A patient with Hirschsprung's disease may develop an enterocolitis. This can be a source of significant morbidity and mortality.


The patient may be known to have Hirschsprung's disease or the patient may first present with the enterocolitis.


Clinical findings:

(1) fever with temperature > 38°C (100.4°F)

(2) diarrhea

(3) abdominal distention

(4) crampy abdominal pain

(5) bloody stools

(6) prostration

(7) lethargy


A patient with entercolitis should have a complete workup to identify an underlying pathogen such as Clostridium difficile, Cryptosporidium or enterocyte adherent E. coli..


Risk factors for enterocolitis:

(1) trisomy 21

(2) diagnosis of Hirschsprung's disease after 1 week of age


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