Over the past four decades, enterocolitis has been a major cause of morbidity and mortality in infants and children with Hirschsprung's disease. The mean incidence is 25%, but the range is great (from 17 to 50%) and may be differently estimated, depending on the manner in which it is diagnosed. Mortality rates range from 0 to 33%, probably reflecting differences in the diagnostic criteria.12 Mortality also appears to be associated with other factors, such as trisomy 21. The classic clinical manifestations described for enterocolitis include abdominal distension, explosive diarrhea, vomiting, fever, lethargy, rectal bleeding and shock.13
Abdominal radiographs show the intestinal 'cutoff' sign in the rectosigmoidal region with absence of air distally. Other common findings are small-bowel dilatation in 74% of patients and multiple air-fluid levels.14 Because of the risk of perforation, contrast enema should not be performed in the presence of clinical enterocolitis.
Postoperative enterocolitis has been associated with a fairly high rate of mortality in several series. In fact, when examining the deaths related to
Diagnosis with differential 261
Hirschsprung's disease, several groups found that approximately 50% of deaths resulted from complications directly related to an enterocolitis episode.15,16
Rectal washouts should be the initial approach in the care of a child, regardless of age, who presents with enterocolitis. Along with washouts, intravenous antibiotics or oral metronidazole (in mild cases) should be used. Should the disease process fail to improve, or the infant's condition deteriorate, the performance of a leveling colostomy should be considered.15,16
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