Hirschsprung's Disease

Published: 2020-10-15

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Inside this episode

Kai, the Library's AI content creator, listened to this episode and mapped who's speaking, the chapters, key claims, and cases. Every item links to the exact moment in the recording.

AI-enriched

Who's speaking

Jason Frischer — guest
Aaron Garrison — guest
Speaker 3 — host
Rod Girardo — host

Chapters

0:00Introduction and Epidemiology — Introduction of speakers and definition of Hirschsprung disease. Discussion of incidence (1 in 5,000), genetic associations including RET gene, family history in 10% of cases, and syndromic associations including trisomy 21, Waardenburg syndrome, and congenital central hypoventilation.
1:55Diagnostic Workup — Review of three diagnostic modalities: contrast enema showing transition zone and rectosigmoid ratio <1.0; rectal biopsy as definitive diagnosis showing absent ganglion cells and hypertrophic nerves; and anorectal manometry demonstrating absent rectoanal inhibitory reflex.
5:08Initial Management and Surgical Goals — Family education, teaching rectal irrigations, and management of neonates versus older children. Three surgical goals: identify extent of aganglionic segment, resect that segment, and restore bowel continuity.
6:59Surgical Techniques — Detailed description of three surgical approaches: Swenson (full thickness anastomosis 1-2 cm above dentate line), Suave (mucosectomy with anastomosis within aganglionic cuff), and Duhamel (posterior approach with common wall creation using stapler).
11:46Complications and Enterocolitis — Early complications including diaper rash, anastomotic leak, and Hirschsprung-associated enterocolitis. Treatment of enterocolitis with fluid resuscitation, digital rectal exam, colonic irrigations, and antibiotics. Long-term prognosis with 80% experiencing constipation and most achieving continence by kindergarten.
14:51Management of Poor Outcomes and Clinical Pearls — Systematic approach to patients not doing well post-operatively, including evaluation for stricture, anatomic problems, or functional issues. Clinical pearls including stopping dissection beyond splenic flexure, ensuring proper exposure and dentate line identification, and teaching families irrigations before discharge.

Key claims

0:07Hirschsprung disease is a congenital developmental anomaly of intestinal ganglion cell migration that results in a functional bowel obstruction — Aaron Garrison
0:35More than 95% of neonates pass meconium within the first 48 hours of life, and failure to pass meconium is typical of Hirschsprung's disease — Jason Frischer
0:35Some infants with Hirschsprung's disease do pass meconium — Jason Frischer
1:03Hirschsprung disease has an incidence of about 1 in 5,000 children — Aaron Garrison
1:03About 10% of children with Hirschsprung disease will have a positive family history — Aaron Garrison
1:03The RET gene is a predisposing genetic condition associated with Hirschsprung disease — Aaron Garrison
1:03Up to 10% of children with Hirschsprung's disease will have trisomy 21 — Aaron Garrison
1:03Only 1 to 2% of patients with trisomy 21 have Hirschsprung disease — Aaron Garrison
1:03Hirschsprung disease is associated with Waardenburg syndrome and congenital central hypoventilation (Ondine's curse) — Aaron Garrison
1:57The classic finding on contrast enema is a transition zone in the rectosigmoid — Jason Frischer
1:57A rectosigmoid ratio of less than 1.0 is suggestive of Hirschsprung's disease — Jason Frischer
1:57Approximately 10% of newborns with Hirschsprung's disease may lack the typical findings on a contrast enema — Jason Frischer
1:57Rectal biopsy is the true definitive diagnosis for Hirschsprung disease — Jason Frischer
1:57Typical biopsy features include absence of ganglion cells, presence of hypertrophic nerves, abnormal pattern of cholinesterase staining, and an absence of calretinine staining — Jason Frischer
1:57An adequate biopsy must be taken from the rectum at least one centimeter above the dentate line and must include both mucosa and submucosal layers — Jason Frischer
4:23In Hirschsprung's disease, anorectal manometry shows a lack of the rectoanal inhibitory reflex (RAIR) — Jason Frischer
4:23Any patient with an absent RAIR must undergo a rectal biopsy for confirmation of the diagnosis — Jason Frischer
5:12Enterocolitis is the life-threatening part of Hirschsprung's disease — Aaron Garrison
6:42There are three goals to the surgical management of Hirschsprung's disease: identify the extent of the aganglionic segment, resect that segment, and restore the bowel to its continuity — Jason Frischer
6:59The contrast enema can be used as a roadmap for surgery but is not always accurate to where the level is — Aaron Garrison
7:36The Swenson procedure involves pulling down the aganglionic bowel and performing a full thickness anastomosis one to two centimeters above the dentate line — Jason Frischer
7:36The Suave procedure involves performing a mucosectomy from a transanal approach slightly above the dentate line and pulling through the normal bowel — Jason Frischer
7:36In the Suave procedure, the aganglionic cuff can become stiff enough to cause an obstruction or an outlet obstruction — Jason Frischer
10:15The Duhamel procedure intentionally leaves a portion of the aganglionic rectum behind and brings the normally ganglionated bowel posterior to that rectum — Aaron Garrison
10:15Children with Duhamel procedure may have higher risks of having constipation and stool withholding — Aaron Garrison
10:15All three surgical techniques (Swenson, Suave, and Duhamel) are equally effective — Aaron Garrison
12:34Hirschsprung's-associated enterocolitis is likely an inflammatory condition secondary to bacterial overgrowth — Jason Frischer
12:34A child with enterocolitis presents with abdominal distension, vomiting, fever, and change in bowel habits that could be either diarrhea or not passing stool at all — Jason Frischer
12:34Treatment of enterocolitis includes fluid resuscitation, digital rectal exam, and colonic irrigations — Jason Frischer
13:54About 80% of kids with Hirschsprung's disease are constipated and will need some kind of management — Aaron Garrison
13:54Most patients with rectosigmoid type Hirschsprung disease should be continent by the time they enter kindergarten — Aaron Garrison
17:10If you're getting past the splenic flexure during surgery for Hirschsprung's disease, stop and await permanent sections and then do a diversion with an ileostomy — Aaron Garrison
17:10Patients tend to outgrow episodes of enterocolitis early in life — Aaron Garrison
18:35All patients should be able to perform irrigations before they get their pull-through and go home — Jason Frischer

This episode was analyzed and enriched by Kai, the Library's AI content creator. Every item links to the moment it comes from — click a timestamp to listen in context.

Hirschsprung Disease: When Absent Ganglion Cells Cause Functional Obstruction

The episode's main topic retold as a plain-language walkthrough — what it is, why it matters, and what the speakers concluded. Written by Kai from the episode transcript and reviewed before publishing.

For the care team · Explainer · AI-written, human-reviewed

Why This Exists as a Discipline

Hirschsprung disease is a congenital developmental anomaly of intestinal ganglion cell migration that results in a functional bowel obstruction 0:07. The aganglionic segment cannot propagate peristalsis, creating a mechanical barrier despite patent lumen. At 1 in 5,000 births, it is common enough that any busy children's hospital sees multiple cases annually 1:03. The condition requires subspecialty management because the diagnosis is subtle, the surgery is technically demanding, and the long-term complications — enterocolitis, constipation, continence issues — require coordinated follow-up that spans years.

The Core Clinical Problem

More than 95% of neonates pass meconium within 48 hours; failure to do so is typical of Hirschsprung disease, though some affected infants do pass meconium 0:35 0:35. The aganglionic bowel is contracted with narrow lumen compared to the dilated, ganglionated bowel proximal to it — this creates the transition zone visible on imaging. The life-threatening complication is Hirschsprung-associated enterocolitis, an inflammatory condition secondary to bacterial overgrowth that presents with abdominal distension, vomiting, fever, and altered bowel habits 12:34 12:34. This must be recognized and treated urgently.

About 10% of affected children have a positive family history, and the RET gene is a known predisposing factor 1:03 1:03. Up to 10% of children with Hirschsprung disease have trisomy 21, though only 1 to 2% of patients with trisomy 21 develop Hirschsprung disease 1:03 1:03. Other associations include Waardenburg syndrome and congenital central hypoventilation 1:03.

Diagnostic Approach

Three modalities are used: contrast enema, rectal biopsy, and anorectal manometry. The contrast enema shows a transition zone in the rectosigmoid and a rectosigmoid ratio less than 1.0, but approximately 10% of newborns with Hirschsprung disease lack typical findings 1:57 1:57 1:57. The study differentiates Hirschsprung disease from meconium ileus and intestinal atresia, and serves as a roadmap for surgery, though it is not always accurate regarding the proximal extent of aganglionosis 6:59.

Rectal biopsy is the definitive diagnosis 1:57. Typical features include absence of ganglion cells, hypertrophic nerves, abnormal cholinesterase staining, and absent calretinine staining 1:57. An adequate biopsy must be taken at least one centimeter above the dentate line and must include both mucosa and submucosa 1:57. Suction technique is used for infants under six months; full-thickness biopsy is considered for older patients.

Anorectal manometry demonstrates absent rectoanal inhibitory reflex (RAIR) in Hirschsprung disease 4:23. However, the reflex may be absent in other conditions, and false positives occur. Any patient with absent RAIR must undergo rectal biopsy for confirmation 4:23. Manometry is useful to rule out Hirschsprung disease, but when positive or undiagnostic, biopsy remains mandatory.

How the Surgical Approach Works

There are three goals: identify the extent of the aganglionic segment, resect that segment, and restore bowel continuity 6:42. Three surgical techniques are used — Swenson, Soave, and Duhamel — and all are equally effective 10:15.

The Swenson procedure involves full-thickness dissection and anastomosis one to two centimeters above the dentate line 7:36. This leaves minimal aganglionic bowel behind. The Soave procedure performs a mucosectomy from a transanal approach slightly above the dentate line, pulling ganglionated bowel through an aganglionic cuff 7:36. The theoretical advantage is less injury to pelvic structures, but the aganglionic cuff can become stiff enough to cause obstruction 7:36. The Duhamel procedure intentionally leaves aganglionic rectum behind and brings ganglionated bowel posterior to it, creating a common wall with a stapler 10:15. Children with Duhamel may have higher rates of constipation and stool withholding 10:15.

If dissection extends past the splenic flexure, stop and await permanent sections, then perform diversion with ileostomy rather than continuing pelvic dissection — this may indicate total colonic Hirschsprung disease 17:10.

Where Practice Is Contested

The choice of surgical technique is not evidence-based but preference-based. The discussants state that all three approaches are equally effective, and surgeons should perform the technique they are most comfortable with 10:15. The length of mucosectomy in Soave procedures has shortened over time, but no consensus threshold exists. Management of older children with dilated colon is individualized — some undergo primary pull-through, others require proximal diversion to allow colonic decompression.

When to Involve This Team

Any neonate who has not passed meconium within 48 hours should be evaluated for Hirschsprung disease. Refer urgently if enterocolitis is suspected — abdominal distension, vomiting, fever, and altered bowel habits in a child with known or suspected Hirschsprung disease require fluid resuscitation, digital rectal exam, colonic irrigations, and often broad-spectrum antibiotics including metronidazole 12:34. About 80% of children with Hirschsprung disease experience constipation requiring management 13:54. Most patients with rectosigmoid disease should be continent by kindergarten 13:54, but patients tend to outgrow episodes of enterocolitis early in life 17:10. Families should be taught rectal irrigations before pull-through surgery and should perform them before bringing the child to the emergency room if enterocolitis is suspected 18:35.

Takeaways from this story

Failure to pass meconium within 48 hours warrants evaluation for Hirschsprung disease, though some affected infants do pass meconium.
Rectal biopsy is the definitive diagnosis; it must include mucosa and submucosa taken at least 1 cm above the dentate line.
Enterocolitis is the life-threatening complication; treat with fluid resuscitation, digital rectal exam, and colonic irrigations.
All three surgical techniques (Swenson, Soave, Duhamel) are equally effective; choice depends on surgeon comfort and experience.
Most patients with rectosigmoid disease achieve continence by kindergarten, though 80% require constipation management.

Topic overview

This discussion covers Hirschsprung disease, a congenital anomaly of intestinal ganglion cell migration causing functional bowel obstruction. The speakers review diagnostic workup including contrast enema, rectal biopsy, and anorectal manometry; three surgical techniques (Swenson, Suave, and Duhamel); and management of Hirschsprung-associated enterocolitis, the life-threatening complication requiring fluid resuscitation, digital rectal exam, and colonic irrigations. Long-term prognosis for rectosigmoid disease is generally good, with approximately 80% of patients experiencing constipation requiring management and most achieving social continence by kindergarten age.

Key takeaways

Rectal biopsy is definitive for Hirschsprung diagnosis; contrast enema misses 10% of cases despite classic transition zone. (1:57)
Enterocolitis is life-threatening; treat with fluid resuscitation, digital rectal exam, and colonic irrigations. (5:12)
All three surgical techniques (Swenson, Suave, Duhamel) are equally effective despite different complication profiles. (10:15)
80% of post-operative patients experience constipation; most achieve social continence by kindergarten age. (13:54)
Adequate rectal biopsy must be >1cm above dentate line with mucosa and submucosa to assess for ganglion cells. (1:57)

Keywords

Hirschsprung disease trisomy 21 Waardenburg syndrome congenital central hypoventilation Ondine's curse meconium ileus intestinal atresia enterocolitis gastroenteritis constipation total colonic Hirschsprung disease contrast enema rectal biopsy anorectal manometry rectal irrigations

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Transcript

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Hi, I'm Jason Frischer. And I'm Aaron Garrison. We're both pediatric surgeons at Cincinnati Children's. Today we're going to review Hirschsprung's disease. Hirschsprung disease is a congenital developmental anomaly of intestinal ganglion cell migration that results in a functional bowel obstruction. I'm here today with Dr. Jason Frischer and Dr. Aaron Garrison, both pediatric colorectal surgeons at Cincinnati Children's, to discuss Hirschsprung disease. How does Hirschsprung's disease typically present? More than 95% of neonates pass meconium within the first 48 hours of life, and failure to pass meconium is typical of Hirschsprung's disease. However, some infants with Hirschsprung's disease do pass meconium. If an infant who has not passed meconium in the first 48 hours of life presents to your clinic, the possibility of Hirschsprung's disease should be entertained. How common is this condition? Are there any predisposing conditions or familial associations? So Hirschsprung disease is actually relatively common in a busy children's hospital. It's got an incidence of about 1 in 5,000 children. So it is something that does come up quite frequently. Unfortunately, about 10% of children will have a positive family history and means there is some predisposing genetic conditions such as the RET gene that can go along with that. So typically, if we have patients with strong family history, we will have them refer to genetics for evaluation and screening for other potential malignancies. As many of you know, Hirschsprung's disease is associated with trisomy 21, and up to 10% of children with Hirschsprung's disease will have trisomy 21, but only 1 to 2% of patients with trisomy 21 have Hirschsprung disease. There are a few other syndromes that are associated with Hirschsprung disease, including Wordenberg syndrome, congenital central hyperventilation, which is known as Ondine's curse, and some others. What diagnostic workups should be performed? The three studies that should be considered for evaluating for Hirschsprung's disease include contrast enema, rectal biopsy, and possibly anorectal manometry. Let's talk about those individually. A contrast enema can be performed in a child with suspected Hirschsprung disease. We typically use a water-soluble contrast enema, and that helps us differentiate between other causes of neonatal intestinal obstruction, such as meconium alias or intestinal atresia. The classic finding is a transition zone in the rectosigmoid, but that transition zone really can be located anywhere within the bowel. Other findings suggestive of Hirschsprung's disease include a rectosigmoid ratio of less than 1.0. What that really means is that the Hirschsprung's disease, the aganglionic bowel, is contracted and is narrow lumen compared to the dilated, ganglionated bowel more proximal. Also, the inability to evacuate the contrast, and in the case of total colonic Hirschsprung's disease, a foreshortened or question mark colon can be seen on the contrast enema. Approximately 10% of newborns with Hirschsprung's disease may lack the typical findings on a contrast enema. So, if there is a clinical suspicion of the disease, a rectal biopsy must be considered. The second part of a diagnostic workup for Hirschsprung's disease, and most important, is the rectal biopsy. It is the true definitive diagnosis. In a child with suspected Hirschsprung's disease, one can perform a rectal biopsy to confirm the diagnosis. Typical features on the biopsy include absence of ganglion cells, presence of hypertrophic nerves, abnormal pattern of cholinesterase staining, and an absence of calretinine staining. To be considered an adequate biopsy, it must be taken from the rectum at least one centimeter above the dentate line and must include both mucosa and submucosal layers. A rectal biopsy can be obtained through suction technique or open full thickness technique. The suction technique is typically used for patients less than six months of age, and one should consider using a full thickness technique for patients older than six months, or when a suction biopsy is inadequate. And would we get anal rectal manometry on all of these patients? Anal rectal manometry is a newer modality that is sometimes used. One must understand the findings for a patient in Hirschsprung's disease, that when doing anal rectal manometry, there is a lack of the recto anal inhibitory reflex, or RARE, R-A-I-R. This reflex may be absent in other conditions as well, and some children have a false positive test. So any patient with an absent RARE must undergo a rectal biopsy for confirmation of the diagnosis. I like to think and use anal rectal manometry to rule out Hirschsprung's disease in some patients, but when it's undiagnostic or even positive, I still perform a biopsy to provide a definitive diagnosis. What should we do once a diagnosis is made? So the first thing I usually do is talk to the family and give them education about the disease, because most of them have never heard of it and they're not sure what to expect. I usually tell them that their child will need this condition managed for life, but that our expectation is that he or she will also live a normal life with some close management and care by the doctors and nurses. The other short-term thing that we teach families to do is rectal irrigations, and this is best taught before you actually do the operation. So getting the family familiar with them, because as we'll talk about later, enterocolitis is the biggest thing that we get concerned about is it's the life-threatening part of Hirschsprung's disease. NICU babies who are diagnosed with Hirschsprung's disease will typically manage with irrigations, antibiotics if they show evidence of enterocolitis, and NPO or NG tubes if they're distended. Many times, though, they're not distended and you can kind of keep them decompressed by doing irrigations, allowing them to have breast milk until you get to the point where they're ready for surgery. Older children are a little bit more difficult in that they've had this problem since birth, and a lot of times the colon has become dilated, and so they're not amenable to just doing a primary pull-through in many cases. So children like that will start on an enema program. Some may need diversion more proximally to give the colon time to decompress. Everyone we manage a little bit differently, but those are a little bit more challenging groups to kind of decide which operation you're going to do and when. What are the goals of surgical management? There are three goals to the surgical management of Hirschsprung's disease. First, identify the extent of the aganglionic segment. Second, resect that segment. And third, restore the bowel to its continuity. What are the principles of surgical management of Hirschsprung disease? So there are a couple things to keep in mind before you do one of these operations, and that's you can use the contrast enema as a roadmap, and most times they are accurate to where the level is, but not always. So there are a few different techniques to obtain your biopsies. We usually do them laparoscopically, or we'll pull the colon out through the umbilicus to take a full thickness biopsy. The goal is to get past the aganglionic segment into the normally innervated bowel that does not have hypertrophic nerves, and ultimately to pull that segment of bowel down through and perform your anastomosis above the dentate line. What are the common surgical options for Hirschsprung's disease? There are three main surgical options that one would consider when performing surgery for Hirschsprung's disease. There's the Swenson, the Suave, and the Duamel. It's important to understand what each procedure entails, as well as the pros and cons of each. Our preferred technique is the Swenson procedure. Essentially, that's where you pull down the aganglionic bowel, you perform your full thickness anastomosis up one to two centimeters above the dentate line. We like that because it leaves really the most minimal amount of aganglionic bowel that you can, and I think it's possible to do the operation without a lot of stretch or injury to pelvic nerves and blood vessels. Some of the time you can do this completely transanally. I prefer doing a combination of laparoscopic and transanal that really minimize the amount of stretch and transanal dissection that you're doing. But it is very important to be cautious about injuring the urethra as it's quite close, especially in boys. The Swave procedure is another technique, very popular, and is actually the performing of a mucosectomy from a transanal approach slightly above the dentate line and pulling through the normal bowel. Again, this can be performed in combination with a abdominal approach, either open or laparoscopic, to do a majority of the dissection. But the actual anastomosis and very distal dissection is a mucosectomy for a determined length, which can be anywhere from one to many centimeters long. I think over the course of time, the length of that mucosectomy has gotten shorter and shorter. The actual anastomosis is performed within a cuff of aganglionic rectum. And so, in essence, the Swave procedure is performing a mucosectomy, leaving a cuff of aganglionic bowel, and you're pulling through ganglionated bowel through that aganglionated cuff down to perform your anastomosis slightly above the dentate line. The pros of this approach is theoretically less injury to pelvic structures, the nerves in that region, and other structures such as the urethra and vagina, which are in close proximity. The cons of this procedure is that there are times when that aganglionic cuff becomes stiff enough to cause an obstruction or an outlet obstruction of your pulled through ganglionated colon. And what about the Duhamel procedure? So, the Duhamel procedure is where you would intentionally leave a portion of the aganglionic rectum behind and bring the normally ganglionated bowel posterior to that rectum. You then make an anastomosis by using a stapler, and that makes a common panel between the two structures. The pro side of this is that you're not dissecting as much in the pelvis, so there may be less injury to pelvic structures. However, the downside that we see is that some of the children can have trouble evacuating that pouch. So, there may be higher risks of having constipation and stool withholding with a Duhamel. Furthermore, where you do your anastomosis, it can often leave a spur if the anastomosis is not large enough. So, sometimes we have to go in and revise that spur or make the anastomosis larger. There are three different surgical techniques that we just reviewed. All of them are equally effective, and one should perform the technique they're most comfortable with. To review, we have the Swenson technique, which is a full thickness dissection and anastomosis. There's the Suave procedure, which is a mucosectomy. You leave a cuff of aganglionic bowel and bring the ganglionated bowel through that cuff of rectum and perform your anastomosis. And then there's the Duhamel procedure, which is performing a pouch with an anastomosis of aganglionic and ganglionated bowel. What are some common complications for these operations? Dr. So, I like to divide these up into the early and the late complications. So, early on, you can see a really bad diaper rash and excoriation. It can often need to be treated like a burn. Obviously, any time you're anastomosing bowel, you have to be worried about a leak, which fortunately is rare in these patients. So, anastomotic leaks, obviously, are usually going to show up in the first week after surgery. Fever, abdominal distension, maybe free air. And that often requires another operation or a more proximal diversion, depending on the extent of the leak. But probably the main thing everyone needs to be aware of is Hirschsprung's-associated enterocolitis. This is truly a life-threatening problem that everybody needs to be educated on. How do you diagnose and treat Hirschsprung's-associated enterocolitis? Hirschsprung's-associated enterocolitis is poorly understood and likely is an inflammatory condition secondary to bacterial overgrowth. A child who presents with enterocolitis shows up to an emergency room with abdominal distension, vomiting, fever, change in bowel habits that could be either diarrhea or not passing stool at all. And this must be recognized as potential enterocolitis and treated urgently. When we treat these patients with enterocolitis, it is important to provide fluid resuscitation, digital rectal exam, and colonic irrigations. Depending on the severity of the enterocolitis, we sometimes add broad-spectrum antibiotics and we usually start these patients on metronidazole. Enterocolitis can present similarly as gastroenteritis, but because of the severity that is potential in a patient with enterocolitis, we treat them as if they have enterocolitis. And again, the key tenants to treating enterocolitis are fluid resuscitation, digital rectal exam, and colonic irrigations. Adding antibiotic coverage, whether it be through metronidazole or broad-spectrum antibiotics, is dependent on the severity of the presentation. What is the long-term prognosis for these patients? When families ask me what is their prognosis, I think it's hard to tell from the beginning because Hirschsprung's disease is such a spectrum of disease and presentations, and kids who have more syndromic types really are a different category. I would say the most common rectosigmoid type patients, though, really should do well and should be continent by the time they enter kindergarten. I do mention that about 80% of kids with Hirschsprung's disease are constipated and will need some kind of management. I think we do manage their constipation pretty aggressively here. Managing that early and aggressively, I think we prevent some of the complications and things that can show up later, such as when the bowel gets distended and defunctionalized. So assuming that the operation's been done well and that there's not any transition zone or strictures, we expect that most of the patients are going to do very well and be in kindergarten socially continent. For a patient who isn't doing well after a pull through, what are your management options? Great question. I think you have to have a systemic and organized plan when taking care of patients who aren't doing well after surgery for Hirschsprung's disease. And the first and foremost is to take obviously a good history and a good physical exam. If you find that there's a stricture at your anastomosis from a prior pull through for Hirschsprung's disease, then that needs to be addressed either through dilations or revising the pull through procedure. If you don't see a stricture on your physical exam, then typically I get a contrast enema and perform a rectal exam under anesthesia and repeat biopsy to further investigate these patients. Then you have to define whether it's anatomic problem or a functional problem. So anatomic problems can be a twist in your pull through, an obstructing cuff, a non-functioning DUAML pouch, or you could see from your biopsy you have a transition zone or continued aganglionic segment causing a functional obstruction and therefore causing problems in the patient who already had a pull through. For patients with an anatomic problem, you have to revise the pull through typically. For patients who do not have an anatomic problem, a patient can undergo bowel management if necessary, or Botox injection to relieve sphincter tone if necessary, and sometimes even further management or workup, including motility studies, may be required to further delineate if there's a segment of colon that's just not functioning properly. These are very complicated patients and a systematic approach with a physical exam and then a contrast study and an exam under anesthesia helps you start to delineate these patients. And then further treatment either with bowel management or motility studies may be required to really work up these few patients that really struggle after pull through for Hirschsprung's disease. To see our guideline on Hirschsprung's associated enterocolitis, either see the link in our outline or in the state current app, see the suspected Hirschsprung's associated enterocolitis guidelines. Darison, what are your clinical pearls for trainees out there? Things that they should know right off the top of their head when it comes to Hirschsprung's disease? I think there's a couple things or potential traps that you can fall into. So if you're in surgery doing an operation for Hirschsprung's disease and you keep leveling and you're getting past the splenic flexure, I think that's a point that I've been taught to stop and await your permanent sections and then do a diversion with an ileostomy. The reason being you don't want to start pulling through bowel that you don't need to remove. And you also don't want to start doing pelvic dissections when you're potentially dealing with a total colonic Hirschsprung's situation. I think one of the other ones is just, you know, continuing to be aware that you could have a patient with total colonic Hirschsprung's even if the contrast enema doesn't quite reflect that. And being prepared to do an ileostomy and not rushing to do something in the neonatal period, kind of taking a more long-term approach to it. I think we've all seen patients who kind of struggle with episodes of enterocolitis early in life and they ultimately just do tend to kind of outgrow it. So don't necessarily feel like there's something wrong, that you did something wrong, as long as you've kind of taken the steps that Dr. Frischer outlined above and made sure that there's nothing anatomically causing these episodes. Then making sure the family knows how to treat colitis, making sure there's no stricture, and being aggressive about medication, sometimes starting laxatives pretty early on can help quite a bit. I didn't know, Frischer, what pearls would you give? There are so many pearls, but a few that I think of are exposure. I think when doing this, the transanal dissection, having good exposure, we like to use the Lone Star Retractor. Some people use just sutures to expose the anal canal, but I think good visualization of the anal canal is important, which brings me to my next point, which is understanding the anatomy of the anal canal and really being able to identify the dentate line. So I think it is imperative that you identify the dentate line because your dissection transanally, whether that be through a Swensen, Suave, or Duhamel technique, you need to make sure that you're starting your dissection above or proximal to the dentate line in order to get a good result. When we perform our pull-throughs, we usually like to straighten out the colon and sort of remove that sigmoid or S-curve in the sigmoid to allow for sort of a straight-line evacuation path for the stool and also allows for easier irrigations when needed for patients with potential enterocolitis. Which leads me to the last point, which I think is one of the most important points, which is prior to performing a pull-through procedure, I ensure that our families are instructed and can perform irrigations so that if their child happens to have enterocolitis or develop signs and symptoms of enterocolitis, the number one treatment is irrigations. And I would have my families perform irrigations before they even bring the patient to the emergency room. And so all patients, before they get their pull-through and when they're ready to go home, are able to perform irrigations before the pull-through and go home with the proper equipment and supplies to do the irrigations. This is Rod Girardo from Cincinnati Children's Hospital, the contributing editor for this audio chapter. What else do you want to hear? Let us know in the Stay Current app, Twitter, Facebook, or Instagram pages. And remember, knowledge should be free.

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