Hirschsprung’s Disease (HD) and Enterocolitis

ExclamationWe all will recognize that delayed passage of meconium (no BM in the first 48 hours of life) is concerning for Hirschsprung’s Disease. Well, that should mean that it isn’t an entity that I need to consider in the ED (they will be diagnosed before hospital discharge). If only life were so clear-cut.

It is possible for the Hirschsprung’s Disease to be diagnosed later on in childhood.

  • Older infants and children that present with “chronic constipation” that does not respond to the typical bowel concoctions should have this condition on their differential diagnosis list.
    • Needing a daily enema is never a good thing.
  • Often the abdomen will be distended and there may be palpable loops of bowel. Additionally, the rectal exam often has no stool in the vault and after removing your digit there is an explosion of “stuff.” I am not a huge advocate for rectal exams, but this is one of the few times when the exam is useful.

So, yes, you may diagnosis it in the ED. But once you diagnosis it and refer them to the surgeon and the segment is removed, the condition is cured and all is well… right? Again, not so fast.

Enterocolitis and Hirschsprung’s Disease

 

  • The classic findings are:
    • abdominal distension,
    • fever, and
    • foul smelling stool.
  • Also commonly seen: vomiting, explosive diarrhea, lethargy, rectal bleeding, and overt shock.
  • The presentation of Enterocolitis can be similar before and after surgical correction.
    • Patients that are diagnosed with HD after their 1st week of life are at greater risk for developing enterocolitis (so that kid you see in the ED).
  •   The patient may present initially with Enterocolitis, but may still develop it after surgical management. One recent report described enterocolitis leading to death in patients from 3 weeks to 20 months after surgical repair.

Moral of the story: the child that has had surgery for his or her HD has not been made “normal” and should still be considered as potentially having a significant and life-threatening condition.

The child with Hirschsprung’s Disease, even s/p surgery, should not be considered to have “gastroenteritis” until enterocolitis is thoroughly evaluated (including discussing with your friendly Peds Surgeon).

 

Marty, T.L., M.E. Matlak, M. Hendrickson, R.E. Black, and D.G. Johnson : Unexpected death from enterocolitis after surgery for Hirschsprung’s disease. Pediatrics, 96:118, 1995

Elhalaby, E.A., Coran, A.G., Blane, C.E., Hirschi, R.B., Teitelbaum, D.H. : Enterocolitis Associated with Hirschsprung’s Disease: A Clinical-Radiological Characterization Based on 168 Patients. Journal of Pediatric Surgery, 30:1; 1995, pp. 76-83.

Sean Fox

I enjoy taking care of patients and I finding it endlessly rewarding to help train others to do the same. I trained at the Combined Emergency Medicine and Pediatrics residency program at University of Maryland, where I had the tremendous fortune of learning from world renown educators and clinicians. Now I have the unbelievable honor of working with an unbelievably gifted group of practitioners at Carolinas Medical Center. I strive every day to inspire my residents as much as they inspire me.

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