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Severe colitis in late-onset Hirschsprung's disease
1Division of Pediatric Gastroenterology and Nutrition, New England Medical Center, Boston, MA 02111.
Journal of Clinical Gastroenterology
|August 1, 1987
Summary
Hirschsprung's disease typically presents in infancy with constipation. This case highlights a rare late-onset presentation, emphasizing the importance of considering Hirschsprung's disease in older children with severe colitis and gastrointestinal bleeding.
Area of Science:
- Pediatric Gastroenterology
- Surgical Neonatology
- Clinical Genetics
Background:
- Hirschsprung's disease (HSCR) is a congenital disorder characterized by the absence of ganglion cells in the distal bowel, leading to functional obstruction.
- Typical presentation involves constipation and abdominal distension within the first few months of life.
- Enterocolitis is a severe complication with high mortality, usually occurring in early infancy.
Observation:
- A case report detailing a child with a previously normal bowel habit.
- The child developed constipation at 16 months of age.
- Severe colitis and chronic lower gastrointestinal bleeding were observed at 3.5 years of age.
Findings:
- The diagnosis of Hirschsprung's disease was made at 3.5 years of age, a significantly later presentation than typical.
- This case challenges the conventional understanding of the age of onset for Hirschsprung's disease symptoms.
- Late diagnosis underscores the variability in clinical presentation.
Implications:
- Highlights the need for increased clinical suspicion for Hirschsprung's disease in older children presenting with severe colitis and gastrointestinal bleeding.
- Suggests that diagnostic delays can occur in atypical cases, potentially impacting patient outcomes.
- Emphasizes the importance of a comprehensive diagnostic approach in pediatric gastrointestinal disorders.