Hirschsprung Disease: Update Course 2013
With Dr. Jacob Langer & Dr. Jason Fisher & Dr. Christine Thayer · hosted by Dr. Todd Ponsky · StayCurrentMD
Cued at 16:09 · stops at 16:54 · 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 newborn bowel obstruction with distal air, contrast enema should precede upper GI unless clinical presentation strongly suggests malrotation
False positive contrast enemas showing transition zones can occur in newborns without Hirschsprung disease
In Mana Proctor's series, 8% of cases with apparent short transition zone on imaging had pathologically higher transition zones (long transition zone concept)
Female patients may have higher risk of long-segment disease, with 50-50 incidence of long-segment in girls
Prone positioning for pull-through is easier on surgeon's back and neck compared to lithotomy
Manometry showing normal recto-anal inhibitory reflex rules out Hirschsprung disease in older children without need for biopsy
Absence of recto-anal inhibitory reflex on manometry requires biopsy confirmation due to false positives
Two definitions exist for ultra-short segment Hirschsprung: (1) absence of recto-anal inhibitory reflex with normal ganglion cells on biopsy (internal sphincter achalasia, not true Hirschsprung), and (2) very short segment of aganglionosis
For ultra-short segment with confirmed aganglionosis, pull-through is preferred over myectomy
Most 16-year-olds with newly diagnosed Hirschsprung require diversion due to massive bowel dilation
Laparoscopic Duhamel is preferred for older patients because thickened rectum makes transanal dissection difficult and causes excessive sphincter stretch
Adult anal canal is 3-4 centimeters long (possibly 5 cm), compared to 1 cm in infants; biopsies at 3 cm in adults may be from anal canal where ganglion cells are normally absent
Transitional epithelium (not normal rectal mucosa) should be seen on biopsy if specimen is truly from anal canal
Most cecal perforations in Hirschsprung disease occur with shorter-segment disease (not total colonic), as cecum distends most and perforates like in rectal cancer
For cecal perforation, close perforation and create loop ileostomy without frozen section of ileum, as total colonic disease is unlikely
In long-segment disease, delay pull-through 6-12 months until stoma output thickens to prevent severe perianal excoriation
Pull-throughs using only cecum (very short colonic segment) have poor outcomes with stasis and enterocolitis; ileal Duhamel may be preferable
If transition zone is at hepatic flexure (not just cecum), preserve the colon and perform Duhamel
When bringing right colon down, flip it over rather than rotating; preserve marginal artery and let anatomy determine orientation
Manometry is not reliably performed until age 5-6 years
For 3-year-old with massive dilation, divert and attempt bowel decompression; resect dilated segment if it does not collapse after 6-8 months
Transanal resection of massively dilated colon in older children causes enormous sphincter stretch and impairs postoperative continence; laparoscopic dissection to pelvic floor is preferred
Older children are less likely to have bowel shrinkage after diversion, but 3-year-olds may still respond
Colonoscopic biopsies can miss Hirschsprung disease; rectal biopsy is more reliable
For recurrent enterocolitis, first rule out distal obstruction and residual aganglionosis with exam under anesthesia and biopsies
Chronic oral metronidazole is used liberally for recurrent enterocolitis; some patients require it for months, and symptoms recur when discontinued
Botox injection decreases number of hospitalizations for enterocolitis, though it does not work in all patients
Tissue diagnosis is mandatory before surgery for Hirschsprung disease; contrast enema alone is insufficient
In critically ill patients with enterocolitis, surgery may be necessary before pathology results are available (typically takes until Wednesday if specimen obtained Friday)
Hypertrophic nerves should not be present in normal anal canal, even though ganglion cells are absent there
In children over age 2-3 years, perform open rectal biopsy under general anesthesia rather than office suction biopsy due to patient cooperation issues
Manometry is reliable for diagnosing Hirschsprung in older children when biopsies may be unreliable
For enterocolitis 6 months post-pull-through with fever, distention, and diarrhea but no peritonitis, treat with IV fluids, broad-spectrum antibiotics, and rectal irrigations
Incidence of enterocolitis in children with trisomy 21 is double that of genetically normal children with Hirschsprung disease