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Pseudomembranous colitis following resection for Hirschsprung's disease
C E Bagwell1, M R Langham, S M Mahaffey
1Division of Pediatric Surgery, University of Florida College of Medicine, Gainesville 32610-0286.
Insights
Pseudomembranous colitis (PMC) can be a severe complication after Hirschsprung
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Infectious Diseases
Background:
- Hirschsprung's disease is a congenital condition characterized by the absence of ganglion cells in the colon, leading to functional obstruction.
- Enterocolitis is a serious complication of Hirschsprung's disease, often associated with significant morbidity and mortality.
- Pseudomembranous colitis (PMC) is a severe form of enterocolitis that can occur postoperatively.
Observation:
- Four cases of PMC developing 1-18 months after definitive surgery for Hirschsprung's disease are reported.
- Patients presented with symptoms indistinguishable from typical Hirschsprung's enterocolitis, including fever, abdominal distention, and diarrhea.
- Two cases had a fulminant course, resulting in death from septic shock, despite vancomycin therapy.
Findings:
- Extensive colonic pseudomembranes were observed in postmortem examinations.
- Clostridium difficile toxin was identified in all cases, but treatment was often initiated late.
- Two survivors required prolonged hospitalization and diverting enterostomy due to the severity of PMC.
Implications:
- PMC is a virulent complication of Hirschsprung's disease, even after surgical correction.
- Early detection and prompt treatment of C. difficile are crucial for improving outcomes.
- Prophylactic or early empiric vancomycin treatment for Hirschsprung's enterocolitis is recommended due to high associated risks.
Abstract:
Enterocolitis is the most common cause of significant morbidity and death in Hirschsprung's disease. Although most cases respond to nasogastric decompression, antibiotics, and colonic evacuation, some children have an unusually fulminant or protracted clinical course. Four cases are reported of pseudomembranous colitis (PMC) that developed 1 to 18 months (mean, 8 months) after definitive surgery for Hirschsprung's disease (Soave endorectal pull-though, 2; Duhamel procedure, 2). While all children presented with fever, abdominal distention, and diarrhea, indistinguishable from typical Hirschsprung's enterocolitis, the clinical course was fulminant in two cases, both of whom died of septic shock. Postmortem examination in both showed extensive colonic pseudomembranes despite identification of Clostridium difficile toxin and subsequent vancomycin therapy (initiated late in the clinical course). Two children in the series had protracted hospitalizations and eventually required diverting enterostomy despite recognition of C difficile toxin and treatment with enteral vancomycin, in one child necessitating multiple courses of antibiotic therapy. Awareness of the virulence of PMC associated with Hirschsprung's disease (even after definitive resection) should prompt submission of stool specimens from any child who presents with enterocolitis for both C difficile culture and toxin levels. On the basis of our experience it is our policy to initiate a prompt course of vancomycin by rectal lavage or nasogastric tube in all children with Hirschsprung's enterocolitis, pending culture results, in view of the significant morbidity and mortality exemplified by cases in this review.