Hirschsprung Disease Part 2
With Dr. Mark Levitt · hosted by Dr. Todd Ponsky · Marc Levitt
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
The vast majority of patients with Hirschsprung disease do extremely well after pull-through, with no emptying problems and normal bowel control
Problem patients after pull-through divide into two types: obstruction patients who cannot empty, and soiling patients
Enterocolitis after a well-done pull-through is not uncommon, particularly in babies, because babies have very tight sphincters capable of staying tight for many hours
After about age one, patients should learn to empty and relax their sphincters with a more normal bowel movement pattern
Evaluation of post-pull-through obstruction involves a contrast study of the colon and an examination under anesthesia
Anatomic causes of post-pull-through obstruction include distal stricture, obstructing cuff, atonic Duhamel pouch, twisted pull-through (up to 360 degrees), and dilated distal segment
Pathologic cause of obstruction: pull-through not done to ganglionated bowel with normal-sized nerves (transition zone)
Healthy pull-through segment requires ganglion cells present and nerve roots no bigger than 40 microns; larger nerves indicate transition zone bowel that might not function
Treatment for post-pull-through enterocolitis: hydration, intravenous metronidazole (most effective antibiotic), and aggressive irrigations two to three times daily
Irrigation technique: 10-20 cc per kilo into large Foley (size 20-22), allow to drip out, repeat while moving tube to wash colon interior
Metronidazole has same efficacy IV or PO because in both cases it is excreted in the bile
In diverted colon (with ileostomy), metronidazole will not work for colitis because drug exits via ileostomy; vancomycin enemas needed instead
Approximately 15-20% of patients can have an enterocolitis episode within the first year after pull-through, but after one year they should not be having enterocolitis
On contrast study, pull-through should hug the sacrum; if diverted forward by space-occupying mass, suspect obstructing cuff
Obstructing cuff may be palpable on digital rectal exam as rubbery thick rubber-band structure around pull-through along sacral hollow, but is outside the lumen and not visible on endoscopy
For transition zone pull-through causing obstruction, management is redo pull-through to healthy ganglionated bowel, often requiring removal of retained sigmoid curve
For obstructing cuff, dissect between bowel and cuff, then make second plane outside cuff in Swenson plane; remove posterior-lateral ring (not entire circumference) to break the ring and solve obstruction
Myectomies that have been traditionally successful may have been cutting the Soave cuff rather than internal sphincter, but technique varies widely between surgeons
Myectomies can hurt skeletal muscle and leave patient incontinent; strongly argue against them
Post-pull-through sphincter problems are relatively rare compared to anatomic problems; not usually the sphincter causing obstruction
Many Hirschsprung patients have tight sphincters on anorectal manometry, with powerful internal sphincter that fails to relax
For obstructed child with no anatomic problem and confirmed sphincter dysfunction on manometry, Botox is indicated
Botox acts as temporary myectomy; preferred over permanent myectomy because it wears off as child learns sphincter coordination, avoiding permanent incontinence risk
Botox timing strategy: inject, then at 4-8 weeks start aggressive laxatives as Botox wears off, helping child learn appropriate bowel movement pattern
Botox can temporarily improve cuff obstruction if injection migrates to cuff level, but patient will recur because cuff must be removed for long-term fix
Anorectal manometry can distinguish sphincter dysfunction (1 cm high-tone zone) from sphincter plus cuff (3-4 cm high-tone zone)
Redo of Duhamel pouch is probably the hardest operation in Hirschsprung disease due to pelvic fibrosis from stapled connection
All patients with Hirschsprung disease should be able to empty spontaneously and should be clean
Patients with Hirschsprung disease are born with normal anal canal and normal sphincters; if anything, sphincters are too good
No Hirschsprung patient is born with missing anal canal or weak sphincter; when present, these are iatrogenic from surgeon starting transanal dissection too low and invading dentate line
Sphincter destruction can result from overstretching during aggressive transabdominal or transanal dissection
Capacity for bowel control in soiling patients determined by sphincter integrity (assessed by visual exam, digital exam, anorectal manometry) and dentate line preservation
Contrast study serves as 'poor man's colonic manometry': narrow non-dilated pull-through suggests hypermotility; dilated pull-through suggests hypomotility
Soiling patient with intact anal canal/sphincter and non-dilated colon: hypermotile, needs slowing with constipating diet, loperamide, water-soluble fiber
Soiling patient with intact anal canal/sphincter and dilated colon: hypomotile, needs laxatives to speed up, has capacity for bowel control
Soiling patient with destroyed anal canal/sphincter and non-dilated colon: hypermotile without continence capacity, needs small-volume enema plus constipating regimen
Soiling patient with destroyed anal canal/sphincter and dilated colon: needs larger volume enema, no hypermotility treatment
Hirschsprung soiling differs from anorectal malformation incontinence because Hirschsprung patients ought to have good anal canal and sphincter; if absent, it relates to surgery
Patients with capacity for bowel control (intact sphincter/anal canal) may receive bridge enema program for social continence while transitioning to appropriate medical regimen
Surgical management for soiling patients without capacity for bowel control: Malone appendicostomy or cecostomy