Radiology and Image Diagnosis of Hirschsprung Disease
With Dr. Steven Kraus & Dr. Rodrigo Ocelami & Dr. Alberto Peña · hosted by Dr. Todd Ponsky · StayCurrentMD
Cued at 24:22 · stops at 25:07 · press play
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
In the 1970s fluoroscopy was done by everybody very well; nowadays with MRI, CT, and ultrasound there has been a shift and fluoroscopy is almost a lost art.
Multiple abnormalities can be seen on plain radiographs in Hirschsprung disease: distal bowel obstruction, dilated colon, and bowel mucosal irregularities.
In a newborn you cannot tell the difference between colon and small bowel on plain radiograph; you can only say there are multiple dilated loops suggesting distal bowel obstruction.
Bubbles of meconium in newborn bowel are not necessarily pneumatosis; this is a fairly common appearance for meconium distributed throughout bowel.
The most common causes of distal bowel obstruction in neonates are Hirschsprung disease, small left colon syndrome (meconium plug syndrome/immature colon), anorectal malformation, meconium ileus, and ileal atresia—these five make up about 99% of cases.
Bowel wall thickening and striations on plain radiograph may indicate spasm and suggest Hirschsprung disease; with bowel thickening you must also think of enterocolitis.
Seeing air in the rectum does not rule out Hirschsprung disease.
The radiological diagnosis of enterocolitis is possible with a plain abdominal radiograph.
Air-fluid levels in the colon on cross-table or decubitus view are a sign of inflammatory process or enterocolitis in the colon.
Enterocolitis in a newborn is Hirschsprung disease until proven otherwise.
Total intestinal Hirschsprung disease is very, very rare; Dr. Kraus had seen only one case.
The contrast enema in a newborn does not always allow making the diagnosis or ruling out Hirschsprung disease.
The false negative rate may be lower in the hands of experienced fluoroscopists, but taking all comers the rate is 20-25%.
Total colonic Hirschsprung disease is a very difficult diagnosis to make on enema.
Short segment disease is also difficult to diagnose, partly due to technique and how the enema is performed.
If you find a high transition zone on enema, the actual pathologic transition could be anywhere; repeat enemas in long segment disease are futile and will not give better indication of transition location.
In a patient with longer segment disease, plan the operation thinking the transition may be high rather than relying on the first enema to determine the correct operation.
Dr. Ocelami's technique: insert rectal tube only 2-3 centimeters into the rectum, never use a Foley catheter inside the rectum, hold the buttocks tight or ask older child to hold contrast.
In the neonatal period use water soluble contrast diluted 50% contrast and 50% saline, infuse very gently and very slowly with a syringe (not gravity) under fluoroscopy to avoid distending the aganglionic segment.
After the neonatal period, fill only up to the transverse colon; if the studied segment is normal in distension, caliber, and mucosa, and the splenic flexure shows no suggestion of total colonic disease, stop at the transverse colon.
Obtain two images in left lateral decubitus, two in right lateral decubitus, and two in AP position; remove the tube to get a good look at the rectum without the tube.
The main goal is to find the transition zone; as soon as you find it, stop the contrast because the diagnosis is made.
The inversion of the rectal sigmoid index is very helpful and valued as a sign.
The affected or aganglionic segment will be spastic; if you give contrast with too much volume or too fast you will miss the spasticity of the affected segment.
Irregularity of the bowel and enterocolitis are good signs to look for on enema.
Always talk to the family after the study; ask for good hydration after using barium or water soluble contrast to help the child evacuate and avoid dehydration.
Always show the family the contrast used so when white stuff comes out they know what is going on.
The main points of technique: never use Foley inside the rectum, inject contrast very slowly and gently using fluoroscopy all the time, look for the signs, and talk to the family before and after the study.
Dr. Kraus uses iodinated water soluble contrast with osmolality about 400, which is hyperosmotic and similar to agents used to clean the colon; it helps make the diagnosis and attempts to clean the colon.
If contrast stays in little neonates they can get dehydrated and run into trouble; the neonatal ICU must be aware of this.
Dr. Kraus uses gravity infusion from a bag with very large tubing at a moderate pace (not slow) to show distal and proximal parts quickly and see the transition zone rapidly.
Early maximal distention is best to see the transition zone; if you wait a long time you can distend the distal aganglionic part because it is soft tissue, not a lead pipe.
If the colon in the neonate looks small, fill the entire colon and attempt to reflux into the terminal ileum to make other diagnoses if present.
Dr. Kraus uses a Foley catheter in full-term infants (12-14 French) and smaller size in premature infants.
On a true lateral image the femurs are on top of each other and the presacral space is well seen; you really want to attempt to get that view.
On frontal view make sure you see the tube but also see distal to it; do not cut off the rectum or you will miss a very distal transition zone.
In a normal neonate the proximal colon toward the splenic flexure is always a little bit smaller than the rectum.
If you have a Foley catheter blown up in the distal rectum to prevent leakage, you will miss short segment Hirschsprung disease every single time.
Use a tube without a balloon, or if you use a balloon push it in further so it does not block the end of the colon.
Rectosigmoid transition cases are usually concordant pathologically and radiologically.
In total colonic Hirschsprung the rectum does not look bigger than the rest of the colon like it should be; when you see a colon that is one smallish size all the way through, think about total colonic disease.
The enema in a premature infant does not follow the rules; it could be immature and look small, so you cannot tell the difference between immaturity and Hirschsprung.
Contrast enemas can be done in premature infants; anything greater than 35 to 36 weeks gestational age is reasonable for diagnostic accuracy.
If you go back further in gestational age when necrotizing enterocolitis becomes much more prevalent, you will not have the same diagnostic accuracy.
If the rectum and sigmoid transition is at about S2 or distal to S1-S2, it is distal rectal disease; if more proximal than that, it is typical rectosigmoid transition.
Small left colon syndrome (meconium plug syndrome) usually has a transition at the splenic flexure that is very abrupt.
A case with small rectum on scout, small colon to splenic flexure, and meconium plugs appeared to be small left colon but was actually Hirschsprung disease with total colonic aganglionosis and terminal ileum transition.
If you have a proximal transition zone you cannot accurately say where that transition is; this should make you think about doing something more invasive rather than just transanal approach.
Dr. Ponsky always performs suction rectal biopsy in almost any patient who needed a contrast enema to rule out distal obstruction, whether meconium plug, small colon, or other cause.
Dr. Ponsky performs rectal biopsy even in meconium ileus cases.
Dr. Peña would not do biopsy if sure it is meconium ileus, but would do biopsy for a picture of small left colon because he does not know how to distinguish it from Hirschsprung.
In Soave procedure there is partial thickness dissection leaving a cuff of tissue; if the cuff is prominent it causes a very wide presacral space best seen on lateral view.
It is very important in post-surgical patients to get a really good true lateral view of the rectum to assess presacral space.
The Duhamel procedure is a chimera of part of the aganglionic segment distally with more proximally the ganglionic segment, not put side to side or end to end but a patchwork.
In symptomatic Duhamel patients you see an extra pouch anteriorly containing stool; enlargement of this pouch and its impression on the ganglionic bowel causes the obstruction.
Dr. Peña wonders why Duhamel patients get dilated rectum; by definition Hirschsprung disease is a condition where the aganglionic segment does not get distended even after 10-15 years, yet in these patients the rectum is very stretched and fecally impacted.
Dr. Peña suspects that all patients who developed the traditional Duhamel pouch may never have had Hirschsprung disease; he invites surgeons to orient the resected pouch and have pathologists study whether there are ganglion cells in that rectum.
Dr. Collins has seen resected Duhamel pouches that had both ganglionic and aganglionic parts, but does not know what their contrast enemas looked like.
Dr. Peña states that if he sees a patient with a contrast enema showing redundant stool-filled colon with normal rectum, he will not take a rectal biopsy because it is a waste of time and the patient has no Hirschsprung disease.
Dr. Peña states there is no way to differentiate so-called ultra-short segment Hirschsprung from idiopathic constipation.