The Colorectal Quiz Episode 10: Total Colonic Hirschsprung Disease Part 1
With Dr. Jason Fisher & Dr. Mark Levitt · hosted by Dr. Rod Gerardo & Dr. Amanda Jensen · Colorectal Channel
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
10% of newborns with meconium plug have Hirschsprung disease
Newborns with meconium plug should receive biopsy for Hirschsprung disease to avoid missing the diagnosis and having the child suffer months of constipation, poor feeding, and distension
In a newborn with meconium plug and Hirschsprung disease, the initial contrast enema shows what appears to be a meconium plug but is actually a segment of Hirschsprung disease
In total colonic Hirschsprung disease, contrast enema shows an amorphous, cylindrical colon without the classic narrowing at the rectum compared to the sigmoid seen in typical Hirschsprung disease
The age cutoff for switching from suction rectal biopsy to full-thickness biopsy is around 6 months
At 10 months of age, full-thickness biopsy is preferred over suction biopsy to ensure a definitive diagnosis
Suction biopsies are problematic when they do not provide a definitive diagnosis, requiring a subsequent trip to the OR for formal biopsy
If the patient is doing well and can be managed with irrigations, it is preferable to have final pathology diagnosis of Hirschsprung disease from rectal biopsy before entering the abdomen
Standard biopsy locations during operative mapping include rectosigmoid, proximal sigmoid around the left colon, splenic flexure/transverse colon, and right colon or hepatic flexure area
After meconium passage, obtaining another contrast image will show the characteristic appearance of Hirschsprung disease
Common causes of failure to pass meconium include Hirschsprung disease, meconium plug syndrome, meconium ileus, and anorectal malformation
Rare causes of failure to pass meconium include small left colon syndrome, hypothyroidism, opiates in the newborn system, magnesium sulfate from maternal preterm labor treatment, milk protein allergy, and microcolon intestinal hypoperistalsis syndrome
Rectal biopsies should be attempted preoperatively before proceeding to the operating room
If the patient is not doing well with irrigations, a procedure such as ileostomy or leveling colostomy is necessary to relieve pressure
Loop ostomies should be avoided in Hirschsprung disease because stool will likely spill into the non-functional part of the colon
A transition zone can often be visualized during surgery (open or laparoscopic), but when it cannot be seen, biopsies should be obtained at multiple levels
It is acceptable to not perform a definitive procedure if only frozen section is available and there is uncertainty about the frozen section diagnosis
Surgeons can wait for final pathology and return another day to perform the pull-through procedure if needed