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Late diagnosis of Hirschsprung disease--patient characteristics and results
Kjetil Juul Stensrud1, Ragnhild Emblem, Kristin Bjørnland
1Department of Paediatric Surgery, Oslo University Hospital, Oslo, Norway. kstensru@ous-hf.no
Insights
Late diagnosis of Hirschsprung disease (HD) in children leads to frequent early complications, including anastomotic leakage. A diverting stoma may be beneficial for these patients, though long-term outcomes are similar to early-diagnosed cases.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Medical Diagnostics
Background:
- Hirschsprung disease (HD) is a congenital condition affecting the large intestine.
- Delayed diagnosis of HD, particularly after age 3, presents unique surgical challenges.
- Understanding outcomes in late-diagnosed HD is crucial for optimizing patient care.
Purpose of the Study:
- To characterize children with Hirschsprung disease diagnosed after age 3.
- To evaluate the postoperative results and complications in this patient group.
Main Methods:
- Retrospective analysis of patients diagnosed with HD after 3 years of age between 1998 and 2011.
- Prospective registration of patient characteristics and surgical outcomes.
- Assessment of early and long-term postoperative functional results.
Main Results:
- Eleven children diagnosed after age 3 were included; most had rectosigmoid disease.
- Three patients with a diverting ileostomy experienced complications.
- Early postoperative complications, including anastomotic leakage, were common (5/11 patients).
- At 3-year follow-up, 7/11 had normal bowel function, but 3/11 experienced soiling.
Conclusions:
- Late diagnosis of Hirschsprung disease is associated with a high rate of early postoperative complications.
- Consideration of a diverting stoma is recommended for late-diagnosed HD.
- Long-term functional outcomes in late-diagnosed HD are comparable to those in neonatally treated patients.
Purpose:
The aim of the present study was to describe the characteristics and the postoperative results of children diagnosed as having Hirschsprung disease (HD) after the age of 3 years.
Methods:
All patients with HD diagnosed after the age of 3 years in our hospital from 1998 to 2011 were included. Patient characteristics and postoperative results were prospectively registered.
Results:
Eleven children were included. Age at diagnosis was 3.0 to 9.6 years. Ten patients had rectosigmoid disease, whereas 1 had total colonic aganglionosis. Three children were given a diverting ileostomy before the pull-through procedure, and all 3 had ileostomy-related complications. Early postoperative complications were seen in 5 children, of whom 2 had anastomotic leakage. At final follow-up, with a median of 3 years postoperatively, 7 had normal bowel function, 1 had frequent loose stools, and 3 were soiling.
Conclusions:
Early postoperative complications, especially anastomotic leakage, occurred frequently in children with late-diagnosed HD. Therefore, a diverting stoma should be considered in these patients. The long-term functional results were comparable with those seen in children operated on as neonates.
