From
Colorectal Channel
The Colorectal Quiz Episode 3.5: Proximal Hirschsprung Disease
hosted by Dr. Rod Gerardo & Dr. Todd Ponsky
Chapter 1 of 5 · Case-Based Learning
Case recap
Introduction and Case Recap
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Host summaries · secondary, not cited in answers
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 plain abdominal x-ray showed a big right colon, prominent transverse colon, and a compressed left colon with small lumen, along with possible small bowel dilation.
It is hard on a newborn film to really discern small and large bowel, and you can get fooled.
A baby with bilious emesis and non-classic abdominal x-ray could have had distal air and then had a volvulus, requiring urgent upper GI to rule out malrotation before contrast enema.
A limited upper GI was performed and ruled out malrotation in this child.
The contrast enema showed an impressive right colon with transverse colon tapering off, and a transition zone probably somewhere in the transverse colon.
Patients with proximal Hirschsprung disease are at risk of perforation, usually in the cecum.
To get a perforation, you need distension, and if you have a transition zone at the hepatic flexure, then all the pressure is in the right colon.
A baby that doesn't have a competent ileosecal valve might be saved from perforation because pressure can decompress into the small bowel.
You are obligated at some point, maybe after resuscitation, to get a rectal biopsy in a patient with suspected Hirschsprung disease.
If the transition zone is at the hepatic flexure, you can predict that enough pressure builds up in the right colon to have the cecum perforate.
You rarely get a perforation in a more standard sigmoid level transition zone.
A suction rectal biopsy confirmed the diagnosis of Hirschsprung disease in this patient.
Proximal Hirschsprung disease and distal Hirschsprung disease require two different operative approaches.
A one-day-old full-term infant weighing 3.9 kilograms presented with significant abdominal distension and bilious emesis.
If a child is sick with bilious emesis and distension, resuscitation should be the first step before diagnostic workup.