# Contrast Enema for Hirschsprung Disease — GCMD Library

Dr. Steven Kraus discusses his contrast enema techniques in Hirschsprung disease. He discusses gravity infusion, lateral rectosigmoid image, AP rectosigmoid, use of Foley catheters and rectal tubes, and transition zone accuracy.

Type: video · 11 min · posted 2019-01-11
Canonical: https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034

## Chapters
- [0:00](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=0) Contrast enema technique for Hirschsprung diagnosis
- [2:43](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=163) Normal anatomy and Hirschsprung variants
- [4:18](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=258) Gestational age limitations
- [4:52](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=292) Interactive case: small left colon vs long-segment Hirschsprung
- [8:27](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=507) Panel debate: rectal biopsy indications

## Statements
- "Iodinated water-soluble contrast with osmolality approximately 400 is used, similar to colon prep agents" (clinical) [0:03](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=3)
- "Hypertonic contrast helps make the diagnosis and attempts to clean the colon" (clinical) [0:21](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=21)
- "Neonates can become dehydrated if contrast remains in the colon due to hyperosmotic effect" (clinical) [0:26](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=26)
- "Gravity infusion from a bag with large-bore tubing is used rather than injection" (clinical) [0:41](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=41)
- "Moderate-pace infusion (not slow) allows rapid visualization of distal and proximal segments to identify transition zone" (clinical) [1:03](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=63)
- "Lateral rectosigmoid imaging is performed" (clinical) [1:24](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=84)
- "Early maximal distention is best for seeing transition zone; delayed imaging can distend the aganglionic distal segment and obscure the transition" (clinical) [1:31](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=91)
- "AP rectosigmoid image is obtained; if colon appears small in neonate, entire colon is filled with attempt to reflux into terminal ileum to identify other diagnoses" (clinical) [1:51](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=111)
- "12-14 French Foley catheter is used in full-term neonates; smaller size in premature infants" (clinical) [2:08](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=128)
- "Normal rectum is larger than proximal colon (toward splenic flexure)" (clinical) [2:43](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=163)
- "Inflated Foley balloon positioned in distal rectum will miss ultra-short segment Hirschsprung every time" (clinical) [3:01](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=181)
- "Tube without balloon should be used, or balloon should be pushed further proximally to avoid obscuring distal colon" (clinical) [3:15](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=195)
- "Rectosigmoid transition Hirschsprung cases are usually concordant pathologically and radiologically" (clinical) [3:30](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=210)
- "Rectosigmoid transition is located at approximately S2 vertebral level" (clinical) [5:51](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=351)
- "Distal rectal Hirschsprung is distal to S1-S2; more proximal is typical rectosigmoid transition" (clinical) [5:55](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=355)
- "Small left colon syndrome typically has transition at splenic flexure that is very abrupt" (clinical) [6:20](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=380)
- "Rectosigmoid index (rectum larger than sigmoid) is a useful principle but insufficient—must image to splenic flexure" (clinical) [7:54](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=474)
- "Proximal transition zones on contrast enema cannot accurately predict histologic transition location" (clinical) [9:10](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=550)
- "Uncertain proximal transition should prompt more invasive surgical approach (laparoscopic or open) rather than transanal" (opinion) [9:28](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=568)
- "Contrast enema is reliable for diagnosis at gestational age 35-36 weeks and above" (clinical) [4:30](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=270)
- "Below 35-36 weeks gestational age, enema does not follow diagnostic rules due to colonic immaturity and overlap with necrotizing enterocolitis" (clinical) [4:08](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=248)
- "Rectal biopsy should be performed in almost any patient requiring contrast enema for distal obstruction, including meconium plug and small left colon" (opinion) [10:18](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=618)
- "Rectal biopsy may be omitted in clear meconium ileus with reflux into terminal ileum and clinical improvement" (opinion) [10:44](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=644)
- "Rectal biopsy would not be performed if diagnosis is clearly meconium (ileus)" — Pena (opinion) [10:58](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=658)
- "Rectal biopsy should be performed for small left colon appearance because it cannot be reliably distinguished from Hirschsprung" — Pena (opinion) [11:03](https://library.globalcastmd.com/watch/contrast-enema-for-hirschsprung-disease-1034?t=663)

## Transcript
To describe a little bit about our technique. Our techniques are very similar. We use an iodinated contrast. It's a water soluble contrast. It is a little bit hyper osmotic, and a lot of the radiologists in particular aren't really aware of all the osmoalities of all the different contrasts, but the one we use is about 400. So it's all, it's very similar to these agents that you use to clean the colon out. So not only does it help to make the diagnosis. But it also will attempt to clean the colon. And in the neonates, it's important to know that, uh, because if the contrast does stay there, uh, the, the little babies, the little neonates can get dehydrated and then they could have run into trouble. And so we make sure that the, uh, the floor, the neonatal ICU knows that. In any event, we use a rectal, uh, a rectal tube in the neonates, um, we tend, and I'll show pictures of them, uh, we use gravity infusion rather than injection. I'm not sure if it really matters a whole lot, but we use a gravity gravity drip, not from a bottle, but from a bag that has very large IV, very large tubing. And actually we get actually infuse the, the fluid at a rate that's not really slow. It's actually a moderate pace to actually show the distal part and the proximal part quickly so that you can actually see a rapid, a rapid picture of the of the transition zone. I feel that that's, you know, fairly important. We do the Lateral rectosigmoid image in the lateral view, as does my, uh, my colleague in Chicago. Early maximal distention is actually the best to see the transition zone because if you wait a long time, you can actually get distention of the distal part, which is, uh, which is the, the, the, uh, the Hirsch involving the Hirschsprung's disease, which is the distal part. And that's because it's not a lead pipe. It is a soft piece of tissue, and you can, uh, distend it. We get an AP image of the rectosigmoid, uh, and again, if the colon and the neonate looks small, we actually fill the entire colon and attempt to reflux into the terminal ileum to make other. Diagnosis, uh, if they are there. Here's the patient in the left side down position, as you can see there. There are the tubes. In a neonate. I tend to use a Foley catheter in a full-term infant, about 12 to 14 French, and in a premature infant, if it's are a little bit premature, a little bit less, um. Here's a normal contrast enema for your, for an example. You can see the nice, well distended rectum. The presacral space is well seen. And why is it well seen? Because the femurs are on top of each other. It's a true lateral image, and you really would like to attempt to get that. Then we get a frontal view, as you can see here, all the way down, making sure that you do see the tube, but you also see a little distal to it. You don't want to cut off the rectum to miss a very distal transition. transition zone, a very distal Hirschprus, but notice that the proximal colon is much always a little bit smaller here in the toward the splendid fletcher than the rectum, and that's a normal appearance. Here's a Hirschsprung disease patient, or at least an enema that reflects Hirschsprung's disease. Note that you can see a very short segment. And if you have a Foley catheter that's blown up in here and you put the Foley here to prevent leakage, you'll miss this every single time. And that's how sometimes the diagnosis of Hirschsprung disease is not made. They consider this rest of the colon a normal size, and it's negative. So you have to make sure you use some kind of a tube that doesn't have a balloon on it. Or at least when you have the balloon in, push the balloon in a little further so it doesn't block the end of the colon. Here's the left side. On the right side here, here's a rectosigmoid transition, the typical rectosigmoid Hirschprung's disease. These are usually concordant pathologically and radiologically. Now here's a patient who has long segment disease. Note that the rectum is very small. Here's the Foley catheter blown outside and held up against the recti anus to prevent the leakage. And you can see that there is actually a very, very small, irregular, spastic type of colon to the level of the splenic flexure. And here's total colonic Hirschsprung's disease. The rectum does look pretty small. It's not bigger than the rest of the colon like it should be. And so this, when you see a colon that's one smallish size all the way through, you have to think about total colonic disease. But you could say, well, if this is a little bit of a premature infant, it's just immature, it could be. And actually the enema in a in a in a premature infant does not follow the rules. It could be immature and look small, so you really can't tell the difference. What age would you go down to to do or size would you go down to to do a contrast enema? Well, we can do contrast enemas in even young premature infants. That would be worthwhile, that would actually give you a reasonable result. Um, I would probably say anything greater than 35 to 36 weeks. If you go back further and you get into patients with necrotizing enterocolitis and that when that becomes much more, uh, uh, uh, uh, uh, prevalent, then I think you're not gonna have the same, uh, diagnostic, uh, accuracy. I'm looking at this contrast, Emma, where's the transition zone? Is it proximal 1 or distal 2? So you're going to get a poll to vote. That's Rectosigmoid, descending column, transverse column, ascending column. Everybody can vote. And we have more than now we have a oh now we changed. So actually while we're putting the pole up, could you go back and show that film again? OK, so here's the enema. It's in a right lateral position. It's not left lateral, but here's the enema and So we have 90% saying rectosigmoid. Well, that's good because that's where it is. That's where the radiographic one is, right? So here you can see that there is a transition at the rectosigmoid. The rectum and sigmoid transition is about S2 or so. So if it's a distal in the rectum, it's going to be distal to S1, S2. If it's more proximal than that, then that's the typical rectosigmoid transition. Um, now, here's a 2 year old male, I'm sorry, a 2 day old male with failure to pass meconium, and here's an enema. So There's the lateral view And there's the frontal view. So what do we call this one? Now remember too that there are other diagnoses, and one of the other diagnoses in a patient of this age is small left colon. Usually their transition is at the splenic flexure, and usually it is very abrupt, like this case. So what does everybody think? Actually we don't have the the pull for that. So actually, why don't we say, do we think this is, I can make one right now. So what do we do we think this is small left colon or meconian plug syndrome, or do we think this is Hirschsprung's disease at the splenic flexure, which is a long segment. So maybe long segment versus small left colon. OK, because this is instructive. And it actually it look, it looks at the principle of is the transition zone accurate in long segment disease. 1, small left colon. 2, meconium plug. Oh, OK. And then long segment disease, is that OK? I can change it. That's good. OK. And we have people voting in all options. Good. Looks like the majority is seeing small colon. Yes, and, and that's, that's what I thought, right? Right. That's the, that's the radiologic, um, dogma is a picture like this. This is a slam dunk, OK. And if you look at the lateral view. The rectum is or isn't bigger than the sigmoid. This is a good point about the rectosigmoid index. Is the rectosigmoid index always good? And I say no. I mean, it's, it's a good principle. If you have a bigger rectum than sigmoid, it's probably normal, but don't stop there. Go all the way up to the splenic flexure. And in this particular case, this patient. Has what has a small rectum on the scout, a small colon to the splenic flexure, and meconium plugs. There's meconium plugs in there, so this is small left colon. Well, is it? No, this was Hirschsprung disease. In fact, can you tell me where the histologic transition should be? So based on that picture that we just saw, This picture, where do you think the transition is? Is it here or is it somewhere else? So a lot of people are saying the transition is at the splenic Fletcher. OK. And actually a lot of, well, actually, About half the people are saying proximal to this planet Fletcher. Well, actually, that's pretty good. OK, so here's the picture. The answer was that this was total. colonic a ganglionosis and in fact the transition was in the terminal ileum. So I just want to bring home the point that if you have a proximal transition zone, you really can't accurately say where that transition is. And I'm not sure how they booked this case, if they did it transantally or if they did it an open procedure or laparoscopically, but I do know that it was a transition zone that was much more proximal, and I think that would make you. Uh, should make you think about doing something that's more invasive rather than just the transianal approach because you really might not know where it is. And actually, actually, as, uh, Dr. Collins was saying, is doing, uh, biopsies and going through the colon, uh, maybe even, you know, the laparoscopic approach would have been good probably for this case as well, right, uh, Todd, right. But can I ask the panels, if you get a, and we get this every once in a while, you get a contrast sentiment and it looks like small left colon. Is anyone going to not perform a rectal biopsy? I always do. So other than the game of am I right at getting question, can I make it even more broad? I would do it. I do it in almost any distal, any patient that needed a contrast enema. To rule out a distal obstruction, I'm going to be doing a suction rectal biopsy, whether it's a meconium plug or a small colon. Or whatever it is, I'm going to, I'm going to be doing a suction rectal biopsy. What about Maconium ileus? Even meconium milius. Yep, definitely. Would you not? I see a blank stare. I think if it's clearly a case of meconium ileus and you reflux into the terminal ileum and you're able to get a response and the patient clinically improves, yeah, I'm not going to do it. I haven't done a rectal biopsy. Dr. Pena, any thoughts? Perhaps I would not do it if I'm sure that it is meconium. I would not do it. But what about a picture of a small left colon? Would you always do a, I would do a biopsy. I would do it because I, I, I don't know how to distinguish this from this one.

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Not medical advice · citation policy: https://library.globalcastmd.com/ai