Does Delayed Diagnosis of Hirschsprung Disease Impact Post-operative and Functional Outcomes? A Multi-Center Review From the Pediatric Colorectal and Pelvic Learning Consortium
The Pediatric Colorectal and Pelvic Learning Consortium conducted a multi-center retrospective review from 2017 to 2023 examining the relationship between delayed diagnosis of Hirschsprung disease and postoperative/functional outcomes.
The study included 679 patients with Hirschsprung disease from 14 different sites.
Increased age at diagnosis was associated with a greater likelihood of undergoing fecal diversion after initial pull-through procedure.
Increased age at diagnosis was associated with an increased risk of constipation or incontinence requiring intervention postoperatively.
No association was found between age at diagnosis and 30-day complication rate after initial pull-through.
No association was found between age at diagnosis and need for pull-through revision.
Delayed diagnosis of Hirschsprung disease affects certain postoperative and functional outcomes in patients.
The Colorectal Quiz Episode 8: Motility Disorders Part 1
Failure of medical management is defined as appropriate treatment with no appropriate response
Patients who cannot take treatment (e.g., kids with autism or other cognitive problems) are considered to have failed medical management
Patients with persistent symptoms or pain with treatment, or failure to grow, are considered to have failed medical management
Failure of retrograde enemas is considered failure of medical management
Reliance on rectal therapy or retrograde enemas with continued soiling causing significant effect on functioning is considered failure of medical management
General pediatricians typically prescribe osmotic laxatives (MiraLax, lactulose) and stimulant laxatives (Senna, bisacodyl), but there are other medications GI specialists use that pediatricians are not accustomed to prescribing
Some children stool every day but don't completely evacuate
Initial evaluation includes history (triggers, stooling frequency, sensation of complete emptying), physical exam (abdominal distension, palpable stool, rectal exam), and diagnostic imaging (abdominal X-ray, water-soluble contrast enema)
In patients with long-standing constipation where appropriate treatments have been tried and failed, the first step is diagnostic studies including contrast enema to assess colonic dilation, redundancy, and ensure normal ratio
Water-soluble contrast (not barium) is used for contrast enemas because it helps empty the colon and acts as a cleanout for patients starting new medical therapy
Contrast studies are not great predictors of how patients will respond to medical or surgical management; normal-looking colons may not respond while abnormal-appearing colons may respond well
The rectoanal inhibitory reflex (RAIR) is the response where the internal anal sphincter relaxes when the rectum becomes distended with stool
Host summaryRod summarizing the discussion — not the host's own clinical position9:11
Anorectal manometry uses a catheter with sensors measuring pressure and a balloon to assess anal sphincter function and its relationship to the rectum
High pressures on anorectal manometry may suggest underlying inability to relax, causing a functional obstruction
Patients with Hirschsprung disease have an absent rectoanal inhibitory reflex (RAIR)
Anorectal manometry provides information about defecation dynamics by having patients bear down, squeeze, and attempt to defecate, comparing sensation to defecate with internal anal sphincter response
Doctor Levitt states he doesn't know how he survived without anorectal manometry testing
Expert opinionMarc Levitt10:47
In the past, surgeons did not understand the major role the sphincter played in many patients
Expert opinionJason Frischer10:51
Colonic motility assessment is critical because in the past, colons or sigmoid colons were resected based on appearance, but patients had motility disorders and did not need resection
Many dilated colons will respond to treatment
Anorectal manometry is critical for determining whether a patient needs surgery or resection, as patients with motility disorders do not need surgery
Host summaryRod summarizing the discussion — not the host's own clinical position12:03
Colorectal Quiz Episode 16: Bowel Management in Spinal Patients - Need for a Urologist Part 2
When a 7 cm appendix is shared between urology and colorectal surgery, the urologist typically takes 6.25 cm leaving only 0.75 cm for colorectal use
Urologists typically take a 70/30 split when sharing the appendix
Expert opinionMarc Levitt1:38