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Appendiceal perforation: a potentially lethal initial mode of presentation of Hirschsprung's disease
A Sarioğlu1, F C Tanyel, N Büyükpamukçu
1Department of Pediatric Surgery, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
Insights
Appendiceal perforation (AP) is a rare complication in infants with Hirschsprung's disease. Longer aganglionic segments in Hirschsprung's disease patients may increase the risk of appendiceal perforation.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Clinical Medicine
Background:
- Hirschsprung's disease is a congenital disorder characterized by the absence of ganglion cells in the distal bowel.
- Appendiceal perforation (AP) is a serious complication, particularly in neonates and infants.
Observation:
- Two cases of appendiceal perforation (AP) were diagnosed at initial admission among 302 patients with Hirschsprung's disease between 1976 and 1993.
- Both affected patients were under two months of age, with one exhibiting total colonic aganglionosis and the other long-segment Hirschsprung's disease.
Findings:
- In both cases, appendiceal perforation occurred at the base of the appendix, with periappendicitis noted but no mucosal involvement.
- The findings suggest a correlation between the length of the aganglionic segment in Hirschsprung's disease and the risk of developing appendiceal perforation.
Implications:
- This study highlights the importance of considering appendiceal perforation in the differential diagnosis of abdominal emergencies in infants with Hirschsprung's disease.
- Early recognition and management of AP in this vulnerable population are crucial for improving patient outcomes.
- Further research is warranted to elucidate the precise mechanisms linking longer aganglionic segments to an increased risk of AP.
Abstract:
In Hacettepe University Children's Hospital, between 1976 and 1993 two patients among 302 with Hirschsprung's disease were diagnosed with appendiceal perforation (AP) at initial admission. Both patients were less than 2 months of age. One of them was a boy with total colonic aganglionosis and the latter a girl with long-segment disease. In both cases the site of AP was the base, and periappendicitis without mucosal involvement was detected. The present cases and review of the literature suggest that longer aganglionic segment carries a higher risk of AP.