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Hirschsprung's disease. Identification of risk factors for enterocolitis
D H Teitelbaum1, S J Qualman, D A Caniano
1Department of Surgery, Ohio State University College of Medicine, Columbus.
Insights
Hirschsprung
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Genetics
Background:
- Hirschsprung's disease is a congenital disorder affecting the large intestine.
- Hirschsprung's-associated enterocolitis (HAEC) is a serious complication.
- Understanding risk factors and outcomes for HAEC is crucial for patient management.
Purpose of the Study:
- To identify risk factors for developing Hirschsprung's-associated enterocolitis (HAEC).
- To analyze the incidence and characteristics of HAEC in a pediatric cohort.
- To evaluate outcomes, including morbidity and hospitalization duration, for neonates with HAEC.
Main Methods:
- Retrospective review of 80 pediatric patients diagnosed with Hirschsprung's disease between 1975 and 1985.
- Analysis of patient demographics, diagnostic delays, genetic factors (trisomy 21), and surgical procedures.
- Comparison of outcomes between patients with and without HAEC, and those who developed HAEC pre- or post-operatively.
Main Results:
- Enterocolitis developed in 24% of patients (19/80).
- Significant risk factors for HAEC included delayed diagnosis (>1 week) and trisomy 21.
- HAEC post-operatively was associated with anorectal stricture; neonates with HAEC had higher morbidity (30%) and longer hospital stays.
Conclusions:
- Delayed diagnosis and trisomy 21 are key risk factors for HAEC.
- While mortality is low, HAEC significantly increases morbidity and resource utilization in neonates.
- Post-operative HAEC may be linked to anorectal strictures, warranting further investigation.
Abstract:
From 1975 to 1985, 80 infants and children were treated at a major pediatric hospital for Hirschsprung's disease, 19 (24%) of whom developed enterocolitis. In 9 neonates (18%) and 4 infants (29%) enterocolitis was present at diagnosis of Hirschsprung's disease, while 4 children acquired enterocolitis following a pull-through procedure. Significant risk factors for development of Hirschsprung's-associated enterocolitis (HAEC) were delay in diagnosis beyond 1 week of age and the presence of trisomy 21. HAEC did not occur more frequently in patients with long-segment aganglionosis, nor did an initial episode of HAEC confer a higher risk of recurrent enterocolitis. HAEC following a pull-through procedure was correlated with an anorectal stricture in three of four cases. Although neonates with HAEC had a low mortality rate (5%), their morbidity rate was 30% and their hospitalization was twice as long as neonates without enterocolitis.