Hirschsprung Disease: Update Course 2013
With Dr. Dr. Langer & Dr. Dr. Thayer · hosted by Dr. Todd Ponsky · StayCurrentMD
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 newborn bowel obstruction with distended abdomen and distal air, start with contrast enema rather than upper GI
Bilious emesis typically prompts upper GI, but newborn distal obstruction is an exception where contrast enema is more informative
Tissue diagnosis is absolutely required before operating for Hirschsprung disease
False positive contrast enemas showing transition zones in newborns without Hirschsprung disease have been documented in published literature
In rare cases of severe enterocolitis, a patient may require emergency diversion before tissue diagnosis is available, particularly if presenting on Friday with pathology results not available until Wednesday
Female patients with Hirschsprung disease may have higher rates of long-segment disease than males
The incidence of long-segment Hirschsprung disease in girls is fifty-fifty
Myectomy is an extremely difficult technical operation with poor success rates in the speaker's experience
For older children (age 5-6 and up), manometry showing normal recto-anal inhibitory reflex rules out Hirschsprung disease without need for biopsy
Absence of recto-anal inhibitory reflex on manometry requires biopsy because there can be false absence of the reflex
There are two definitions of ultra-short segment Hirschsprung disease: (1) absence of recto-anal inhibitory reflex with normal ganglion cells (internal sphincter achalasia, not true Hirschsprung), and (2) very short segment of aganglionosis
The anal canal in an adult is 3 to 4 centimeters long, compared to 1 centimeter in an infant, which affects biopsy interpretation
Hypertrophic nerves should not be present in normal anal canal, even though there is normal dropout of ganglion cells
Most cecal perforations from Hirschsprung disease are not total colonic disease but shorter segment disease where the cecum becomes distended and perforates
In long-segment Hirschsprung disease, wait 6 to 12 months before pull-through to allow stoma output to thicken and prevent severe perianal excoriation
When transition zone is in ascending colon (just cecum remaining), results of pulling cecum down are poor due to stasis and enterocolitis; better to do ileal Duhamel
If transition zone is at hepatic flexure rather than ascending colon, preserve the colon and bring it down
In older children (age 2-3 and up), suction rectal biopsy should not be performed; instead do open rectal biopsy under general anesthesia
In 3-year-old with severely dilated colon from chronic Hirschsprung disease, the dilated segment is unlikely to collapse and should be resected
Attempting to resect severely dilated colon transanally causes enormous stretch on the sphincter and impairs postoperative continence
Dilated colon in 3-year-old with Hirschsprung disease can shrink down with diverting stoma and irrigations over 6-8 months
The older the child, the less likely severely dilated colon will shrink down with diversion
A patient at a well-known Ohio institution had multiple normal colonoscopic biopsies but subsequent rectal biopsy showed aganglionosis and hypertrophic nerves
The incidence of enterocolitis is significantly higher (approximately double) in children with trisomy 21 compared to genetically normal children with Hirschsprung disease
Chronic oral metronidazole is used liberally for recurrent enterocolitis and some children need it for three months or longer
Botox injection decreased the number of hospitalizations for enterocolitis in published study, though it does not always work
Dr. Langer has published articles in a Seminars in Pediatric Surgery issue on Hirschsprung disease less than a year ago
A significant portion of fellows at a Washington DC course said they would get upper GI first in newborn with bilious emesis before contrast enema
8% of cases in Mana Proctor's paper showed short-appearing transition zones on imaging that were actually long-segment disease pathologically
Dr. Pena and Dr. Levitt do not believe in ultra-short segment Hirschsprung disease and do not perform myectomies