
Determining which enema works for each child is a trial and error process that usually takes about one week.
Open
Statement
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
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).
Open
Statement
The enema should be done at the same time every day and the entire process (administration, holding, evacuation) should last one hour.
Open
Statement
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.
Open
Statement
Enema frequency is always once daily; concentration is adjusted rather than volume or frequency.
Open
Quotation
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.
Open
Statement
The Malone procedure (appendicostomy) is not the treatment for fecal incontinence; the treatment is finding the enema that works. The appendicostomy provides independence.
Open
Statement
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.
Open
Statement
For constipation management, disimpaction is required before determining laxative dosage to avoid severe cramping.
Open
Statement
Disimpaction protocol consists of 3 enemas per day for 3 days; most children are disimpacted after day 3.
Open
Statement
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).
Open
Statement
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.
Open
Statement
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.
Open
Andrea's statements about Cloaca 3 statements
Cloaca - Prental Imaging & Diagnosis - Counseling
Statement
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
Open
Quotation
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.
Open
Statement
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
Open
Andrea's statements about Colorectal / ARM & Hirschsprung 267 statements
Open the Colorectal / ARM & Hirschsprung collection →
Cloaca - Prental Imaging & Diagnosis - Counseling
Statement
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
Open
Quotation
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.
Open
Statement
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
Open
History of Hirschsprung Disease
Statement
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.
Open
Statement
Harald Hirschsprung was a pediatrician who developed the hydrostatic reduction of ileocolonic intussusception.
Open
Statement
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
Resection of the distal non-dilated portion was not used as a treatment for Hirschsprung disease prior to 1946.
Open
Statement
Barium enema technique became the standard diagnostic test for Hirschsprung disease in 1948, with Dr. Swenson involved in this publication.
Open
Statement
Barry Shandling, who worked in Canada, proposed punch biopsies for newborns that required no closure nor anesthesia.
Open
Statement
Dr. Syndergaard from Sweden performed the first successful operation for total colonic aganglionosis in 1953, doing a colon resection with an ileo-anal anastomosis.
Open
Statement
Dr. Swenson observed that when patients had a colostomy the obstruction was relieved, but after closing the colostomy the disease returned.
Open
Statement
Dr. Swenson scoped from the rectum when the patient had a colostomy and saw there was no true obstruction.
Open
Statement
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.
Open
Statement
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.
Open
Surgical Procedures for Hirschsprung Disease
Statement
Transanal approach results in absolutely no scar, and patients have minimal postoperative pain.
Open
Statement
Transanal approach results in absolutely no scar, and patients have minimal postoperative pain.
Open
Statement
Patients with total colonic aganglionosis have suboptimal long-term results and a high incidence of complications.
Open
Statement
Patients with total colonic aganglionosis have suboptimal long-term results and a high incidence of complications.
Open
Statement
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.
Open
Statement
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.
Open
Statement
Pouch pull-through is not recommended for patients with Hirschsprung disease due to obstructive symptoms.
Open
Statement
Pouch pull-through is not recommended for patients with Hirschsprung disease due to obstructive symptoms.
Open
Statement
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.
Open
Statement
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.
Open
Statement
For total colonic aganglionosis diagnosed in a healthy newborn, perform colectomy with straight ileoanal anastomosis and ileostomy at presentation.
Open
Statement
For total colonic aganglionosis diagnosed in a healthy newborn, perform colectomy with straight ileoanal anastomosis and ileostomy at presentation.
Open
Statement
Close the ileostomy in total colonic aganglionosis only when the child is toilet-trained for urine and willing to accept rectal irrigation.
Open
Statement
Close the ileostomy in total colonic aganglionosis only when the child is toilet-trained for urine and willing to accept rectal irrigation.
Open
Statement
Patients with total colonic aganglionosis have a higher risk for enterocolitis, and the best treatment for enterocolitis is rectal irrigation.
Open
Statement
Patients with total colonic aganglionosis have a higher risk for enterocolitis, and the best treatment for enterocolitis is rectal irrigation.
Open
Statement
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.
Open
Statement
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.
Open
Statement
Leaving unused colon in total colonic aganglionosis can lead to enterocolitis from mucus accumulation and infection, and it's difficult to irrigate.
Open
Statement
Leaving unused colon in total colonic aganglionosis can lead to enterocolitis from mucus accumulation and infection, and it's difficult to irrigate.
Open
Statement
The PHOX2B gene provides instructions for making a protein that acts early in development to promote nerve cell formation and regulate neuron maturation.
Open
Statement
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.
Open
Statement
PHOX2B mutation is associated with congenital central hypoventilation syndrome, neuroblastoma, and Hirschsprung disease.
Open
Statement
PHOX2B mutation is associated with congenital central hypoventilation syndrome, neuroblastoma, and Hirschsprung disease.
Open
Statement
Patients with congenital central hypoventilation syndrome need tracheostomy and assisted ventilation during sleep.
Open
Statement
Patients with congenital central hypoventilation syndrome need tracheostomy and assisted ventilation during sleep.
Open
Statement
The association of Hirschsprung disease and anorectal malformation is very bad because every patient will be fecally incontinent.
Open
Statement
The association of Hirschsprung disease and anorectal malformation is very bad because every patient will be fecally incontinent.
Open
Statement
Due to Hirschsprung disease, we will resect the natural reservoir (rectosigmoid), so patients with both conditions will be fecally incontinent.
Open
Statement
Due to Hirschsprung disease, we will resect the natural reservoir (rectosigmoid), so patients with both conditions will be fecally incontinent.
Open
Statement
It is very important to discuss guaranteed fecal incontinence with parents prior to surgery for combined Hirschsprung and anorectal malformation.
Open
Statement
It is very important to discuss guaranteed fecal incontinence with parents prior to surgery for combined Hirschsprung and anorectal malformation.
Open
Outcomes and Complications in Hirschsprung Disease
Quotation
A beautiful scar because your suture is very beautiful. It's nothing compared to all the tragic things that can happen if you. With exposure, so I'm all in favor. Do your midline laparotomy, do a good surgery, make sure you have. Supply and the patient will be happy.
Open
Statement
A beautiful scar from limited exposure is nothing compared to the tragic complications that can result from inadequate exposure during surgery.
Open
Quotation
A beautiful scar because your suture is very beautiful. It's nothing compared to all the tragic things that can happen if you. With exposure, so I'm all in favor. Do your midline laparotomy, do a good surgery, make sure you have. Supply and the patient will be happy.
Open
Statement
A beautiful scar from limited exposure is nothing compared to the tragic complications that can result from inadequate exposure during surgery.
Open
Statement
Midline incisions are preferable to transverse incisions for colonic surgery because they can be extended if needed and provide excellent exposure for mobilizing splenic and hepatic flexures.
Open
Statement
Midline incisions are preferable to transverse incisions for colonic surgery because they can be extended if needed and provide excellent exposure for mobilizing splenic and hepatic flexures.
Open
Statement
In Hirschsprung disease, anal dilations are started 1 month after surgery (deeper anastomosis) compared to 14 days in anorectal malformations (skin-level anastomosis); dilations begin only if a ring-like anastomosis is palpable on digital rectal exam.
Open
Statement
In Hirschsprung disease, anal dilations are started 1 month after surgery (deeper anastomosis) compared to 14 days in anorectal malformations (skin-level anastomosis); dilations begin only if a ring-like anastomosis is palpable on digital rectal exam.
Open
Statement
For Hirschsprung dilations, start with a Hegar size close to the goal (e.g., size 12 if goal is 13), advancing weekly, because the anastomosis is usually adequate; this differs from anorectal malformations where you start with very small dilators.
Open
Statement
For Hirschsprung dilations, start with a Hegar size close to the goal (e.g., size 12 if goal is 13), advancing weekly, because the anastomosis is usually adequate; this differs from anorectal malformations where you start with very small dilators.
Open
Statement
For patients with hypermotility (tendency to diarrhea) and fecal incontinence, bowel management consists of 250 mL saline-only enemas; laxatives are contraindicated as they worsen incontinence.
Open
Statement
For patients with hypermotility (tendency to diarrhea) and fecal incontinence, bowel management consists of 250 mL saline-only enemas; laxatives are contraindicated as they worsen incontinence.
Open
Statement
Laxatives do not produce immediate, predictable bowel movements; they cause multiple unpredictable bowel movements, making fecally incontinent patients worse.
Open
Quotation
Laxatives do not produce immediate, predictable bowel movements; if we knew that you would take the laxative and you would have the immediate effect, meaning I take the pill and I will immediately go to the to the toilet and have a bowel movement, that would be perfect, but unfortunately we don't have this drug available in the market.
Open
Quotation
Laxatives do not produce immediate, predictable bowel movements; if we knew that you would take the laxative and you would have the immediate effect, meaning I take the pill and I will immediately go to the to the toilet and have a bowel movement, that would be perfect, but unfortunately we don't have this drug available in the market.
Open
Statement
Laxatives do not produce immediate, predictable bowel movements; they cause multiple unpredictable bowel movements, making fecally incontinent patients worse.
Open
Quotation
If you take laxative, you'll have many bowel movements at unpredictable time, so if you're giving laxatives to a patient that is fecally incontinent, you're just making this patient worse.
Open
Quotation
If you take laxative, you'll have many bowel movements at unpredictable time, so if you're giving laxatives to a patient that is fecally incontinent, you're just making this patient worse.
Open
Statement
Giving laxatives to force bowel movements while simultaneously giving drugs to decrease bowel motility (like Imodium) is giving drugs with opposite effects that do not work.
Open
Statement
Giving laxatives to force bowel movements while simultaneously giving drugs to decrease bowel motility (like Imodium) is giving drugs with opposite effects that do not work.
Open
Anorectal Malformations with Dr. Andrea Bischoff
Statement
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.
Open
Quotation
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.
Open
Statement
In a newborn baby, a normal caliber anus should accommodate a number 12 Hegar dilator.
Open
Quotation
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.
Open
Statement
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.
Open
Statement
30% of patients with anorectal malformation will have cardiac anomalies, but in only 10% of them are these anomalies hemodynamically significant.
Open
Statement
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
Tethered cord has more influence on the urinary tract rather than the gastrointestinal tract in terms of prognosis.
Open
Statement
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.
Open
Statement
The advantage of primary newborn repair is that bowel preparation is not required since meconium is considered sterile.
Open
Quotation
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.
Open
Statement
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.
Open
Statement
Patients with anorectal malformation have one chance to have the right operation; secondary operations or re-operations usually change the prognosis for bowel control.
Open
Quotation
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.
Open
Statement
An 8 French feeding tube should be used to catheterize a suspected vestibular fistula if it cannot be visualized.
Open
Statement
The ideal colostomy should be totally diverting, located in the descending colon to leave enough distal bowel for pull-through and avoid prolapse.
Open
Statement
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.
Open
Statement
During colostomy creation, all distal bowel should be irrigated with plenty of normal saline to remove all meconium.
Open
Statement
For high pressure distal colostogram, a number 8 French Foley catheter is normally used in the mucous fistula.
Open
Statement
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.
Open
Statement
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.
Open
Statement
In rectal urethral prostatic fistula, the common wall with urethra is shorter than bulbar fistula but more rectal dissection is needed to gain length.
Open
Statement
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.
Open
Quotation
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.
Open
Statement
Cloaca patients have never been seen with disorder of sexual differentiation; they are all females with normal ovaries.
Open
Quotation
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.
Open
Statement
Cloaca with common channel less than 3 centimeters can be repaired posterior sagittally with total urogenital mobilization.
Open
Quotation
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.
Open
Statement
Rectal perineal fistula patients with normal sacrum and no tethered cord have 100% chance of bowel control.
Open
Statement
Malformations with better prognosis for bowel control will suffer from more constipation.
Open
Statement
Rectal vestibular fistula patients with normal sacrum and no tethered cord have 95% chance of bowel control.
Open
Statement
Cloaca with common channel less than 3 centimeters and normal sacrum has generally about 70% chance of bowel control.
Open
Statement
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.
Open
Quotation
We think that the only indication to keep a colostomy would be incapacity to form solid stool.
Open
Statement
Patients prefer the quality of life of a pull-through with bowel management rather than with a colostomy.
Open
Quotation
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
Determining which enema works for each child is a trial and error process that usually takes about one week.
Open
Statement
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
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).
Open
Statement
The enema should be done at the same time every day and the entire process (administration, holding, evacuation) should last one hour.
Open
Statement
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.
Open
Statement
Enema frequency is always once daily; concentration is adjusted rather than volume or frequency.
Open
Statement
The Malone procedure (appendicostomy) is not the treatment for fecal incontinence; the treatment is finding the enema that works. The appendicostomy provides independence.
Open
Quotation
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.
Open
Statement
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.
Open
Statement
For constipation management, disimpaction is required before determining laxative dosage to avoid severe cramping.
Open
Statement
Disimpaction protocol consists of 3 enemas per day for 3 days; most children are disimpacted after day 3.
Open
Statement
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).
Open
Statement
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.
Open
Statement
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.
Open
Hirschsprung Disease: Surgical Procedures
Statement
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
Patients with total colonic aganglionosis have higher risk for enterocolitis, and the best treatment for enterocolitis is rectal irrigation.
Open
Statement
If urinary sodium is less than 20 millimoles per liter in a patient with ileostomy, start oral sodium replacement.
Open
Quotation
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
PHOX2B mutation is associated with congenital central hypoventilation syndrome, neuroblastoma, and Hirschsprung disease.
Open
Statement
Patients with congenital central hypoventilation syndrome (Ondine's curse) need tracheostomy and assisted ventilation during sleep because they stop breathing when they fall asleep.
Open
Statement
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.
Open
Quotation
The association of HR and anorectal malformation is a very bad association because every patient will be fecally incontinent.
Open
Hirschsprung Disease: Cases and Complications
Statement
The pathologist at the speaker's institution immediately reports the size of nerves on frozen section, and will state if they believe the specimen is still in a transition zone.
Open
Statement
The pathologist at the speaker's institution immediately reports the size of nerves on frozen section, and will state if they believe the specimen is still in a transition zone.
Open
Statement
Midline laparotomy incisions are preferred over transverse incisions for Hirschsprung surgery because they provide excellent exposure, can be extended if needed, and allow mobilization of splenic and hepatic flexures when necessary.
Open
Statement
Midline laparotomy incisions are preferred over transverse incisions for Hirschsprung surgery because they provide excellent exposure, can be extended if needed, and allow mobilization of splenic and hepatic flexures when necessary.
Open
Statement
The speaker's institution starts anal dilations in Hirschsprung patients one month after surgery, only if a ring-like anastomosis is palpable on digital rectal exam; if the anastomosis is soft, no dilations are performed.
Open
Statement
The speaker's institution starts anal dilations in Hirschsprung patients one month after surgery, only if a ring-like anastomosis is palpable on digital rectal exam; if the anastomosis is soft, no dilations are performed.
Open
Statement
The speaker's protocol for post-Hirschsprung pull-through is to keep patients on oral Flagyl (metronidazole) and rectal irrigations, tapering both down over time.
Open
Statement
The speaker's protocol for post-Hirschsprung pull-through is to keep patients on oral Flagyl (metronidazole) and rectal irrigations, tapering both down over time.
Open
Statement
In anorectal malformations, anal dilations start with a very small Hegar and increase weekly; in Hirschsprung, dilations can start near the goal size (e.g., size 12 if goal is 13) because the anastomosis is deeper and usually less tight.
Open
Statement
In anorectal malformations, anal dilations start with a very small Hegar and increase weekly; in Hirschsprung, dilations can start near the goal size (e.g., size 12 if goal is 13) because the anastomosis is deeper and usually less tight.
Open
Statement
Anal dilations are performed twice daily in both anorectal malformations and Hirschsprung disease, typically with diaper changes.
Open
Statement
Anal dilations are performed twice daily in both anorectal malformations and Hirschsprung disease, typically with diaper changes.
Open
Statement
For hypermotile (diarrhea-prone) Hirschsprung patients with fecal incontinence, the correct bowel management is small-volume saline enemas only (250 mL), not laxatives, fiber, or enemas with additives.
Open
Statement
For hypermotile (diarrhea-prone) Hirschsprung patients with fecal incontinence, the correct bowel management is small-volume saline enemas only (250 mL), not laxatives, fiber, or enemas with additives.
Open
Statement
Laxatives do not produce immediate, predictable bowel movements—they cause multiple unpredictable bowel movements, which worsens incontinence in fecally incontinent patients.
Open
Statement
Laxatives do not produce immediate, predictable bowel movements—they cause multiple unpredictable bowel movements, which worsens incontinence in fecally incontinent patients.
Open
Quotation
If we knew that you would take the laxative and you would have the immediate effect, meaning I take the pill and I will immediately go to the to the toilet and have a bowel movement, that would be perfect. But unfortunately we don't have this drug available in the market.
Open
Quotation
If we knew that you would take the laxative and you would have the immediate effect, meaning I take the pill and I will immediately go to the to the toilet and have a bowel movement, that would be perfect. But unfortunately we don't have this drug available in the market.
Open
Statement
Giving laxatives to force bowel movements while simultaneously giving Imodium to decrease motility is counterproductive because the drugs have opposite effects.
Open
Statement
Giving laxatives to force bowel movements while simultaneously giving Imodium to decrease motility is counterproductive because the drugs have opposite effects.
Open
Statement
Published experience with sacral nerve stimulation (InterStim) in anorectal malformations is limited to only 10 patients in the literature, without specification of malformation type or sacral quality, making results impossible to evaluate.
Open
Statement
Published experience with sacral nerve stimulation (InterStim) in anorectal malformations is limited to only 10 patients in the literature, without specification of malformation type or sacral quality, making results impossible to evaluate.
Open
Error Traps and Culture of Safety in Anorectal Malformations
Quotation
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.
Open
Quotation
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
Patients with anorectal malformations need long-term follow-up in colorectal, urology, and gynecology.
Open
Statement
Patients with anorectal malformations need long-term follow-up in colorectal, urology, and gynecology.
Open
Anorectal Malformations with Dr. Andrea Bischoff
Quotation
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.
Open
Statement
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.
Open
Statement
In a newborn baby, a normal caliber anus should accommodate a number 12 Hegar dilator.
Open
Quotation
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.
Open
Statement
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.
Open
Statement
30% of patients with anorectal malformation will have cardiac anomalies, but in only 10% of them are these anomalies hemodynamically significant.
Open
Statement
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
Tethered cord has more influence on the urinary tract rather than the gastrointestinal tract in terms of prognosis.
Open
Statement
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.
Open
Statement
The advantage of primary newborn repair is that bowel preparation is not required since meconium is considered sterile.
Open
Quotation
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.
Open
Statement
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.
Open
Quotation
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.
Open
Statement
Patients with anorectal malformation have one chance to have the right operation; secondary operations or re-operations usually change the prognosis for bowel control.
Open
Statement
An 8 French feeding tube should be used to catheterize a suspected vestibular fistula if it cannot be visualized.
Open
Statement
The ideal colostomy should be totally diverting, located in the descending colon to leave enough distal bowel for pull-through and avoid prolapse.
Open
Statement
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.
Open
Statement
During colostomy creation, all distal bowel should be irrigated with plenty of normal saline to remove all meconium.
Open
Statement
For high pressure distal colostogram, a number 8 French Foley catheter is normally used in the mucous fistula.
Open
Statement
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.
Open
Statement
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.
Open
Statement
In rectal urethral prostatic fistula, the common wall with urethra is shorter than bulbar fistula but more rectal dissection is needed to gain length.
Open
Statement
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.
Open
Quotation
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.
Open
Quotation
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.
Open
Statement
Cloaca patients have never been seen with disorder of sexual differentiation; they are all females with normal ovaries.
Open
Statement
Cloaca with common channel less than 3 centimeters can be repaired posterior sagittally with total urogenital mobilization.
Open
Quotation
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.
Open
Statement
Rectal perineal fistula patients with normal sacrum and no tethered cord have 100% chance of bowel control.
Open
Statement
Malformations with better prognosis for bowel control will suffer from more constipation.
Open
Statement
Rectal vestibular fistula patients with normal sacrum and no tethered cord have 95% chance of bowel control.
Open
Statement
Cloaca with common channel less than 3 centimeters and normal sacrum has generally about 70% chance of bowel control.
Open
Quotation
We think that the only indication to keep a colostomy would be incapacity to form solid stool.
Open
Statement
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.
Open
Statement
Patients prefer the quality of life of a pull-through with bowel management rather than with a colostomy.
Open
Statement
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.
Open
Quotation
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
Determining which enema works for each child is a trial and error process that usually takes about one week.
Open
Statement
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
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).
Open
Statement
The enema should be done at the same time every day and the entire process (administration, holding, evacuation) should last one hour.
Open
Statement
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.
Open
Statement
Enema frequency is always once daily; concentration is adjusted rather than volume or frequency.
Open
Statement
The Malone procedure (appendicostomy) is not the treatment for fecal incontinence; the treatment is finding the enema that works. The appendicostomy provides independence.
Open
Quotation
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.
Open
Statement
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.
Open
Statement
For constipation management, disimpaction is required before determining laxative dosage to avoid severe cramping.
Open
Statement
Disimpaction protocol consists of 3 enemas per day for 3 days; most children are disimpacted after day 3.
Open
Statement
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).
Open
Statement
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.
Open
Statement
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.
Open
Andrea's statements about Enterocolitis 21 statements
Open the Enterocolitis collection →
Outcomes and Complications in Hirschsprung Disease
Statement
A beautiful scar from limited exposure is nothing compared to the tragic complications that can result from inadequate exposure during surgery.
Open
Quotation
A beautiful scar because your suture is very beautiful. It's nothing compared to all the tragic things that can happen if you. With exposure, so I'm all in favor. Do your midline laparotomy, do a good surgery, make sure you have. Supply and the patient will be happy.
Open
Statement
Midline incisions are preferable to transverse incisions for colonic surgery because they can be extended if needed and provide excellent exposure for mobilizing splenic and hepatic flexures.
Open
Statement
In Hirschsprung disease, anal dilations are started 1 month after surgery (deeper anastomosis) compared to 14 days in anorectal malformations (skin-level anastomosis); dilations begin only if a ring-like anastomosis is palpable on digital rectal exam.
Open
Statement
For Hirschsprung dilations, start with a Hegar size close to the goal (e.g., size 12 if goal is 13), advancing weekly, because the anastomosis is usually adequate; this differs from anorectal malformations where you start with very small dilators.
Open
Statement
For patients with hypermotility (tendency to diarrhea) and fecal incontinence, bowel management consists of 250 mL saline-only enemas; laxatives are contraindicated as they worsen incontinence.
Open
Quotation
Laxatives do not produce immediate, predictable bowel movements; if we knew that you would take the laxative and you would have the immediate effect, meaning I take the pill and I will immediately go to the to the toilet and have a bowel movement, that would be perfect, but unfortunately we don't have this drug available in the market.
Open
Statement
Laxatives do not produce immediate, predictable bowel movements; they cause multiple unpredictable bowel movements, making fecally incontinent patients worse.
Open
Quotation
If you take laxative, you'll have many bowel movements at unpredictable time, so if you're giving laxatives to a patient that is fecally incontinent, you're just making this patient worse.
Open
Statement
Giving laxatives to force bowel movements while simultaneously giving drugs to decrease bowel motility (like Imodium) is giving drugs with opposite effects that do not work.
Open
Hirschsprung Disease: Cases and Complications
Statement
The pathologist at the speaker's institution immediately reports the size of nerves on frozen section, and will state if they believe the specimen is still in a transition zone.
Open
Statement
Midline laparotomy incisions are preferred over transverse incisions for Hirschsprung surgery because they provide excellent exposure, can be extended if needed, and allow mobilization of splenic and hepatic flexures when necessary.
Open
Statement
The speaker's institution starts anal dilations in Hirschsprung patients one month after surgery, only if a ring-like anastomosis is palpable on digital rectal exam; if the anastomosis is soft, no dilations are performed.
Open
Statement
The speaker's protocol for post-Hirschsprung pull-through is to keep patients on oral Flagyl (metronidazole) and rectal irrigations, tapering both down over time.
Open
Statement
In anorectal malformations, anal dilations start with a very small Hegar and increase weekly; in Hirschsprung, dilations can start near the goal size (e.g., size 12 if goal is 13) because the anastomosis is deeper and usually less tight.
Open
Statement
Anal dilations are performed twice daily in both anorectal malformations and Hirschsprung disease, typically with diaper changes.
Open
Statement
For hypermotile (diarrhea-prone) Hirschsprung patients with fecal incontinence, the correct bowel management is small-volume saline enemas only (250 mL), not laxatives, fiber, or enemas with additives.
Open
Statement
Laxatives do not produce immediate, predictable bowel movements—they cause multiple unpredictable bowel movements, which worsens incontinence in fecally incontinent patients.
Open
Quotation
If we knew that you would take the laxative and you would have the immediate effect, meaning I take the pill and I will immediately go to the to the toilet and have a bowel movement, that would be perfect. But unfortunately we don't have this drug available in the market.
Open
Statement
Giving laxatives to force bowel movements while simultaneously giving Imodium to decrease motility is counterproductive because the drugs have opposite effects.
Open
Statement
Published experience with sacral nerve stimulation (InterStim) in anorectal malformations is limited to only 10 patients in the literature, without specification of malformation type or sacral quality, making results impossible to evaluate.
Open
Andrea's statements about Hirschsprung disease 45 statements
Open the Hirschsprung disease collection →
History of Hirschsprung Disease
Statement
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.
Open
Statement
Harald Hirschsprung was a pediatrician who developed the hydrostatic reduction of ileocolonic intussusception.
Open
Statement
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
Resection of the distal non-dilated portion was not used as a treatment for Hirschsprung disease prior to 1946.
Open
Statement
Barium enema technique became the standard diagnostic test for Hirschsprung disease in 1948, with Dr. Swenson involved in this publication.
Open
Statement
Barry Shandling, who worked in Canada, proposed punch biopsies for newborns that required no closure nor anesthesia.
Open
Statement
Dr. Syndergaard from Sweden performed the first successful operation for total colonic aganglionosis in 1953, doing a colon resection with an ileo-anal anastomosis.
Open
Statement
Dr. Swenson observed that when patients had a colostomy the obstruction was relieved, but after closing the colostomy the disease returned.
Open
Statement
Dr. Swenson scoped from the rectum when the patient had a colostomy and saw there was no true obstruction.
Open
Statement
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.
Open
Statement
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.
Open
Surgical Procedures for Hirschsprung Disease
Statement
Transanal approach results in absolutely no scar, and patients have minimal postoperative pain.
Open
Statement
Patients with total colonic aganglionosis have suboptimal long-term results and a high incidence of complications.
Open
Statement
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.
Open
Statement
Pouch pull-through is not recommended for patients with Hirschsprung disease due to obstructive symptoms.
Open
Statement
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.
Open
Statement
For total colonic aganglionosis diagnosed in a healthy newborn, perform colectomy with straight ileoanal anastomosis and ileostomy at presentation.
Open
Statement
Close the ileostomy in total colonic aganglionosis only when the child is toilet-trained for urine and willing to accept rectal irrigation.
Open
Statement
Patients with total colonic aganglionosis have a higher risk for enterocolitis, and the best treatment for enterocolitis is rectal irrigation.
Open
Statement
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.
Open
Statement
Leaving unused colon in total colonic aganglionosis can lead to enterocolitis from mucus accumulation and infection, and it's difficult to irrigate.
Open
Statement
The PHOX2B gene provides instructions for making a protein that acts early in development to promote nerve cell formation and regulate neuron maturation.
Open
Statement
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.
Open
Statement
PHOX2B mutation is associated with congenital central hypoventilation syndrome, neuroblastoma, and Hirschsprung disease.
Open
Statement
Patients with congenital central hypoventilation syndrome need tracheostomy and assisted ventilation during sleep.
Open
Statement
The association of Hirschsprung disease and anorectal malformation is very bad because every patient will be fecally incontinent.
Open
Statement
Due to Hirschsprung disease, we will resect the natural reservoir (rectosigmoid), so patients with both conditions will be fecally incontinent.
Open
Statement
It is very important to discuss guaranteed fecal incontinence with parents prior to surgery for combined Hirschsprung and anorectal malformation.
Open
Hirschsprung Disease: Surgical Procedures
Statement
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
Patients with total colonic aganglionosis have higher risk for enterocolitis, and the best treatment for enterocolitis is rectal irrigation.
Open
Statement
If urinary sodium is less than 20 millimoles per liter in a patient with ileostomy, start oral sodium replacement.
Open
Statement
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.
Open
Quotation
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.
Open
Statement
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.
Open
Statement
PHOX2B mutation is associated with congenital central hypoventilation syndrome, neuroblastoma, and Hirschsprung disease.
Open
Statement
Patients with congenital central hypoventilation syndrome (Ondine's curse) need tracheostomy and assisted ventilation during sleep because they stop breathing when they fall asleep.
Open
Quotation
The association of HR and anorectal malformation is a very bad association because every patient will be fecally incontinent.
Open
Statement
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.
Open
Andrea's statements about Total Colonic Aganglionosis 27 statements
Open the Total Colonic Aganglionosis collection →
Surgical Procedures for Hirschsprung Disease
Statement
Transanal approach results in absolutely no scar, and patients have minimal postoperative pain.
Open
Statement
Patients with total colonic aganglionosis have suboptimal long-term results and a high incidence of complications.
Open
Statement
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.
Open
Statement
Pouch pull-through is not recommended for patients with Hirschsprung disease due to obstructive symptoms.
Open
Statement
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.
Open
Statement
For total colonic aganglionosis diagnosed in a healthy newborn, perform colectomy with straight ileoanal anastomosis and ileostomy at presentation.
Open
Statement
Close the ileostomy in total colonic aganglionosis only when the child is toilet-trained for urine and willing to accept rectal irrigation.
Open
Statement
Patients with total colonic aganglionosis have a higher risk for enterocolitis, and the best treatment for enterocolitis is rectal irrigation.
Open
Statement
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.
Open
Statement
Leaving unused colon in total colonic aganglionosis can lead to enterocolitis from mucus accumulation and infection, and it's difficult to irrigate.
Open
Statement
PHOX2B mutation is associated with congenital central hypoventilation syndrome, neuroblastoma, and Hirschsprung disease.
Open
Statement
Patients with congenital central hypoventilation syndrome need tracheostomy and assisted ventilation during sleep.
Open
Statement
The association of Hirschsprung disease and anorectal malformation is very bad because every patient will be fecally incontinent.
Open
Statement
Due to Hirschsprung disease, we will resect the natural reservoir (rectosigmoid), so patients with both conditions will be fecally incontinent.
Open
Statement
It is very important to discuss guaranteed fecal incontinence with parents prior to surgery for combined Hirschsprung and anorectal malformation.
Open
Hirschsprung Disease: Surgical Procedures
Statement
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.
Open
Statement
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.
Open
Statement
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.
Open
Statement
Patients with total colonic aganglionosis have higher risk for enterocolitis, and the best treatment for enterocolitis is rectal irrigation.
Open
Statement
If urinary sodium is less than 20 millimoles per liter in a patient with ileostomy, start oral sodium replacement.
Open
Quotation
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.
Open
Statement
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.
Open
Statement
Patients with congenital central hypoventilation syndrome (Ondine's curse) need tracheostomy and assisted ventilation during sleep because they stop breathing when they fall asleep.
Open
Statement
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.
Open
Quotation
The association of HR and anorectal malformation is a very bad association because every patient will be fecally incontinent.
Open
Summaries Andrea gave as host · 19 summaries
Recaps of other experts' statements, not Andrea's own clinical position.
Summaries Andrea gave as host · Colorectal / ARM & Hirschsprung 15 summaries
Open the Colorectal / ARM & Hirschsprung collection →
History of Hirschsprung Disease
Host summary
Andrea Bischoff summarizes what Dr. Alberto Pena said: 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.
Open
Host summary
Andrea Bischoff summarizes what Dr. Alberto Pena said: Harald Hirschsprung was a pediatrician who developed the hydrostatic reduction of ileocolonic intussusception.
Open
Host summary
Andrea Bischoff summarizes what Dr. Alberto Pena said: 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.
Open
Host summary
Andrea Bischoff summarizes what Dr. Alberto Pena said: 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.
Open
Host summary
Andrea Bischoff summarizes what Dr. Alberto Pena said: 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.
Open
Host summary
Andrea Bischoff summarizes what Dr. Alberto Pena said: Resection of the distal non-dilated portion was not used as a treatment for Hirschsprung disease prior to 1946.
Open
Host summary
Andrea Bischoff summarizes what Dr. Alberto Pena said: Barium enema technique became the standard diagnostic test for Hirschsprung disease in 1948, with Dr. Swenson involved in this publication.
Open
Host summary
Andrea Bischoff summarizes what Dr. Alberto Pena said: Barry Shandling, who worked in Canada, proposed punch biopsies for newborns that required no closure nor anesthesia.
Open
Host summary
Andrea Bischoff summarizes what Dr. Alberto Pena said: Dr. Syndergaard from Sweden performed the first successful operation for total colonic aganglionosis in 1953, doing a colon resection with an ileo-anal anastomosis.
Open
Host summary
Andrea Bischoff summarizes what Dr. Alberto Pena said: Dr. Swenson observed that when patients had a colostomy the obstruction was relieved, but after closing the colostomy the disease returned.
Open
Host summary
Andrea Bischoff summarizes what Dr. Alberto Pena said: Dr. Swenson scoped from the rectum when the patient had a colostomy and saw there was no true obstruction.
Open
Host summary
Andrea Bischoff summarizes what Dr. Alberto Pena said: 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.
Open
Host summary
Andrea Bischoff summarizes what Dr. Alberto Pena said: 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.
Open
Surgical Procedures for Hirschsprung Disease
Host summary
Andrea Bischoff summarizing the discussion: The PHOX2B gene provides instructions for making a protein that acts early in development to promote nerve cell formation and regulate neuron maturation.
Open
Host summary
Andrea Bischoff summarizing the discussion: 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.
Open
Summaries Andrea gave as host · Total Colonic Aganglionosis 4 summaries
Open the Total Colonic Aganglionosis collection →
Surgical Procedures for Hirschsprung Disease
Host summary
Andrea Bischoff summarizing the discussion: The PHOX2B gene provides instructions for making a protein that acts early in development to promote nerve cell formation and regulate neuron maturation.
Open
Host summary
Andrea Bischoff summarizing the discussion: 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.
Open
Hirschsprung Disease: Surgical Procedures
Host summary
Andrea Bischoff summarizing the discussion: 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.
Open
Host summary
Andrea Bischoff summarizing the discussion: PHOX2B mutation is associated with congenital central hypoventilation syndrome, neuroblastoma, and Hirschsprung disease.
Open