Enterocolitis is the most serious complication of Hirschsprung disease. Early management of these patients can decrease the severity of this complication. Hirschsprung disease is more common in patients with trisomy 21 than in the general population. Furthermore, the risk of developing enterocolitis is higher in this population. We report on an infant with trisomy 21 who developed enterocolitis as a complication of Hirschsprung disease that was diagnosed late when the infant presented with septic shock leading to death of the patient.
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