StayCurrentMD · Panel Discussion and Case Presentation Part II: Pediatric Bowel Management 2013
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Video33 min·Published May 2013Older

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

With Dr. Alp Numoglu & Dr. Michael Alshaus & Dr. Alberto Pena · StayCurrentMD
Cued at 12:56 · stops at 13:41 · press play
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What the experts said53 expert statements · 11 host summaries
For total colonic Hirschsprung disease, Duhamel procedure is preferred initially.
ClinicalAlp Numoglu
For shorter-segment Hirschsprung disease, laparoscopic biopsy to establish the level, followed by laparoscopic-assisted pelvic dissection and perirectal dissection to join the dissection lines.
ClinicalAlp Numoglu
Transanal approach after de la Torre technique used for the last 40 cases, with laparoscopy in some cases to confirm ganglionosis level.
ClinicalStephanie
For total colonic Hirschsprung disease, delaying the pull-through and performing ileoanal anastomosis similar to total colectomy patients, with protective ileostomy.
ClinicalMichael Alshaus
Pure transanal approach used for shorter-segment disease and some redo pull-throughs when ganglion cell location is accurately determined.
ClinicalMichael Alshaus
In Spain, de la Torre technique is used; for total colonic aganglionosis, Lester Martin procedure is used.
ClinicalAlberto Peña
About half of the 11 partners do Soave, the other half do Swenson procedures; laparoscopic leveling is performed, some use umbilical incisions depending on contrast enema findings.
Clinical
Some partners doing Soave are switching to 'Soaven'—a very short Soave cuff transitioning to Swenson plane a couple centimeters above the dentate line.
Clinical
When the anal canal is destroyed, the patient will not have bowel control; with total colonic aganglionosis producing liquid stool, there is no bowel management possible.
ClinicalAlberto Peña
A permanent stoma is indicated when the anal canal is destroyed and the patient has total colonic aganglionosis.
ClinicalAlberto Peña
Sometimes constipating diet and fiber are tried to convince parents that there is no other option except permanent stoma; this is one of the few indications for permanent stoma.
ClinicalAlberto Peña
For a patient previously operated for Hirschsprung disease suffering from enterocolitis with normal rectal biopsy, rectal irrigation is the treatment.
ClinicalAlp Numoglu
Before surgery, parents must demonstrate rectal irrigations to nursing staff on the floor and be signed off before proceeding to surgery.
ClinicalMonica
For patients seen in clinic while waiting for surgical date, irrigation teaching and demonstration occur in clinic.
ClinicalMonica
Parents are taught rectal irrigation on the ward by nurses; personal control is performed to ensure parents know how to irrigate before hospital discharge and before surgery.
ClinicalStephanie
Common irrigation problems: parents hesitant to advance catheter far enough, or not using enough saline to get clear return before finishing.
ClinicalMonica
In the background of Hirschsprung disease, enterocolitis is not simple gastroenteritis; children are often taken to other medical centers and treated as simple gastroenteritis by doctors unaware of enterocolitis.
ClinicalAlp Numoglu
Parents are continuously taught to return to the specialist hospital for enterocolitis, not general hospitals.
ClinicalAlp Numoglu
Families are made very comfortable with irrigations before going to the emergency room, because many places are uncomfortable with irrigations in general.
ClinicalMonica
Most pediatricians worldwide do not know the difference between enterocolitis and gastroenteritis; they do not understand the entity called post-Hirschsprung enterocolitis.
OpinionAlberto Peña
Pediatricians do not understand why irrigations are necessary; they see dilated bowel on X-ray and think it is intestinal obstruction, not recognizing the entity itself.
OpinionAlberto Peña
When starting dissection 2 cm above the dentate line and pulling bowel through, the upper mucosa is often damaged, and the anastomosis ends up about 1 cm above the dentate line.
ClinicalAlberto Peña
Dr. Pena does not believe that leaving 1-2 cm of rectal mucosa is the simple cause of enterocolitis.
OpinionAlberto Peña
A group in New York doing neonatal Soave primary procedures reported zero enterocolitis; when Dr. Pena followed some of those patients, many had fecal incontinence.
ClinicalAlberto Peña
If you produce fecal incontinence in a patient, enterocolitis is zero; a patient with destroyed anal canal is equivalent to a stoma, and patients with stomas rarely have enterocolitis.
ClinicalAlberto Peña
A good operation preserving the sphincter and anal canal creates sphincter closure, which creates stasis, and stasis produces enterocolitis.
ClinicalAlberto Peña
Dr. Pena prefers to deal with enterocolitis rather than fecal incontinence; fecal incontinence is for life.
OpinionAlberto Peña
GI doctors recently started doing more anorectal manometry; it is rare for a post-op Hirschsprung patient to have normal anorectal manometry, leading to misinformation.
ClinicalMichael Alshaus
Some Hirschsprung patients are told by GI doctors they have chronic bacterial overgrowth syndrome and started on antibiotics, when they likely have enterocolitis; antibiotics alone are not the total solution.
ClinicalMichael Alshaus
Young parents often feel irrigations are a chore and tough on their babies; it is crucial to instruct them, stress the importance, and teach excellent technique.
ClinicalMichael Alshaus
Always use saline for irrigations, not regular water; importantly, warm the saline, especially for neonatal babies, to keep body temperature normal.
ClinicalMonica
Dr. Pena's incidence of enterocolitis in pull-through patients is about 30%.
EpidemiologicalAlberto Peña
Using Duhamel technique, there is a low incidence of enterocolitis, but no explanation for this.
EpidemiologicalStephanie
Resecting the rectosigmoid introduces a major pathophysiological change; children with perfect pull-throughs preserving the anal canal sometimes have toilet-training problems without explanation.
ClinicalAlberto Peña
Hyperactive children with attention deficit disorder have more toilet-training problems because they have a piece of colon that does not act like a reservoir, connected to the rectum and moving constantly; significant cooperation from the child is required.
ClinicalAlberto Peña
Even with a perfect operation, patients may have certain toilet-training problems; if the anal canal is destroyed, they will be totally incontinent; partial anal canal destruction causes more problems.
ClinicalAlberto Peña
In manometry studies of the colon, migrating complexes or high-amplitude contractions stop in the sigmoid colon in most people and do not go to the rectum; after pull-through, these are moved down to the anus.
ClinicalMichael Alshaus
Toilet-training Hirschsprung patients do not always get the same warning of impending bowel movement and do not have as much time; must factor this in and use the gastrocolic reflex.
ClinicalMichael Alshaus
Dr. Pena does not find rectal manometry useful in Hirschsprung disease, constipation, or anorectal malformations after many years of experience.
OpinionAlberto Peña
Patients are discharged with three irrigations per day and metronidazole (Flagyl); every month the number of irrigations is decreased.
ClinicalAlberto Peña
If decreasing irrigations causes recurrent enterocolitis and the patient does not tolerate lack of irrigation, and by 6 months post-op the patient is still on irrigations, parents become very nervous; at that point, other options are discussed.
ClinicalAlberto Peña
For refractory enterocolitis, another option is further resection of normal ganglionic colon to remove more.
ClinicalAlberto Peña
Hirschsprung disease is much more than ganglion cells vs. no ganglion cells; we do not know why some patients never have enterocolitis and toilet-train early, behaving like normal children, while others have severe enterocolitis from day one.
OpinionAlberto Peña
'Benign Hirschsprung disease' patients present at 8-10 years old with classic imaging and abdominal distention but never had enterocolitis, grew and developed normally, and do very well after surgery.
ClinicalAlberto Peña
In the United States, earlier diagnosis is being made of patients with 'bad Hirschsprung'—enterocolitis from day one, very sick, and high incidence of enterocolitis after surgery.
EpidemiologicalAlberto Peña
There is much we do not know about Hirschsprung disease; the story is much more than absent ganglion cells, and taking bowel with normal ganglion cells down does not mean that bowel is 100% normal.
OpinionAlberto Peña
Some believe that ganglionic bowel may have neuronal intestinal dysplasia, but this is a very controversial histopathological diagnosis; we do not know what is wrong and must learn much more.
OpinionAlberto Peña
Another option for obstructive Soave cuff is laparotomy or laparoscopy to split the cuff in front of the sacrum without resection.
ClinicalAlp Numoglu
Dr. Pena is skeptical that the cuff produces obstruction; to believe it, he would need to see the cuff producing real obstruction manifested by very dilated colon above the cuff, which is very unusual.
OpinionAlberto Peña
Dr. Mark Levitt has experience dealing with obstructive cuffs transanally; laparoscopic approach is not a bad idea but Dr. Pena has never heard of it being done laparoscopically.
ClinicalAlberto Peña
Patients with total fecal incontinence have no enterocolitis; all operations moving toward fecal incontinence (myectomies, myotomies, Botox, massive dilatation, putting 3 fingers in the rectum) are temporary or permanent moves toward incontinence.
ClinicalAlberto Peña
Patients subjected to myotomies, myectomies, or repeated Botox injections eventually develop more severe fecal incontinence.
ClinicalAlberto Peña
Dr. Pena does not believe in myectomy/myotomy/Botox procedures and does not use Botox.
OpinionAlberto Peña
In Italy, Soave approach was used initially, but switched to transanal approach in the last 2 years with laparoscopic biopsies; very satisfied with results.
Host summary
For a patient with total colonic aganglionosis, previously operated, suffering from fecal incontinence and severe diaper rash, with destroyed anal canal, treatment is a permanent stoma.
Host summary
The only contraindication for irrigation is a recent operation; after a recent operation, the surgeon who operated should perform irrigation immediately post-op to avoid perforating the anastomosis.
Host summary
When re-biopsying patients suspected of having a transition zone or aganglionic pull-through, biopsy as high as possible above the anastomosis to avoid the problem of finding aganglionic tissue at the anastomosis level.
Host summary
Metronidazole (Flagyl) is given orally for better effect; when tapering, sometimes given with irrigation through the rectum.
Host summary
After a biopsy, wait 48 hours before starting rectal irrigation; patients are taught irrigation in clinic first, then biopsy is done later so it is not a fresh incision.
Host summary
Fecal incontinence is more frequently seen in Swenson and Soave operations compared to Duhamel and Rehbein.
Host summary